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Sample records for adult epithelioid sarcoma

  1. Epithelioid sarcoma of the spine: case report and literature review.

    PubMed

    Chamadoira, Clara; Pereira, Paulo; Silva, Pedro S; Castro, Ligia; Vaz, Rui

    2014-01-01

    Epithelioid sarcomas are rare mesenchymal neoplasms mainly arising in the limbs of young adults. We report the case of a 24-year-old male presenting low back pain radiating to both lower limbs, constipation and urinary retention. The MRI scan showed an intraspinal lesion extending from L4 to S2. Surgery resulted in gross total removal of the extradural lesion and partial removal of the intradural component. The immunohistological study of the lesion was consistent with an epithelioid sarcoma. The patient was submitted to radiotherapy and chemotherapy, but a local recurrence of the lesion and dissemination along the neuraxis were observed 3 months after surgery. Despite treatment, the patient died 4 months after the surgical procedure due to multiorgan failure. Despite there being isolated reports of epithelioid sarcomas appearing in the spine, this is, to our knowledge, the first case with intradural extension. PMID:24674671

  2. Epithelioid sarcoma of the spine: case report and literature review.

    PubMed

    Chamadoira, Clara; Pereira, Paulo; Silva, Pedro S; Castro, Ligia; Vaz, Rui

    2014-01-01

    Epithelioid sarcomas are rare mesenchymal neoplasms mainly arising in the limbs of young adults. We report the case of a 24-year-old male presenting low back pain radiating to both lower limbs, constipation and urinary retention. The MRI scan showed an intraspinal lesion extending from L4 to S2. Surgery resulted in gross total removal of the extradural lesion and partial removal of the intradural component. The immunohistological study of the lesion was consistent with an epithelioid sarcoma. The patient was submitted to radiotherapy and chemotherapy, but a local recurrence of the lesion and dissemination along the neuraxis were observed 3 months after surgery. Despite treatment, the patient died 4 months after the surgical procedure due to multiorgan failure. Despite there being isolated reports of epithelioid sarcomas appearing in the spine, this is, to our knowledge, the first case with intradural extension.

  3. Bacillary (epithelioid) angiomatosis and concurrent Kaposi's sarcoma in acquired immunodeficiency syndrome.

    PubMed

    Berger, T G; Tappero, J W; Kaymen, A; LeBoit, P E

    1989-11-01

    Two patients with acquired immunodeficiency syndrome developed simultaneous Kaposi's sarcoma and bacillary (epithelioid) angiomatosis. The distinguishing clinical and histologic features of these two vascular proliferations associated with human immunodeficiency virus disease are described. The lesions of bacillary (epithelioid) angiomatosis contained bacteria, while the lesions of Kaposi's sarcoma did not. With erythromycin therapy, the lesions of bacillary (epithelioid) angiomatosis cleared, while those of Kaposi's persisted. Bacillary (epithelioid) angiomatosis, a treatable but potentially fatal opportunistic infection of human immunodeficiency virus disease, should be considered in the differential diagnosis of vascular lesions in immunosuppressed patients.

  4. Sorafenib in Treating Patients With Metastatic, Locally Advanced, or Recurrent Sarcoma

    ClinicalTrials.gov

    2014-05-07

    Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Leiomyosarcoma; Adult Malignant Fibrous Histiocytoma; Adult Neurofibrosarcoma; Adult Synovial Sarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Uterine Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma; Uterine Carcinosarcoma; Uterine Leiomyosarcoma

  5. Hemin toxicity in a human epithelioid sarcoma cell line.

    PubMed

    Braverman, S; Helson, C; Helson, L

    1995-01-01

    The major cytotoxic component of hemin was identified as metal free protoporphyrin IX in an epithelioid sarcoma cell line (VA-ES-BJ) and a glioblastoma cell line (U-373 MG) by exposing the cell lines to the iron chelator deferoxamine, tin-protoporphyrin IX, and protoporphyrin IX. The contribution of lipid peroxidation and free radical generation to toxicity was examined using DL-buthionine-[S,R]-sulfoximine (BSO), and 21-aminosteroid (lazaroid, U74500A). Hemin caused significantly greater toxicity in VA-ES-BJ than in U-373 MG. While exogenous PpIX was more toxic than hemin in both cell lines, this toxicity was not due to iron depletion following intracellular heme formation since ferric citrate did not reverse PpIX toxicity. Pre-treatment with BSO enhanced hemin toxicity in the VA-ES-BJ cell line but not in U-373 MG, suggesting different modes of toxicity in the two cell lines. Exposure to lazaroid protected only VA-ES-BJ from protoporphyrin-induced toxicity implicating a specific sensitivity to lipid peroxidation and/or free radical generation by this cell line. These characteristics of the VA-ES-BJ cell line distinguish it from the glioblastoma and emphasize its utility for exploring cytotoxic effects of hemin and its precursors.

  6. Proximal-type epithelioid sarcoma: a new case report and literature review

    PubMed Central

    Echchaoui, Abdelmoughit; Sadrati, Yassine; Elbir, Youssef; Elktaibi, Abderrahim; Benyachou, Malika; Mazouz, Samir El; Gharib, Nour-eddine; Abbassi, Abdellah

    2016-01-01

    Proximal-type epithelioid sarcoma is a rare soft tissue neoplasm which arises from the more proximal part of body and occurs more often in young people; the definite diagnosis depends mainly on the pathological examination; early detection and complete excision remain the foundation of treatment. Due to its aggressive behavior, high capacity of recurrence and the great ability to metastasize, a careful clinical long-term monitoring is required. We report a new case of a 20 years old girl, presented with proximal-type epithelioid sarcoma in her right scapular region, confirmed by pathological examination and removed surgically without recurrence or metastasis at eighteen months of follow-up. PMID:27800093

  7. Observation, Radiation Therapy, Combination Chemotherapy, and/or Surgery in Treating Young Patients With Soft Tissue Sarcoma

    ClinicalTrials.gov

    2014-09-08

    Adult Alveolar Soft-part Sarcoma; Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Extraskeletal Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Malignant Hemangiopericytoma; Adult Malignant Mesenchymoma; Adult Neurofibrosarcoma; Adult Synovial Sarcoma; Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Neurofibrosarcoma; Childhood Synovial Sarcoma; Dermatofibrosarcoma Protuberans; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  8. Depsipeptide (Romidepsin) in Treating Patients With Metastatic or Unresectable Soft Tissue Sarcoma

    ClinicalTrials.gov

    2016-09-12

    Adult Alveolar Soft-part Sarcoma; Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Extraskeletal Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Malignant Hemangiopericytoma; Adult Malignant Mesenchymoma; Adult Neurofibrosarcoma; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Gastrointestinal Stromal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Adult Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  9. Expression of lung resistance protein in epithelioid sarcoma in vitro and in vivo.

    PubMed

    Kusakabe, H; Iwasaki, H; Sano, K; Kiyokane, K

    2000-06-01

    The incidence of epithelioid sarcoma among patients with malignant soft tissue tumors is small, but the rates of recurrence and metastasis of this type of sarcoma are high. To date, effective chemotherapy for advanced epithelioid sarcoma has not been established and, furthermore, epithelioid sarcoma is known to exhibit multidrug resistance (MDR). The chemosensitivities to anticancer agents of two cell lines established from epithelioid sarcoma were examined in this study. The results showed that the ES-OMC-MN and SFT-8606 cell lines were resistant to vincristine (IC50 1190 nM and 872 nM, respectively) and Adriamycin (IC50 921 nM and 650 nM, respectively), but sensitive to actinomycin D (IC50 < 10 nM). P-glycoprotein (p-Gp) and MDR-associated protein (MRP) were not expressed in these cell lines, but a high expression level of lung resistance protein (LRP) was observed. The original tumor tissues from which the two cell lines were established were also found to be LRP-positive but not to express p-Gp or MRP. Their chemosensitivities to Adriamycin were not significantly altered in the presence of 2.5 microg/ml anti-LRP antibody (LRP-56), but the IC50 of vincristine was much less (IC50 128 nM and 27 nM, respectively) than that for an untreated cell line. It is thus suggested that the vincristine resistance in the two cell lines is LRP-mediated. Since cyclosporin A, known to be a modifier of p-Gp, also induced reversal of vincristine resistance in the ES-OMC-MN and SFT-8606 cell lines (IC50 6.2 nM and 17 nM, respectively), it is suggested that cyclosporin A acts as a modifier of MDR mediated by LRP.

  10. Epithelioid Sarcoma of the Vulva in a 17-year-old Pregnant Woman

    PubMed Central

    Rai, Har; Odunsi, Kunle; Kesterson, Joshua; Morrison, Carl; Chan, Ada; Mhawech-Fauceglia, Paulette

    2016-01-01

    Epithelioid sarcoma of the vulva is an extremely rare neoplasm with aggressive behavior and poor outcome. Herein, we report a case of vulvar epithelioid sarcoma presenting in a 17-year-old very early in her pregnancy. The patient presented with an asymptomatic nodule of the right labia majora of 1-year duration. Computerized tomographic scans showed enlarged inguinal lymph nodes and numerous lung nodules. Positron emission tomography was performed and revealed no suspicious lesions for metastatic disease. The patient underwent local excision of her vulvar lesion. On the basis of morphology and extensive immunohistochemistry, the lesion was classified as epithelioid sarcoma. The patient was referred to radiation therapy and upon evaluation, she was found to be in her sixth week of gestation. The patient continued with her pregnancy and underwent a lymph node dissection, which was positive. We review the literature of only the well-documented cases extensively studied by immunohistochemical analysis. We summarize the clinical presentation, clinical impression, treatment modalities, and outcomes of these cases. On account of the rarity of this disease in the vulva, there is no clear consensus on treatment modalities, but it seems that early aggressive surgical resection is the treatment of choice, with the role of adjuvant therapy to be determined. PMID:19384081

  11. Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma

    ClinicalTrials.gov

    2016-06-09

    Adult Alveolar Soft Part Sarcoma; Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; Adult Epithelioid Hemangioendothelioma; Adult Epithelioid Sarcoma; Adult Extraskeletal Myxoid Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Mesenchymoma; Adult Malignant Peripheral Nerve Sheath Tumor; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Adult Unclassified Pleomorphic Sarcoma; Chondrosarcoma; Clear Cell Sarcoma of the Kidney; Conjunctival Kaposi Sarcoma; Dermatofibrosarcoma Protuberans; Gastrointestinal Stromal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Adult Unclassified Pleomorphic Sarcoma of Bone; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Kaposi Sarcoma; Recurrent Osteosarcoma; Recurrent Uterine Corpus Sarcoma; Small Intestine Leiomyosarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma; Unclassified Pleomorphic Sarcoma of Bone

  12. Epithelioid inflammatory myofibroblastic sarcoma in abdominal cavity: a case report and review of literature.

    PubMed

    Wu, Hui; Meng, Yu-Hong; Lu, Ping; Ning, Hao-Yong; Hong, Liu; Kang, Xiao-Ling; Duan, Min-Gang

    2015-01-01

    In this study, we present a rare and difficult case of epithelioid inflammatory myofibroblastic sarcoma (EIMS) in abdominal cavity. A 47-year-old female presented as left upper abdominal pain for 6 months and abdominal distention for 1 month. CT examination showed a solid mass in the left upper intra-abdomen. Grossly, the tumor was found in the mesenterium of colon with the size of 7.5 × 6.5 × 3.5 cm, and was solid and gray-yellowish in the cut surface. Focal myxomatous appearance was observed. Microscopically, stromal myxoid change together with prominant infiltrated lymphocytes, neutrophils and eosinophils were found in the tumor, and the tumor cells were round, epithelioid with vesicular nuclei, large prominant nucleoli and high mitotic rate. Immunohistochemically, strong diffused positive for vimentin, desmin, ALK (nuclear membrane staining pattern) and AAT, focally positive for CD99 and CD30, were showed, Ki67 index was about 20%; Especially, WT-1 and D240 were focally expressed in this tumor. FISH analysis showed rearrangement of ALK, and reverse-transcription polymerase chain reaction (RT-PCR) analysis was used to detect the fusion location of the RANBP2 and ALK gene. The diagnosis of EMIS was made based on its location, typical morphology, the immunohistochemical features especially the nuclear membranous immunostaining of ALK and rearrangement of RANBP2-ALK. The tumor showed higher aggressive behaviors and a poor prognosis. The differential diagnosis and other treatments of EMIS are also discussed in the present study. This finding may increase the case information of EMIS.

  13. General Information about Adult Soft Tissue Sarcoma

    MedlinePlus

    ... Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version General Information About Adult Soft Tissue Sarcoma Go to Health ... the PDQ Adult Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  14. Cixutumumab and Doxorubicin Hydrochloride in Treating Patients With Unresectable, Locally Advanced, or Metastatic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2016-05-16

    Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; Adult Epithelioid Sarcoma; Adult Extraskeletal Myxoid Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Mesenchymoma; Adult Malignant Peripheral Nerve Sheath Tumor; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Adult Undifferentiated High Grade Pleomorphic Sarcoma of Bone; Childhood Angiosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Malignant Peripheral Nerve Sheath Tumor; Childhood Pleomorphic Rhabdomyosarcoma; Childhood Rhabdomyosarcoma With Mixed Embryonal and Alveolar Features; Childhood Synovial Sarcoma; Dermatofibrosarcoma Protuberans; Malignant Adult Hemangiopericytoma; Malignant Childhood Hemangiopericytoma; Metastatic Childhood Soft Tissue Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Untreated Childhood Rhabdomyosarcoma

  15. Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-Rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery

    ClinicalTrials.gov

    2016-11-04

    Adult Fibrosarcoma; Alveolar Soft Part Sarcoma; Angiomatoid Fibrous Histiocytoma; Atypical Fibroxanthoma; Clear Cell Sarcoma of Soft Tissue; Epithelioid Malignant Peripheral Nerve Sheath Tumor; Epithelioid Sarcoma; Extraskeletal Myxoid Chondrosarcoma; Extraskeletal Osteosarcoma; Fibrohistiocytic Neoplasm; Glomus Tumor of the Skin; Inflammatory Myofibroblastic Tumor; Intimal Sarcoma; Leiomyosarcoma; Liposarcoma; Low Grade Fibromyxoid Sarcoma; Low Grade Myofibroblastic Sarcoma; Malignant Cutaneous Granular Cell Tumor; Malignant Peripheral Nerve Sheath Tumor; Malignant Triton Tumor; Mesenchymal Chondrosarcoma; Myxofibrosarcoma; Myxoid Chondrosarcoma; Myxoinflammatory Fibroblastic Sarcoma; Nerve Sheath Neoplasm; PEComa; Pericytic Neoplasm; Plexiform Fibrohistiocytic Tumor; Sclerosing Epithelioid Fibrosarcoma; Stage IB Soft Tissue Sarcoma; Stage IIB Soft Tissue Sarcoma; Stage III Soft Tissue Sarcoma; Stage IV Soft Tissue Sarcoma; Synovial Sarcoma; Undifferentiated (Embryonal) Sarcoma; Undifferentiated High Grade Pleomorphic Sarcoma of Bone

  16. Doxorubicin With Upfront Dexrazoxane for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2016-07-11

    Sarcoma, Soft Tissue; Soft Tissue Sarcoma; Undifferentiated Pleomorphic Sarcoma; Leiomyosarcoma; Liposarcoma; Synovial Sarcoma; Myxofibrosarcoma; Angiosarcoma; Fibrosarcoma; Malignant Peripheral Nerve Sheath Tumor; Epithelioid Sarcoma

  17. Penile pseudomyogenic hemangioendothelioma/epithelioid sarcoma-like hemangioendothelioma with a novel pattern of SERPINE1-FOSB fusion detected by RT-PCR--report of a case.

    PubMed

    Ide, Yoshi-Hiro; Tsukamoto, Yoshitane; Ito, Takaaki; Watanabe, Takahiro; Nakagawa, Noboru; Haneda, Takashi; Nagai, Makoto; Yamanishi, Kiyofumi; Hirota, Seiichi

    2015-05-01

    We experienced a rare case of penile mesenchymal tumor in a 43-year-old Japanese man. At least three nodules were observed around the penis. The tumors were composed of spindle- to oval-shaped atypical cells with and without prominent nucleoli. These cells were like myogenic cells, but negative for myogenic markers. They were positive for endothelial markers, such as ERG, Fli1 and CD31. They were also positive for nuclear and cytoplasmic FOSB which are not expressed in epithelioid hemangioendothelioma or epithelioid sarcoma. These pathological and immunohistochemical findings strongly suggested pseudomyogenic hemangioendothelioma/epithelioid sarcoma-like hemangioendothelioma (PHE/ES-HE). Since a recent report directly proved that two cases of PHE/ES-HE have SERPINE1-FOSB fusion gene by reverse transcription-polymerase chain reaction (RT-PCR), we examined whether the fusion gene is present or not in the present case by RT-PCR using fresh frozen surgical material. Sequencing of the PCR product revealed that this case has SERPINE1-FOSB fusion. The fusion pattern of our case was different from those of two previously reported cases. In our case, 86 nucleotides of SERPINE1 intron 1 were inserted between SERPINE1 exon 1 and the middle portion of FOSB exon 1, and a putative translation start codon was identified in SERPINE1 intron 1. Thus, this is the third case of PHE/ES-HE with SERPINE1-FOSB fusion proved by RT-PCR.

  18. Trial of Dasatinib in Advanced Sarcomas

    ClinicalTrials.gov

    2016-10-12

    Rhabdomyosarcoma; Malignant Peripheral Nerve Sheath Tumors; Chondrosarcoma; Sarcoma, Ewing's; Sarcoma, Alveolar Soft Part; Chordoma; Epithelioid Sarcoma; Giant Cell Tumor of Bone; Hemangiopericytoma; Gastrointestinal Stromal Tumor (GIST)

  19. A Phase II, Multicenter Study of the EZH2 Inhibitor Tazemetostat in Adult Subjects With INI1-Negative Tumors or Relapsed/Refractory Synovial Sarcoma

    ClinicalTrials.gov

    2016-09-20

    Malignant Rhabdoid Tumors (MRT); Rhabdoid Tumors of the Kidney (RTK); Atypical Teratoid Rhabdoid Tumors (ATRT); Selected Tumors With Rhabdoid Features; Synovial Sarcoma; INI1-negative Tumors; Malignant Rhabdoid Tumor of Ovary; Renal Medullary Carcinoma; Epithelioid Sarcoma

  20. [Radiotherapy of adult soft tissue sarcoma].

    PubMed

    Le Péchoux, C; Moureau-Zabotto, L; Llacer, C; Ducassou, A; Sargos, P; Sunyach, M P; Thariat, J

    2016-09-01

    Incidence of soft tissue sarcoma is low and requires multidisciplinary treatment in specialized centers. The objective of this paper is to report the state of the art regarding indications and treatment techniques of main soft tissue sarcoma localisations.

  1. [Radiotherapy of adult soft tissue sarcoma].

    PubMed

    Le Péchoux, C; Moureau-Zabotto, L; Llacer, C; Ducassou, A; Sargos, P; Sunyach, M P; Thariat, J

    2016-09-01

    Incidence of soft tissue sarcoma is low and requires multidisciplinary treatment in specialized centers. The objective of this paper is to report the state of the art regarding indications and treatment techniques of main soft tissue sarcoma localisations. PMID:27523415

  2. Consistent SMARCB1 Homozygous Deletions in Epithelioid Sarcoma and in a Subset of Myoepithelial Carcinomas can be Reliably Detected by FISH in Archival Material

    PubMed Central

    Le Loarer, Francois; Zhang, Lei; Fletcher, Christopher D; Ribeiro, Agnes; Singer, Samuel; Italiano, Antoine; Neuville, Agnes; Houlier, Aurélie; Chibon, Frederic; Coindre, Jean-Michel; Antonescu, Cristina R

    2014-01-01

    Epithelioid sarcomas (ES) are mesenchymal neoplasms subclassified into distal and proximal subtypes based on their distinct clinical presentations and histologic features. Consistent loss of SMARCB1 nuclear expression has been considered as the hallmark abnormality for both subtypes, a feature shared with atypical teratoid/rhabdoid tumor of infancy (ATRT). While virtually all ATRTs harbor underlying SMARCB1 somatic or germline alterations, mechanisms of SMARCB1 inactivation in ES are less well defined. To further define mechanisms of SMARCB1 inactivation a detailed molecular analysis was performed on 40 ES (25 proximal and 15 distal ES, with classic morphology and negative SMARCB1 expression) for their genomic status of SMARCB1 and related genes encoding the SWI/SNF subunits (PBRM1, BRG1, BRM, SMARCC1/2 and ARID1A) by FISH using custom BAC probes. An additional control group was included spanning a variety of 41 soft tissue neoplasms with either rhabdoid/epithelioid features or selected histotypes previously shown to lack SMARCB1 by IHC. Furthermore, 12 ES were studied by array CGH (aCGH) and an independent TMA containing 50 additional ES cases was screened for Aurora Kinase A (AURKA) and cyclin D1 immunoexpression. Homozygous SMARCB1 deletions were found by FISH in 36/40 ES (21/25 proximal-type). One of the distal-type ES displayed homozygous SMARCB1 deletion in the tumor cells, along with a heterozygous deletion within normal tissue, finding confirmed by array CGH. None of the proximal ES lacking homozygous SMARCB1 deletions displayed alterations in other SWI/SNF subunits gene members. Among controls, only the SMARCB1-immunonegative myoepithelial carcinomas displayed SMARCB1 homozygous deletions in 3/5 cases, while no gene specific abnormalities were seen among all other histologic subtypes of sarcomas tested regardless of the SMARCB1 protein status. There was no consistent pattern of AURKA and Cyclin D1 expression. The array CGH was successful in 9/12 ES

  3. Kasabach-Merrit syndrome and adult hepatic epithelioid hemangioendothelioma an unusual association.

    PubMed

    Frider, Bernardo; Bruno, Andrés; Selser, Julio; Vanesa, Rodríguez; Pascual, Pablo; Bistoletti, Raquel

    2005-02-01

    Kasabach-Merrit syndrome (KMS) is very rarely observed in adults associated with visceral hemangiomas. Hepatic epithelioid hemangioendothelioma (HEHE) is a very rare clinical entity with an intermediate malignant potential and a mortality rate of 20-30%. We described a case of KMS associated with HEHE in a 70 year old male patient who presented coagulation abnormalities, low platelet count and high fibrin degradation products that were typical features of KMS. Histopathology of the hepatic tissue and a positive immunostaining for endothelial markers confirmed the diagnosis of an HEHE. The first description of KMS was made in children in 1940. It was described as a "consumptive coagulopathy with capillary hemangiomas". However, recent reports have shown that this condition could be associated with kaposiform hemangioendotheliomas, an aggressive form of giant hemangioma. A giant hemangioma in adults as well as an hemangioendothelioma in children could be associated KMS, but an HEHE has been rarely reported in association with this syndrome in adult patients. PMID:15664259

  4. Adult Intramedullary Ewing Sarcoma of the Proximal Hip

    PubMed Central

    Jasti, Siva; Rafferty, William; Lackman, Richard

    2014-01-01

    Ewing sarcoma of bone is classically a permeative lesion in the diaphysis of long bones in children. While they occur primarily in children and adolescents, they can be seen in young adults in their 20s, but these are typically seen in flat bones. The permeative nature of the lesion can elicit new bone formation creating a partially sclerotic appearance, cortical expansion presenting as a “Codman triangle,” or have an “onion-skin” type of aggressive periosteal reaction/periostitis. Ewing sarcoma is rarely seen without an associated soft-tissue mass and is even rarer to just have benign-appearing periostitis (e.g., thick, uniform, or wavy cortex). We present such a case of Ewing sarcoma in a young adult confined to just the medullary metadiaphysis without cortical erosion or soft-tissue mass. To the best of our knowledge, this is the first case to be reported in the radiology literature. PMID:25024862

  5. Wiki-Based Clinical Practice Guidelines for the Management of Adult Onset Sarcoma: A New Paradigm in Sarcoma Evidence

    PubMed Central

    Neuhaus, S. J.; Thomas, D.; Desai, J.; Vuletich, C.; von Dincklage, J.; Olver, I.

    2015-01-01

    In 2013 Australia introduced Wiki-based Clinical Practice Guidelines for the Management of Adult Onset Sarcoma. These guidelines utilized a customized MediaWiki software application for guideline development and are the first evidence-based guidelines for clinical management of sarcoma. This paper presents our experience with developing and implementing web-based interactive guidelines and reviews some of the challenges and lessons from adopting an evidence-based (rather than consensus-based) approach to clinical sarcoma guidelines. Digital guidelines can be easily updated with new evidence, continuously reviewed and widely disseminated. They provide an accessible method of enabling clinicians and consumers to access evidence-based clinical practice recommendations and, as evidenced by over 2000 views in the first four months after release, with 49% of those visits being from countries outside of Australia. The lessons learned have relevance to other rare cancers in addition to the international sarcoma community. PMID:25784832

  6. Epigenetic regulation of SMARCB1 By miR-206, -381 and -671-5p is evident in a variety of SMARCB1 immunonegative soft tissue sarcomas, while miR-765 appears specific for epithelioid sarcoma. A miRNA study of 223 soft tissue sarcomas.

    PubMed

    Sápi, Zoltán; Papp, Gergő; Szendrői, Miklós; Pápai, Zsuzsanna; Plótár, Vanda; Krausz, Thomas; Fletcher, Christopher D M

    2016-10-01

    Complete/partial loss of SMARCB1 nuclear-immunopositivity is characteristic of a certain subset of soft tissue sarcomas (STSs). Our previous work showed that oncomiRs-206,-381, and 671-5p could silence the SMARCB1 mRNA and protein expression and that they display significant overexpression in epithelioid sarcomas (ESs). MiR-765 was overexpressed too, but functionally was inactive in the silencing. In the current work, using quantitative PCR, we conducted a miRNA study of 51 ESs, 20 rhabdoid tumors (RTs), 20 synovial sarcomas (SSs), 15 malignant peripheral nerve sheath tumors (MPNSTs), 11 myoepithelial carcinomas (MECs), and 10 extraskeletal myxoid chondrosarcomas (EMCSs) with complete/partial loss of SMARCB1 nuclear immunostain, in contrast to controls (SMARCB1-immunopositive) of 96 STSs, 13 melanomas and 10 sarcomatoid carcinomas. The SMARCB1 genetic status of ESs was determined by MLPA and FISH. A subset of ESs (5/51) showed biallelic deletion of SMARCB1 with no overexpression of any miRNA, suggesting these tumors could be the counterpart of pediatric RT, at least genetically. Another subset (5/51) was genetically either intact or monoallelic deleted with at least threefold overexpression of one of miR-206,-381,-671-5p, suggesting epigenetic regulation only. 39/51 ESs had a biallelic deletion (>20% by FISH and/or by MLPA) but with overexpressed miR-206,-381, and 671-5p, suggesting intratumoral heterogeneity, i.e., both genetic and epigenetic regulation. At least threefold overexpression of one of miR-206,-381, and 671-5p was detected in all MPNSTs, EMCSs, SSs and 7 MCs. Except for ESs, four SSs and one MPNST, there was no event above threefold overexpression of miR-765 among all 195 tested tumors. Our results suggest a general role of miR-206,-381, and 671-5p in SMARCB1 gene silencing of ES, MC, EMCS, MPNST and SS. In the future, miR-765 could possibly be a diagnostic tool for ES because of its 97% specificity and 80% sensitivity. © 2016 Wiley Periodicals, Inc.

  7. Epigenetic regulation of SMARCB1 By miR-206, -381 and -671-5p is evident in a variety of SMARCB1 immunonegative soft tissue sarcomas, while miR-765 appears specific for epithelioid sarcoma. A miRNA study of 223 soft tissue sarcomas.

    PubMed

    Sápi, Zoltán; Papp, Gergő; Szendrői, Miklós; Pápai, Zsuzsanna; Plótár, Vanda; Krausz, Thomas; Fletcher, Christopher D M

    2016-10-01

    Complete/partial loss of SMARCB1 nuclear-immunopositivity is characteristic of a certain subset of soft tissue sarcomas (STSs). Our previous work showed that oncomiRs-206,-381, and 671-5p could silence the SMARCB1 mRNA and protein expression and that they display significant overexpression in epithelioid sarcomas (ESs). MiR-765 was overexpressed too, but functionally was inactive in the silencing. In the current work, using quantitative PCR, we conducted a miRNA study of 51 ESs, 20 rhabdoid tumors (RTs), 20 synovial sarcomas (SSs), 15 malignant peripheral nerve sheath tumors (MPNSTs), 11 myoepithelial carcinomas (MECs), and 10 extraskeletal myxoid chondrosarcomas (EMCSs) with complete/partial loss of SMARCB1 nuclear immunostain, in contrast to controls (SMARCB1-immunopositive) of 96 STSs, 13 melanomas and 10 sarcomatoid carcinomas. The SMARCB1 genetic status of ESs was determined by MLPA and FISH. A subset of ESs (5/51) showed biallelic deletion of SMARCB1 with no overexpression of any miRNA, suggesting these tumors could be the counterpart of pediatric RT, at least genetically. Another subset (5/51) was genetically either intact or monoallelic deleted with at least threefold overexpression of one of miR-206,-381,-671-5p, suggesting epigenetic regulation only. 39/51 ESs had a biallelic deletion (>20% by FISH and/or by MLPA) but with overexpressed miR-206,-381, and 671-5p, suggesting intratumoral heterogeneity, i.e., both genetic and epigenetic regulation. At least threefold overexpression of one of miR-206,-381, and 671-5p was detected in all MPNSTs, EMCSs, SSs and 7 MCs. Except for ESs, four SSs and one MPNST, there was no event above threefold overexpression of miR-765 among all 195 tested tumors. Our results suggest a general role of miR-206,-381, and 671-5p in SMARCB1 gene silencing of ES, MC, EMCS, MPNST and SS. In the future, miR-765 could possibly be a diagnostic tool for ES because of its 97% specificity and 80% sensitivity. © 2016 Wiley Periodicals, Inc

  8. Undifferentiated embryonal sarcoma of the liver in an adult patient

    PubMed Central

    Lee, Kyu Ho; Maratovich, Mussin Nadiar; Lee, Kyoung-Bun

    2016-01-01

    Undifferentiated embryonal sarcoma of the liver (UESL) is rare primary hepatic sarcoma and is known to occur in pediatric patients. This case is the UESL occurred in a 51-year old male patient. Multilocular cystic lesion was composed of primitive spindle cells without specific differentiation. This rare case would help to review differential diagnosis of primary sarcoma in liver and cystic neoplasm of the liver. PMID:27377912

  9. Studying Genes in Tissue Samples From Younger and Adolescent Patients With Soft Tissue Sarcomas

    ClinicalTrials.gov

    2016-05-13

    Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Neurofibrosarcoma; Childhood Synovial Sarcoma; Chordoma; Desmoid Tumor; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Recurrent Childhood Soft Tissue Sarcoma

  10. Relationship between dose of injected /sup 239/Pu and bone sarcoma mortality in young adult beagles

    SciTech Connect

    Peterson, A.V.; Prentice, R.L.; Marek, P.

    1982-04-01

    The effect of /sup 239/Pu on bone sarcoma mortality in young adult beagles is examined using data from a follow-up study conducted at the Radiobiology Laboratory at the University of Utah. A (proportional-hazards) model, which specifies that bone sarcoma mortality depends on both dose of injected plutonium and time since injection as the product of a dose factor and a time factor, is used both for describing the data and for performing inference about the relationship between dose of plutonium and bone sarcoma mortality. Relative bone sarcoma mortality rates are found to be approximately linear as a function of dose. There is evidence that relative bone sarcoma mortality decreases with time since injection.

  11. Adult Primary Spinal Epidural Extraosseous Ewing's Sarcoma: A Case Report and Review of the Literature

    PubMed Central

    Thomas, Cheddhi; Modrek, Aram S.; Bayin, N. Sumru; Snuderl, Matija; Schiff, Peter B.

    2016-01-01

    Background. Extraosseous Ewing's sarcoma in the spinal epidural space is a rare malignancy, especially in adults. Case Presentation. A 40-year-old male presented with back pain and urinary hesitancy. MRI revealed a thoracic extradural mass with no osseous involvement. He underwent surgery for gross total resection of the mass, which was diagnosed as Ewing's sarcoma. He was subsequently treated with chemoradiotherapy. He remains disease-free 1 year after surgery. Review of the literature indicated only 45 previously reported cases of spinal epidural extraosseous Ewing's sarcoma in adults. Conclusions. Extraosseous Ewing's sarcoma in the spinal epidural space is a rare clinical entity that should be included in the differential for spinal epidural masses. Its treatment is multidisciplinary but frequently requires surgical intervention due to compressive neurologic symptoms. Gross total resection appears to correlate with improved outcomes. PMID:27610254

  12. Adult Primary Spinal Epidural Extraosseous Ewing's Sarcoma: A Case Report and Review of the Literature.

    PubMed

    Bustoros, Mark; Thomas, Cheddhi; Frenster, Joshua; Modrek, Aram S; Bayin, N Sumru; Snuderl, Matija; Rosen, Gerald; Schiff, Peter B; Placantonakis, Dimitris G

    2016-01-01

    Background. Extraosseous Ewing's sarcoma in the spinal epidural space is a rare malignancy, especially in adults. Case Presentation. A 40-year-old male presented with back pain and urinary hesitancy. MRI revealed a thoracic extradural mass with no osseous involvement. He underwent surgery for gross total resection of the mass, which was diagnosed as Ewing's sarcoma. He was subsequently treated with chemoradiotherapy. He remains disease-free 1 year after surgery. Review of the literature indicated only 45 previously reported cases of spinal epidural extraosseous Ewing's sarcoma in adults. Conclusions. Extraosseous Ewing's sarcoma in the spinal epidural space is a rare clinical entity that should be included in the differential for spinal epidural masses. Its treatment is multidisciplinary but frequently requires surgical intervention due to compressive neurologic symptoms. Gross total resection appears to correlate with improved outcomes. PMID:27610254

  13. Adult Primary Spinal Epidural Extraosseous Ewing's Sarcoma: A Case Report and Review of the Literature

    PubMed Central

    Thomas, Cheddhi; Modrek, Aram S.; Bayin, N. Sumru; Snuderl, Matija; Schiff, Peter B.

    2016-01-01

    Background. Extraosseous Ewing's sarcoma in the spinal epidural space is a rare malignancy, especially in adults. Case Presentation. A 40-year-old male presented with back pain and urinary hesitancy. MRI revealed a thoracic extradural mass with no osseous involvement. He underwent surgery for gross total resection of the mass, which was diagnosed as Ewing's sarcoma. He was subsequently treated with chemoradiotherapy. He remains disease-free 1 year after surgery. Review of the literature indicated only 45 previously reported cases of spinal epidural extraosseous Ewing's sarcoma in adults. Conclusions. Extraosseous Ewing's sarcoma in the spinal epidural space is a rare clinical entity that should be included in the differential for spinal epidural masses. Its treatment is multidisciplinary but frequently requires surgical intervention due to compressive neurologic symptoms. Gross total resection appears to correlate with improved outcomes.

  14. An increased incidence of Hodgkin's lymphoma in patients with adult-onset sarcoma

    PubMed Central

    2012-01-01

    Background Sarcomas are rare, often fatal malignancies of connective tissues that can occur in genetic predisposition syndromes or result from carcinogen exposure. Hodgkin's lymphoma (HL) is not known to contribute to any recognised familial cancer syndrome comprising sarcomas, but is known to be associated with a variety of second cancers, including sarcomas. This study describes the prevalence of HL in families affected by sarcoma. Methods The International Sarcoma Kindred Study (ISKS) is a prospective cohort of 561 families ascertained via a proband with adult-onset sarcoma. Cancer-specific standardised incidence ratios (SIR) for multiple primary malignancies in probands were estimated. Clinical characteristics of individuals reporting both sarcoma and HL were described. Standardised incidence ratios for the occurrence of cancer in ISKS families were also estimated. Results Multiple primary cancers were reported in 16% of probands, significantly higher than in the general population. The risk of HL in probands was increased 15.8-fold (95%CI 7.9-31.6) and increased risks were also seen for breast cancer (SIR 2.9, 95%CI 1.9-4.4) and thyroid cancer (SIR 8.4, 95%CI 4.2-16.8). In 8 probands with both HL and sarcoma, the diagnosis of HL preceded that of sarcoma in 7 cases, and occurred synchronously in one case. Only 3 cases of sarcoma occurred in or close to prior radiotherapy fields. The overall incidence of HL in the ISKS cohort was not significantly increased by comparison with age- and gender-specific population estimates (SIR 1.63, 95%CI 1.05-2.43), suggesting that the association between HL and sarcomas did not extend to other family members. The age of onset of non-sarcoma, non-HL cancers in families affected by both HL and sarcoma was younger than the general population (56.2 y vs 65.6 y, P < 0.0001). Conclusions The basis for the association between HL and sarcomas may include the carcinogenic effects of therapy combined with excellent survival rates for HL

  15. Isolated Limb Perfusion of Melphalan With or Without Tumor Necrosis Factor in Treating Patients With Soft Tissue Sarcoma of the Arm or Leg

    ClinicalTrials.gov

    2012-03-14

    Stage IVB Adult Soft Tissue Sarcoma; Stage IIB Adult Soft Tissue Sarcoma; Stage IIC Adult Soft Tissue Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Stage IVA Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma

  16. Penile Epithelioid Angiomatoid Nodule.

    PubMed

    Pirpiris, Athina; Gilbourd, Daniel; Ranasinghe, Anudini; Dill, Tony; Lynnhtun, Kyaw; Rindani, Rahul

    2015-10-01

    Cutaneous epithelioid angiomatoid nodule is a rare clinical entity that is common on the trunk and limbs. This is the first report of penile cutaneous epithelioid angiomatoid nodule. Although it is a benign entity, it must be differentiated from vascular neoplasms, as it can bear similar clinical and pathologic features. PMID:26171823

  17. Oral and maxillofacial sclerosing epithelioid fibrosarcoma: report of five cases.

    PubMed

    Folk, Gretchen S; Williams, Stephen B; Foss, Robert B; Fanburg-Smith, Julie C

    2007-09-01

    Sclerosing epithelioid fibrosarcoma (SEF) has distinctive morphology and occurs mainly in deep soft tissue of adult extremities. Approximately 59 cases of SEF have been reported, with only 12 previously described in head and neck locations. Lesions involving the oral and maxillofacial region (OMFR) and intraosseous examples are rare. We present five cases of OMFRSEF. The OMF Pathology Department Registry was searched for cases coded from 1990 to the present as "SEF," "fibrosarcoma not otherwise specified" or "neoplasm of uncertain histiogenesis." Inclusion required OMFR location, an abundantly sclerotic sarcoma with epithelioid features, and lack of other phenotype by immunohistochemistry. Five cases of SEF included 3 males and 2 females. The age of the patients were: 19, 22, 35, 47 and 47 years. Tumor location included the infra-temporal fossa, buccal mucosa (recurrence extending into bone), anterior mandible (intraosseous primary, focally extending into soft tissue), and left parotid and submandibular gland (with metaplastic bone) regions. Tumor sizes ranged from 1.0 to 5.7 cm, median 3.5 cm. Histologically, the tumors were well delineated and multinodular, separated by fibrous septae. The spindled to primarily epithelioid tumor cells formed moderately cellular sheets and cords of irregularly contoured medium to large, round to oval, occasionally overlapping nuclei, indistinct nucleoli, wispy eosinophilic (retracting) cytoplasm, and distinctive cytoplasmic borders, embedded in osteoid-like stroma. Hemangiopericytoid (HPC-like) vessels were observed. Despite numerous apoptotic cells, mitoses were generally low; necrosis was present in two cases. Three tumors were graded as 2/3 and two 1/3. Immunohistochemically, the tumor cells were positive for vimentin, 1 case focally for CD34, whereas all cases were negative for S100 protein, keratins, EMA, desmin, and SMA. Wide or radical excision was performed with no adjuvant therapy. Follow-up revealed that 4 cases recurred

  18. Cixutumumab and Temsirolimus in Treating Younger Patients With Recurrent or Refractory Sarcoma

    ClinicalTrials.gov

    2016-05-26

    Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Gliosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Neurofibrosarcoma; Childhood Synovial Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Osteosarcoma

  19. Sorafenib in Treating Patients With Soft Tissue Sarcomas (Extremity Sarcoma Closed to Entry as of 5/30/07)

    ClinicalTrials.gov

    2014-04-01

    Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Osteosarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  20. Oral epithelioid hemangioendothelioma

    PubMed Central

    Bhattacharya, Preeti Tomar; Guledgud, Mahima V.; Patil, Karthikeya

    2015-01-01

    Epithelioid hemangioendothelioma (HE) is an intermediate malignant potential vascular neoplasm with uncertain clinical behavior, wide variations in microscopic findings, and prognosis. According to the World Health Organization (2002) classification, epithelioid HE has been considered under malignant tumors which rarely metastasize. The epithelioid variant, the most aggressive one, has similar gender predilection and sporadic occurrence in children. The patients usually present with an asymptomatic oral mass whereas few cases may report with the painful bleeding lesion. We attempt to present a case in an adolescent male with previously never described biological behavior, diverse histopathological features, and immunohistochemistry findings. PMID:26681871

  1. Epithelioid angiomyolipoma: imaging appearances.

    PubMed

    Bharwani, N; Christmas, T J; Jameson, C; Moat, N; Sohaib, S A

    2009-12-01

    Epithelioid angiomyolipoma is a recently described rare variant of renal angiomyolipoma. It can occur in patients with or without tuberous sclerosis, and may potentially be malignant. We report the imaging findings from two cases of epithelioid angiomyolipoma: the first in a patient with tuberous sclerosis complex, arising in a horse-shoe kidney and growing into the inferior vena cava and right atrium; the second in a 62-year-old hypertensive man.

  2. AZD0530 in Treating Patients With Recurrent Locally Advanced or Metastatic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2015-07-02

    Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Rhabdomyosarcoma; Dermatofibrosarcoma Protuberans; Endometrial Stromal Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Uterine Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma; Uterine Carcinosarcoma; Uterine Leiomyosarcoma

  3. [Bacillary epithelioid angiomatosis in advanced HIV infection].

    PubMed

    Hettmannsperger, U; Soehnchen, R; Gollnick, H; Detmar, M; Orfanos, C E

    1993-12-01

    A patient with advanced HIV infection developed multiple angiomatous papules and nodules on the upper chest within a few days. At first sight the lesions resembled disseminated Kaposi's sarcoma; the differential diagnosis, however, included eruptive haemangiomas and pyogenic granulomas. Such distinct clinical characteristics as the collarette-like desquamation at the borders of the tumours led to the suspicion of bacillary epithelioid angiomatosis in HIV infection, which was then confirmed by histology and ultrastructural demonstration of bacillary colonies within the lesions. Under systemic antibiotic treatment, marked regression of the lesions was quickly observed within 1 week and complete regression occurred after 4 weeks. It is important to consider bacillary angiomatosis in HIV infection in the differential diagnosis of Kaposi's sarcoma, and it is a separate entity in the form of angioproliferation caused by bacteria.

  4. Oral mucosal bacillary epithelioid angiomatosis in a patient with AIDS associated with rapid alveolar bone loss: case report.

    PubMed

    Glick, M; Cleveland, D B

    1993-05-01

    Oral manifestations of bacillary epithelioid angiomatosis have been described in the literature, but without histopathologic evidence confirming the presence of the etiologic bacilli. The clinical and histopathologic similarities between bacillary epithelioid angiomatosis and Kaposi's sarcoma may have contributed to confusion in diagnosis and treatment of the latter. Furthermore, inclusion of bacillary epithelioid angiomatosis in the differential diagnosis of proliferative vascular lesions may help to clarify the etiology, pathology and epidemiology of these lesions. This article is the first report of the intraoral manifestation of bacillary epithelioid angiomatosis with histopathologic documentation of the causative pathogen.

  5. Using a family history questionnaire to identify adult patients with increased genetic risk for sarcoma

    PubMed Central

    Schiavi, A.; Lavigne, J.; Turcotte, R.; Kasprzak, L.; Dumas, N.; Chong, G.; Freeman, C.; Alameldin, M.; Galiatsatos, P.; Palma, L.; Foulkes, W.D.

    2015-01-01

    Background Sarcomas in adults can be associated with hereditary cancer syndromes characterized by early-onset predisposition to numerous types of cancer. Because of variability in familial presentation and the largely unexplained genetic basis of sarcomas, ascertainment of patients for whom a genetics evaluation is most indicated poses challenges. We assessed the utility of a Sarcoma Clinic Genetic Screening (scgs) questionnaire in facilitating that task. Methods Between 2008 and 2012, 169 patients (median age: 53 years; range: 17–88 years) completed a self-administered scgs questionnaire. A retrospective chart review was completed for all respondents, and descriptive statistics were reported. Probands were divided into two groups depending on whether they did or did not report a family history of Li–Fraumeni syndrome–type cancers. Results A family history of cancer (as far as 3rd-degree relatives) was reported in 113 of 163 sarcoma patients (69%). Eeles Li–Fraumeni–like (lfl) criteria were fulfilled in 46 probands (28%), Chompret lfl in 21 (13%), Birch lfl in 8 (5%), and classic Li–Fraumeni in none. In the 10 probands tested for TP53 mutations, 1 pathogenic mutation was found. Further investigation of selected families led to the discovery of germline mutations in MLH1, MSH2, and APC genes in 3 individuals. Conclusions The scgs questionnaire was useful for ascertaining probands with sarcoma who could benefit from a genetic assessment. The tool allowed us to identify high-risk families fitting the criteria for lfl and, surprisingly, other hereditary cancer syndromes. Similar questionnaires could be used in other cancer-specific clinics to increase awareness of the genetic component of these cancers. PMID:26628864

  6. Treatment of severe or progressive Kaposi's sarcoma in HIV-infected adults

    PubMed Central

    Gbabe, Oluwatoyin F; Okwundu, Charles I; Dedicoat, Martin; Freeman, Esther E

    2014-01-01

    Background Kaposi's sarcoma remains the most common cancer in Sub-Saharan Africa and the second most common cancer in HIV-infected patients worldwide. Since the introduction of highly active antiretroviral therapy (HAART), there has been a decline in its incidence. However, Kaposi's sarcoma continues to be diagnosed in HIV-infected patients. Objectives To assess the added advantage of chemotherapy plusHAART compared toHAART alone; and the advantages of different chemotherapy regimens in HAART and HAART naive HIV infected adults with severe or progressive Kaposi's sarcoma. Search methods We searched the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE and GATEWAY, the WHO Clinical Trials Registry Platform and the US National Institutes of Health's Clinical Trials.gov for ongoing trials and the Aegis archive of HIV/AIDS for conference abstracts. An updated search was conducted in July 2014. Selection criteria Randomised trials and observational studies evaluating the effects of any chemotherapeutic regimen in combination with HAART compared to HAART alone, chemotherapy versus HAART, and comparisons between different chemotherapy regimens. Data collection and analysis Two review authors assessed the studies independently and extracted outcome data. We used the risk ratio (RR) with a 95% confidence interval (CI) as the measure of effect. We did not conduct meta-analysis as none of the included trials assessed identical chemotherapy regimens. PMID:25221796

  7. Adjuvant Radiotherapy for Pediatric and Young Adult Nonrhabdomyosarcoma Soft-Tissue Sarcoma

    SciTech Connect

    Smith, Kristy B.; Indelicato, Daniel J.; Knapik, Jacquelyn A.; Lagmay, Joanne P.; Morris, Christopher; Kirwan, Jessica M.; Zlotecki, Robert A.; Scarborough, Mark T.; Gibbs, C. Parker; Marcus, Robert B.

    2011-09-01

    Purpose: To evaluate the prognostic factors, outcomes, and complications in patients aged {<=}30 years with resectable nonrhabdomyosarcoma soft-tissue sarcoma treated at the University of Florida with radiotherapy (RT) during a 34-year period. Methods and Materials: A total of 95 pediatric or young adult patients with nonrhabdomyosarcoma soft-tissue sarcoma were treated with curative intent with surgery and RT at the University of Florida between 1973 and 2007. The most common histologic tumor subtypes were synovial sarcoma in 22 patients, malignant fibrous histiocytoma in 19, and malignant peripheral nerve sheath tumor in 11 patients. The mean age at RT was 22 years (range, 6-30). Of the 95 patients, 73 had high-grade tumors; 45 had undergone preoperative RT and 50 postoperative RT. The prognostic factors for survival, local recurrence, and distant recurrence were analyzed. Results: The median follow-up was 7.2 years (range, 0.4-30.5). The actuarial 5-year local control rate was 88%. A microscopically negative margin was associated with superior local control. Although 83% of local recurrence cases initially developed in the absence of metastases, all patients with local failure ultimately died of their disease. The actuarial estimate of 5-year overall survival and disease-free survival was 65% and 63%, respectively. Of all the deaths, 92% were disease related. An early American Joint Committee on Cancer stage, tumor <8 cm, and the absence of neurovascular invasion were associated with superior disease-free survival. The National Cancer Institute Common Toxicity Criteria, version 3, Grade 3-4 treatment complication rate was 9%. No secondary malignancies were observed. Conclusion: In the present large single-institution study, we found positive margins and locally advanced features to be poor prognostic factors for both local progression and survival. The results from the present study have helped to characterize the therapeutic ratio of RT in pediatric and young

  8. Therapeutic Angiotensin-(1-7) in Treating Patients With Metastatic Sarcoma That Cannot Be Removed By Surgery

    ClinicalTrials.gov

    2013-12-10

    Bone Cancer; Chondrosarcoma; Clear Cell Sarcoma of the Kidney; Metastatic Osteosarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Osteosarcoma; Recurrent Uterine Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma

  9. Targeted therapies in rare sarcomas: IMT, ASPS, SFT, PEComa, and CCS.

    PubMed

    Stacchiotti, Silvia; Marrari, Andrea; Dei Tos, Angelo P; Casali, Paolo G

    2013-10-01

    This article highlights the data currently available on the activity of targeted medical treatment in a subgroup of rare entities within soft tissue sarcomas, including inflammatory myofibroblastic tumor, alveolar soft part sarcoma, solitary fibrous tumor, malignant perivascular epithelioid cell tumor (PEComa), and clear cell sarcoma.

  10. Alisertib in Treating Patients With Advanced or Metastatic Sarcoma

    ClinicalTrials.gov

    2016-02-02

    Myxofibrosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Leiomyosarcoma; Recurrent Liposarcoma; Recurrent Malignant Peripheral Nerve Sheath Tumor; Recurrent Undifferentiated Pleomorphic Sarcoma; Stage III Soft Tissue Sarcoma; Stage IV Soft Tissue Sarcoma

  11. Osteosarcomas and adult soft tissue sarcomas: is there a place for high LET radiation therapy?

    PubMed

    Chauvel, P

    1992-04-01

    The treatment policy for non-operable or unresectable osteosarcoma and adult soft tissue sarcoma remains unclear or controversial, despite the progress achieved in multimodality treatments. The poor results obtained by radiotherapy alone led to consider these tumours as radioresistant and to use high Linear Energy Transfer (LET) particles, such as neutrons. These particles benefit from a higher Relative Biological Efficiency (RBE) and other biological properties tending to decrease radioresistance phenomenas. From the non randomized studies previously published, neutron-therapy seems to give better local control rates, compared to photons and/or electrons. But these results are not strongly convincing, due to the large heterogeneities in patient recruitment, histological types, sizes, sites and moreover to the high complication rates encountered in some studies, even if they are mainly imputable to the use of low energy machines. The use of high-energy hospital-based accelerators combined to the possibilities of accurate dose distribution offered by conformal therapy, the potential value of light ion beam therapy combining the dose distribution advantages of protons to the biological properties of high LET particles, represent the directions in which progresses might be made for further improvement of non-operable or unresectable osteosarcoma and soft tissue sarcomas treatment results. PMID:1622850

  12. Epithelioid osteosarcoma of the scapula.

    PubMed

    Herget, G W; Otto, C; Kurz, P; Uhl, M; Adler, C-P; Südkamp, N P; Hauschild, O

    2014-01-01

    Epithelioid and epithelial neoplasms of bone are rare. They include different epithelioid variants of vascular lesions, osteoblastoma, chondroblastoma and most importantly metastatic carcinoma. Up to now, only few cases of epithelioid osteosarcoma were described. In this case the authors report a 53-year-old patient presented with a medical history of chronic shoulder pain for 3 years. Magnetic resonance imaging (MRI and computed tomography (CT) showed a destructive, partially calcified osseous lesion of the scapula with expansion into the surrounding soft tissue, suggestive of a primary bone tumor. Histologically, the tumor consisted of epithelioid cells with expression of cytokeratine and the lesion was primarily diagnosed as metastatic carcinoma. With regard to the MRI morphology untypical for metastatic disease the histopathologic slides were re-evaluated and detection of tumor osteoid led to the diagnosis of epithelioid osteosarcoma. Chemotherapy was initiated, however follow-up imaging studies showed rapidly progressive disease of both primary tumor and lung metastases. In conclusion, epithelioid neoplasms of the bone are extremetumourly rare and must be distinguished from metastatic carcinoma. Despite the presence of cytokeratine positive cells a thorough histological evaluation is mandatory and osteoid detection is essential in order to establish the correct diagnosis and further treatment. Key words: osteosarcoma, epithelioid, aneurysmal bone cyst, chondrosarcoma, pathology, immunohistochemistry. PMID:25137500

  13. Hepatic undifferentiated (embryonal) sarcoma in an adult: a case report and review of the literature.

    PubMed

    Tokunaga, Y; Ryo, J; Hoppou, T; Kitaoka, A; Tokuka, A; Osumi, K; Tanaka, T

    2000-11-01

    Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignant tumour with a poor prognosis. The absence of specific symptoms, the rapid tumour growth, the normality of the common tumour markers, and the consequential delay in the diagnosis often result in significant enlargement of the tumour. To our knowledge, there have been only 42 reported cases of USL in adults worldwide during the 40 years since the clinicopathological entity of USL was defined. We report here a 27-year-old male with USL who has been treated successfully with surgical resection and adjuvant chemotherapy using ifosfamide, adriamycin and cisplatin. Although the prognosis of the disease remains generally poor, long-term survival has been achieved recently in patients with a combination of surgery and multi-agent chemotherapy.

  14. General Information about Kaposi Sarcoma

    MedlinePlus

    ... Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma Go to Health Professional Version ... the PDQ Adult Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  15. General Information About Uterine Sarcoma

    MedlinePlus

    ... Research Uterine Sarcoma Treatment (PDQ®)–Patient Version General Information About Uterine Sarcoma Go to Health Professional Version ... the PDQ Adult Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  16. Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma

    ClinicalTrials.gov

    2016-09-23

    Adult Rhabdomyosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Chordoma; Desmoid Tumor; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Previously Untreated Childhood Rhabdomyosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  17. Whole Lung Irradiation for Adults With Pulmonary Metastases From Ewing Sarcoma

    SciTech Connect

    Casey, Dana L.; Alektiar, Kaled M.; Gerber, Naamit K.; Wolden, Suzanne L.

    2014-08-01

    Purpose: To evaluate feasibility and patterns of failure in adult patients with Ewing sarcoma (ES) treated with whole lung irradiation (WLI) for pulmonary metastases. Methods and Materials: Retrospective review of all ES patients treated at age 18 or older with 12-15 Gy WLI for pulmonary metastases at a single institution between 1990 and 2014. Twenty-six patients met the study criteria. Results: The median age at WLI was 23 years (range, 18-40). The median follow-up time of the surviving patients was 3.8 years (range, 1.0-9.6). The 3-year cumulative incidence of pulmonary relapse (PR) was 55%, with a 3-year cumulative incidence of PR as the site of first relapse of 42%. The 3-year event-free survival (EFS) and overall survival (OS) were 38 and 45%, respectively. Patients with exclusively pulmonary metastases had better outcomes than did those with extrapulmonary metastases: the 3-year PR was 45% in those with exclusively lung metastases versus 76% in those with extrapulmonary metastases (P=.01); the 3-year EFS was 49% versus 14% (P=.003); and the 3-year OS was 61% versus 13% (P=.009). Smoking status was a significant prognostic factor for EFS: the 3-year EFS was 61% in nonsmokers versus 11% in smokers (P=.04). Two patients experienced herpes zoster in the radiation field 6 and 12 weeks after radiation. No patients experienced pneumonitis or cardiac toxicity, and no significant acute or late sequelae were observed among the survivors. Conclusion: WLI in adult patients with ES and lung metastases is well tolerated and is associated with freedom from PR of 45% at 3 years. Given its acceptable toxicity and potential therapeutic effect, WLI for pulmonary metastases in ES should be considered for adults, as it is in pediatric patients. All patients should be advised to quit smoking before receiving WLI.

  18. Epidemiology and survivorship of soft tissue sarcomas in adults: a national cancer database reporta

    PubMed Central

    Corey, Robert M; Swett, Katrina; Ward, William G

    2014-01-01

    The National Cancer Data Base (NCDB) of the American College of Surgeons gather demographic and survival data on ∼70% of cancers in the USA. We wanted to investigate the demographic and survivorship data on this potentially more representative cohort of patients with soft tissue sarcomas. We selected 34 of the most commonly encountered soft tissue sarcomas reported to the NCDB, provided that each entity contained a minimum of 50 cases. This report summarizes the demographic and survivorship data on 63,714 patients with these 34 histologically distinct soft tissue sarcomas reported to the NCDB from 1998 to 2010. The overall survivorships of these sarcomas were near the lower limits of many prior reports due to the all-inclusive, minimally biased inclusion criteria. The overall best prognosis was Dermatofibrosarcoma NOS (not otherwise specified). (5-year survivorship 92%). The worst prognosis was Dedifferentiated Chondrosarcoma (5-year survivorship 19%). New observations included Biphasic Synovial Sarcoma demonstrating a better 5-year survivorship (65%) compared to spindle-cell synovial sarcoma (56%, P < 0.031) and Synovial Sarcoma, NOS (52%, P < 0.001). The demographic and 2- and 5-year survivorship data for all 34 soft tissue sarcomas are presented herein. This extent of demographic and survival data in soft tissue sarcomas is unprecedented. Because of the large number of cases and the inclusive nature of the NCDB, without restriction to certain stages, categories, or treatments, it is less subject to selection bias. Therefore, these data are thought to be more reflective of the true overall prognosis given the current management of sarcoma across the NCDB contributing sites. PMID:25044961

  19. Modulation of u-PA, MMPs and their inhibitors by a novel nutrient mixture in adult human sarcoma cell lines.

    PubMed

    Roomi, M Waheed; Kalinovsky, Tatiana; Niedzwiecki, Aleksandra; Rath, Matthias

    2013-07-01

    Adult sarcomas are highly aggressive tumors that are characterized by high levels of matrix metalloproteinase (MMP)-2 and -9 secretions that degrade the ECM and basement membrane, allowing cancer cells to spread to distal organs. Proteases play a key role in tumor cell invasion and metastasis by digesting the basement membrane and ECM components. Strong clinical and experimental evidence demonstrates association of elevated levels of u-PA and MMPs with cancer progression, metastasis and shortened patient survival. MMP activities are regulated by specific tissue inhibitors of metalloproteinases (TIMPs). Our main objective was to study the effect of a nutrient mixture (NM) on the activity of u-PA, MMPs and TIMPs in various human adult sarcomas. Human fibrosarcoma (HT-1080), chondrosarcoma (SW-1353), liposarcoma (SW-872), synovial sarcoma (SW-982) and uterine leimyosarcoma (SK-UT-1) cell lines (ATCC) were cultured in their respective media and treated at confluence with NM at 0, 50, 100, 250, 500 and 1,000 µg/ml. Analysis of u-PA activity was carried out by fibrin zymography, MMPs by gelatinase zymography and TIMPs by reverse zymography. Fibrosarcoma, chondrosarcoma, liposarcoma and leiomyosarcoma cancer cell lines expressed u-PA, which was inhibited by NM in a dose-dependent manner. However, no bands corresponding to u-PA were detected for synovial sarcoma cells. On gelatinase zymography, fibrosarcoma, chondrosarcoma, liposarcoma and synovial sarcoma showed bands corresponding to MMP-2 and MMP-9 with enhancement of MMP-9 with PMA (100 ng/ml) treatment. Uterine leiomyosarcoma showed strong bands corresponding to inactive and active MMP-9 and a faint band corresponding to MMP-9 dimer induced with PMA treatment, but no MMP-2 band. NM inhibited their expression in a dose-dependent manner. Activity of TIMPs was upregulated by NM in all cancer cell lines in a dose-dependent manner. Analysis revealed a positive correlation between u-PA and MMPs and a negative correlation

  20. Undifferentiated embryonal sarcoma of the liver in a middle-aged adult with systemic lupus erythematosus

    PubMed Central

    2013-01-01

    Adult primary undifferentiated embryonal sarcoma of the liver (UESL) is a rare disease. While the etiology of UESL remains largely unknown, association with systemic inflammatory disorders has been observed. Here, we report a case of UESL in a 46-year-old woman with systemic lupus erythematosus (SLE) and without chronic hepatitis or liver cirrhosis. Systematic review of the publicly available English language medical literature identified only 27 cases of UESL in patients aged >45 years and none with SLE. Our patient presented with abdominal pain and had a 2-year history of SLE. Abdominal ultrasonography and enhanced computed tomography revealed a solid mass in the right lobe of the liver. Presumptive diagnosis of atypical hepatocellular carcinoma was made and the patient was treated with segmentectomy of S5 and S4a and cholecystectomy. The final diagnosis of UESL was made according to the pathology results. Since SLE patients may be at increased risk of malignancy, it is possible that the SLE pathogenesis may have contributed to the development of UESL in our patient. According to this case, UESL should be considered when SLE patients present with hepatic space-occupying lesions. PMID:24073982

  1. FDG-PET/CT Imaging Predicts Histopathologic Treatment Responses after Neoadjuvant Therapy in Adult Primary Bone Sarcomas

    DOE PAGES

    Benz, Matthias R.; Czernin, Johannes; Tap, William D.; Eckardt, Jeffrey J.; Seeger, Leanne L.; Allen-Auerbach, Martin S.; Dry, Sarah M.; Phelps, Michael E.; Weber, Wolfgang A.; Eilber, Fritz C.

    2010-01-01

    Purpose . Tmore » he aim of this study was to prospectively evaluate whether FDG-PET allows an accurate assessment of histopathologic response to neoadjuvant treatment in adult patients with primary bone sarcomas. Methods . Twelve consecutive patients with resectable, primary high grade bone sarcomas were enrolled prospectively. FDG-PET/CT imaging was performed prior to the initiation and after completion of neoadjuvant treatment. Imaging findings were correlated with histopathologic response. Results . Histopathologic responders showed significantly more pronounced decreases in tumor FDG-SUVmax from baseline to late follow up than non-responders ( 64 ± 19 % versus 29 ± 30 %, resp.; P = .03 ). Using a 60% decrease in tumor FDG-uptake as a threshold for metabolic response correctly classified 3 of 4 histopathologic responders and 7 of 8 histopathologic non-responders as metabolic responders and non-responders, respectively (sensitivity, 75%; specificity, 88%). Conclusion . These results suggest that changes in FDG-SUVmax at the end of neoadjuvant treatment can identify histopathologic responders and non-responders in adult primary bone sarcoma patients.« less

  2. Epithelioid Angiosarcoma With Metastatic Disease After Endovascular Therapy of Abdominal Aortic Aneurysm

    SciTech Connect

    Schmehl, Joerg; Scharpf, Marcus; Brechtel, Klaus; Kalender, Guenay; Heller, Stephan; Claussen, Claus D.; Lescan, Mario

    2012-02-15

    Malignancies of the aortic wall represent a rare condition, and only a few reports have covered cases of sarcomas arising at the site of a prosthesis made of Dacron. A coincidence with endovascular repair has only been reported in one case to date. We report a patient with epithelioid angiosarcoma and metastatic disease, which was found in an aneurysmal sac after endovascular aortic repair for abdominal aortic aneurysm.

  3. Biphasic solitary fibrous tumour: a report of two cases with epithelioid features.

    PubMed

    Awasthi, R; O'Neill, J K; Keen, C E; Sarsfield, P T L; Devaraj, V S; Stone, C A; Smith, M E F

    2006-03-01

    We present two cases of solitary fibrous tumour (SFT) showing biphasic morphology with a spectrum of malignant epithelioid components. Slides prepared from formalin-fixed and paraffin-embedded tissue from both cases were stained with haematoxylin and eosin and by immunohistochemistry. Interphase fluorescent in situ hybridisation studies were performed in both cases using paraffin-embedded tissue to look for the t(X;18) translocation, thereby to exclude synovial sarcoma. Both cases showed biphasic morphology with some areas having typical benign spindled SFT morphology (including CD34 expression) and other areas having a malignant epithelioid appearance. In one of the cases, the epithelioid area, which was well circumscribed and showed packeting of cell groups, demonstrated expression of cytokeratin and epithelial cadherin but not of CD34. In the second case, the immunophenotype of the epithelioid component was similar to that of the benign SFT component. These findings suggest that epithelioid change in SFT shows a range of differentiation at one end, similar to that of a standard SFT, and at the other end, possibly acquiring epithelial characteristics.

  4. Solitary fibrous tumor of the thigh with epithelioid features: a case report

    PubMed Central

    Martorell, Miguel; Pérez-Vallés, Ana; Gozalbo, Francisco; Garcia-Garcia, Jose Angel; Gutierrez, Jair; Gaona, John

    2007-01-01

    Background Extrapleural Solitary Fibrous tumors (SFTs) have been increasingly reported. The retroperitoneum, deep soft tissues of proximal extremities, abdominal cavity, trunk, head and neck are the most common extraserosal locations reported. Microscopically they show a wide range of morphological features, and so the differential diagnosis is extensive. Immunohistochemically, they commonly express CD34, vimentin, bcl-2 and CD99. Epithelial membrane antigen (EMA) and smooth muscle actin (SMA) may occasionally be expressed. Epithelioid morphology in extrapleural SFT has only very occasionally been described (five cases reported), some of them with biphasic pattern and others with malignant characteristics. Case presentation A SFT of the thigh with epithelioid areas in a 63 year old woman is reported. Microscopically the tumor showed areas hypo and hipercellular. At the periphery of the hipercellular areas there were nodules composed of epithelioid cells. Immunohistochemically both the spindle and epithelioid cells were positive for CD34, vimentin, bcl-2 and CD99. Epithelial, neural and muscular markers were negative. Molecular study was done and ruled out a synovial sarcoma. Conclusion Ten cases of SFT of the thigh have been reported but to our knowledge this is the first case with epithelioid morphology affecting the extremities. Identification of this pattern of SFT is of importance, to avoid misdiagnosis with other more aggressive conditions in soft tissue. PMID:17577399

  5. Long-Term Neurotoxicity of Chemotherapy in Adolescents and Young Adults Treated for Bone and Soft Tissue Sarcomas

    PubMed Central

    Connolly, Sean; Latoufis, Christos; Eagle, Karen; Ash, Catherine M.; Fowler, Clare; Souhami, Robert L.

    1998-01-01

    Purpose. To study the long-term neurotoxicity of chemotherapy in adolescents and young adults treated for bone and soft tissue sarcomas. Patients and Methods. Thirty-six adolescents and young adults (median age 17 years) were examined following chemotherapy for bone and soft tissue sarcomas. Twenty-nine (29/36) had received cisplatin (median 400 mg/m2), 15/36 ifosfamide (median 20 g/m2), and 12/36 vincristine (median 16 mg). Neurotoxicity was assessed at a median of 8 months (range, 1–54 months) after completion of chemotherapy by clinical examination, nerve conduction studies, audiograms and autonomic function tests. The same nerve conduction studies were carried out in 20 normal volunteers to define normal ranges in this age group. Results. Sixteen patients (44%) had a significant reduction in deep tendon reflexes, and this clinical parameter correlated well with abnormalities detected in nerve conduction studies. Vibration perception threshold (VPT) was raised in 20/36 patients (55%) and this was the most sensitive single test in the assessment of neuropathy. There was a significant correlation between VPT and cumulative cisplatin dose received in mg/m-2 (r=0.607, p<0.01). Ten of 29 patients (35%) had abnormal nerve conduction studies with a pattern characteristic of sensory axonal neuropathy. No patient complained of auditory symptoms, but minor high tone hearing loss was detected by audiograms in 5/28 patients who had received cisplatin. No patients had symptoms of autonomic neuropathy, but autonomic function tests showed minor abnormalities in 4/22 patients tested, and all had received cisplatin. Conclusions. This study demonstrates significant, although asymptomatic, long-term neurotoxicity of cisplatin in adolescents and young adults receiving chemotherapy for bone and soft tissue sarcomas. Follow-up studies are planned to assess whether these neurological deficits improve with time. PMID:18521240

  6. Occupational factors and risk of adult bone sarcomas: a multicentric case-control study in Europe.

    PubMed

    Merletti, Franco; Richiardi, Lorenzo; Bertoni, Franco; Ahrens, Wolfgang; Buemi, Antoine; Costa-Santos, Cristina; Eriksson, Mikael; Guénel, Pascal; Kaerlev, Linda; Jöckel, Karl-Heinz; Llopis-Gonzalez, Agustin; Merler, Enzo; Miranda, Ana; Morales-Suárez-Varela, Maria M; Olsson, Håkan; Fletcher, Tony; Olsen, Jorn

    2006-02-01

    We investigated the association between occupational factors and risk of bone sarcoma, a rare tumor with a largely unknown aetiology. A multicentric case-control study was conducted in 7 European countries in 1995-97. Ninety-six cases aged 35-69 years with a centrally reviewed diagnosis of bone sarcoma (68 chondrosarcomas and 28 osteosarcomas) were compared to 2,632 population (68%) or colon cancer (32%) controls. Subjects were interviewed to obtain information on occupational, medical and reproductive history, smoking and alcohol consumption and selected exposures including use of pesticides. Response proportions were 90% among cases and 66% among controls. Odds ratios (OR) and 95% confidence intervals (CI) were estimated for selected categories of job titles and branches of industry and for use of pesticides. We found an increased OR for bone sarcoma among blacksmiths, toolmakers, machine-tool operators (OR = 2.14, 95% CI 1.08-4.26), woodworkers (OR = 2.68, 95% CI 1.36-5.29) and construction workers (OR = 1.62, 95% CI 0.92-2.87). Ever users of pesticide had an OR of 2.33 (95% CI 1.31-4.13), with similar risks for exposure to insecticides and exposure to herbicides. Neither duration of employment in any of the analyzed occupational categories nor duration of use of pesticides showed an increasing trend in the risk of bone sarcoma. ORs of bone sarcoma were 1.03 (95% CI 0.23-4.57), 3.13 (95% CI 1.26-7.76) and 1.44 (95% CI 0.43-4.85) for the first, second and third tertile of days of use of pesticides. Our study suggests that novel and previously reported (woodworking) occupational factors play a role in the aetiology of bone sarcomas.

  7. Pediatric Sarcomas.

    PubMed

    Williams, Regan F; Fernandez-Pineda, Israel; Gosain, Ankush

    2016-10-01

    Pediatric sarcomas are a heterogeneous group of tumors accounting for approximately 10% of childhood solid tumors. Treatment is focused on multimodality therapy, which has improved the prognosis over the past two decades. Current regimens focus on decreasing treatment for low-risk patients to decrease the long-term side effects while maximizing therapy for patients with metastatic disease to improve survival. Pediatric sarcomas can be divided into soft tissue sarcomas and osseous tumors. Soft tissue sarcomas are further delineated into rhabdomyosarcomas, which affect young children and nonrhabdomyosarcomas, which are most common in adolescents. The most common bone sarcomas are osteosarcomas and Ewing's sarcoma. PMID:27542645

  8. Ewing sarcoma

    MedlinePlus

    Bone cancer - Ewing sarcoma; Ewing family of tumors; Primitive neuroectodermal tumors (PNET); Bone neoplasm - Ewing sarcoma ... NCCN clinical practice guidelines in oncology (NCCN guidelines): bone cancer. Updated 2016. www.nccn.org/professionals/physician_gls/ ...

  9. Primary Renal Sclerosing Epithelioid Fibrosarcoma: Report of Two Cases with EWSR1-CREB3L1 Gene Fusion

    PubMed Central

    Argani, Pedram; Lewin, Jack R.; Edmonds, Pamela; Netto, George J.; Prieto-Granada, Carlos; Zhang, Lei; Jungbluth, Achim A.; Antonescu, Cristina R.

    2014-01-01

    We report the first two genetically confirmed cases of primary renal sclerosing epithelioid fibrosarcoma (SEF), occurring in a 17 year-old male and a 61 year-old female. In both cases, the tumors demonstrated the typical epithelioid clear cell morphology associated with extensive hyalinizing fibrosis, raising the differential diagnosis of solitary fibrous tumor, metanephric stromal tumor, and the sclerosing variant of clear cell sarcoma of the kidney. Both neoplasms demonstrated diffuse immunoreactivity for MUC4, a highly specific marker for SEF, and both demonstrated evidence of rearrangement of both the EWSR1 and CREB3L1 genes which have recently shown to be fused in this entity. Both neoplasms presented with metastatic disease. Primary renal SEF represents yet another translocation-associated sarcoma now shown to arise primarily in the kidney. PMID:25353281

  10. Adjuvant epirubicin with or without Ifosfamide for adult soft-tissue sarcoma.

    PubMed

    Petrioli, Roberto; Coratti, Andrea; Correale, Pierpaolo; D'Aniello, Carlo; Grimaldi, Luca; Tanzini, Gabriello; Civitelli, Serenella; Marsili, Stefania; Messinese, Simona; Marzocca, Giuseppe; Pirtoli, Luigi; Francini, Guido

    2002-10-01

    This randomized study compared the efficacy of epirubicin-based adjuvant chemotherapy on the disease-free interval (DFI) and overall survival of patients with high-risk soft-tissue sarcomas. After curative surgery, 43 of the 88 enrolled patients were assigned to surgery with or without radiotherapy and 45 to surgery plus chemotherapy (26 epirubicin, 19 epirubicin + ifosfamide) with or without radiotherapy. The trial closed prematurely because of poor patient accrual. There was a statistical significant difference in the 5-year disease-free survival of the patients receiving adjuvant chemotherapy with or without radiotherapy (69%) and that of those treated with surgery with or without radiotherapy (44%) ( p= 0.01). The 5-year survival of the patients treated with adjuvant chemotherapy with or without radiotherapy was 72% as against 47% of those treated with surgery with or without radiotherapy ( p= 0.06). The power of the study was 0.65 for both the DFI and overall survival. The results of the study suggest a possible advantage of epirubicin-based adjuvant chemotherapy in patients with soft-tissue sarcoma at high risk of relapse. PMID:12393986

  11. Local Mesenchymal Stem/Progenitor Cells Are a Preferential Target for Initiation of Adult Soft Tissue Sarcomas Associated with p53 and Rb Deficiency

    PubMed Central

    Choi, Jinhyang; Curtis, Stephen J.; Roy, David M.; Flesken-Nikitin, Andrea; Nikitin, Alexander Yu.

    2010-01-01

    The cell of origin and pathogenesis of the majority of adult soft tissue sarcomas (STS) remains poorly understood. Because mutations in both the P53 and RB tumor suppressor genes are frequent in STS in humans, we inactivated these genes by Cre-loxP–mediated recombination in mice with floxed p53 and Rb. Ninety-three percent of mice developed spindle cell/pleomorphic sarcomas after a single subcutaneous injection of adenovirus carrying Cre-recombinase. Similar to human STS, these sarcomas overexpress Cxcr4, which contributes to their invasive properties. Using irradiation chimeras generated by transplanting bone marrow cells from mice carrying either the Rosa26StoploxPLacZ or the Z/EG reporter, as well as the floxed p53 and Rb genes, into irradiated p53loxP/loxPRbloxP/loxP mice, it was determined that sarcomas do not originate from bone marrow–derived cells, such as macrophages, but arise from the local resident cells. At the same time, dermal mesenchymal stem cells isolated by strict plastic adherence and low levels of Sca-1 expression (Sca-1low, CD31negCD45neg) have shown enhanced potential for malignant transformation according to soft agar, invasion, and tumorigenicity assays, after the conditional inactivation of both p53 and Rb. Sarcomas formed after transplantation of these cells have features typical for undifferentiated high-grade pleomorphic sarcomas. Taken together, our studies indicate that local Sca-1low dermal mesenchymal stem/progenitor cells are preferential targets for malignant transformation associated with deficiencies in both p53 and Rb. PMID:20864684

  12. Jaw Dysfunction Related to Pterygoid and Masseter Muscle Dosimetry After Radiation Therapy in Children and Young Adults With Head-and-Neck Sarcomas

    SciTech Connect

    Krasin, Matthew J.; Wiese, Kristin M.; Spunt, Sheri L.; Hua, Chia-ho; Daw, Najat; Navid, Fariba; Davidoff, Andrew M.; McGregor, Lisa; Merchant, Thomas E.; Kun, Larry E.; McCrarey, Lola; and others

    2012-01-01

    Purpose: To investigate the relationship between jaw function, patient and treatment variables, and radiation dosimetry of the mandibular muscles and joints in children and young adults receiving radiation for soft-tissue and bone sarcomas. Methods and Materials: Twenty-four pediatric and young adult patients with head-and-neck sarcomas were treated on an institutional review board-approved prospective study of focal radiation therapy for local tumor control. Serial jaw depression measurements were related to radiation dosimetry delivered to the medial and lateral pterygoid muscles, masseter muscles, and temporomandibular joints to generate mathematical models of jaw function. Results: Baseline jaw depression was only influenced by the degree of surgical resection. In the first 12 weeks from initiation of radiation, surgical procedures greater than a biopsy, administration of cyclophosphamide containing chemotherapy regimes, and large gross tumor volumes adversely affected jaw depression. Increasing dose to the pterygoid and masseter muscles above 40 Gy predicted loss of jaw function over the full course of follow-up. Conclusions: Clinical and treatment factors are related to initial and subsequent jaw dysfunction. Understanding these complex interactions and the affect of specific radiation doses may help reduce the risk for jaw dysfunction in future children and young adults undergoing radiation therapy for the management of soft-tissue and bone sarcomas.

  13. Evaluation of Dose-Intense Ifosfamide, with and Without Edatrexate, in Adults with Sarcoma

    PubMed Central

    Schwartz, Gary M.; Leung, Denis; Sugarman, Alison; Bertino, Joseph R.

    1999-01-01

    Purpose. To define the maximally tolerated dose (MTD) of ifosfamide when given with G-CSF on an every other week schedule, and to define the MTD of edatrexate that can be given every two weeks with an intense schedule of ifosfamide. Patients and Methods. Forty-one patients with metastatic or unresectable, locally advanced sarcoma participated in this 2-step phase I trial.The starting dose of ifosfamide was 10 gm/m2 given by continuous intravenous infusion over 4 days every 2 weeks.When the MTD was defined, edatrexate, beginning at a dose of 40 mg/m2 intravenously every 2 weeks was added in subsequent cohorts of patients. Results. Myelosuppression was the most prominent toxicity. Fatigue, nausea, and vomiting were observed in the majority of patients. Ifosfamide 12 gm/m2 given every 2 weeks approached or exceeded the MTD. Edatrexate 100 mg/m2 could be given safety as an intravenous bolus with ifosfamide 10 gm/m2 every 2 weeks. Therapeutic responses were observed in patients with measurable disease. Conclusions. This study demonstrates the feasibility of administering a dose-intense schedule of ifosfamide alone or ifosfamide with edatrexate that might be applied in the adjuvant or neo-adjuvant setting. PMID:18521274

  14. Hepatic Epithelioid Hemangioendothelioma in an Eight Year old - A Case Report

    PubMed Central

    Nayal, Bhavna; Nagel, Bhawna; Rao, Lakshmi

    2014-01-01

    Hepatic epithelioid haemangio-endothelioma (HEHE) is a rare vascular tumour of endothelial origin. The etiology of this tumour is unknown and has a variable clinical outcome. It usually affects adults and is extremely rare in children. Histologically, HEHE is characterized by epithelioid to dendritic tumour cells with intracytoplasmic lumina containing RBCs and a myxoid to sclerotic stroma. Being a rare entity, awareness and a high degree of suspicion is required to correctly identify this tumour. We report a case of hepatic hemangioendothelioma in an 8-year-old child. PMID:25653958

  15. Hepatic Epithelioid Hemangioendothelioma in an Eight Year old - A Case Report.

    PubMed

    Vasudevan, Geetha; Nayal, Bhavna; Nagel, Bhawna; Rao, Lakshmi

    2014-12-01

    Hepatic epithelioid haemangio-endothelioma (HEHE) is a rare vascular tumour of endothelial origin. The etiology of this tumour is unknown and has a variable clinical outcome. It usually affects adults and is extremely rare in children. Histologically, HEHE is characterized by epithelioid to dendritic tumour cells with intracytoplasmic lumina containing RBCs and a myxoid to sclerotic stroma. Being a rare entity, awareness and a high degree of suspicion is required to correctly identify this tumour. We report a case of hepatic hemangioendothelioma in an 8-year-old child. PMID:25653958

  16. Retroperitoneal Sarcomas.

    PubMed

    Porpiglia, Andrea S; Reddy, Sanjay S; Farma, Jeffrey M

    2016-10-01

    Retroperitoneal sarcomas are rare tumors, representing only 15% of all sarcomas. The mainstay of therapy is surgical resection with negative margins. However, this is challenging because of the late presentation of many of these tumors and involvement with adjacent structures. Decisions on radiation therapy and chemotherapy should be made in a multidisciplinary setting at a tertiary referral center.

  17. [Extraskeletal Ewing's sarcoma].

    PubMed

    Baram, J; Tichler, T; Nass, D; Brenner, H J

    1992-01-01

    5 patients diagnosed as having extraskeletal Ewing's sarcoma have been referred to our adult oncology unit since 1980. All were men, ranging in age from 18-57 (mean 32 years). The primary tumor was located on the trunk in 4 and in an extremity in 1. Wide tumor excision was feasible in only 2. 3 died within 27 months and 2 are alive, 13 and 67 months, respectively, following diagnosis. This study demonstrates the highly aggressive nature of extraskeletal Ewing's sarcoma and the need for early diagnosis and efficient chemotherapy.

  18. [Epithelioid hemangioendothelioma: an uncommon liver tumor].

    PubMed

    Pareja, Eugenia; Cortés, Miriam; Rayon, Miguel; Moya, Angel; Mir, Jose

    2010-01-01

    We report the case of a female patient who was referred to our unit because of a solid liver tumor, suggestive of metastasis. After biopsy, the patient was diagnosed with epithelioid hemangioendothelioma of the liver. Epithelioid hemangioendothelioma is a rare entity with an unpredictable, potentially fatal, clinical course and outcome. Due to its rarity, this entity should be considered when a solitary hepatic lesion is detected and should be included in the differential diagnosis with liver metastases. We highlight the infrequency of this tumor, its presentation as a solitary hepatic lesion and the indication of surgical treatment. We describe the clinical and pathological characteristics of epithelioid hemangioendothelioma of the liver and report a new case of this entity. The distinct therapeutic options are discussed.

  19. Primary pleuropulmonary synovial sarcoma.

    PubMed

    Mirzoyan, Michael; Muslimani, Ala'a; Setrakian, Sebouh; Swedeh, Mohamed; Daw, Hamed A

    2008-09-01

    Pleuropulmonary synovial sarcoma (PPSS) is increasingly recognized as a subtype of sarcoma because of the recent identification of a distinctive chromosomal translocation specific to synovial sarcoma. Soft-tissue synovial sarcoma is far more common than PPSS and typically develops in para-articular locations of the extremities, affects young and middle-aged adults, with no difference in distribution between the sexes, and has well-documented radiologic manifestations. Pleuropulmonary synovial sarcoma can arise in the chest wall, heart, mediastinum, pleura, or lung, and it shares patient demographics and several imaging features with its soft-tissue counterpart. Patients present with a cough, chest pain, or dyspnea. On chest radiographs, PPSS typically appears as a sharply marginated mass with uniform opacity, based in the pleura or in the lung, and often accompanied by an ipsilateral pleural effusion. Computed tomographic images show a well-circumscribed, heterogeneously enhanced lesion without associated involvement of bone and without calcifications (except in the case of a chest wall primary tumor). Magnetic resonance imaging provides superior demonstration of nodular soft tissue and multilocular fluid-filled internal components of PPSS, in addition to peripheral rim enhancement after the intravenous administration of a gadoliniumbased contrast material such as gadopentetate dimeglumine. Current treatment consists of surgical resection followed by chemotherapy, radiation therapy, or both. PMID:18824448

  20. Suprasellar epithelioid hemangioendothelioma: Case report and review of the literature

    PubMed Central

    Barger, James; Tanweer, Omar; Liechty, Benjamin; Snuderl, Matija; Jafar, Jafar J.

    2016-01-01

    Background: Epithelioid hemangioendothelioma (EHE) is a rare sarcoma of vascular origin, which is clinically and histologically intermediate between benign hemangioma and angiosarcoma. It is most commonly found in the liver, lung, and bone, however, 46 intracranial cases have been reported in the literature, of which this is the fifth reported suprasellar tumor. Case Description: A 45-year-old woman developed progressive lethargy, somnolence, and memory decline over the course of 6 months. On computed tomography (CT), she was found to have a large hypothalamic mass and underwent subtotal resection via a bifrontal craniotomy. Conclusions: While primary intracranial EHE is an uncommon presentation of a rare tumor, the suprasellar region does not seem to be an unusual location when it does occur. Prognosis is generally good, and may be better for primary intracranial disease than that for EHE originating elsewhere. Surgery is the first line of therapy, with variable benefit from adjuvant chemotherapy or radiation when total resection is not possible. Chemotherapeutic approaches in current use are directed at preventing endothelial proliferation. PMID:27656318

  1. Suprasellar epithelioid hemangioendothelioma: Case report and review of the literature

    PubMed Central

    Barger, James; Tanweer, Omar; Liechty, Benjamin; Snuderl, Matija; Jafar, Jafar J.

    2016-01-01

    Background: Epithelioid hemangioendothelioma (EHE) is a rare sarcoma of vascular origin, which is clinically and histologically intermediate between benign hemangioma and angiosarcoma. It is most commonly found in the liver, lung, and bone, however, 46 intracranial cases have been reported in the literature, of which this is the fifth reported suprasellar tumor. Case Description: A 45-year-old woman developed progressive lethargy, somnolence, and memory decline over the course of 6 months. On computed tomography (CT), she was found to have a large hypothalamic mass and underwent subtotal resection via a bifrontal craniotomy. Conclusions: While primary intracranial EHE is an uncommon presentation of a rare tumor, the suprasellar region does not seem to be an unusual location when it does occur. Prognosis is generally good, and may be better for primary intracranial disease than that for EHE originating elsewhere. Surgery is the first line of therapy, with variable benefit from adjuvant chemotherapy or radiation when total resection is not possible. Chemotherapeutic approaches in current use are directed at preventing endothelial proliferation.

  2. Kaposi's Sarcoma

    MedlinePlus

    Kaposi's sarcoma (KS) is a cancer that causes patches of abnormal tissue to grow under the skin, in the lining of ... of cancer cells, blood vessels, and blood cells. KS is caused by infection with human herpesvirus-8 ( ...

  3. 18F-FLT Positron Emission Tomography and Diffusion-Weighted Magnetic Resonance Imaging in Planning Surgery and Radiation Therapy and Measuring Response in Patients With Newly Diagnosed Ewing Sarcoma

    ClinicalTrials.gov

    2016-03-15

    Adult Supratentorial Primitive Neuroectodermal Tumor (PNET); Ewing Sarcoma of Bone; Extraosseous Ewing Sarcoma; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Untreated Childhood Supratentorial Primitive Neuroectodermal Tumor

  4. Myeloid sarcoma of the orbit without systemic recurrence of disease in an adult: A clinicopathological case report.

    PubMed

    Grigalunas, Alexander L; Mizen, Thomas R

    2016-01-01

    Myeloid sarcoma (MS), also known as granulocytic sarcoma (GS) or chloroma (named for the greenish hue attributed to the pigment of myeloperoxidase [MPO]), is a rare solid tumor with a predilection for the orbit. MS usually occurs in conjunction with acute myeloid leukemia (AML), myelodysplastic syndrome (MDS), or myeloproliferative disorder (MPS) and may be the harbinger of disease. Therefore, prompt diagnosis is essential for patient survival. We present a rare case of a 61 year old with an isolated orbital MS without active leukemia.

  5. Epithelioid hemangioendothelioma of hypopharynx: A rare presentation.

    PubMed

    Sancheti, Sankalp; Singh, Jiten N; Malik, Akshat; Devi, Kalpana Th; Jain, Shivani

    2016-01-01

    Epithelioid hemangioendothelioma is a rare vascular neoplasm of borderline or low-grade malignant potential uncommonly seen in head and neck region and has not been reported in the hypopharynx. We present here a case of epithelioid hemangioendothelioma arising from the hypopharynx in a young female patient who presented with progressive dysphagia for 1-year and difficulty in breathing for 1-month. This is first reported case in published English literature to the best of our knowledge. Recognition of this borderline entity is necessary because of its potential for malignant transformation and recurrence. A wide excision and regular clinical follow-up would be an appropriate treatment protocol. The role of other therapeutic modalities such as chemotherapy and/or radiotherapy is not yet well established. PMID:27433056

  6. Epithelioid Hemangioendothelioma of the Distal Radius: A Case Report.

    PubMed

    Duncan, Scott F M; Krochmal, Daniel J; Craft, Randall O; Merritt, Marianne V; Smith, Anthony A

    2007-01-01

    Epithelioid hemangioendothelioma is a rare vascular tumor with cytologic behavior between angiosarcoma and hemangioma. We present the case of a 58-year-old male with primary epithelioid hemangioendothelioma of the distal radius measuring 6.2 × 5 cm with extension into the pronator quadratus and brachioradialis muscles. We discuss our approach to performing a limb-sparing resection combined with reconstruction to preserve upper extremity function. A review of the clinical, radiographic, and pathologic features of epithelioid hemangioendothelioma is also presented.

  7. Bartonella Species Bacteremia in Two Patients with Epithelioid Hemangioendothelioma ▿

    PubMed Central

    Mascarelli, Patricia E.; Iredell, Jonathan R.; Maggi, Ricardo G.; Weinberg, Guy; Breitschwerdt, Edward B.

    2011-01-01

    Bartonella henselae and B. koehlerae bacteremia was documented in two epithelioid hemangioendothelioma patients and B. koehlerae bacteremia in an asymptomatic partner of one of the patients. Considering the biology and clinically variable natural history of epithelioid hemangioendothelioma, these results suggest that chronic Bartonella infection could have a role in the development of this vascular neoplasm. Bartonella spp. are known to induce vasoproliferative tumors in immunocompromised patients and may play a role in the development of epithelioid hemangioendothelioma in immunocompetent patients. PMID:21918021

  8. Imaging Features of Primary Tumors and Metastatic Patterns of the Extraskeletal Ewing Sarcoma Family of Tumors in Adults: A 17-Year Experience at a Single Institution

    PubMed Central

    Huh, Jimi; Park, Seong Joon; Kim, Hyoung Jung; Lee, Jong Seok; Ha, Hyun Kwon; Tirumani, Sree Harsha; Ramaiya, Nikhil H.

    2015-01-01

    Objective To comprehensively analyze the spectrum of imaging features of the primary tumors and metastatic patterns of the Extraskeletal Ewing sarcoma family of tumors (EES) in adults. Materials and Methods We performed a computerized search of our hospital's data-warehouse from 1996 to 2013 using codes for Ewing sarcoma and primitive neuroectodermal tumors as well as the demographic code for ≥ 18 years of age. We selected subjects who were histologically confirmed to have Ewing sarcoma of extraskeletal origin. Imaging features of the primary tumor and metastatic disease were evaluated for lesion location, size, enhancement pattern, necrosis, margin, and invasion of adjacent organs. Results Among the 70 patients (mean age, 35.8 ± 15.6 years; range, 18-67 years) included in our study, primary tumors of EES occurred in the soft tissue and extremities (n = 20), abdomen and pelvis (n = 18), thorax (n = 14), paravertebral space (n = 8), head and neck (n = 6), and an unknown primary site (n = 4). Most primary tumors manifested as large and bulky soft-tissue masses (mean size, 9.0 cm; range, 1.3-23.0 cm), frequently invading adjacent organs (45.6%) and showed heterogeneous enhancement (73.7%), a well-defined (66.7%) margin, and partial necrosis/cystic degeneration (81.9%). Notably, 29 patients had metastatic disease detected at their initial diagnosis. The most frequent site of metastasis was lymph nodes (75.9%), followed by bone (31.0%), lung (20.7%), abdominal solid organs (13.8%), peritoneum (13.8%), pleura (6.9%), and brain (3.4%). Conclusion Primary tumors of EES can occur anywhere and mostly manifest as large and bulky, soft-tissue masses. Lymph nodes are the most frequent metastasis sites. PMID:26175577

  9. Histiocytic sarcoma

    PubMed Central

    Machado, Eduardo Silva; de Miranda, Ana Carolina; Escopelli, Ticiane; Caron, Ruggero; Escopelli, Alessandra Cristhina

    2011-01-01

    A 59-year-old white woman, SC, after being treated for pneumonia, presented with an increase in the size of lymph nodes. The immunohistochemical examination diagnosed histiocytic sarcoma. Relapse occurred 12 months after starting chemotherapy. The patient evolved with febrile neutropenia, septic shock and death. PMID:23284265

  10. Adult soft tissue sarcoma

    MedlinePlus

    ... certain chemicals, such as vinyl chloride or certain herbicides Having swelling in the arms or legs for ... Bethesda, MD 20894 U.S. Department of Health and Human Services National Institutes of Health Page last updated: ...

  11. Combination Chemotherapy in Treating Patients With Non-Metastatic Extracranial Ewing Sarcoma

    ClinicalTrials.gov

    2016-02-19

    Adult Supratentorial Primitive Neuroectodermal Tumor (PNET); Childhood Supratentorial Primitive Neuroectodermal Tumor; Ewing Sarcoma of Bone; Extraosseous Ewing Sarcoma; Extraosseous Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Peripheral Primitive Neuroectodermal Tumor of the Kidney; Untreated Childhood Supratentorial Primitive Neuroectodermal Tumor

  12. Cabozantinib-S-Malate in Treating Younger Patients With Recurrent, Refractory, or Newly Diagnosed Sarcomas, Wilms Tumor, or Other Rare Tumors

    ClinicalTrials.gov

    2016-10-10

    Adrenal Cortex Carcinoma; Adult Alveolar Soft Part Sarcoma; Adult Clear Cell Sarcoma of Soft Parts; Adult Hepatocellular Carcinoma; Adult Rhabdomyosarcoma; Adult Soft Tissue Sarcoma; Childhood Alveolar Soft Part Sarcoma; Childhood Central Nervous System Neoplasm; Childhood Clear Cell Sarcoma of Soft Parts; Childhood Hepatocellular Carcinoma; Childhood Rhabdomyosarcoma; Childhood Soft Tissue Sarcoma; Childhood Solid Neoplasm; Ewing Sarcoma; Hepatoblastoma; Hepatocellular Carcinoma; Recurrent Adrenal Cortex Carcinoma; Recurrent Adult Hepatocellular Carcinoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Alveolar Soft Part Sarcoma; Recurrent Childhood Central Nervous System Neoplasm; Recurrent Childhood Hepatocellular Carcinoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Ewing Sarcoma; Recurrent Hepatoblastoma; Recurrent Renal Cell Carcinoma; Recurrent Rhabdomyosarcoma; Recurrent Solid Neoplasm; Renal Cell Carcinoma; Thyroid Gland Medullary Carcinoma; Wilms Tumor

  13. Isolated Limb Perfusion With Melphalan in Treating Patients With Stage IIIB-IV Melanoma or Sarcoma

    ClinicalTrials.gov

    2015-07-22

    Basal Cell Carcinoma of the Skin; Eccrine Carcinoma of the Skin; Recurrent Adult Soft Tissue Sarcoma; Recurrent Melanoma; Recurrent Skin Cancer; Squamous Cell Carcinoma of the Skin; Stage III Adult Soft Tissue Sarcoma; Stage IIIB Melanoma; Stage IIIC Melanoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Melanoma

  14. Sclerosing Epithelioid Fibrosarcoma of the Bone: A Case Report of High Resistance to Chemotherapy and a Survey of the Literature

    PubMed Central

    Grunewald, Thomas G. P.; von Luettichau, Irene; Weirich, Gregor; Wawer, Angela; Behrends, Uta; Prodinger, Peter M.; Jundt, Gernot; Bielack, Stefan S.; Gradinger, Reiner; Burdach, Stefan

    2010-01-01

    Sclerosing epithelioid fibrosarcoma (SEF) is a rare soft tissue sarcoma mostly occurring in extraosseous sites. SEF represents a clinically challenging entity especially because no standardized treatment regimens are available. Intraosseous localization is an additional challenge with respect to the therapeutical approach. We report on a 16-year-old patient with SEF of the right proximal tibia. The patient underwent standardized neoadjuvant chemotherapy analogous to the EURAMOS-1 protocol for the treatment of osteosarcoma followed by tumor resection and endoprosthetic reconstruction. Histopathological analysis of the resected tumor showed >90% vital tumor cells suggesting no response to chemotherapy. Therefore, therapy was reassigned to the CWS 2002 High-Risk protocol for the treatment of soft tissue sarcoma. To date (22 months after diagnosis), there is no evidence of relapse or metastasis. Our data suggest that SEF may be resistant to a chemotherapy regimen containing Cisplatin, Doxorubicin, and Methotrexate, which should be considered in planning treatment for patients with SEF. PMID:20396630

  15. Malignant mesenteric perivascular epithelioid cell neoplasm presenting as an intra-abdominal fistula in a 49-year-old female.

    PubMed

    Kapur, Sakshi; Patel, Napoleon K; Levin, Miles B; Huang, Richard

    2014-01-01

    Perivascular epithelioid cell tumors are rare mesenchymal tumors arising from histologically and immunohistochemically distinctive perivascular epithelioid cells that express both myogenic and melanocytic markers. These tumors are known to arise from different organs in the body and usually have an unpredictable clinical course. We report a case of a 49-year-old female who presented with diffuse abdominal pain, fever, chills, and nonbilious vomiting for a day. Work-up revealed a mesenteric mass measuring 13.5 × 7.7 × 9.5 cm, arising in the mesentery of the hepatic flexure, with adjacent gas suggestive of fistularization into the right colon. An exploratory laparotomy with resection of the mesenteric mass was performed, and the initial histopathology results were compatible with either an adenocarcinoma or a sarcoma; however, because of poor differentiation it was difficult to make a definitive diagnosis. However, final histopathology results revealed a malignant perivascular epithelioid cell tumor (with reservation that a S100 negative metastatic melanoma must be excluded clinically). Following surgery the patient was started on everolimus, an m-TOR inhibitor, and has shown good response to this medication.

  16. Malignant Mesenteric Perivascular Epithelioid Cell Neoplasm Presenting as an Intra-Abdominal Fistula in a 49-Year-Old Female

    PubMed Central

    Patel, Napoleon K.; Levin, Miles B.

    2014-01-01

    Perivascular epithelioid cell tumors are rare mesenchymal tumors arising from histologically and immunohistochemically distinctive perivascular epithelioid cells that express both myogenic and melanocytic markers. These tumors are known to arise from different organs in the body and usually have an unpredictable clinical course. We report a case of a 49-year-old female who presented with diffuse abdominal pain, fever, chills, and nonbilious vomiting for a day. Work-up revealed a mesenteric mass measuring 13.5 × 7.7 × 9.5 cm, arising in the mesentery of the hepatic flexure, with adjacent gas suggestive of fistularization into the right colon. An exploratory laparotomy with resection of the mesenteric mass was performed, and the initial histopathology results were compatible with either an adenocarcinoma or a sarcoma; however, because of poor differentiation it was difficult to make a definitive diagnosis. However, final histopathology results revealed a malignant perivascular epithelioid cell tumor (with reservation that a S100 negative metastatic melanoma must be excluded clinically). Following surgery the patient was started on everolimus, an m-TOR inhibitor, and has shown good response to this medication. PMID:25114821

  17. Epithelioid solitary fibrous tumor of the central nervous system.

    PubMed

    Fu, Jing; Zhang, Rui; Zhang, Hongying; Bu, Hong; Chen, Huijiao; Yin, Xiangli; Zhang, Zhang; Wei, Bing

    2012-01-01

    Epithelioid solitary fibrous tumor (SFT) has recently been reported and is an extremely rare soft-tissue neoplasm. Herein we present an epithelioid SFT attached to the falx cerebri occurring in a Chinese woman. This patient underwent gross-total tumor resection at the age of 30 years and recurred 68 months following the initial total resection. Histologically, the initial lesion exhibited features of classic spindle cell SFT. In contrast, the recurrent tumor demonstrated exclusively epithelioid morphology with significant atypia. Both the original and recurrent lesions showed positivity for vimentin, CD34, Bcl-2, and CD99, whereas were negative for all the remaining antibodies. The epithelioid feature in SFT seems to be associated with a more aggressive clinical behavior in this case and more cases are awaited to verify this possibility. To the best of authors' knowledge, the present case is the first published example of SFT with epithelioid feature in the central nervous system.

  18. Carbon Ion Radiation Therapy Improves the Prognosis of Unresectable Adult Bone and Soft-Tissue Sarcoma of the Head and Neck

    SciTech Connect

    Jingu, Keiichi; Tsujii, Hirohiko; Mizoe, Jun-Etsu; Hasegawa, Azusa; Bessho, Hiroki; Takagi, Ryo; Morikawa, Takamichi; Tonogi, Morio; Tsuji, Hiroshi; Kamada, Tadashi; Yamada, Shogo

    2012-04-01

    Purpose: To evaluate the safety and efficacy of carbon ion radiotherapy (C-ion RT) with 70.4 GyE for unresectable bone and soft-tissue sarcoma of the adult head and neck. Methods and Materials: Twenty-seven patients (mean age, 46.2 years) were enrolled in this prospective study on C-ion RT with 70.4 GyE/16 fractions (fr) between April 2001 and February 2008. The primary end points were acute and late reactions of normal tissues, local control rate, and overall survival rate. The secondary end point was efficacy of the treatment in comparison to historical results with 57.6 or 64.0 GyE/16 fr. Results: The 3-year local control rate and overall survival rate for all patients were 91.8% (95% confidence interval [CI] = 81.0-100%) and 74.1% (95% CI = 57.5-90.6%), respectively. Acute reaction of Grade 3 or more was observed in only 1 patient. With regard to late reactions, visual loss was observed in 1 patient and a Grade 3 reaction of the maxillary bone was observed in 4 patients. A comparison with historical results revealed that the local control rate with 70.4 GyE was significantly higher than that with 57.6 or 64.0 GyE (3-year, 91.8% vs. 23.6%, p < 0.0001). Furthermore, the overall survival with 70.4 GyE tended to be higher than that with 57.6 or 64.0 GyE (3-year, 74.1% vs. 42.9%, p = 0.09). Conclusion: C-ion RT with 70.4 GyE/16 fr for bone and soft-tissue sarcoma of the adult head and neck appears to be effective with acceptable toxicities in comparison to conventional RT and C-ion RT with lower doses.

  19. Sarcomas of Soft Tissue and Bone.

    PubMed

    Ferrari, Andrea; Dirksen, Uta; Bielack, Stefan

    2016-01-01

    The definition of soft tissue and bone sarcomas include a large group of several heterogeneous subtypes of mesenchymal origin that may occur at any age. Among the different sarcomas, rhabdomyosarcoma, synovial sarcoma, Ewing sarcoma and osteosarcoma are aggressive high-grade malignancies that often arise in adolescents and young adults. Managing these malignancies in patients in this age bracket poses various clinical problems, also because different therapeutic approaches are sometimes adopted by pediatric and adult oncologists, even though they are dealing with the same condition. Cooperation between pediatric oncologists and adult medical oncologists is a key step in order to assure the best treatment to these patients, preferably through their inclusion into international clinical trials. PMID:27595362

  20. Synovial Sarcoma With Myoid Differentiation.

    PubMed

    Qassid, Omar; Ali, Ahmed; Thway, Khin

    2016-09-01

    Synovial sarcoma is a malignant mesenchymal tumor with variable epithelial differentiation, which is defined by the presence of a specific t(X;18)(p11.2;q11.2) chromosomal translocation that generates SS18-SSX fusion oncogenes. Synovial sarcoma typically arises within extremity deep soft tissue (particularly around large joints) of young adults, but has been shown to occur at almost any location. When it arises in more unusual sites, such as the abdomen, it can present a significant diagnostic challenge. We describe a case of intraabdominal monophasic synovial sarcoma that immunohistochemically showed strong expression of smooth muscle actin and calponin but only very scanty cytokeratin, and which showed morphologic and immunohistochemical overlap with other spindle cell neoplasms that can arise at this site, such as gastrointestinal stromal tumor and myofibrosarcoma. As correct diagnosis is of clinical and prognostic importance, surgical pathologists should be aware of the potential for synovial sarcoma to occur at a variety of anatomic sites and of its spectrum of immunoreactivity. Synovial sarcoma should be in the differential diagnosis of spindle cell neoplasms with myoid differentiation that do not fall into any definite tumor category, for which there should be a relatively low threshold for performing fluorescence in situ hybridization or reverse transcription-polymerase chain reaction to assess for the specific SS18 gene rearrangement or SS18-SSX fusion transcripts, which remain the diagnostic gold standard. PMID:27106779

  1. EF5 to Evaluate Tumor Hypoxia in Patients With High-Grade Soft Tissue Sarcoma or Mouth Cancer

    ClinicalTrials.gov

    2013-01-15

    Stage I Adult Soft Tissue Sarcoma; Stage I Squamous Cell Carcinoma of the Lip and Oral Cavity; Stage II Adult Soft Tissue Sarcoma; Stage II Squamous Cell Carcinoma of the Lip and Oral Cavity; Stage III Adult Soft Tissue Sarcoma; Stage III Squamous Cell Carcinoma of the Lip and Oral Cavity

  2. Sarcoma Foundation of America

    MedlinePlus

    ... Make a Donation Matching Gifts Sarcoma Dedication Page Stocks and Securities Workplace Giving Year-End Giving FAQ's ... Make a Donation Matching Gifts Sarcoma Dedication Page Stocks and Securities Workplace Giving Year-End Giving

  3. Kaposi's sarcoma in South Africa.

    PubMed

    Sitas, F; Newton, R

    2001-01-01

    Kaposi's sarcoma was endemic in South Africa even before the advent of the human immunodeficiency virus (HIV). Between 1988 and 1996, the incidence of Kaposi's sarcoma in South Africa has risen at least threefold and continues to increase as the HIV epidemic grows. Research from South Africa has shown that infection with human herpesvirus 8 (HHV8) is associated with Kaposi's sarcoma but not with any other major cancer site or type. In addition, the risk of Kaposi's sarcoma increases with increasing antibody titer to HHV8, but, for a given titer, the risk is greater in HIV-seropositive compared with HIV-seronegative individuals. The age- and sex-standardized seroprevalence of HHV8 in black South African hospital patients was found to be slightly more than 30%; the seroprevalence of HHV8 increased with age and was similar in men and in women. The modes of transmission of HHV8 are yet to be fully elucidated. Limited evidence exists for sexual transmission in black South African adults, but mother-to-child and person-to-person transmission in childhood is also likely. Furthermore, the seroprevalence of HHV8 decreases with increasing levels of education and is lower in whites than in blacks, suggesting that factors associated with poverty may be important determinants of transmission. Future research should focus on risk factors for Kaposi's sarcoma in HHV8-infected individuals, on determinants and mode of transmission of HHV8, and on the elucidation of the effect of primary HHV8 infection in adults and in children.

  4. Kaposi's sarcoma in South Africa.

    PubMed

    Sitas, F; Newton, R

    2001-01-01

    Kaposi's sarcoma was endemic in South Africa even before the advent of the human immunodeficiency virus (HIV). Between 1988 and 1996, the incidence of Kaposi's sarcoma in South Africa has risen at least threefold and continues to increase as the HIV epidemic grows. Research from South Africa has shown that infection with human herpesvirus 8 (HHV8) is associated with Kaposi's sarcoma but not with any other major cancer site or type. In addition, the risk of Kaposi's sarcoma increases with increasing antibody titer to HHV8, but, for a given titer, the risk is greater in HIV-seropositive compared with HIV-seronegative individuals. The age- and sex-standardized seroprevalence of HHV8 in black South African hospital patients was found to be slightly more than 30%; the seroprevalence of HHV8 increased with age and was similar in men and in women. The modes of transmission of HHV8 are yet to be fully elucidated. Limited evidence exists for sexual transmission in black South African adults, but mother-to-child and person-to-person transmission in childhood is also likely. Furthermore, the seroprevalence of HHV8 decreases with increasing levels of education and is lower in whites than in blacks, suggesting that factors associated with poverty may be important determinants of transmission. Future research should focus on risk factors for Kaposi's sarcoma in HHV8-infected individuals, on determinants and mode of transmission of HHV8, and on the elucidation of the effect of primary HHV8 infection in adults and in children. PMID:11158199

  5. Excellent clinical outcomes and retention in care for adults with HIV-associated Kaposi sarcoma treated with systemic chemotherapy and integrated antiretroviral therapy in rural Malawi

    PubMed Central

    Herce, Michael E; Kalanga, Noel; Wroe, Emily B; Keck, James W; Chingoli, Felix; Tengatenga, Listern; Gopal, Satish; Phiri, Atupere; Mailosi, Bright; Bazile, Junior; Beste, Jason A; Elmore, Shekinah N; Crocker, Jonathan T; Rigodon, Jonas

    2015-01-01

    Introduction HIV-associated Kaposi sarcoma (HIV-KS) is the most common cancer in Malawi. In 2008, the non-governmental organization, Partners In Health, and the Ministry of Health established the Neno Kaposi Sarcoma Clinic (NKSC) to treat HIV-KS in rural Neno district. We aimed to evaluate 12-month clinical outcomes and retention in care for HIV-KS patients in the NKSC, and to describe our implementation model, which featured protocol-guided chemotherapy, integrated antiretroviral therapy (ART) and psychosocial support delivered by community health workers. Methods We conducted a retrospective cohort study using routine clinical data from 114 adult HIV-KS patients who received ART and ≥1 chemotherapy cycle in the NKSC between March 2008 and February 2012. Results At enrolment 97% of patients (n/N=103/106) had advanced HIV-KS (stage T1). Most patients were male (n/N=85/114, 75%) with median age 36 years (interquartile range, IQR: 29–42). Patients started ART a median of 77 days prior to chemotherapy (IQR: 36–252), with 97% (n/N=105/108) receiving nevirapine/lamivudine/stavudine. Following standardized protocols, we treated 20 patients (18%) with first-line paclitaxel and 94 patients (82%) with bleomycin plus vincristine (BV). Of the 94 BV patients, 24 (26%) failed to respond to BV requiring change to second-line paclitaxel. A Division of AIDS grade 3/4 adverse event occurred in 29% of patients (n/N=30/102). Neutropenia was the most common grade 3/4 event (n/N=17/102, 17%). Twelve months after chemotherapy initiation, 83% of patients (95% CI: 74–89%) were alive, including 88 (77%) retained in care. Overall survival (OS) at 12 months did not differ by initial chemotherapy regimen (p=0.6). Among patients with T1 disease, low body mass index (BMI) (adjusted hazard ratio, aHR=4.10, 95% CI: 1.06–15.89) and 1 g/dL decrease in baseline haemoglobin (aHR=1.52, 95% CI: 1.03–2.25) were associated with increased death or loss to follow-up at 12 months. Conclusions

  6. Synchronous Hepatic Epithelioid Hemangioendothelioma and Hepatocellular Carcinoma

    PubMed Central

    Athanasopoulos, Panagiotis G.; Hadjittofi, Christopher; Luong, Tu Vinh; O’Beirne, James; Sharma, Dinesh

    2015-01-01

    Abstract We would like to report the first case in English literature, to the best of our knowledge, of a synchronous hepatic epithelioid hemangioendothelioma (HEHE) and hepatocellular carcinoma (HCC), as well as to address the current trends and challenges in the management of HEHE. An otherwise well 58-year-old man was referred to his local hepatology service with elevated serum γ-GT levels. Imaging revealed bilobar liver lesions consistent with HEHE, a discrete left lobe lesion suspected as HCC, and multiple pulmonary nodules. Biopsies confirmed HEHE with pulmonary metastases. After multidisciplinary team discussions, the patient was admitted under our team and underwent an uneventful laparoscopic left lateral hepatectomy for suspected HCC, which was confirmed histologically. As part of a watch-and-wait approach to metastatic HEHE, in the first follow-up (3 months postoperatively) the patient was clinically fine and the surveillance CT scan did not show recurrent disease. By presenting this case, we aim to raise awareness that this rare entity can coexist with others, potentially complicating their management. PMID:26313777

  7. [Bacillary epithelioid angiomatosis in AIDS. Two cases].

    PubMed

    Bodokh, I; Lacour, J P; Perrin, C; Rosenthal, E; Dubois, D; Gallais, V; Cassuto, J P; Ortonne, J P

    1992-10-17

    Bacillary angiomatosis is a newly recognized multisystem opportunistic infection seen in the human immunodeficiency virus infection. The disease is marked by papular and nodular vascular skin lesions that clinically resemble Kaposi's sarcoma. Histologically, the lesions are different and show clusters of bacteria showing the structure of Gram negative bacilli staining with Warthin-Starry stain. Transmission electron microscopy shows that the organisms (1 to 2 microns) have a trilamellar wall structure. Treatment with oral erythromycin (2 to 3 g a day) for 2 to 4 weeks rapidly leads to resolution.

  8. Distinction of endometrial stromal sarcomas from 'hemangiopericytomatous' tumors using a panel of immunohistochemical stains.

    PubMed

    Bhargava, Rohit; Shia, Jinru; Hummer, Amanda J; Thaler, Howard T; Tornos, Carmen; Soslow, Robert A

    2005-01-01

    Endometrial stromal sarcomas are low-grade malignant tumors that may pose a diagnostic challenge, especially when they are present in an extrauterine site. Owing to the presence of an arborizing vasculature and cells with an undifferentiated appearance, endometrial stromal sarcomas can be confused with several soft-tissue neoplasms. We studied 17 endometrial stromal sarcomas, eight hemangiopericytomas, 14 solitary fibrous tumors, and 16 synovial sarcomas immunohistochemically, detecting the following antigens: CD10, estrogen receptor, progesterone receptor, bcl-2, CD34, smooth muscle antigen, epithelial membrane antigen and cytokeratin (AE1/AE3). Most endometrial stromal sarcomas stained positively for CD10 (16/17), estrogen receptor (17/17), progesterone receptor (15/17), and bcl-2 (17/17). Staining with antismooth muscle antigen was seen in 11 of 17 cases of endometrial stromal sarcoma, with more intense staining seen in areas showing smooth muscle differentiation. Staining with AE1/3 was seen in four of 17 endometrial stromal sarcomas, with two of the positive cases containing epithelioid cells. None of the endometrial stromal sarcomas expressed epithelial membrane antigen or CD34. More than half of the hemangiopericytomas (4/8) and solitary fibrous tumors (9/14) cases demonstrated CD10 expression either focally or in a patchy cytoplasmic and membranous pattern. Hemangiopericytomas, solitary fibrous tumors, and synovial sarcomas did not express estrogen receptor. Four of eight hemangiopericytomas and seven of 14 solitary fibrous tumors also showed patchy progesterone receptor expression. CD34 expression was identified in six of eight hemangiopericytomas and 13 of 14 solitary fibrous tumors, but we did not find expression of CD34 in synovial sarcoma. Differences between endometrial stromal sarcoma and other soft-tissue tumors were detected for all of the immunohistochemical markers (P<0.05), except anti-bcl-2 and AE1/3. Antibodies against CD10 mark a substantial

  9. Targeted therapy for sarcomas

    PubMed Central

    Forscher, Charles; Mita, Monica; Figlin, Robert

    2014-01-01

    Sarcomas are tumors of mesenchymal origin that make up approximately 1% of human cancers. They may arise as primary tumors in either bone or soft tissue, with approximately 11,280 soft tissue tumors and 2,650 bone tumors diagnosed each year in the United States. There are at least 50 different subtypes of soft tissue sarcoma, with new ones described with ever-increasing frequency. One way to look at sarcomas is to divide them into categories on the basis of their genetic make-up. One group of sarcomas has an identifiable, relatively simple genetic signature, such as the X:18 translocation seen in synovial sarcoma or the 11:22 translocation seen in Ewing’s sarcoma. These specific abnormalities often lead to the presence of fusion proteins, such as EWS-FLI1 in Ewing’s sarcoma, which are helpful as diagnostic tools and may become therapeutic targets in the future. Another group of sarcomas is characterized by complex genetic abnormalities as seen in leiomyosarcoma, osteosarcoma, and undifferentiated sarcoma. It is important to keep these distinctions in mind when contemplating the development of targeted agents for sarcomas. Different abnormalities in sarcoma could be divided by tumor subtype or by the molecular or pathway abnormality. However, some existing drugs or drugs in development may interfere with or alter more than one of the presented pathways. PMID:24669185

  10. Comparative pathology of canine soft tissue sarcomas: possible models of human non-rhabdomyosarcoma soft tissue sarcomas.

    PubMed

    Milovancev, M; Hauck, M; Keller, C; Stranahan, L W; Mansoor, A; Malarkey, D E

    2015-01-01

    Comparative analyses of canine and human soft tissue sarcomas (STSs) are lacking. This study compared the histological and immunohistochemical (labelling for desmin, smooth muscle actin [SMA], CD31, pancytokeratin, S100 and CD34) appearance of 32 archived, formalin-fixed, paraffin wax-embedded canine STS tumour specimens by board-certified veterinary and medical pathologists, both blinded to the other's interpretations. Comparison between the veterinary and human diagnoses revealed a generally consistent pattern of interpretation with few notable variations. Most tumours (13/32) were judged to display similar histomorphological appearance to human low-grade spindle cell sarcomas, appearing non-distinctive and morphologically of a fibroblastic/myofibroblastic type. Five canine cases resembled human liposarcoma, but with atypical desmin-positive epithelioid cells present. Five canine cases resembled human spindle cell sarcoma with myxoid features and two additional cases resembled human myxofibrosarcoma. Seven canine cases were noted to resemble human undifferentiated sarcoma. Findings in the present study demonstrate that canine STSs display histological and immunohistochemical features similar to their human equivalents. Because of these cross-species similarities, a particular opportunity exists to understand the biology and treatment of human STS by potentially including dogs as clinical models. PMID:25435513

  11. Ruptured Hepatic Epithelioid Angiomyolipoma: A Case Report and Literature Review

    PubMed Central

    Tajima, Shogo; Suzuki, Akira; Suzumura, Kiyoshi

    2014-01-01

    A 38-year-old male was admitted to our hospital due to upper abdominal pain. Computed tomography revealed a hepatic angiomyolipoma (AML; 10.5 × 9.5 × 7.0 cm in size), which had ruptured into the space between the liver and the diaphragm. Following transcatheter arterial embolization, surgical resection was performed. The tumor consisted of epithelioid cells (50–60%), mature fat (40–50%), and thickened-wall blood vessels. Considering the amount of epithelioid cells and their positivity for E-cadherin and β-catenin, the tumor was diagnosed as hepatic epithelioid AML. Cases of ruptured hepatic AML are rare. To the best of our knowledge, this is the sixth case reported in the English literature. PMID:24987358

  12. Management of hepatic epithelioid haemangio-endothelioma in children: what option?

    PubMed

    Sharif, K; English, M; Ramani, P; Alberti, D; Otte, J-B; McKiernan, P; Gosseye, S; Jenney, M; de Ville de Goyet, J

    2004-04-19

    Hepatic epithelioid haemangio-endothelioma (HEHE) is an endothelium-derived tumour of low-to-medium grade malignancy. It is predominantly seen in adults and is unresponsive to chemotherapy. Liver transplantation is an accepted indication when the tumour is unresectable. Hepatic epithelioid haemangio-endothelioma is very rare in children and results after transplantation are not reported. The aim of this study is to review the experience of three European centres in the management of HEHE in children. A retrospective review of all paediatric patients with HEHE managed in three European centres is presented. Five children were identified. Four had unresectable tumours. The first had successful resection followed by chemotherapy and is alive, without disease 3 years after diagnosis. One child died of sepsis and one of tumour recurrence in the graft and lungs 2 and 5 months, respectively, after transplant. Two children who had progressive disease with ifosfamide-based chemotherapy have had a reduction in clinical symptoms and stabilisation of disease up to 18 and 24 months after the use of platinum-based chemotherapy. HEHE seems more aggressive in children than reported in adults and the curative role of transplantation must be questioned. Ifosfamide-based chemotherapy was not effective. Further studies are necessary to confirm if HEHE progression in children may be influenced by platinum-based chemotherapy. PMID:15083175

  13. The adhesion molecule CD44v6 is associated with a high risk for local recurrence in adult soft tissue sarcomas

    PubMed Central

    Maula, S; Huuhtanen, R L; Blomqvist, C P; Wiklund, T A; Laurila, P; Ristamäki, R

    2001-01-01

    In many malignant diseases the expression levels of CD44 and its splice variant v6 (CD44v6) have been associated with the prognosis. The purpose of this study was to investigate the clinical significance of CD44 in adult soft tissue sarcomas (STS). 133 STS patients with a limb or superficial trunk tumour treated at the Helsinki University Central Hospital in 1987–1993 with a median follow-up time of 68 months were included in this study. The expression of CD44 and CD44v6 was determined immunohistochemically on paraffin-embedded tumour samples. 95% of the tumours expressed CD44 and CD44v6 was detected in 57%. Strong CD44 expression was associated with low grade (P = 0.04) and small tumour size (P = 0.02). In diploid tumours the CD44 expression was correlated with low S-phase fraction (P = 0.001). High expression of both, CD44 in general as well as that of CD44v6, predicted a higher risk for local recurrence (CD44: P = 0.01 and CD44v6: P = 0.05). Low CD44v6 content of the primary tumour correlated with poor survival (P = 0.02). Determining the expression of CD44 or CD44v6 in a primary STS could be a valuable tool for selecting the group of patients who might benefit from intensified local tumour treatment. © 2001 Cancer Research Campaign http://www.bjcancer.com PMID:11161384

  14. Whole-Body Radiation Therapy, Systemic Chemotherapy, and High-Dose Chemotherapy Followed By Stem Cell Rescue in Treating Patients With Poor-Risk Ewing Sarcoma

    ClinicalTrials.gov

    2015-01-07

    Adult Supratentorial Primitive Neuroectodermal Tumor (PNET); Ewing Sarcoma of Bone; Extraosseous Ewing Sarcoma; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Untreated Childhood Supratentorial Primitive Neuroectodermal Tumor

  15. Can Kaposi Sarcoma Be Prevented?

    MedlinePlus

    ... early? Can Kaposi sarcoma be prevented? Kaposi sarcoma (KS) is caused by the Kaposi sarcoma associated herpesvirus ( ... to protect people against KSHV. For now, preventing KS depends on reducing the chance of becoming infected ...

  16. Post-radiation epithelioid angiosarcoma of the urinary bladder and prostate

    PubMed Central

    Wang, Gang; Black, Peter C.; Skinnider, Brian F.; Hayes, Malcolm M.; Jones, Edward C.

    2016-01-01

    Angiosarcoma of the lower urinary tract is exceedingly rare. A minority of cases are associated with local radiotherapy. Epithelioid angiosarcoma is a variant of angiosarcoma composed of large rounded epithelioid endothelial cells that are positive for cytokeratin on immunostaining. There are only two cases of post-radiation epithelioid angiosarcoma reported in the urinary bladder, and none in the prostate gland. We report a case of epithelioid angiosarcoma involving the urinary bladder and prostate in a patient with a history of radiotherapy for prostatic adenocarcinoma. A brief review of literature regarding post-radiation epithelioid angiosarcomas in the lower urinary tract is discussed.

  17. Skull sclerosing epithelioid fibrosarcoma: A case report and review of the literature

    PubMed Central

    XU, JINGJING; WANG, JIAWEI; ZHANG, MINMING; LI, BAIZHOU

    2016-01-01

    Sclerosing epithelioid fibrosarcoma (SEF) is an unusual variant of fibrosarcoma that was previously considered to be a low-grade tumor with an indolent course. The tumor occurs most commonly in the soft tissue of the limb, trunk, head and neck, and occasionally in the bone and visceral organs. The skull is a rare primary site for SEF, with only 3 cases reported to date. The current study reports a case of SEF occurring in the occipital bone of a 24-year-old man, who lacked neurological symptoms. Imaging revealed a large mass emanating from the occipital bone and involving the superior sagittal sinus, torcular herophili and adjacent brain tissue. Histological and immunohistochemical characteristics confirmed the diagnosis of SEF. The patient experienced local recurrence and distant metastasis at 10 and 15 months, respectively, subsequent to the resection of the primary mass. The current case and review of the literature suggest that skull SEF may behave clinically as an aggressive malignant sarcoma. Radiological findings indicated the biological and histopathological characteristics of the tumor. Thus, its clinical behavior and certain imaging features may suggest this diagnosis. PMID:27123127

  18. The soft tissue sarcomas

    SciTech Connect

    Eilber, F.R.; Morton, D.L.; Sondak, V.K.; Economou, J.S.

    1987-01-01

    New advances in multimodality therapy of sarcomas in all anatomic sites are thoroughly described. Multimodality therapy with limb-salvage surgery for extremity tumors, sarcomas of the head and neck, trunk, intraabdominal, visceral, and genitourinary tract and cardiopulmonary system are presented. Separate sections are devoted to the management of pediatric sarcomas, pulmonary metastasis and to the pathology and radiobiology, chemotherapy, and immunotherapy of sarcomas. The text also stresses the philosophy of achieving adequate local control without radical amputation by combined surgery and chemo/radiotherapy.

  19. Combination Therapy for Advanced Kaposi Sarcoma

    Cancer.gov

    In this clinical trial, adult patients with any form of advanced Kaposi sarcoma will be treated with liposomal doxorubicin and bevacizumab every 3 weeks for a maximum of six treatments.  Patients who respond to this therapy or have stable disease will rec

  20. Anatomic Tumor Location Influences the Success of Contemporary Limb-Sparing Surgery and Radiation Among Adults With Soft Tissue Sarcomas of the Extremities

    SciTech Connect

    Korah, Mariam P.; Deyrup, Andrea T.; Monson, David K.; Oskouei, Shervin V.; Weiss, Sharon W.; Landry, Jerome; Godette, Karen D.

    2012-02-01

    Purpose: To examine the influence of anatomic location in the upper extremity (UE) vs. lower extremity (LE) on the presentation and outcomes of adult soft tissue sarcomas (STS). Methods and Materials: From 2001 to 2008, 118 patients underwent limb-sparing surgery (LSS) and external beam radiotherapy (RT) with curative intent for nonrecurrent extremity STS. RT was delivered preoperatively in 96 and postoperatively in 22 patients. Lesions arose in the UE in 28 and in the LE in 90 patients. Patients with UE lesions had smaller tumors (4.5 vs. 9.0 cm, p < 0.01), were more likely to undergo a prior excision (43 vs. 22%, p = 0.03), to have close or positive margins after resection (71 vs. 49%, p = 0.04), and to undergo postoperative RT (32 vs. 14%, p = 0.04). Results: Five-year actuarial local recurrence-free and distant metastasis-free survival rates for the entire group were 85 and 74%, with no difference observed between the UE and LE cohorts. Five-year actuarial probability of wound reoperation rates were 4 vs. 29% (p < 0.01) in the UE and LE respectively. Thigh lesions accounted for 84% of the required wound reoperations. The distribution of tumors within the anterior, medial, and posterior thigh compartments was 51%, 26%, and 23%. Subset analysis by compartment showed no difference in the probability of wound reoperation between the anterior and medial/posterior compartments (29 vs. 30%, p = 0.68). Neurolysis was performed during resection in (15%, 5%, and 67%, p < 0.01) of tumors in the anterior, medial, and posterior compartments. Conclusions: Tumors in the UE and LE differ significantly with respect to size and management details. The anatomy of the UE poses technical impediments to an R0 resection. Thigh tumors are associated with higher wound reoperation rates. Tumor resection in the posterior thigh compartment is more likely to result in nerve injury. A better understanding of the inherent differences between tumors in various extremity sites will assist in

  1. PCR and RT-PCR analysis of infection and transcriptional activity of walleye dermal sarcoma virus (WDSV) in organs of adult walleyes (Stizostedion vitreum).

    PubMed

    Poulet, F M; Bowser, P R; Casey, J W

    1996-01-01

    The pathogenesis of walleye dermal sarcoma virus (WDSV) infection was investigated in adult walleyes (Stizostedion vitreum). Three tumor-bearing and three tumor-free walleyes were collected in the spring from Oneida Lake, New York, and analyzed for viral infection and transcriptional activity. Specifically, the target organs for viral infection and supporting viral transcriptional activity were determined by assessing for the presence of WDSV DNA and RNA in the brain, liver, kidney, skin, and spleen. For each organ, WDSV DNA and RNA were detected using the polymerase chain reaction (PCR) and reverse transcription PCR (RT-PCR) respectively. Quantitative estimates of the number of viral DNA and RNA copies were obtained in each case by comparing the signal intensity of the sample to that of external controls. WDSV RNA/DNA ratios, based on those quantitative estimates, were computed for each organ. An RNA/DNA ratio of 3 was arbitrarily chosen as the threshold above which there was viral transcriptional activity. Viral DNA was found in all the organs examined from the three tumor-free walleyes. In those three tumor-free walleyes, low levels of WDSV RNA were detected in only one kidney and two spleen samples. In the three tumor-bearing walleyes, viral DNA was found in one brain, one kidney, two liver, and two skin samples. In contrast to the few organs from tumor-free walleyes in which WDSV RNA was detected, in tumor-bearing walleyes WDSV RNA was present in the one brain examined and in 2/3 kidney, 2/3 liver, 3/3 skin, and 3/3 spleen samples. A WDSV RNA/DNA ratio above 3 was obtained in all three tumor-bearing walleyes but in only one tumor-free fish. These data indicated that 1) both tumor-bearing and tumor-free walleyes were infected by WDSV, 2) many cell types were targeted by WDSV and supported viral transcription, and 3) tumor-bearing walleyes harbored a transcriptionally active WDSV, whereas tumor-free walleyes contained mostly silent WDSV DNA.

  2. TERT promoter hotspot mutations are recurrent in myxoid liposarcomas but rare in other soft tissue sarcoma entities

    PubMed Central

    2014-01-01

    Background Recently, recurrent point mutations in the telomerase reverse transcriptase (TERT) promoter region have been found in many human cancers, leading to a new transcription factor binding site, increased induction of TERT and subsequently to telomere maintenance. We determined the prevalence of TERT promoter mutations in soft tissue sarcomas of 341 patients comprising 16 entities and in 16 sarcoma cell lines covering 7 different soft tissue sarcoma types. Methods The sarcoma tissue samples were collected from the archives of the Institute of Pathology, University of Heidelberg and were composed of 39 myxoid liposarcomas (MLS), 61 dedifferentiated liposarcomas, 15 pleomorphic liposarcomas, 27 leiomyosarcomas, 25 synovial sarcomas (SS), 35 malignant peripheral nerve sheath tumors (MPNST), 40 undifferentiated pleomorphic sarcomas, 17 myxofibrosarcomas, 9 low grade fibromyxoid sarcomas, 10 cases of dermatofibrosarcoma protuberans, 31 solitary fibrous tumors (SFT), 8 extraskeletal myxoid chondrosarcomas, 9 angiosarcomas, 6 alveolar soft part sarcomas, 5 clear cell sarcomas and 4 epithelioid sarcomas. Sarcoma cell lines were obtained from the raising laboratories. A 193 bp fragment of the TERT promoter region covering the hot-spot mutations C228T and C250T was amplified, and direct sequencing of the PCR products was performed. Results TERT promoter mutations were detected in 36/341 sarcomas. They were highly recurrent in MLS (29/39; 74%) and were in the present MLS series not associated with the phenotype (myxoid vs. round cell variant), tumor grade, tumor site and patients’ median age or gender. In the remaining cases, TERT promoter mutations were found only in 7/302 sarcoma samples and confined to SFTs (4/31; 13%), MPNSTs (2/35; 6%), and SSs (1/25; 4%). Within the collection of sarcoma cell lines examined, TERT promoter mutations were detected in two MLS and in one of three MPNST cell lines. Conclusions TERT promoter mutations are frequent in MLSs including

  3. Hepatic epithelioid hemangioendothelioma in a patient with hemochromatosis.

    PubMed

    Ahmad, Nisar; Adams, Denise M; Wang, Jiang; Prakash, Rajan; Karim, Nagla Abdel

    2014-09-01

    Hepatic epithelioid hemangioendothelioma (HEHE) is a rare hepatic vascular tumor that represents a diagnostic challenge. The rarity of this neoplasm precludes establishment of a standard-of-care treatment. Risk factors for HEHE are not well-known. Liver transplant remains the most common therapeutic modality for nonmetastatic disease. This article describes a patient who presented with abdominal pain, fatigue, poor appetite, and weight loss. Genetic testing showed that the patient was homozygous for C282Y consistent with hereditary hemochromatosis, and liver biopsy was consistent with malignant epithelioid hemangioendothelioma. The patient was referred for a liver transplant but was deemed inappropriate for transplant secondary to peritoneal studding, with frozen-section analysis showing metastatic disease at the time of surgery. PMID:25190690

  4. Epithelioid Hemangioendothelioma of Tibia and Talus: A Case Report

    PubMed Central

    Kumar, Dharmendra; Jain, Vijay K.; Bhardwaj, Meenakshi; Naik, Ananta K.; Nasa, Ruchi; Arya, Rajendra K.

    2015-01-01

    Epithelioid hemangioendothelioma (EHE) of the bone is extremely uncommon and consists of less than 1% of primary bone tumors. It is characterized by epithelioid endothelial cells and has variable biological behavior. EHE is more likely to occur between 20 and 30 years of age. Approximately half of EHE present with multifocal disease. Since the behavior of these tumors is intermediate, it is important to not misdiagnose EHE as an angiosarcoma. Here we describe the case of a 43-year-old male who presented with pain and swelling of the leg and ankle to Dr. Ram Manohar Lohia Hospital, India. Radiography and computed tomography of the ankle and leg were performed and suggested an expansile lytic lesion involving the distal tibia and talus bone. The lesion was excised and ankle arthrodesis performed. Histological evaluation of the lesion demonstrated the presence of EHE of the tibia and talus. PMID:26366265

  5. Epithelioid Hemangioendothelioma of Tibia and Talus: A Case Report.

    PubMed

    Kumar, Dharmendra; Jain, Vijay K; Bhardwaj, Meenakshi; Naik, Ananta K; Nasa, Ruchi; Arya, Rajendra K

    2015-07-01

    Epithelioid hemangioendothelioma (EHE) of the bone is extremely uncommon and consists of less than 1% of primary bone tumors. It is characterized by epithelioid endothelial cells and has variable biological behavior. EHE is more likely to occur between 20 and 30 years of age. Approximately half of EHE present with multifocal disease. Since the behavior of these tumors is intermediate, it is important to not misdiagnose EHE as an angiosarcoma. Here we describe the case of a 43-year-old male who presented with pain and swelling of the leg and ankle to Dr. Ram Manohar Lohia Hospital, India. Radiography and computed tomography of the ankle and leg were performed and suggested an expansile lytic lesion involving the distal tibia and talus bone. The lesion was excised and ankle arthrodesis performed. Histological evaluation of the lesion demonstrated the presence of EHE of the tibia and talus. PMID:26366265

  6. Management of Breast Sarcoma.

    PubMed

    Hsu, Cary; McCloskey, Susan A; Peddi, Parvin F

    2016-10-01

    Breast sarcomas are exceptionally rare mesenchymal neoplasms composed of many histologic subtypes. Therapy is guided by principles established in the management of extremity sarcomas. The anatomic site does influence treatment decisions, particularly the surgical management. Surgery should be undertaken with the aim of achieving a widely negative margin. Selected patients can be managed with breast-conserving surgery. Breast reconstruction is increasingly being undertaken for selected patients. Radiation therapy and chemotherapy are used selectively for large, high-grade sarcomas for which there is significant concern for local and distant recurrence. PMID:27542642

  7. Epithelioid Trophoblastic Tumor in a Postmenopausal Woman: A Case Report

    PubMed Central

    Park, Jung-Woo

    2016-01-01

    Epithelioid trophoblastic tumor (ETT) is a rare gestational trophoblastic neoplasm composed of intermediate trophoblasts. Most cases of ETT are reported in women of reproductive age following a prior gestation within 2 weeks to 30 years. ETT is extremely rare in postmenopausal women. It is commonly misdiagnosed as a poorly differentiated carcinoma or another type of gestational trophoblastic tumor. We report a case of ETT in a 56-year-old woman that developed 23 years after the patient's last pregnancy. PMID:27152314

  8. Epithelioid hemangioma of the distal humerus with pathologic fracture.

    PubMed

    Kleck, Christopher J; Seidel, Matthew J

    2012-01-16

    Epithelioid hemangioma is a rare tumor that can have bone involvement. Its clinically and radiographically aggressive appearance mimics a malignant neoplasm. Although epitheliod hemangioma has been described as having an aggressive appearance on magnetic resonance imaging (MRI) and plain radiographs, this is the first reported case of pathologic fracture associated with this lesion to our knowledge. This article describes a case of epithelioid hemangioma involving the distal humerus, which initially presented with progressive pain and fracture of the lateral condyle. The aggressive appearance on plain radiographs and MRI suggested a malignant bone tumor. This preliminary diagnosis was confirmed due to the presence of local lymph node spread on positron emission tomography/computed tomography. After a core needle biopsy revealed nondiagnostic tissue, rather than performing a wide resection based on a presumptive malignant diagnosis, we followed the standard diagnostic algorithm and performed an open biopsy with temporary internal stabilization. The tissue sample was adequate and revealed a diagnosis of epithelioid hemangioma. Based on this finding, we were able to proceed with surgical management, including curettage of the lesion, placement of a bone graft, and internal fixation, rather than a wide resection with elbow joint replacement. This article emphasizes the need for careful adherence to the diagnostic algorithm for musculoskeletal tumors. In doing so, a definitive diagnosis was reached, and our patient was able to resume his occupation as a laborer without the restrictions that would have accompanied elbow arthroplasty.

  9. Pleural Epithelioid Hemangioendothelioma: A Case Report and Literature Review.

    PubMed

    Fan, Yingqi; Wang, Faping; Li, Suyun; Ye, Cheng; Ying, Ying; Mao, Hui

    2016-05-01

    Epithelioid hemangioendothelioma (EHE) is a rare neoplasm of vascular origin that can arise in multiple and varied tissue sites. Pleural epithelioid hemangioendothelioma (PEH), a subtype of EHE, is particularly less reported. Herein, we describe a case of PEH presented with left-sided back pain in a 68-year-old female, and her chest CT scan revealed thickening of the left pleura and left pleural effusion, the histological diagnosis was confirmed by both conventional examination and immunohistochemistry. A literature search utilizing PubMed, Embase, Ovid and Cochrane, Wanfang and Chinese National Knowledge infrastructure (CNKI) for PEH was conducted to investigate the characteristics of the disease, 26 related articles were retrieved and 40 cases of PEH were reported. According to available literature, the average age at presentation is 51.8 years and the disease occurred more often in men than women. The etiology of the disease remained unknown. Chest pain, cough, and dyspnea were the common symptoms. Computed tomography usually revealed pleural effusion and pleural thickening. Histological examinations revealed mainly epithelioid cells. Immunohistochemical stains were positive for vascular endothelial markers. PEH tends to have more aggressive behavior than tumors in other locations, thus effective treatment has not yet been established until now. Further studies are needed to analyze the prognostic factors, clinical features and treatment of PEH. PMID:27372473

  10. What Is Uterine Sarcoma?

    MedlinePlus

    ... supporting tissues of the uterus (womb). About the uterus The uterus is a hollow organ, about the ... a baby out during childbirth. Cancers of the uterus and endometrium Sarcomas are cancers that start from ...

  11. Epidemic Kaposi Sarcoma

    MedlinePlus

    ... therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. ... complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information ...

  12. Classic Kaposi Sarcoma

    MedlinePlus

    ... therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. ... complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information ...

  13. A systematic review: perivascular epithelioid cell tumor of gastrointestinal tract

    PubMed Central

    Chen, Zehong; Han, Siqi; Wu, Jialin; Xiong, Minmin; Huang, Yanqiao; Chen, Jianhui; Yuan, Yujie; Peng, Jianjun; Song, Wu

    2016-01-01

    Abstract Perivascular epithelioid cell tumor (PEComa) is a rare entity with distinctive morphology and of expressing myomelanocytic markers. Gastrointestinal tract (GI) is one of the most common anatomic sites of origin and counts for 20% to 25% of all reported cases of perivascular epithelioid cell tumors not otherwise specified (PEComas-NOS). However, the biologic behavior of perivascular epithelioid cell tumors of gastrointestinal tract (GI PEComas-NOS) is still unclear. The aim of conducting this systematic review is to sum up what is known so far of the epidemiology, natural history, management and prognosis of GI PEComas-NOS. A systematic research was performed on PubMed and EMBASE using the following terms: (“perivascular epithelioid cell tumor” or “PEComa”) and (“gastrointestinal tract” or “GI” or “oral ” or “mouth” or “esophagus” or “gullet” or “gastric” or “stomach” or “duodenum” or “jejunum” or “ileum” or “cecum” or “colon” or “colorectal” or “sigmoid” or “rectum” or “anus” or “mesentery”) up to December 1, 2015. Retrieved GI PEComas-NOS publications, which included these terms, contains case reports, case series to case characteristic researches. A total of 168 articles were reviewed, 41 GI PEComa-NOS English studies among which were retrieved for analysis. We reviewed epidemiology, natural history, management and prognosis of GI PEComa-NOS. Generally GI PEComa-NOS is believed to have women predomination. The most frequently involved location is colon with non-specific clinical signs. Pathologically, GI PEComas-NOS shows epithelioid predominance (70%), meanwhile coexpresses melanocytic and muscle markers characteristically, while immunohistochemistry is a useful tool for identify, which indicates that HMB-45 is regarded as the most sensitive reagent. Complete resection served as mainstay of treatment, while chemotherapy should be unanimously considered to apply in malignant

  14. A systematic review: perivascular epithelioid cell tumor of gastrointestinal tract.

    PubMed

    Chen, Zehong; Han, Siqi; Wu, Jialin; Xiong, Minmin; Huang, Yanqiao; Chen, Jianhui; Yuan, Yujie; Peng, Jianjun; Song, Wu

    2016-07-01

    Perivascular epithelioid cell tumor (PEComa) is a rare entity with distinctive morphology and of expressing myomelanocytic markers. Gastrointestinal tract (GI) is one of the most common anatomic sites of origin and counts for 20% to 25% of all reported cases of perivascular epithelioid cell tumors not otherwise specified (PEComas-NOS). However, the biologic behavior of perivascular epithelioid cell tumors of gastrointestinal tract (GI PEComas-NOS) is still unclear. The aim of conducting this systematic review is to sum up what is known so far of the epidemiology, natural history, management and prognosis of GI PEComas-NOS.A systematic research was performed on PubMed and EMBASE using the following terms: ("perivascular epithelioid cell tumor" or "PEComa") and ("gastrointestinal tract" or "GI" or "oral " or "mouth" or "esophagus" or "gullet" or "gastric" or "stomach" or "duodenum" or "jejunum" or "ileum" or "cecum" or "colon" or "colorectal" or "sigmoid" or "rectum" or "anus" or "mesentery") up to December 1, 2015. Retrieved GI PEComas-NOS publications, which included these terms, contains case reports, case series to case characteristic researches.A total of 168 articles were reviewed, 41 GI PEComa-NOS English studies among which were retrieved for analysis. We reviewed epidemiology, natural history, management and prognosis of GI PEComa-NOS. Generally GI PEComa-NOS is believed to have women predomination. The most frequently involved location is colon with non-specific clinical signs. Pathologically, GI PEComas-NOS shows epithelioid predominance (70%), meanwhile coexpresses melanocytic and muscle markers characteristically, while immunohistochemistry is a useful tool for identify, which indicates that HMB-45 is regarded as the most sensitive reagent. Complete resection served as mainstay of treatment, while chemotherapy should be unanimously considered to apply in malignant cases. Eventually, it is necessary for closed and long-term follow-up with endoscope and

  15. Pulmonary artery intimal sarcoma diagnosed using endobronchial ultrasound-guided transbronchial needle aspiration.

    PubMed

    Caraway, Nancy P; Salina, Davide; Deavers, Michael T; Morice, Rodolfo; Landon, Gene

    2015-01-01

    Intimal sarcoma of the pulmonary artery is a rare intraluminal malignant neoplasm that has an aggressive biological behavior, and early diagnosis may improve patient outcome. We describe a case of pulmonary artery intimal sarcoma diagnosed on cytologic material obtained by endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) biopsy with rapid on-site evaluation (ROSE). The aspirate showed loosely cohesive clusters of pleomorphic malignant spindled and epithelioid cells. An immunostain panel did not demonstrate any definitive mesenchymal or epithelial differentiation. The tumor's intraluminal origin was supported by radiographic imaging studies. Subsequently, the patient received preoperative chemotherapy and underwent tumor resection with reconstruction. This report describes the cytomorphologic features of this rare intravascular tumor and demonstrates how EBUS-TBNA with ROSE was instrumental in obtaining optimal cytologic sampling for ancillary studies, thus expediting the management.

  16. Pulmonary artery intimal sarcoma diagnosed using endobronchial ultrasound-guided transbronchial needle aspiration.

    PubMed

    Caraway, Nancy P; Salina, Davide; Deavers, Michael T; Morice, Rodolfo; Landon, Gene

    2015-01-01

    Intimal sarcoma of the pulmonary artery is a rare intraluminal malignant neoplasm that has an aggressive biological behavior, and early diagnosis may improve patient outcome. We describe a case of pulmonary artery intimal sarcoma diagnosed on cytologic material obtained by endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) biopsy with rapid on-site evaluation (ROSE). The aspirate showed loosely cohesive clusters of pleomorphic malignant spindled and epithelioid cells. An immunostain panel did not demonstrate any definitive mesenchymal or epithelial differentiation. The tumor's intraluminal origin was supported by radiographic imaging studies. Subsequently, the patient received preoperative chemotherapy and underwent tumor resection with reconstruction. This report describes the cytomorphologic features of this rare intravascular tumor and demonstrates how EBUS-TBNA with ROSE was instrumental in obtaining optimal cytologic sampling for ancillary studies, thus expediting the management. PMID:25745502

  17. General Information about Ewing Sarcoma

    MedlinePlus

    ... Research Ewing Sarcoma Treatment (PDQ®)–Patient Version General Information About Ewing Sarcoma Go to Health Professional Version ... the PDQ Pediatric Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  18. Epithelioid angiosarcoma of the skin. A malignant tumor mimicking many different neoplasms.

    PubMed

    Santos-Juanes1, Jorge; Vivanco-Allende, Blanca; Galache, Cristina Galache

    2016-01-01

    Epithelioid angiosarcomas are rare malignant mesenchymal tumors. The main problem of these tumors is the complicate clinical and histological diagnosis. We report a case with an immunohistochemical panel. We propose the use of CD31 in the immunohistochemical panel of an undifferenciated tumor with epithelioid features, because it appears to be the only endothelial marker these tumors constantly express. PMID:27617941

  19. Radiologic-Pathologic Correlation: Metastatic Pulmonary Epithelioid Hemangioendothelioma of Bone Primary

    PubMed Central

    Lee, Christine U; Zreik, Riyam T; Boland, Jennifer M; White, Mariah L

    2015-01-01

    Epithelioid hemangioendothelioma is a rare vascular malignancy often characterized by a clinically indolent course and delayed diagnosis. The authors present the radiologic and pathologic features of a case of pulmonary epithelioid hemangioendothelioma which was initially thought to be calcified granulomas. PMID:26430543

  20. The Impact of Radiation on an Unusual Case of Omental Epithelioid Angiosarcoma

    PubMed Central

    Narayanan, Sumana; Parker, Mitchell; Shayo, Jonathan; Zheng, Min; Matulewicz, Theodore; Parker, Glenn

    2015-01-01

    Epithelioid angiosarcoma is a rare high-grade tumor with irregular vascular morphology. We report an unusual case of intra-abdominal epithelioid angiosarcoma affecting the omentum and peritoneal surfaces resulting in significant hemorrhagic and inflammatory changes. As in other cases of this tumor this patient had previously undergone radiation treatment for a history of cervical cancer. PMID:26290766

  1. Sarcomas and pharmacogenetics.

    PubMed

    Biason, Paola; Toffoli, Giuseppe

    2005-09-01

    Sarcomas are a heterogeneous group of tumors, requiring different chemotherapeutic approaches. Recently, several regimens for metastatic tumors were evaluated with respect to the different responses to conventional chemotherapy of the various histologic subtypes of sarcomas. The impact of pharmacogenetics in the progress of chemotherapy appears to be crucial in defining the clinical response to many drugs, such as anthracycline or alkylating agents, that are widely used in treatment regimens for soft tissue sarcomas (STS) or sarcomas of the bone. Polymorphisms of metabolizing enzymes (e.g., cytochrome P450 and glutathione-S-transferase), transporter proteins (reduced folate carrier and P-glycoprotein) or target proteins (thymidylate synthase, methylenetetrahydrofolate reductase, dihydrofolate reductase, and c-KIT) may be responsible for an altered clinical outcome, in terms of both response and toxicity. The administration of new chemotherapeutic agents, such as imatinib for gastrointestinal tumors (GIST), requires the study of genetic polymorphisms possibly affecting the integrity of the target (c-KIT), which may provide valid information regarding possible developments of therapy. For STS and sarcoma of the bone, the genetic markers, which could be unambiguously predictive of the phenotypic profile of patients, are as yet undetermined.

  2. Primary Intracranial Synovial Sarcoma

    PubMed Central

    Li, Luyuan; Sinson, Grant; Mueller, Wade

    2016-01-01

    Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18)(p11.2;q11.2) chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression. PMID:27247811

  3. Primary Intracranial Synovial Sarcoma.

    PubMed

    Patel, Mohit; Li, Luyuan; Nguyen, Ha Son; Doan, Ninh; Sinson, Grant; Mueller, Wade

    2016-01-01

    Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18)(p11.2;q11.2) chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression. PMID:27247811

  4. Pulmonary tumor thrombotic microangiopathy from metastatic epithelioid angiosarcoma

    PubMed Central

    Cakir, Ebru; Yazici, Ulku; Tastepe, Irfan

    2013-01-01

    The lung is most common site for metastatic disease via hematogenous route. Tumor emboli of the vessels of the lung induces fibrocellular and fibromuscular intimal proliferation. These histopathological changes may cause pulmonary tumor trombotic microangiopaty. Few cases are diagnosed antemortem. We report a 60 year old woman with by metastatic epithelioid angiosarcoma involving the lung. Tumor cells were positive for VEGF and topoisomerase II. VEGF may be involved in the pathogenesis pulmonary tumor trombotic microangioapy and topoisomerase II positivity showed sensitivity against catalytic topoisomerase II inhibitors. PMID:23825782

  5. Multicentric myofibroblastic sarcoma

    PubMed Central

    Wechalekar, Mihir Dilip; Ayres, Oliver; Farshid, Gelareh; Clayer, Mark; Cleland, Leslie G

    2014-01-01

    We report a case of synchronous, multicentric low-grade myofibroblastic sarcoma presenting in a 62-year-old man. He initially presented with inflammatory symmetric polyarthritis and adhesive capsulitis of his shoulder and hips bilaterally and did not respond to a trial of disease modifying antirheumatic drugs. Over a period of several years he developed progressive restriction of both knees and nodules on his hands, both knees and back. A biopsy of the nodule on his back was inconclusive and subsequent biopsies on his left and then right knee revealed a spindle cell neoplasm with an infiltrative growth pattern, mitotic figures, positive immunostaining for smooth muscle actin and focal myxoid change consistent with myofibroblastic sarcoma. While myofibroblastic sarcoma has been known to metastasise, to our knowledge, a multifocal presentation of this tumour has not been described previously. PMID:25368122

  6. Simultaneous Clear Cell Sarcomas of the Duodenum and Jejunum

    PubMed Central

    Cruise, Michael

    2016-01-01

    Clear cell sarcoma (CCS) is an uncommon tumor that usually presents as an extremity mass but can rarely manifest as a gastrointestinal tumor with a diverse spectrum of symptoms, most commonly related to a mass effect or ulceration. Herein we report a case in which two separate tumors, one in the duodenum and the other in the jejunum, present concurrently. The subject presented with symptomatic anemia and underwent imaging and endoscopic studies that culminated in the discovery of the two lesions. He subsequently underwent operative treatment with resection of both tumors and made an unremarkable recovery. The resection specimen consisted of two separate clear cell sarcomas with negative margins. Under microscopic evaluation, they demonstrated nested growths of epithelioid cells with scattered spindled cells infiltrating the enteric wall. The neoplastic cells were positive for S100 with scattered expression of Melan A. Florescence in situ hybridization revealed a translocation at the EWRS1 locus. He was disease-free for 30 months following the procedure; then he developed a rapidly progressing metastatic disease with subsequent death 4 months later. PMID:27375743

  7. Epithelioid melanocytic nevus with tubule and pseudoacini formation.

    PubMed

    Uhlenhake, Elizabeth E; Smoller, Bruce R; Gardner, Jerad M; Shalin, Sara C

    2015-03-01

    A 26-year-old female presented with a 7 mm irritated pink-red papule on the left posterior shoulder. A shave biopsy revealed a dermal proliferation of epithelioid cells arranged in small nests with central lumen-like structures resembling glands set in a densely sclerotic stroma. S100 and Melanoma antigen recognized by T cells 1 (MART-1) immunohistochemical positivity confirmed a dermal melanocytic neoplasm. Pan-cytokeratin and cytokeratin 7 were negative within the nests ruling out an adnexal neoplasm or metastatic adenocarcinoma. A Spitz nevus variant characterized by the presence of focal tubular structures (tubular epithelioid cell nevus) has rarely been described in the literature and is of uncertain biological significance. Similar structures have also been observed in Clark/dysplastic nevi and melanoma. Glandular differentiation is seen in a wide variety of benign and malignant epithelial neoplasms; however, melanocytes are not known to be capable of forming true glands. The exact mechanism and significance of this phenomenon are currently unknown. Certain postulations include central melanocyte apoptosis, autocrine or paracrine factor secretion or retraction artifact caused by tissue fixation. This distinctive finding is important to recognize in order to avoid misdiagnosis as a glandular neoplasm.

  8. Deregulation of the Hippo pathway in soft-tissue sarcoma promotes FOXM1 expression and tumorigenesis

    PubMed Central

    Eisinger-Mathason, T. S. Karin; Mucaj, Vera; Biju, Kevin M.; Nakazawa, Michael S.; Gohil, Mercy; Cash, Timothy P.; Yoon, Sam S.; Skuli, Nicolas; Park, Kyung Min; Gerecht, Sharon; Simon, M. Celeste

    2015-01-01

    Genetic aberrations responsible for soft-tissue sarcoma formation in adults are largely unknown, with targeted therapies sorely needed for this complex and heterogeneous family of diseases. Here we report that that the Hippo pathway is deregulated in many soft-tissue sarcomas, resulting in elevated expression of the effector molecule Yes-Associated Protein (YAP). Based on data gathered from human sarcoma patients, a novel autochthonous mouse model, and mechanistic analyses, we determined that YAP-dependent expression of the transcription factor forkhead box M1 (FOXM1) is necessary for cell proliferation/tumorigenesis in a subset of soft-tissue sarcomas. Notably, FOXM1 directly interacts with the YAP transcriptional complex via TEAD1, resulting in coregulation of numerous critical pro-proliferation targets that enhance sarcoma progression. Finally, pharmacologic inhibition of FOXM1 decreases tumor size in vivo, making FOXM1 an attractive therapeutic target for the treatment of some sarcoma subtypes. PMID:26080399

  9. Management of Bone Sarcoma.

    PubMed

    Gutowski, Christina J; Basu-Mallick, Atrayee; Abraham, John A

    2016-10-01

    Treatment of bone sarcoma requires careful planning and involvement of an experienced multidisciplinary team. Significant advancements in systemic therapy, radiation, and surgery in recent years have contributed to improved functional and survival outcomes for patients with these difficult tumors, and emerging technologies hold promise for further advancement. PMID:27542644

  10. Leukosis/Sarcoma Group

    Technology Transfer Automated Retrieval System (TEKTRAN)

    The leukosis/sarcoma (L/S) group of diseases designates a variety of transmissible benign and malignant neoplasms of chickens caused by members that belong to the family Retroviridae. Because the expansion of the literature on this disease, it is no longer feasible to cite all relevant publications ...

  11. Leukosis/Sarcoma Group

    Technology Transfer Automated Retrieval System (TEKTRAN)

    The leukosis/sarcoma (L/S) group of diseases designates a variety of transmissible benign and malignant neoplasms of chickens caused by members that belong to the family Retroviridae. Lymphoid leukosis has been the most common form of L/S group of diseases seen in field flocks, although myeloid leuk...

  12. Microscopic endometrial perivascular epithelioid cell nodules: a case report with the earliest presentation of a uterine perivascular epithelioid cell tumor.

    PubMed

    Fang, Chia-Lang; Lin, Yun-Ho; Chen, Wei-Yu

    2012-09-03

    Perivascular epithelioid cell (PEC) tumors (PEComas) are a family of related mesenchymal tumors composed of PECs which co-express melanocytic and smooth muscle markers. Although their distinctive histologic, immunohistochemical, ultrastructural, and genetic features have been clearly demonstrated, their histogenesis and normal counterpart remain largely unknown. Precursor lesions of PEComas have rarely been reported. We herein describe a tuberous sclerosis patient with microscopic PEC nodules in the endometrium of adenomyosis, pelvic endometriosis, an ovarian endometriotic cyst, and the endometrium of the uterine cavity. The nodules showed a mixture of spindle-shaped and epithelioid cells concentrically arranged around small arteries. The cells exhibited uniform nuclei, light eosinophilic cytoplasm, and immunoreactivity with HMB-45 and CD10. Some nodules revealed continuity with a PEComa in the myometrium. These findings support microscopic endometrial PEC nodules possibly being precursor lesions of uterine PEComas. The wide distribution of the nodules in the pelvis may be related to the multicentricity of PEComas in tuberous sclerosis patients. Owing to the immunoreactivity with CD10, microscopic endometrial PEC nodules may be misinterpreted as endothelial stromal cells unless melanocytic markers are stained. To the best of our knowledge, this is a case with the earliest manifestation of PEC lesions occurring in the endometrium. Virtual slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/9658280017862643.

  13. Ambiguous presentations of pulmonary epithelioid hemangioendothelioma: Two case reports of a rare pulmonary malignancy.

    PubMed

    Soo, Chun Ian; Ng, Boon Hau; Tan, Eng Liang; Abdul Hamid, Faisal

    2016-01-01

    Pulmonary epithelioid hemangioendothelioma is an uncommon lung malignancy of endothelial origin. Besides demonstrating unpredictable presentation features and prognosis, the paucity of established treatment guidelines remains a challenge in managing these patients. We present two patients. The first patient presented with chronic productive cough over 1-year duration. He was initially diagnosed and showed partial response to treatment for cardiac failure. A persistent right upper zone consolidation on chest radiograph prompted further investigations which revealed the diagnosis of pulmonary epithelioid hemangioendothelioma. The second patient presented with right-sided hemiparesis for 1-month duration. Initial computer tomography scan of the brain showed findings of distant metastatic foci. Subsequent investigations revealed pulmonary epithelioid hemangioendothelioma as the primary lesion. Both patients succumbed without any treatment due to rapid progression of the disease. We believe that pulmonary epithelioid hemangioendothelioma is undoubtedly rarely reported in south-east Asia region. In these two case reports, the patients were diagnosed in west and east Malaysia, respectively, in the same year (2015). Both cases highlight the increasing prevalence of pulmonary epithelioid hemangioendothelioma. We postulate that this could possibly be secondary to the advancement in diagnostic capabilities and improved healthcare facilities available in this region. Late presentation of pulmonary epithelioid hemangioendothelioma generally results in grave prognosis. Further investigations are required to elucidate the nature of progression and therapeutic options for patients with pulmonary epithelioid hemangioendothelioma. PMID:27489719

  14. Immunotherapy of Childhood Sarcomas.

    PubMed

    Roberts, Stephen S; Chou, Alexander J; Cheung, Nai-Kong V

    2015-01-01

    Pediatric sarcomas are a heterogeneous group of malignant tumors of bone and soft tissue origin. Although more than 100 different histologic subtypes have been described, the majority of pediatric cases belong to the Ewing's family of tumors, rhabdomyosarcoma and osteosarcoma. Most patients that present with localized stage are curable with surgery and/or chemotherapy; however, those with metastatic disease at diagnosis or those who experience a relapse continue to have a very poor prognosis. New therapies for these patients are urgently needed. Immunotherapy is an established treatment modality for both liquid and solid tumors, and in pediatrics, most notably for neuroblastoma and osteosarcoma. In the past, immunomodulatory agents such as interferon, interleukin-2, and liposomal-muramyl tripeptide phosphatidyl-ethanolamine have been tried, with some activity seen in subsets of patients; additionally, various cancer vaccines have been studied with possible benefit. Monoclonal antibody therapies against tumor antigens such as disialoganglioside GD2 or immune checkpoint targets such as CTLA-4 and PD-1 are being actively explored in pediatric sarcomas. Building on the success of adoptive T cell therapy for EBV-related lymphoma, strategies to redirect T cells using chimeric antigen receptors and bispecific antibodies are rapidly evolving with potential for the treatment of sarcomas. This review will focus on recent preclinical and clinical developments in targeted agents for pediatric sarcomas with emphasis on the immunobiology of immune checkpoints, immunoediting, tumor microenvironment, antibody engineering, cell engineering, and tumor vaccines. The future integration of antibody-based and cell-based therapies into an overall treatment strategy of sarcoma will be discussed.

  15. Epithelioid and fusiform blue nevus of chronically sun-damaged skin, an entity distinct from the epithelioid blue nevus of the Carney complex.

    PubMed

    Yazdan, Pedram; Haghighat, Zahra; Guitart, Joan; Gerami, Pedram

    2013-01-01

    Epithelioid blue nevus (EBN) was first described in patients with Carney complex (CNC) and subsequently shown to also occur sporadically. Over 50% of patients with CNC harbor mutations in the gene PRKAR1A, which codes for protein kinase A regulatory subunit 1α (R1α) involved in the signaling pathway regulating melanogenesis and melanocytic proliferation. Immunohistochemical expression of R1α has been shown to be absent in the majority of pigmented epithelioid melanocytomas and all CNC-associated EBNs but present in melanomas and other melanocytic nevi. We have observed several examples of EBN occurring in chronically sun-damaged (CSD) skin with a predominance of epithelioid morphology but also containing a component of fusiform and conventional blue nevus cells, which we have termed epithelioid and fusiform blue nevus of CSD skin. Several of these cases demonstrated notable pleomorphism and nuclear atypia with rare mitotic activity raising concern for the possibility of melanoma; however, the clinical outcomes, detailed histologic review, and molecular results were most consistent with a benign melanocytic neoplasm. We report our clinical, histopathologic, immunohistochemistry, and fluorescence in situ hybridization experience with this distinct entity of epithelioid and fusiform blue nevus and demonstrate that it is a unique subtype of blue nevus occurring on CSD skin with a higher frequency of an associated conventional blue nevus component compared with EBN and without association with CNC or loss of R1α expression typically found in pigmented epithelioid melanocytoma and CNC-associated EBN. We also postulate that the epithelioid pattern may represent a subclone of the conventional blue nevus component induced by chronic UV damage. PMID:22892599

  16. Bone Sarcomas: From Biology to Targeted Therapies

    PubMed Central

    Gaspar, Nathalie; Di Giannatale, Angela; Geoerger, Birgit; Redini, Françoise; Corradini, Nadège; Enz-Werle, Natacha; Tirode, Franck; Marec-Berard, Perrine; Gentet, Jean-Claude; Laurence, Valérie; Piperno-Neumann, Sophie; Oberlin, Odile; Brugieres, Laurence

    2012-01-01

    Primary malignant bone tumours, osteosarcomas, and Ewing sarcomas are rare diseases which occur mainly in adolescents and young adults. With the current therapies, some patients remain very difficult to treat, such as tumour with poor histological response to preoperative CT (or large initial tumour volume for Ewing sarcomas not operated), patients with multiple metastases at or those who relapsed. In order to develop new therapies against these rare tumours, we need to unveil the key driving factors and molecular abnormalities behind the malignant characteristics and to broaden our understanding of the phenomena sustaining the metastatic phenotype and treatment resistance in these tumours. In this paper, starting with the biology of these tumours, we will discuss potential therapeutic targets aimed at increasing local tumour control, limiting metastatic spread, and finally improving patient survival. PMID:23226965

  17. Head and Neck Sarcomas: Analysis of the SEER Database

    PubMed Central

    Peng, Kevin A.; Grogan, Tristan; Wang, Marilene B.

    2015-01-01

    Objective To summarize the epidemiology of sarcomas occurring in the head and neck and identify prognostic factors for patient survival. Study Design and Setting Cross-sectional analysis of the National Cancer Institute’s Surveillance, Epidemiology and End Results (SEER) program. Methods The SEER 18 registries, comprising sarcoma diagnoses made from 1973 to 2010, were queried for sarcomas arising in the head and neck. Pediatric and adult patients were analyzed separately, and multivariate and propensity-matched analyses were performed to identify predictors of disease-specific survival. Results In all, 11,481 adult cases and 1244 pediatric cases were identified. In adults, the most common histologic subtypes were malignant fibrous histiocytoma (MFH), Kaposi sarcoma, and hemangiosarcoma, while in the pediatric cohort, the most common histologic subtypes were rhabdomyosar-coma, MFH, and osteosarcoma. Cause-specific 2-, 5-, and 10-year survival rates were 76%, 66%, and 61% for adults and 84%, 73%, and 71% for pediatric patients. Multivariate analysis performed for adults revealed that male gender, absence of radiation therapy, and stage I disease were associated with improved cause-specific survival reaching statistical significance. However, a propensity-matched model demonstrated no significant difference in cause-specific survival between patients who received radiation and those who did not. Conclusion Sarcomas, a heterogeneous group of malignant mesenchymal tumors, are uncommonly found in the head and neck. This study represents the largest analysis of patients with head and neck sarcomas in the literature and demonstrates the impact of age, gender, primary site, histology, and radiation status on overall prognosis. PMID:25135525

  18. Epithelioid haemangioma: a rare cause of painful erections and sleep deprivation.

    PubMed

    Lucky, M A; McGuinness, L A; Floyd, M S; Azhar, U; Shanks, J H; Li, C; Shenjere, P; Nonaka, D; Robinson, L Q; Parr, N J

    2014-09-01

    Epithelioid haemangioma of the penis is a rare condition which usually presents a solid single nodule. We report a case in a 43-year-old man who presented with painful erections and sleep disturbance with two palpable penile nodules. Magnetic resonance imaging with an artificially induced erection revealed these as individual lesions, and local excision was successfully undertaken. Pathological diagnosis of epithelioid haemangioma was confirmed with positive staining for CD31. Although rare, penile epithelioid haemangioma should be considered as a differential in an atypical penile mass. Induction in of an artificial erection prior to MRI can aid diagnosis and treatment is typically with surgical excision.

  19. Drugs Approved for Soft Tissue Sarcoma

    MedlinePlus

    ... Professionals Questions to Ask about Your Treatment Research Drugs Approved for Soft Tissue Sarcoma This page lists ... soft tissue sarcoma that are not listed here. Drugs Approved for Soft Tissue Sarcoma Cosmegen (Dactinomycin) Dactinomycin ...

  20. PERIVASCULAR EPITHELIOID TUMOURS (PEComas) OF THE GYNAECOLOGICAL TRACT

    PubMed Central

    Conlon, Niamh; Soslow, Robert A.; Murali, Rajmohan

    2016-01-01

    Perivascular epithelioid tumour (PEComas) of the gynaecological tract are rare tumours which were first recognised and diagnosed within the last twenty years. They represent a unique diagnostic challenge with regard to their accurate and reproducible distinction from more common entities such as smooth muscle tumours of the uterine corpus. In this review article we trace the development of the concept of the PEComa tumour family, highlight what is known about extra-gynaecological tract PEComa at an immunohistochemical, molecular and therapeutic level and then present a summary of all reported cases of gynaecological tract PEComa to date. In the summary, we highlight rare subtypes of gynaecological tract PEComa, and compare the performances of extant prognostic classification systems for malignancy in these tumours. PMID:25750268

  1. Chemoembolization followed by orthotopic liver transplant for epithelioid hemangioendothelioma.

    PubMed

    St Peter, Shawn D; Moss, Adyr A; Huettl, Eric A; Leslie, Kevin O; Mulligan, David C

    2003-12-01

    Hepatic epithelioid hemangioendothelioma (HEHE) is a rare liver tumor with an indolent course relative to other hepatic malignancies. Over the past two decades, primary treatment for these lesions has been defined as resection for localized disease, or transplantation for diffuse and multifocal tumors. No published report to date has described effective pre- or post-operative adjuvant treatment for this disease. In this report, we present the first case of HEHE effectively managed with chemoembolization followed by transplantation, documenting objective tumor response to embolization. Furthermore, diagnosis for this lesion can easily be mistaken, directing management in erroneous directions. This case illustrates diagnostic pitfalls affiliated with the work-up of this tumor. PMID:14756273

  2. Epithelioid hemangioendothelioma of the spine. Report of two cases.

    PubMed

    Aquilina, Kristian; Lim, Christopher; Kamel, Mahmoud Hamdy; Marks, Charles J; O'Sullivan, Michael G; Keohane, Catherine

    2005-11-01

    Epithelioid hemangioendothelioma (EH) is a rare tumor of vascular origin. The authors describe two cases of spinal EH, one involving the T-10 vertebra and the second involving the upper cervical spine. In the first case the patient underwent resection of the tumor; this case represents the longest reported follow-up period for spinal EH. In the second case, extensive involvement of C-2, C-3, and C-4 as well as encasement of both vertebral arteries precluded safe tumor resection, and posterior occipitocervical stabilization was performed. The patient subsequently died of metastatic disease. The findings in these two cases underscore the difficulty in predicting the clinical behavior of spinal EH based solely on histological and clinical features as well as the uncertainty of the roles of surgery, chemotherapy, and radiotherapy in the oncological management of a spinal tumor for which clinical data are very limited.

  3. [Multifocal epithelioid angiosarcoma of bone with lung metastases].

    PubMed

    Pacheco, C; Albalá, M D; Blanco, M; Hidalgo, F J

    2014-01-01

    Angiosarcoma is a rare mesenchymal neoplasm that may arise from vascular or lymphatic tissue. Bone primary angiosarcoma is extremely rare, representing less than 1% of all angiosarcomas. It́s a very aggressive neoplasm and patients have metastatic disease at initial diagnosis in a large percentage of cases. On radiographs, these lesions are usually aggressive osteolytic lesions, commonly with soft-tissue mass extension, and tumoral enhancement on CT or MR imaging. The appearance of the bone scan is variable, describing studies with tracer uptake or low uptake. These tumours are more often found in the long bones, but spinal involvement has been reported in 10% of patients. There are a few reports in the literature of bone angiosarcoma with lung metastases. We present a patient with multifocal epithelioid angiosarcoma (spine and ribs) and multiple lung metastasis, evidenced by CT and conventional bone scintigraphy, with a fast growth.

  4. Epithelioid granulomas in Hodgkin's disease--prognostic significance.

    PubMed

    Chopra, R; Rana, R; Zachariah, A; Mahajan, M K; Prabhakar, B R

    1995-10-01

    Prognostic significance of non-caseating epithelioid granulomas in association with Hodgkin's disease has been studied. Such granulomas were found in 15 of the total of 104 cases of Hodgkin's disease encountered between Jan. 1981 and June 1990. These 15 patients were compared with 30 concurrent patients of Hodgkin's disease who did not have associated granulomas. All the patients were initially staged, treated and followed up for a period of two years. There was no significant difference in overall survival rate between the granuloma group and the control group. However, in relapse free survival rate in advanced stages of the disease (III & IV), although the difference between granuloma group and the control group was not statistically significant (p = 0.11), yet the relapse free survival curves revealed a tendency towards better survival with lesser number of relapses and longer remissions in granuloma group.

  5. Endosialin expression in soft tissue sarcoma as a potential marker of undifferentiated mesenchymal cells

    PubMed Central

    Thway, Khin; Robertson, David; Jones, Robin L; Selfe, Joanna; Shipley, Janet; Fisher, Cyril; Isacke, Clare M

    2016-01-01

    Background: Soft tissue sarcomas are a group of neoplasms with differentiation towards mesenchymal tissue, many of which are aggressive and chemotherapy resistant. Histology and immunoprofiles often overlap with neoplasms of other lineages, and establishing an accurate histopathological diagnosis is crucial for correct management, and therapeutic stratification. The endosialin cell surface glycoprotein is predominantly expressed by stromal fibroblasts and pericytes in epithelial neoplasms; however, tumour cell expression has been reported in small series of sarcomas. Methods: We assessed endosialin expression by immunohistochemistry in a large set of 514 human soft tissue sarcomas. Results: Tumour cell endosialin expression was seen in 89% of undifferentiated pleomorphic sarcomas (104/117), 77% adult fibrosarcomas/spindle cell sarcomas (20/26), 62% synovial sarcomas (37/60), 51% leiomyosarcomas (94/185) and 31% rhabdomyosarcomas (39/126). Conclusions: Endosialin immunohistochemistry has potential to distinguish undifferentiated and poorly differentiated sarcomas from other poorly differentiated, non-mesenchymal neoplasms. A Phase II trial randomising patients with advanced sarcomas to receive chemotherapy with/without an endosialin therapeutic antibody has recently completed enrolment. Endosialin expression could be used to select patients for such clinical trials. Based on our results, patients with undifferentiated pleomorphic sarcoma may be particularly suitable for such a therapeutic approach. PMID:27434038

  6. Ultrastructural characteristics of type A epithelioid cells during BCG-granulomatosis and treatment with lysosomotropic isoniazid.

    PubMed

    Shkurupii, V A; Kozyaev, M A; Nadeev, A P

    2006-04-01

    We studied BCG-granulomas, their cellular composition, and ultrastructure of type A epithelioid cells in the liver of male BALB/c mice with spontaneous granulomatous inflammation. The animals received free isoniazid or isoniazid conjugated with lysosomotropic intracellularly prolonged matrix (dialdehyde dextran, molecular weight 65-75 kDa). Lysosomotropic isoniazid was accumulated in the vacuolar apparatus of epithelioid cells and produced a stimulatory effect on plastic processes in these cells.

  7. Potential molecular targets for Ewing's sarcoma therapy.

    PubMed

    Jully, Babu; Rajkumar, Thangarajan

    2012-10-01

    Ewing's sarcoma (ES) is a highly malignant tumor of children and young adults. Modern therapy for Ewing's sarcoma combines high-dose chemotherapy for systemic control of disease, with advanced surgical and/or radiation therapeutic approaches for local control. Despite optimal management, the cure rate for localized disease is only approximately 70%, whereas the cure rate for metastatic disease at presentation is less than 30%. Patients who experience long-term disease-free survival are at risk for significant side-effects of therapy, including infertility, limb dysfunction and an increased risk for second malignancies. The identification of new targets for innovative therapeutic approaches is, therefore, strongly needed for its treatment. Many new pharmaceutical agents have been tested in early phases of clinical trials in ES patients who have recurrent disease. While some agents led to partial response or stable disease, the percentages of drugs eliciting responses or causing an overall effect have been minimal. Furthermore, of the new pharmaceuticals being introduced to clinical practice, the most effective agents also have dose-limiting toxicities. Novel approaches are needed to minimize non-specific toxicity, both for patients with recurrence and at diagnosis. This report presents an overview of the potential molecular targets in ES and highlights the possibility that they may serve as therapeutic targets for the disease. Although additional investigations are required before most of these approaches can be assessed in the clinic, they provide a great deal of hope for patients with Ewing's sarcoma. PMID:23580819

  8. 17-N-Allylamino-17-Demethoxygeldanamycin in Treating Patients With Advanced Epithelial Cancer, Malignant Lymphoma, or Sarcoma

    ClinicalTrials.gov

    2013-02-06

    AIDS-related Peripheral/Systemic Lymphoma; AIDS-related Primary CNS Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Chondrosarcoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Nodal Marginal Zone B-cell Lymphoma; Ovarian Sarcoma; Primary Central Nervous System Non-Hodgkin Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Osteosarcoma; Recurrent Small Lymphocytic Lymphoma; Recurrent Uterine Sarcoma; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage IV Adult Burkitt Lymphoma; Stage IV Adult Diffuse Large Cell Lymphoma; Stage IV Adult Diffuse Mixed Cell Lymphoma; Stage IV Adult Diffuse Small Cleaved Cell Lymphoma; Stage IV Adult Hodgkin Lymphoma; Stage IV Adult Immunoblastic Large Cell Lymphoma; Stage IV Adult Lymphoblastic Lymphoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Adult T-cell Leukemia/Lymphoma; Stage IV Cutaneous T-cell Non-Hodgkin Lymphoma; Stage IV Grade 1 Follicular Lymphoma; Stage IV Grade 2 Follicular Lymphoma; Stage IV Grade 3 Follicular Lymphoma; Stage IV Mantle Cell Lymphoma; Stage IV Marginal Zone Lymphoma; Stage IV Mycosis Fungoides/Sezary Syndrome; Stage IV Small

  9. Renal metastases from osteogenic sarcoma

    SciTech Connect

    Ayres, R.; Curry, N.S.; Gordon, L.; Bradford, B.F.

    1985-01-01

    A clinically and radiographically unsuspected ossified renal metastasis from a primary osteogenic sarcoma was identified by computed tomography (CT) and radionuclide bone scan. These imaging modalities play an important adjunctive role in the evaluation and follow-up of patients with primary osteogenic sarcoma.

  10. Diagnostic Study of Tumor Characteristics in Patients With Ewing's Sarcoma

    ClinicalTrials.gov

    2013-06-20

    Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  11. Collecting and Storing Biological Samples From Patients With Ewing Sarcoma

    ClinicalTrials.gov

    2016-05-16

    Askin Tumor; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  12. Expression profiling of synovial sarcoma by cDNA microarrays: association of ERBB2, IGFBP2, and ELF3 with epithelial differentiation.

    PubMed

    Allander, Susanne V; Illei, Peter B; Chen, Yidong; Antonescu, Cristina R; Bittner, Mike; Ladanyi, Marc; Meltzer, Paul S

    2002-11-01

    Synovial sarcoma is an aggressive spindle cell sarcoma with two major histological subtypes, biphasic and monophasic, defined respectively by the presence or absence of areas of glandular epithelial differentiation. It is characterized by a specific chromosomal translocation, t(X;18)(p11.2;q11.2), which juxtaposes the SYT gene on chromosome 18 to either the SSX1 or the SSX2 gene on chromosome X. The chimeric SYT-SSX products are thought to function as transcriptional proteins that deregulate gene expression, thereby providing a putative oncogenic stimulus. We investigated the pattern of gene expression in synovial sarcoma using cDNA microarrays containing 6548 sequence-verified human cDNAs. A tissue microarray containing 37 synovial sarcoma samples verified to bear the SYT-SSX fusion was constructed for complementary analyses. Gene expression analyses were performed on individual tumor samples; 14 synovial sarcomas, 4 malignant fibrous histiocytomas, and 1 fibrosarcoma. Statistical analysis showed a distinct expression profile for the group of synovial sarcomas as compared to the other soft tissue sarcomas, which included variably high expression of ERBB2, IGFBP2, and IGF2 in the synovial sarcomas. Immunohistochemical analysis of protein expression in tissue microarrays of 37 synovial sarcomas demonstrated strong expression of ERBB2 and IGFBP2 in the glandular epithelial component of biphasic tumors and in solid epithelioid areas of some monophasic tumors. Fluorescence in situ hybridization analysis indicated that the ERBB2 overexpression was not because of gene amplification. Differentially expressed genes were also found in a comparison of the expression profiles of the biphasic and monophasic histological subgroups of synovial sarcoma, notably several keratin genes, and ELF3, an epithelial-specific transcription factor gene. Finally, we also noted differential overexpression of several neural- or neuroectodermal-associated genes in synovial sarcomas relative to

  13. Ki67 index is an independent prognostic factor in epithelioid but not in non-epithelioid malignant pleural mesothelioma: a multicenter study

    PubMed Central

    Ghanim, B; Klikovits, T; Hoda, M A; Lang, G; Szirtes, I; Setinek, U; Rozsas, A; Renyi-Vamos, F; Laszlo, V; Grusch, M; Filipits, M; Scheed, A; Jakopovic, M; Samarzija, M; Brcic, L; Stancic–Rokotov, D; Kern, I; Rozman, A; Dekan, G; Klepetko, W; Berger, W; Glasz, T; Dome, B; Hegedus, B

    2015-01-01

    Background: Estimating the prognosis in malignant pleural mesothelioma (MPM) remains challenging. Thus, the prognostic relevance of Ki67 was studied in MPM. Methods: Ki67 index was determined in a test cohort of 187 cases from three centres. The percentage of Ki67-positive tumour cells was correlated with clinical variables and overall survival (OS). The prognostic power of Ki67 index was compared with other prognostic factors and re-evaluated in an independent cohort (n=98). Results: Patients with Ki67 higher than median (>15%) had significantly (P<0.001) shorter median OS (7.5 months) than those with low Ki67 (19.1 months). After multivariate survival analyses, Ki67 proved to be—beside histology and treatment—an independent prognostic marker in MPM (hazard ratio (HR): 2.1, P<0.001). Interestingly, Ki67 was prognostic exclusively in epithelioid (P<0.001) but not in non-epithelioid subtype. Furthermore, Ki67 index was significantly lower in post-chemotherapy samples when compared with chemo-naive cases. The prognostic power was comparable to other recently published prognostic factors (CRP, fibrinogen, neutrophil-to-leukocyte ratio (NLR) and nuclear grading score) and was recapitulated in the validation cohort (P=0.048). Conclusion: This multicentre study demonstrates that Ki67 is an independent and reproducible prognostic factor in epithelioid but not in non-epithelioid MPM and suggests that induction chemotherapy decreases the proliferative capacity of MPM. PMID:25633038

  14. Liver transplantation for hepatic epithelioid hemangioendothelioma: The Canadian multicentre experience

    PubMed Central

    Nudo, Carmine G; Yoshida, Eric M; Bain, Vincent G; Marleau, Denis; Wong, Phil; Marotta, Paul J; Renner, Eberhard; Watt, Kymberly D; Deschênes, Marc

    2008-01-01

    INTRODUCTION: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare entity. At the present time, there is no standardized effective therapy. Liver transplantation (LT) has emerged as a treatment for this rare tumour. OBJECTIVE: To evaluate the outcome of liver transplantation for HEHE at eight centres across Canada. METHODS: The charts of patients who were transplanted for HEHE at eight centres across Canada were reviewed. RESULTS: A total of 11 individuals (eight women and three men) received a LT for HEHE. All LTs were performed between 1991 and 2005. The mean (± SD) age at LT was 38.7±13 years. One patient had one large liver lesion (17 cm × 14 cm × 13 cm), one had three lesions, one had four lesions and eight had extensive (five or more) liver lesions. One patient had spleen involvement and two had involved lymph nodes at the time of transplantation. The mean duration of follow-up was 78±63 months (median 81 months). Four patients (36.4%) developed recurrence of HEHE with a mean time to recurrence of 25±25 months (median 15.6 months) following LT. The calculated survival rate following LT for HEHE was 82% at five years. CONCLUSIONS: The results of LT for HEHE are encouraging, with a recurrence rate of 36.4% and a five-year survival rate of 82%. Further studies are needed to help identify patients who would benefit most from LT for this rare tumour. PMID:18925305

  15. Contrast-enhanced ultrasound of histologically proven hepatic epithelioid hemangioendothelioma

    PubMed Central

    Dong, Yi; Wang, Wen-Ping; Cantisani, Vito; D’Onofrio, Mirko; Ignee, Andre; Mulazzani, Lorenzo; Saftoiu, Adrian; Sparchez, Zeno; Sporea, Ioan; Dietrich, Christoph F

    2016-01-01

    AIM: To analyze contrast-enhanced ultrasound (CEUS) features of histologically proven hepatic epithelioid hemangioendothelioma (HEHE) in comparison to other multilocular benign focal liver lesions (FLL). METHODS: Twenty-five patients with histologically proven HEHE and 45 patients with histologically proven multilocular benign FLL were retrospectively reviewed. Four radiologists assessed the CEUS enhancement pattern in consensus. RESULTS: HEHE manifested as a single (n = 3) or multinodular (n = 22) FLL. On CEUS, HEHE showed rim-like (18/25, 72%) or heterogeneous hyperenhancement (7/25, 28%) in the arterial phase and hypoenhancement (25/25, 100%) in the portal venous and late phases (PVLP), a sign of malignancy. Eighteen patients showed central unenhanced areas (18/25, 72%); in seven patients (7/25, 28%), more lesions were detected in the PVLP. In contrast, all patients with hemangioma and focal nodular hyperplasia showed hyperenhancement as the most distinctive feature (P < 0.01). CONCLUSION: CEUS allows for characterization of unequivocal FLL. By analyzing the hypoenhancement in the PVLP, CEUS can determine the malignant nature of HEHE. PMID:27217705

  16. Common Presentation with Uncommon Diagnosis: Multifocal Epithelioid Hemangioendothelioma.

    PubMed

    Kundu, Susmita; Misra, Swapnendu; Biswas, Debabani; Mitra, Ritabrata; Naskar, Bidisha Ghosh

    2015-11-01

    A young female patient presenting with recurrent hemoptysis, neck swelling, and mediastinal mass mimicking lymphadenopathy was admitted to the Institute of Post Graduate Medical Education and Research and SSKM hospital, Kolkata, India. Clinical features, radiological studies, fibre optic bronchoscopy, and fine needle aspiration cytology from the neck swelling created a diagnostic dilemma until surgical resection and immunohistochemistry reports confirmed the diagnosis of multifocal epithelioid hemangioendothelioma, a rare vascular tumor with intermediate malignancy potential. Because it is a slow-progressing disease and due to the non-availability of standard chemotherapy, the patient, and her legal guardian, opted for palliative care only. She was asymptomatic for four years but again presented with hemoptysis, reappearance of the neck swelling on the same side, and a mediastinal mass compressing the superior vena cava and right pulmonary artery. This report describes the diagnostic problems and therapeutic challenges in the management of this rare tumor over a four-year follow-up period. The clinical course emphasizes the highly unpredictable nature of this tumor. PMID:26674363

  17. Germline PTPRD mutations in Ewing sarcoma: biologic and clinical implications.

    PubMed

    Jiang, Yunyun; Janku, Filip; Subbiah, Vivek; Angelo, Laura S; Naing, Aung; Anderson, Peter M; Herzog, Cynthia E; Fu, Siqing; Benjamin, Robert S; Kurzrock, Razelle

    2013-06-01

    Ewing sarcoma occurs in children, adolescents and young adults. High STAT3 levels have been reported in approximately 50% of patients with Ewing sarcoma, and may be important in tumorigenesis. Protein tyrosine phosphatase delta (PTPRD) is a tumor suppressor that inhibits STAT3 activation. To date, while somatic mutations in PTPRD have been reported in diverse tumors, germline mutations of PTPRD have not been investigated in Ewing sarcoma or other cancers. We identified a novel germline mutation in the PTPRD gene in three of eight patients (37.5%) with metastatic Ewing sarcoma. Although the functional impact in two of the patients is unclear, in one of them the aberration was annotated as a W775stop germline mutation, and would be expected to lead to gene truncation and, hence, loss of the STAT3 dephosphorylation function of PTPRD. Since STAT3 is phosphorylated after being recruited to the insulin growth factor receptor (IGF-1R), suppression of IGF-1R could attenuate the enhanced STAT3 activation expected in the presence of PTPRD mutations. Of interest, two of three patients with germline PTPRD mutations achieved durable complete responses following treatment with IGF-1R monoclonal antibody-based therapies. Our pilot data suggest that PTPRD germline mutations may play a role in the development of Ewing sarcoma, a disease of young people, and their presence may have implications for therapy.

  18. Adoptive cell therapy for sarcoma

    PubMed Central

    Mata, Melinda; Gottschalk, Stephen

    2015-01-01

    Current therapy for sarcomas, though effective in treating local disease, is often ineffective for patients with recurrent or metastatic disease. To improve outcomes, novel approaches are needed and cell therapy has the potential to meet this need since it does not rely on the cytotoxic mechanisms of conventional therapies. The recent successes of T-cell therapies for hematological malignancies have led to renewed interest in exploring cell therapies for solid tumors such as sarcomas. In this review, we will discuss current cell therapies for sarcoma with special emphasis on genetic approaches to improve the effector function of adoptively transferred cells. PMID:25572477

  19. Primary Epithelioid Angiosarcoma of Spleen: A Case Report and Review of Literature

    PubMed Central

    Abdallah, Rania Abdallah; Asaad, Nancy Yousef; Al-Sharaky, Dalia Rifaat; Alhanafy, Alshimaa Mahmoud

    2016-01-01

    Splenic angiosarcomas are usually secondary tumours, and only few primary cases have been encountered. We report a unique primary case of epithelioid angiosarcoma arising in the spleen in a male patient 55-year-old and presented to our hospital as a medical emergency with acute abdomen and haemorrhagic ascitis. CT revealed splenic focal lesion and suggested that this abdominal haemorrhage was due to ruptured splenic haemangioma, thus abdominal exploration and splenectomy were done. The histopathological examination showed an infiltrating ill-defined growth formed of high grade epithelioid cells arranged in sheet-like growth pattern, with occasional papillary appearance. The presence of rudimentary vascular channels lined by epithelioid endothelial cells with occasional intraluminal erythrocytes suggested vascular tumour origin. The neoplastic cells showed diffuse expression of CD31 together with focal expression of cytokeratin (CK) and CD34. Because of its epithelioid morphology and unmistakable positivity for CK, this case may be easily misdiagnosed as a metastatic carcinoma, which is not uncommon finding in the spleen. Epithelioid angiosarcoma is a rare type of vascular tumour in the spleen, which co-expresses vascular and epithelial markers making its distinction from metastatic carcinoma is sometimes difficult. PMID:26894076

  20. Hepatic epithelioid hemangioendothelioma: resection or transplantation, which and when?

    PubMed

    Ben-Haim, M; Roayaie, S; Ye, M Q; Thung, S N; Emre, S; Fishbein, T A; Sheiner, P M; Miller, C M; Schwartz, M E

    1999-11-01

    Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor with an unpredictable course and prognosis. The aim of this study is to describe our experience with liver resection, as well as transplantation, in the treatment of this tumor. We retrospectively analyzed the clinical features, pathological findings, and postoperative results in a series of 11 patients presenting between 1990 and 1998. Five patients (45%) presented with abdominal pain, 3 patients (27%) with jaundice and ascites, and the rest were asymptomatic. Computed tomography or magnetic resonance imaging showed localized lesions in 2 patients (18%) and multifocal disease in the others. Seven patients (64%) had extrahepatic lesions, detected either by preoperative imaging or discovered at exploration. Two resections of apparently localized lesions were followed by rapid and aggressive recurrence. Five patients were treated with transplantation, including 1 patient who had previously undergone resection. Of these 5 patients, 2 patients are currently free of detectable disease, 1 patient who had severe ascites and jaundice is now asymptomatic with stable extrahepatic lesions, and 2 patients (including 1 who had previously undergone a resection) died of tumor recurrence. One patient with advanced tumor died while waiting for transplantation. The remaining 4 patients are free of symptoms and have stable hepatic and extrahepatic disease. HEHE is nearly always multifocal, and our results with resection were dismal. Because of the unpredictable nature of the tumor, the indications for transplantation in patients without liver-related symptoms should be carefully evaluated. Nevertheless, extrahepatic disease should not be an absolute contraindication for liver transplantation in patients with severe liver dysfunction. PMID:10545542

  1. Drugs Approved for Kaposi Sarcoma

    Cancer.gov

    This page lists cancer drugs approved by the Food and Drug Administration (FDA) for Kaposi sarcoma. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries.

  2. Treatment Options for Kaposi Sarcoma

    MedlinePlus

    ... therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. ... complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information ...

  3. Treatment Option Overview (Kaposi Sarcoma)

    MedlinePlus

    ... therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. ... complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information ...

  4. [Bacillary (epithelioid) angiomatosis with a nodular presentation and self-limited evolution].

    PubMed

    Teira, R; López, I; Zubero, Z; Muñoz, J; Careaga, J M; Santamaría, J M

    1993-06-12

    Bacillary angiomatosis (epithelioid) is a recently described clinicopathologic syndrome, principally associated to infection by the human immunodeficiency virus. The case of a patient who was seen for fever and the appearance of four painful, erythematous and indurated subcutaneous nodules on the anteroexternal face of the right lower extremity 15 days previously is presented. No microorganisms were observed by microbiologic and histologic techniques however the latter showed a vascular proliferation with prominent endothelium of epithelioid morphology and notable interstitial inflammatory reaction according to the pattern described as characteristic of epithelioid angiomatosis. The fever and the nodules disappeared spontaneously. The clinical and histopathologic characteristics of this disease as well as the recent contributions with respect to the identification of the possible causative bacillus are discussed.

  5. Malignant renal epithelioid angiomyolipoma: A case report and review of the literature

    PubMed Central

    GUO, BAOYIN; SONG, HUALIN; YUE, JIULING; LI, GANG

    2016-01-01

    Malignant renal epithelioid angiomyolipoma (EAML) is rare, and currently there is no malignant criteria for its pathological diagnosis. In the present study, the case of a patient who suffered malignant renal EAML and underwent nephrectomy is reported. The histological patterns of the tumor were composed of sheets or nests of large polygonal epithelioid cells and thick-walled blood vessels, with clear mitoses. Immunohistochemistry demonstrated that the epithelioid and smooth muscle cells characteristically expressed human melanoma black-45, epithelial membrane antigen and actin. Pathological evaluation revealed malignant EAML with regional lymph node metastases. Magnetic resonance imaging and X-ray examination identified multiple liver and lung nodules at 16 months post-surgery. Since the patient did not respond to the initial treatment with doxorubicin and cisplatin, sorafenib was subsequently administered. However, the treatment was not effective, and the patient succumbed to multiple metastases six months later. PMID:26870174

  6. Epithelioid angiosarcoma of the kidney: A case report and literature review.

    PubMed

    Liu, Hongyun; Huang, Xingang; Chen, Hua; Wang, Xuechun; Chen, Lei

    2014-09-01

    Epithelioid angiosarcoma (EAS) is a rare disease which presents a great diagnostic challenge. The present study reports a case of EAS in the kidney in a 75-year-old male who presented with gross hematuria. An abdominal computed tomography scan revealed space-occupying lesions of the right kidney and renal cell carcinoma was suspected. Histological examination of the resected specimens showed pleomorphic epithelioid cells with vesicular nuclei, prominent nucleoli and eosinophilic cytoplasm that lined irregular vascular spaces. Immunohistochemical staining revealed that the tumor cells were positive for AE1/AE3, cytokeratin (CK) 7, vimentin, cluster of differentiation (CD) 31 and E-cadherin, but showed no staining for CD10, CD34, factor VIII, CK20, carcinoembryonic antigen or desmin. Based on the histopathological and immunohistochemical findings, the patient was diagnosed with epithelioid angiosarcoma. Postoperative radiation therapy was administered and no recurrence was observed six months after surgery. PMID:25120677

  7. Epithelioid angiosarcoma of the adrenal gland associated with chronic arsenical intoxication?

    PubMed

    Livaditou, A; Alexiou, G; Floros, D; Filippidis, T; Dosios, T; Bays, D

    1991-03-01

    Epithelioid angiosarcoma is a rare tumor quite recently described. There is no accurate epidemiological study of this tumor. Among the internal organs, the liver is the one most frequently affected with angiosarcoma while there is no reference to the adrenal gland as a primary site. It is well known that the direct exposure to arsenicals (especially of vineyard cultivators) may be an important causative factor in the pathogenesis of the disease. A 59-year-old male vineyard cultivator with an epithelioid angiosarcoma of the right adrenal gland is described. The histologic characteristics as well as the immunohistochemical profile of the tumor are presented and the literature is briefly reviewed.

  8. Typical CT and MRI signs of hepatic epithelioid hemangioendothelioma

    PubMed Central

    GAN, LU; CHANG, RUIPING; JIN, HUALAN; YANG, LI

    2016-01-01

    To investigate the typical magnetic resonance imaging (MRI) and computed tomography (CT) features of hepatic epithelioid hemangioendothelioma (HEH), the CT and MRI findings of 14 histopathologically confirmed cases of HEH were retrospectively analyzed. Non-contrast and dynamic contrast-enhanced scans were conducted in all cases. A total of 229 lesions were detected in the 14 cases. All cases were classified as one of three types: (i) Solitary nodular type (1 case, 7%); (ii) multifocal nodular type (11 cases, 79%); or (iii) diffuse type (2 cases, 14%). The diameter of the lesions ranged from 5 to 105 mm. For the first two types (solitary and multifocal nodular types), the CT findings included low density lesions with clear margins on non-contrast scans, centripetal enhancement in arterial phase, and homogeneous enhancement in the portal venous and delay phases. The findings of non-contrast MRI scans for these two types included low signal intensity on T1-weighted images, heterogeneous high signal intensity on T2-weighted images, and heterogeneous high signal intensity on diffusion-weighted images. The lesions were predominantly located in submarginal areas. On contrast-enhanced MRI, the findings for the first two types included peripheral ring-like enhancement with a central low signal intensity (‘black target-like’ sign) and a central enhanced core surrounded by a low signal intensity halo (‘white target-like’ sign). The findings for the third HEH type (diffuse type) on CT and MRI scans included low density or heterogeneous signal intensity lesions involving regions of part or the whole liver, coalescent lesions (‘strip-like’ sign), and gradual enhancement along central vessels (‘lollipop’ sign). Collectively, these findings indicate that the ‘white target-like’ sign, ‘black target-like’ sign, ‘lollipop’ sign and ‘strip-like’ sign, in addition to capsular contraction and submarginal location, on CT and MRI imaging may have

  9. General Information about Childhood Soft Tissue Sarcoma

    MedlinePlus

    ... Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version General Information About Childhood Soft Tissue Sarcoma Go to Health ... the PDQ Pediatric Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  10. Childhood Soft Tissue Sarcoma: Treatment Information

    MedlinePlus

    ... Germ Cell Tumors Kidney/Wilms Tumor Liver Cancer Neuroblastoma Osteosarcoma Rhabdomyosarcoma Skin Cancer Soft Tissue Sarcoma Thyroid ... Tumor Liver Cancer Lymphoma (Non-Hodgkin) Lymphoma (Hodgkin) Neuroblastoma Osteosarcoma Retinoblastoma Rhabdomyosarcoma Skin Cancer Soft Tissue Sarcoma ...

  11. Determinants of Toxicity, Patterns of Failure, and Outcome Among Adult Patients With Soft Tissue Sarcomas of the Extremity and Superficial Trunk Treated With Greater Than Conventional Doses of Perioperative High-Dose-Rate Brachytherapy and External Beam Radiotherapy

    SciTech Connect

    San Miguel, Inigo; San Julian, Mikel; Cambeiro, Mauricio; Sanmamed, Miguel Fernandez; Vazquez-Garcia, Blanca; Pagola, Maria; Gaztanaga, Miren; Martin-Algarra, Salvador; Martinez-Monge, Rafael

    2011-11-15

    Purpose: The present study was undertaken to determine factors predictive of toxicity, patterns of failure, and survival in 60 adult patients with soft tissue sarcomas of the extremity and superficial trunk treated with combined perioperative high-dose-rate brachytherapy and external beam radiotherapy. Methods and Materials: The patients were treated with surgical resection and perioperative high-dose-rate brachytherapy (16 or 24 Gy) for negative and close/microscopically positive resection margins, respectively. External beam radiotherapy (45 Gy) was added postoperatively to reach a 2-Gy equivalent dose of 62.9 and 72.3 Gy, respectively. Adjuvant chemotherapy with ifosfamide and doxorubicin was given to patients with advanced high-grade tumors. Results: Grade 3 toxic events were observed in 18 patients (30%) and Grade 4 events in 6 patients (10%). No Grade 5 events were observed. A location in the lower limb was significant for Grade 3 or greater toxic events on multivariate analysis (p = .013), and the tissue volume encompassed by the 150% isodose line showed a trend toward statistical significance (p = .086). The local control, locoregional control, and distant control rate at 9 years was 77.4%, 69.5%, and 63.8%, respectively. On multivariate analysis, microscopically involved margins correlated with local control (p = .036) and locoregional control (p = .007) and tumor size correlated with distant metastases (p = .004). The 9-year disease-free survival and overall survival rate was 47.0% and 61.5%, respectively. Multivariate analysis showed poorer disease-free survival rates for patients with tumors >6 cm (p = .005) and microscopically involved margins (p = .043), and overall survival rates decreased with increasing tumor size (p = .011). Conclusions: Grade 3 or greater wound complications can probably be decreased using meticulous treatment planning to decrease the tissue volume encompassed by the 150% isodose line, especially in lower limb locations

  12. Talimogene Laherparepvec and Radiation Therapy in Treating Patients With Newly Diagnosed Soft Tissue Sarcoma That Can Be Removed by Surgery

    ClinicalTrials.gov

    2016-10-04

    Leiomyosarcoma; Liposarcoma; Sarcoma Differentiation Score 2; Sarcoma Differentiation Score 3; Stage IA Soft Tissue Sarcoma; Stage IB Soft Tissue Sarcoma; Stage IIA Soft Tissue Sarcoma; Stage IIB Soft Tissue Sarcoma; Undifferentiated Pleomorphic Sarcoma

  13. Malignant Perivascular Epithelioid Cell Neoplasm (PEComa) of the Pelvis: A Case Report.

    PubMed

    D'Andrea, David; Hanspeter, Esther; D'Elia, Carolina; Martini, Thomas; Pycha, Armin

    2016-05-01

    Perivascular epithelioid cell neoplasms (PEComa) are rare mesenchymal tumors that can occur in any part of the body and have unpredictable pathological behavior. They are usually benign, but may be malignant. We present a case of malignant PEComa of the pelvic retroperitoneum treated with radical surgery.

  14. [Unusual lung localization of histiocytic sarcoma].

    PubMed

    Aichaouia, C; Daboussi, S; Haddaoui, A; Moatemri, Z; Farah, S; Khadraoui, M; Bouzaiene, A; Cheikh, R

    2012-10-01

    Histiocytic sarcoma, proliferation araising from immunoregulatory effector system cells, is a very rare and recently recognised tumor. Diagnosis is based on immunohistochemistry and molecular genetic techniques, which allow to distinguish histiocytic sarcoma from lymphocytic proliferation, such as non-Hodgkin's. We report this rare case of multivisceral histiocytic sarcoma revealed by lung localization and for which the evolution was fatal.

  15. Granulocytic sarcoma masquerading as Ewing's sarcoma: a diagnostic dilemma.

    PubMed

    Haresh, Kunhi Parambath; Joshi, Nikhil; Gupta, Chaitali; Prabhakar, Ramachandran; Sharma, Daya Nand; Julka, Pramod Kumar; Rath, Goura Kishor

    2008-01-01

    An eleven-year-old boy presented with a swelling in his left elbow. Radiologically the features were that of an Ewing's sarcoma involving the ulna. Histopathology showed small round cell tumor strongly positive for Monoclonal Imperial Cancer research fund 2 (MIC2) antigen. Similar cells in the bone marrow were involved with MIC2 positivity. The patient developed skin lesions, which on biopsy were found to be chloromas. The initial biopsies were reevaluated with special stains revealing granulocytic sarcomas in acute myeloid leukemia masquerading as Ewing's due to its MIC2 positivity. The possibility of myeloid neoplasms should be considered routinely with known MIC2 positive round cell tumors. PMID:18923208

  16. Prognostic relevance of CCN3 in Ewing sarcoma.

    PubMed

    Perbal, Bernard; Lazar, Noureddine; Zambelli, Diana; Lopez-Guerrero, Jose Antonio; Llombart-Bosch, Antonio; Scotlandi, Katia; Picci, Piero

    2009-10-01

    Ewing sarcoma is a highly aggressive malignant bone tumor occurring preferentially in children and young adults. At present, only clinical features, such as patient age, presence of clinically evident metastases at diagnosis, and poor response to neoadjuvant chemotherapy, are widely accepted as prognostic indicators in Ewing sarcoma. In this study, we assessed the prognostic value of CCN3 (Nov), a matricellular protein that play crucial roles in bone formation. Polyclonal antibodies directed against each of the different CCN3 modules were used to identify variant CCN3 proteins in tumors and to draw potential relationships between the expression of these variants and the outcome of patients with Ewing sarcoma. Our results confirmed that expression of the full-length CCN3 in Ewing sarcoma is associated to a worse prognostic. Furthermore, we report a possible relationship between the expression of a CCN3 protein lacking an internal module (von Willebrand factor type C) and sensitivity to radiotherapy. We hypothesize that the increased level of variant CCN3 in the tumor cells reduces their tumorigenic potential and results in better outcome.

  17. From the archives of the AFIP: Pleuropulmonary synovial sarcoma.

    PubMed

    Frazier, Aletta Ann; Franks, Teri J; Pugatch, Robert D; Galvin, Jeffrey R

    2006-01-01

    Pleuropulmonary synovial sarcoma (PPSS) is increasingly recognized as a subtype of sarcoma because of the recent identification of a distinctive chromosomal translocation specific to synovial sarcoma. Soft-tissue synovial sarcoma is far more common than PPSS and typically develops in para-articular locations of the extremities; affects young and middle-aged adults, with no difference in distribution between the sexes; and has well-documented radiologic manifestations. PPSS may arise in the chest wall, heart, mediastinum, pleura, or lung, and it shares patient demographics and several imaging features with its soft-tissue counterpart. Patients present with a cough, chest pain, or dyspnea. On chest radiographs, PPSS typically appears as a sharply marginated mass with uniform opacity, based either in the pleura or in the lung, and often accompanied by an ipsilateral pleural effusion. Computed tomographic images show a well-circumscribed heterogeneously enhanced lesion without associated involvement of bone and without calcifications (except in the case of a chest wall primary tumor). Magnetic resonance imaging provides superior demonstration of nodular soft tissue and multilocular fluid-filled internal components of PPSS, in addition to peripheral rim enhancement after the intravenous administration of a gadolinium-based contrast material such as gadopentetate dimeglumine. Current treatment consists of surgical resection followed by chemotherapy, radiation therapy, or both. PMID:16702463

  18. SYNOVIAL SARCOMA OF THE LARYNX.

    PubMed

    Javed, Nabila; Iqbal, Javed

    2015-01-01

    Synovial sarcoma is a mesenchymal spindle cell tumour that displays variable epithelial differentiation. It most commonly occurs in lower extremities. Head and neck is a rare site for synovial sarcoma accounting for less than 10%. Larynx is an extremely rare site and only 16 cases with laryngeal location have been reported. Immunohistochemistry is important for correct diagnosis. Surgical excision of the tumour with clear margins and local radiotherapy is effective in local control. Chemotherapy is indicated in the presence of distant metastasis. Case of a 16 years old female is presented with hoarseness of voice and mass in supraglottic region. Lateral pharangotomy and excision of mass revealed synovial sarcoma. She had been treated with adjuvant radiotherapy in September 2012. She was fine and coming for regular follow up.

  19. Testicular myeloid sarcoma: case report

    PubMed Central

    Zago, Luzia Beatriz Ribeiro; Ladeia, Antônio Alexandre Lisbôa; Etchebehere, Renata Margarida; de Oliveira, Leonardo Rodrigues

    2013-01-01

    Myeloid sarcomas are extramedullary solid tumors composed of immature granulocytic precursor cells. In association with acute myeloid leukemia and other myeloproliferative disorders, they may arise concurrently with compromised bone marrow related to acute myeloid leukemia, as a relapsed presentation, or occur as the first manifestation. The testicles are considered to be an uncommon site for myeloid sarcomas. No therapeutic strategy has been defined as best but may include chemotherapy, radiotherapy and/or hematopoietic stem cell transplantation. This study reports the evolution of a patient with testicular myeloid sarcoma as the first manifestation of acute myeloid leukemia. The patient initially refused medical treatment and died five months after the clinical condition started. PMID:23580888

  20. CIC-rearranged Sarcomas: A Study of 20 Cases and Comparisons With Ewing Sarcomas.

    PubMed

    Yoshida, Akihiko; Goto, Keisuke; Kodaira, Makoto; Kobayashi, Eisuke; Kawamoto, Hiroshi; Mori, Taisuke; Yoshimoto, Seiichi; Endo, Otone; Kodama, Narihito; Kushima, Ryoji; Hiraoka, Nobuyoshi; Motoi, Toru; Kawai, Akira

    2016-03-01

    The CIC gene rearrangement exists in a subset of small round cell sarcomas. As the nosologic relationship of these sarcomas to Ewing sarcomas remains undetermined, we examined 20 CIC-rearranged sarcomas to compare their clinicopathologic features with those of Ewing sarcomas. The CIC-rearranged sarcomas were from a group of 14 men and 6 women with a median age of 24.5 years. The primary tumor sites included the limbs, trunk wall, internal trunk, lung, cerebrum, and pharynx. A comparison of the demographic and clinical characteristics of the 20 patients with CIC-rearranged sarcomas with those of the 53 near-consecutive patients with EWSR1-rarranged Ewing sarcomas showed that there were no differences with respect to their ages and sexes. Although none of the CIC-rearranged sarcomas arose in the bone, 40% of the Ewing sarcomas primarily affected the skeleton. The overall survival of patients with Ewing sarcomas was significantly better than that for patients with CIC-rearranged sarcomas. A histologic comparison of the CIC-rearranged sarcomas with 20 EWSR1-rearranged Ewing sarcomas showed significantly higher degrees of lobulation, nuclear pleomorphism, the prominence of the nucleoli, spindle cell elements, and myxoid changes in the CIC-rearranged sarcomas. Distinguishing immunohistochemical features included heterogenous CD99 reactivity, nuclear WT1 expression, and calretinin expression in the CIC-rearranged sarcomas and NKX2.2 expression in the Ewing sarcomas. CIC-rearranged sarcomas are distinct from Ewing sarcomas clinically, morphologically, and immunohistochemically, and they should be considered a separate entity rather than being grouped within the same family of tumors.

  1. Primary Pulmonary Ewing’s Sarcoma: Rare Cause of Superior Vena Cava Syndrome in Children

    PubMed Central

    Atwal, Swapndeep Singh; Garga, Umesh Chandra

    2014-01-01

    Ewing’s sarcoma is a common malignant bone tumour presenting in children and young adults. Rarely extra- skeletal soft tissues and visceral organs can also be the site of origin of Ewing’s sarcoma. Primary pulmonary Ewing’s sarcoma is an extremely rare malignancy which occurs in the paediatric population. We report an unusual case of primary pulmonary Ewing’s sarcoma in a nine year old girl who presented with features of superior vena cava syndrome in the emergency department. The diagnosis was confirmed pathologically both by light microscopy and immunohistochemistry. The patient was put on chemotherapy and surgery was planned but the patient expired within three days of starting chemotherapy. PMID:25302247

  2. Mast cell sarcoma: clinical management.

    PubMed

    Weiler, Catherine R; Butterfield, Joseph

    2014-05-01

    Mast cell sarcoma is a disorder that results in abnormal mast cells as identified by morphology, special stains, and in some publications, c-kit mutation analysis. It affects animal species such as canines more commonly than humans. In humans it is a very rare condition, with variable clinical presentation. There is no standard therapy for the disorder. It can affect any age group. It is occasionally associated with systemic mastocytosis and/or urticaria pigmentosa. The prognosis of mast cell sarcoma in published literature is very poor in humans.

  3. Extra osseous primary Ewing's sarcoma.

    PubMed

    Ali, Syed Asad; Muhammad, Agha Taj; Soomro, Abdul Ghani; Siddiqui, Akmal Jamal

    2010-01-01

    The case of 20 years old boy with an extra osseous Ewing's sarcoma is described. He was initially diagnosed as a case of infiltrative malignant tumour of left suprarenal gland on the basis of preoperative workup but postoperative biopsy of surgically excised specimen confirmed Extra-osseous Ewing's Sarcoma (EES) suprarenal gland with no evidence of malignancy on skeletal scintiscan, bone marrow aspirate and histopathology Suprarenal location of primary EES is unknown and probably has not been reported in literature. We report a unique case of EES.

  4. Treatment Options for Adult Soft Tissue Sarcoma

    MedlinePlus

    ... superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle ... superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle ...

  5. Treatment Option Overview (Adult Soft Tissue Sarcoma)

    MedlinePlus

    ... superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle ... superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle ...

  6. Stages of Adult Soft Tissue Sarcoma

    MedlinePlus

    ... superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle ... superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle ...

  7. Prognostic features of renal sarcomas (Review)

    PubMed Central

    ÖZTÜRK, HAKAN

    2015-01-01

    The aim of the present review was to evaluate the prognostic features of primary sarcomas of the kidney. A literature review was conducted using a number of databases, including Medline (PubMed) and Scopus, for studies published between January 1992 and December 2013. Of the studies published in English, those describing the prognostic features of primary sarcomas of the kidney were recorded. The electronic search was limited to the following keywords: Sarcoma, renal sarcoma, prognosis, diagnosis, immunohistochemistry, genetic and survey. Subsequent to the search, no review articles and/or meta-analyses associated with the prognosis of primary sarcomas of the kidney were identified. In total, 31 studies, which consisted of case studies, case series and studies concerned with the overall prognosis of urological soft-tissue sarcomas, were reviewed. Primary sarcoma of the kidney has a poor prognosis compared with other sarcomas of the urogenital system. In addition to the surgical excision of renal sarcomas, pathological, molecular and genetic prognostic factors are also considered. Due to the small number of cases, previous studies have not randomized the prognostic features of primary sarcomas of the kidney. The elucidation of the so-called ‘chaotic’ genetic and molecular basis of renal sarcomas will help to predict patient prognoses. Surgical excision is the most significant parameter for determining the prognosis of sarcomas of the kidney. However, sarcomas also exhibit prognostic features that are based upon pathological, genetic and molecular factors. The present review suggests that additional factors may be important in predicting the prognosis of patients with renal sarcomas, and that clinicians should plan treatment and follow-up regimens according to these factors. PMID:25663853

  8. Xenograft and genetically engineered mouse model systems of osteosarcoma and Ewing's sarcoma: tumor models for cancer drug discovery

    PubMed Central

    Sampson, Valerie B; Kamara, Davida F; Kolb, E Anders

    2014-01-01

    Introduction There are > 75 histological types of solid tumors that are classified into two major groups: bone and soft-tissue sarcomas. These diseases are more prevalent in children, and pediatric sarcomas tend to be highly aggressive and rapidly progressive. Sarcomas in adults may follow a more indolent course, but aggressive tumors are also common. Sarcomas that are metastatic at diagnosis, or recurrent following therapy, remain refractory to current treatment options with dismal overall survival rates. A major focus of clinical trials, for patients with sarcoma, is to identify novel and more effective therapeutic strategies targeted to genomic or proteomic aberrations specific to the malignant cells. Critical to the understanding of the potential for targeted therapies are models of disease that are representative of clinical disease and predictive of relevant clinical responses. Areas covered In this article, the authors discuss the use of mouse xenograft models and genetically engineered mice in cancer drug discovery. The authors provide a special focus on models for the two most common bone sarcomas: osteosarcoma (OS) and Ewing's sarcoma (ES). Expert opinion Predicting whether a new anticancer agent will have a positive therapeutic index in patients with OS and ES remains a challenge. The use of mouse sarcoma models for understanding the mechanisms involved in the response of tumors to new treatments is an important step in the process of drug discovery and the development of clinically relevant therapeutic strategies for these diseases. PMID:23844615

  9. Gemcitabine Hydrochloride, Docetaxel, and Radiation Therapy in Treating Patients With Uterine Sarcoma That Has Been Removed By Surgery

    ClinicalTrials.gov

    2015-01-16

    Stage IA Uterine Sarcoma; Stage IB Uterine Sarcoma; Stage IC Uterine Sarcoma; Stage IIA Uterine Sarcoma; Stage IIB Uterine Sarcoma; Stage IIIA Uterine Sarcoma; Stage IIIB Uterine Sarcoma; Stage IIIC Uterine Sarcoma; Stage IVA Uterine Sarcoma; Stage IVB Uterine Sarcoma; Uterine Corpus Leiomyosarcoma

  10. Visceral bacillary epithelioid angiomatosis: possible manifestations of disseminated cat scratch disease in the immunocompromised host: a report of two cases.

    PubMed

    Kemper, C A; Lombard, C M; Deresinski, S C; Tompkins, L S

    1990-08-01

    Opportunistic infection with the causative agent of cat scratch disease may be responsible for an unusual vascular proliferative lesion, referred to as bacillary epithelioid angiomatosis, previously described only in human immunodeficiency virus (HIV)-infected patients. We present a case of an HIV-infected patient with bacillary epithelioid angiomatosis involving the liver and bone marrow causing progressive hepatic failure. We also report a case of a cardiac transplant recipient with hepatic and splenic bacillary epithelioid angiomatosis manifesting as a fever of unknown origin, a previously unreported event in a non-HIV-infected patient. These cases represent the first documentation of bacillary epithelioid angiomatosis with visualization of cat scratch-like organisms involving internal organs.

  11. Kaposi's sarcoma of the penis.

    PubMed

    Houston, W; Pontin, A; Kuhn, T; Mambo, N

    1975-06-01

    The 11th case of Kaposi's sarcoma of the glans penis is reported. Aetiology and treatment are discussed and evidence adduced to support a conservative surgical approach by local excision, supplemented if necessary by low dosage irradiation and possibly methotrexate infusion.

  12. Molecular Approaches to Sarcoma Therapy

    PubMed Central

    Olsen, R. J.; Tarantolo, S. R.

    2002-01-01

    Soft tissue sarcomas comprise a heterogeneous group of aggressive tumors that have a relatively poor prognosis. Although conventional therapeutic regimens can effectively cytoreduce the overall tumor mass, they fail to consistently achieve a curative outcome. Alternative gene-based approaches that counteract the underlying neoplastic process by eliminating the clonal aberrations that potentiate malignant behavior have been proposed. As compared to the accumulation of gene alterations associated with epithelial carcinomas, sarcomas are frequently characterized by the unique presence of a single chromosomal translocation in each histological subtype. Similar to the Philadelphia chromosome associated with CML, these clonal abnormalities result in the fusion of two independent unrelated genes to generate a unique chimeric protein that displays aberrant activity believed to initiate cellular transformation. Secondary gene mutations may provide an additional growth advantage that further contributes to malignant progression. The recent clinical success of the tyrosine kinase inhibitor, STI571, suggests that therapeutic approaches specifically directed against essential survival factors in sarcoma cells may be effective. This review summarizes published approaches targeting a specific molecular mechanism associated with sarcomagenesis. The strategy and significance of published translational studies in six distinct areas are presented. These include: (1) the disruption of chimeric transcription factor activity; (2) inhibition of growth stimulatory post-translational modifications; (3) restoration of tumor suppressor function; (4) interference with angiogenesis; (5) induction of apoptotic pathways; and (6) introduction of toxic gene products. The potential for improving outcomes in sarcoma patients and the conceptual obstacles to be overcome are discussed. PMID:18521343

  13. Differentially Expressed miRNAs in Ewing Sarcoma Compared to Mesenchymal Stem Cells: Low miR-31 Expression with Effects on Proliferation and Invasion

    PubMed Central

    Karnuth, Bianca; Dedy, Nicolas; Spieker, Tilmann; Lawlor, Elizabeth R.; Gattenlöhner, Stefan; Ranft, Andreas; Dirksen, Uta; Jürgens, Heribert; Bräuninger, Andreas

    2014-01-01

    Ewing sarcoma, the second most common bone tumor in children and young adults, is an aggressive malignancy with a strong potential to metastasize. Ewing sarcoma is characterised by translocations encoding fusion transcription factors with an EWSR1 transactivation domain fused to an ETS family DNA binding domain. microRNAs are post-transcriptional regulators of gene expression and aberrantly expressed microRNAs have been identified as tumor suppressors or oncogenes in most cancer types. To identify potential oncogenic and tumor suppressor microRNAs in Ewing sarcoma, we determined and compared the expression of 377 microRNAs in 40 Ewing sarcoma biopsies, 6 Ewing sarcoma cell lines and mesenchymal stem cells, the putative cellular origin of Ewing sarcoma, from 6 healthy donors. Of the 35 differentially expressed microRNAs identified (fold change >4 and q<0.05), 19 were higher and 16 lower expressed in Ewing sarcoma. In comparisons between Ewing sarcoma samples with EWS-FLI or EWS-ERG translocations, with differing dissemination characteristics and of primary samples and metastases no significantly differential expressed microRNAs were detected using various stringency criteria. For miR-31, the microRNA with lowest expression in comparison to mesenchymal stem cells, functional analyses were performed to determine its potential as a tumor suppressor in Ewing sarcoma. Two of four miR-31 transfected Ewing sarcoma cell lines showed a significantly reduced proliferation (19% and 33% reduction) due to increased apoptosis in one and increased length of G1-phase in the other cell line. All three tested miR-31 transfected Ewing sarcoma cell lines showed significantly reduced invasiveness (56% to 71% reduction). In summary, we identified 35 microRNAs differentially expressed in Ewing sarcoma and demonstrate that miR-31 affects proliferation and invasion of Ewing sarcoma cell lines in ex vivo assays. PMID:24667836

  14. Sapanisertib or Pazopanib Hydrochloride in Treating Patients With Locally Advanced or Metastatic Sarcoma

    ClinicalTrials.gov

    2016-10-31

    High Grade Sarcoma; Metastatic Leiomyosarcoma; Metastatic Malignant Peripheral Nerve Sheath Tumor; Metastatic Synovial Sarcoma; Metastatic Undifferentiated Pleomorphic Sarcoma; Myxofibrosarcoma; Recurrent Leiomyosarcoma; Recurrent Malignant Peripheral Nerve Sheath Tumor; Recurrent Synovial Sarcoma; Recurrent Undifferentiated Pleomorphic Sarcoma; Uterine Corpus Leiomyosarcoma

  15. Malignant epithelioid hemangioendothelioma of the lip: a case report and comprehensive literature review.

    PubMed

    Robinson, Allen A; Tolentino, Lucilene F; Uyanne, Jettie; Melrose, Raymond; Calhoun, Colonya C

    2014-04-01

    Malignant epithelioid hemangioendothelioma (MEH), also known as high-risk epithelioid hemangioendothelioma, is a low- to intermediate-grade vascular malignancy originally described as a vascular neoplasm of endothelial origin. This very rare vascular neoplasm has been described mainly in soft tissue, but also in various organs and locations, including the liver, lung, brain, colon, lymph nodes, peritoneum, spleen, bone, skin, heart, soft tissues, and vascular system. Several cases have been described in the head and neck, including the submandibular gland, parotid gland, nasal cavity, parapharyngeal space, maxilla, maxillary sinus, occipital bone, oral cavity, thyroid gland, neck, scalp, larynx, and mandible. This case report is the first description of MEH presenting as an exophytic lower-lip lesion.

  16. EWS/FLI1 Target Genes and Therapeutic Opportunities in Ewing Sarcoma.

    PubMed

    Cidre-Aranaz, Florencia; Alonso, Javier

    2015-01-01

    Ewing sarcoma is an aggressive bone malignancy that affect children and young adults. Ewing sarcoma is the second most common primary bone malignancy in pediatric patients. Although significant progress has been made in the treatment of Ewing sarcoma since it was first described in the 1920s, in the last decade survival rates have remained unacceptably invariable, thus pointing to the need for new approaches centered in the molecular basis of the disease. Ewing sarcoma driving mutation, EWS-FLI1, which results from a chromosomal translocation, encodes an aberrant transcription factor. Since its first characterization in 1990s, many molecular targets have been described to be regulated by this chimeric transcription factor. Their contribution to orchestrate Ewing sarcoma phenotype has been reported over the last decades. In this work, we will focus on the description of a selection of EWS/FLI1 targets, their functional role, and their potential clinical relevance. We will also discuss their role in other types of cancer as well as the need for further studies to be performed in order to achieve a broader understanding of their particular contribution to Ewing sarcoma development.

  17. EWS/FLI1 Target Genes and Therapeutic Opportunities in Ewing Sarcoma

    PubMed Central

    Cidre-Aranaz, Florencia; Alonso, Javier

    2015-01-01

    Ewing sarcoma is an aggressive bone malignancy that affect children and young adults. Ewing sarcoma is the second most common primary bone malignancy in pediatric patients. Although significant progress has been made in the treatment of Ewing sarcoma since it was first described in the 1920s, in the last decade survival rates have remained unacceptably invariable, thus pointing to the need for new approaches centered in the molecular basis of the disease. Ewing sarcoma driving mutation, EWS–FLI1, which results from a chromosomal translocation, encodes an aberrant transcription factor. Since its first characterization in 1990s, many molecular targets have been described to be regulated by this chimeric transcription factor. Their contribution to orchestrate Ewing sarcoma phenotype has been reported over the last decades. In this work, we will focus on the description of a selection of EWS/FLI1 targets, their functional role, and their potential clinical relevance. We will also discuss their role in other types of cancer as well as the need for further studies to be performed in order to achieve a broader understanding of their particular contribution to Ewing sarcoma development. PMID:26258070

  18. EWS/FLI1 Target Genes and Therapeutic Opportunities in Ewing Sarcoma.

    PubMed

    Cidre-Aranaz, Florencia; Alonso, Javier

    2015-01-01

    Ewing sarcoma is an aggressive bone malignancy that affect children and young adults. Ewing sarcoma is the second most common primary bone malignancy in pediatric patients. Although significant progress has been made in the treatment of Ewing sarcoma since it was first described in the 1920s, in the last decade survival rates have remained unacceptably invariable, thus pointing to the need for new approaches centered in the molecular basis of the disease. Ewing sarcoma driving mutation, EWS-FLI1, which results from a chromosomal translocation, encodes an aberrant transcription factor. Since its first characterization in 1990s, many molecular targets have been described to be regulated by this chimeric transcription factor. Their contribution to orchestrate Ewing sarcoma phenotype has been reported over the last decades. In this work, we will focus on the description of a selection of EWS/FLI1 targets, their functional role, and their potential clinical relevance. We will also discuss their role in other types of cancer as well as the need for further studies to be performed in order to achieve a broader understanding of their particular contribution to Ewing sarcoma development. PMID:26258070

  19. Utility of characteristic 'Weak to Absent' INI1/SMARCB1/BAF47 expression in diagnosis of synovial sarcomas.

    PubMed

    Rekhi, Bharat; Vogel, Ulrich

    2015-07-01

    Recently, very few studies have shown value of immunohistochemical (IHC) expression of INI1/SMARCB1 in diagnosis of synovial sarcomas (SSs). This study was aimed at testing reproducibility and utility of this finding. Sixty-eight SSs and 147 other tumours, in the form of various biopsies, were tested for IHC expression of INI1. Twenty-six SSs were further confirmed with positive SS18 rearrangement. Forty monophasic spindle cell type (58.8%), 13 biphasic (19.1%), 12 poorly differentiated (17.6%) and three calcifying SSs (4.4%) were positive for epithelial membrane antigen (EMA) (46/62) (74.1%), pan cytokeratin (AE1/AE3) (31/47) (65.9%), cytokeratin (CK7) (20/31) (64.5%), BCL2 (62/66) (93.9%), MIC2 (61/63) (96.8%), transducin-like enhancer of split 1 (TLE1) (29/31) (93.5%) and CK19 (14/24) (58.3%). INI1 expression was 'weak to absent' in 60/68 (88.2%) SSs; in 1/3 atypical ossifying fibromyxoid tumours (AOFMTs) and in 3/10 (30%) malignant peripheral nerve sheath tumours (MPNSTs) of various types. INI1 was completely absent in 10/10 (100%) epithelioid sarcomas (ESs), 4/4 (100%) malignant rhabdoid tumours, single paediatric undifferentiated sarcoma, 5/19 (26.3%) myoepithelial carcinomas and in 2/4 (50%) epithelioid-subtype of MPNSTs. Remaining 100 tumours, including 12 Ewing sarcomas, 15 carcinomas, eight solitary fibrous tumours (SFT), seven extraskeletal myxoid chondrosarcomas, three fibrosarcomas and other tumours retained INI1 expression. A unique 'weak to absent' IHC expression of INI1 is highly sensitive (88.2%) and specific (97.3%) for a SS, irrespective of its subtypes and types of biopsies. This can be considered useful in diagnosing SSs, especially in settings lacking molecular and/or cytogenetic analysis. A similar INI1 expression is shared by certain AOFMTs and MPNSTs.

  20. Utility of characteristic 'Weak to Absent' INI1/SMARCB1/BAF47 expression in diagnosis of synovial sarcomas.

    PubMed

    Rekhi, Bharat; Vogel, Ulrich

    2015-07-01

    Recently, very few studies have shown value of immunohistochemical (IHC) expression of INI1/SMARCB1 in diagnosis of synovial sarcomas (SSs). This study was aimed at testing reproducibility and utility of this finding. Sixty-eight SSs and 147 other tumours, in the form of various biopsies, were tested for IHC expression of INI1. Twenty-six SSs were further confirmed with positive SS18 rearrangement. Forty monophasic spindle cell type (58.8%), 13 biphasic (19.1%), 12 poorly differentiated (17.6%) and three calcifying SSs (4.4%) were positive for epithelial membrane antigen (EMA) (46/62) (74.1%), pan cytokeratin (AE1/AE3) (31/47) (65.9%), cytokeratin (CK7) (20/31) (64.5%), BCL2 (62/66) (93.9%), MIC2 (61/63) (96.8%), transducin-like enhancer of split 1 (TLE1) (29/31) (93.5%) and CK19 (14/24) (58.3%). INI1 expression was 'weak to absent' in 60/68 (88.2%) SSs; in 1/3 atypical ossifying fibromyxoid tumours (AOFMTs) and in 3/10 (30%) malignant peripheral nerve sheath tumours (MPNSTs) of various types. INI1 was completely absent in 10/10 (100%) epithelioid sarcomas (ESs), 4/4 (100%) malignant rhabdoid tumours, single paediatric undifferentiated sarcoma, 5/19 (26.3%) myoepithelial carcinomas and in 2/4 (50%) epithelioid-subtype of MPNSTs. Remaining 100 tumours, including 12 Ewing sarcomas, 15 carcinomas, eight solitary fibrous tumours (SFT), seven extraskeletal myxoid chondrosarcomas, three fibrosarcomas and other tumours retained INI1 expression. A unique 'weak to absent' IHC expression of INI1 is highly sensitive (88.2%) and specific (97.3%) for a SS, irrespective of its subtypes and types of biopsies. This can be considered useful in diagnosing SSs, especially in settings lacking molecular and/or cytogenetic analysis. A similar INI1 expression is shared by certain AOFMTs and MPNSTs. PMID:25912315

  1. What's New in Soft Tissue Sarcomas Research and Treatment?

    MedlinePlus

    ... Topic Additional resources for soft tissue sarcoma What`s new in soft tissue sarcoma research and treatment? Research ... develop. This information is already being applied to new tests to diagnose and classify sarcomas. This is ...

  2. A 3-dimensional in vitro model of epithelioid granulomas induced by high aspect ratio nanomaterials

    PubMed Central

    2011-01-01

    Background The most common causes of granulomatous inflammation are persistent pathogens and poorly-degradable irritating materials. A characteristic pathological reaction to intratracheal instillation, pharyngeal aspiration, or inhalation of carbon nanotubes is formation of epithelioid granulomas accompanied by interstitial fibrosis in the lungs. In the mesothelium, a similar response is induced by high aspect ratio nanomaterials, including asbestos fibers, following intraperitoneal injection. This asbestos-like behaviour of some engineered nanomaterials is a concern for their potential adverse health effects in the lungs and mesothelium. We hypothesize that high aspect ratio nanomaterials will induce epithelioid granulomas in nonadherent macrophages in 3D cultures. Results Carbon black particles (Printex 90) and crocidolite asbestos fibers were used as well-characterized reference materials and compared with three commercial samples of multiwalled carbon nanotubes (MWCNTs). Doses were identified in 2D and 3D cultures in order to minimize acute toxicity and to reflect realistic occupational exposures in humans and in previous inhalation studies in rodents. Under serum-free conditions, exposure of nonadherent primary murine bone marrow-derived macrophages to 0.5 μg/ml (0.38 μg/cm2) of crocidolite asbestos fibers or MWCNTs, but not carbon black, induced macrophage differentiation into epithelioid cells and formation of stable aggregates with the characteristic morphology of granulomas. Formation of multinucleated giant cells was also induced by asbestos fibers or MWCNTs in this 3D in vitro model. After 7-14 days, macrophages exposed to high aspect ratio nanomaterials co-expressed proinflammatory (M1) as well as profibrotic (M2) phenotypic markers. Conclusions Induction of epithelioid granulomas appears to correlate with high aspect ratio and complex 3D structure of carbon nanotubes, not with their iron content or surface area. This model offers a time- and cost

  3. A Histomorphologic Grading System That Predicts Overall Survival in Diffuse Malignant Peritoneal Mesothelioma With Epithelioid Subtype

    PubMed Central

    Blackham, Aaron U.; Levine, Edward; Russell, Greg; Votanopoulos, Konstantinos I.; Stewart, John H.; Shen, Perry; Geisinger, Kim R.; Sirintrapun, Sahussapont J.

    2016-01-01

    Diffuse malignant peritoneal mesothelioma (MPeM) is rare and arises from peritoneal serosal surfaces. Although it shares similar histomorphology with its counterpart, malignant pleural mesothelioma, etiologies, clinical courses, and therapies differ. Nuclear grading and level of mitoses have been correlated with prognosis in malignant pleural mesothelioma with epithelioid subtype. Whether nuclear grading and level of mitoses correlate with prognosis in MPeM is still unknown. Our study utilizes a 2 tier system incorporating nuclear features and level of the mitoses to stratify cases of MPeM with epithelioid subtype. Fifty-one cases of MPeM with clinical follow-up underwent retrospective microscopic review. From that subset, 46 cases were of epithelioid subtype, which were then stratified into a low-grade or high-grade tier. Survival times were calculated on the basis of Kaplan-Meier analysis. The low-grade tier had higher overall survival with a median of 11.9 years and 57% at 5 years when compared with the high-grade tier with a median of 3.3 years and 21% at 5 years (P=0.002). Although not statistically significant, the low-grade tier had higher progression-free survival with a median of 4.7 years and 65% at 5 years when compared with the high-grade tier with a median of 1.9 years and 35% at 5 years (P=0.089). Our study is first to specifically evaluate and correlate nuclear features and level of mitoses with overall survival in MPeM with epithelioid subtype. PMID:27438989

  4. Primary Pulmonary Epithelioid Hemangioendothelioma: A Rare Cause of PET-Negative Pulmonary Nodules

    PubMed Central

    Cazzuffi, Riccardo; Calia, Nunzio; Ravenna, Franco; Pasquini, Claudio; Saturni, Sara; Cavallesco, Giorgio Narciso; Quarantotto, Francesco; Rinaldi, Rosa; Cogo, Annaluisa; Caramori, Gaetano; Papi, Alberto

    2011-01-01

    We report here a case of primary pulmonary epithelioid hemangioendothelioma diagnosed in a 67-year-old Caucasian man, presenting with exertion dyspnoea, dry cough, and multiple bilateral pulmonary nodules revealed by computed tomography. At the 18F-fluorodeoxyglucose positron emission tomography, these nodules were negative. The histopathological diagnosis was made on a pulmonary wedge resection (performed during video-thoracoscopic surgery). PMID:21869893

  5. The lollipop sign: a new cross-sectional sign of hepatic epithelioid hemangioendothelioma.

    PubMed

    Alomari, Ahmad I

    2006-09-01

    In four cases of hepatic epithelioid hemangioendothelioma (HEHE), multiple liver lesions were depicted on enhanced computed tomography (CT) and/or magnetic resonance imaging (MRI) scans. A peculiar sign, a hepatic or portal vein terminating at or just within the periphery of some of the liver lesions, was noted in all four; the images look like a lollipop. Imaging findings of HEHE can suggest other diseases, but this sign buttressed by other radiological findings, seems to be specific for this entity. PMID:16644166

  6. Combination capecitabine and bevacizumab in the treatment of metastatic hepatic epithelioid hemangioendothelioma.

    PubMed

    Lau, Augustine; Malangone, Steve; Green, Myke; Badari, Ambuga; Clarke, Kathryn; Elquza, Emad

    2015-07-01

    Hepatic epithelioid hemangioendothelioma (HEHE) is a rare, often misdiagnosed vascular neoplasm with clinical behaviors that range from indolent to highly aggressive. Even when the appropriate diagnosis is achieved, the best treatment for HEHE has not been defined or standardized, further complicating the care of these patients. We present a diagnostically challenging case of HEHE where we utilized capecitabine and bevacizumab as another novel treatment option. PMID:26136854

  7. Cutaneous perivascular epithelioid cell tumors: A review on an infrequent neoplasm

    PubMed Central

    Llamas-Velasco, Mar; Requena, Luis; Mentzel, Thomas

    2016-01-01

    “Perivascular epithelioid cutaneous” cell tumors (PEComa) are a family of mesenchymal tumors with shared microscopic and immunohistochemical properties: They exhibit both smooth muscle cell and melanocytic differentiation. Non-neoplastic counterpart of PEComa’s cells are unknown, as well as the relationship between extracutaneous PEComa and primary cutaneous ones. We will review the clinical setting, histopathologic features, chromosomal abnormalities, differential diagnosis and treatment options for cutaneous PEComa. PMID:27019799

  8. Combination capecitabine and bevacizumab in the treatment of metastatic hepatic epithelioid hemangioendothelioma

    PubMed Central

    Malangone, Steve; Green, Myke; Badari, Ambuga; Clarke, Kathryn; Elquza, Emad

    2015-01-01

    Hepatic epithelioid hemangioendothelioma (HEHE) is a rare, often misdiagnosed vascular neoplasm with clinical behaviors that range from indolent to highly aggressive. Even when the appropriate diagnosis is achieved, the best treatment for HEHE has not been defined or standardized, further complicating the care of these patients. We present a diagnostically challenging case of HEHE where we utilized capecitabine and bevacizumab as another novel treatment option. PMID:26136854

  9. Cathepsin K expression in the spectrum of perivascular epithelioid cell (PEC) lesions of the kidney.

    PubMed

    Martignoni, Guido; Bonetti, Franco; Chilosi, Marco; Brunelli, Matteo; Segala, Diego; Amin, Mahul B; Argani, Pedram; Eble, John N; Gobbo, Stefano; Pea, Maurizio

    2012-01-01

    The perivascular epithelioid cell (PEC) is a unique cell type coexpressing contractile proteins (mainly α-smooth muscle actin), melanocytic markers, including microphthalmia-associated transcription factor (MITF), and estrogen and progesterone receptors. It is constantly present in a group of tumors called PEComas. Renal PEComas include the common angiomyolipoma as well as less common lesions such as microscopic angiomyolipoma, intraglomerular lesions, angiomyolipoma with epithelial cysts, epithelioid angiomyolipoma, oncocytoma-like angiomyolipoma and lymphangioleiomyomatosis of the renal sinus. It has been demonstrated that most of these lesions are determined by mutations affecting genes of the tuberous sclerosis complex, tuberous sclerosis 1 (TSC1) and tuberous sclerosis 2 (TSC2), with eventual deregulation of the RHEB/MTOR/RPS6KB2 pathway, and it has been observed that some PEComas regressed during sirolimus therapy, an MTOR inhibitor. Recently, overexpression of MITF has been related to the expression of the papain-like cysteine protease cathepsin K in osteoclasts where it has inhibited MTOR. The aim of this study is to evaluate cathepsin K immunohistochemically in the entire spectrum of PEComa lesions in the kidney. The study population consisted of 84 renal PEComa lesions, including 5 composed predominantly of fat (lipoma-like angiomyolipoma), 15 almost exclusively composed of spindle-shaped smooth muscle cells (leiomyoma-like angiomyolipoma) and 31 common angiomyolipomas composed of a mixture of fat, spindle and epithelioid smooth muscle cells, and abnormal thick-walled blood vessels, 15 microscopic angiomyolipomas, 5 intraglomerular lesions, 2 oncocytoma-like angiomyolipomas, 8 epithelioid angiomyolipomas, 2 angiomyolipomas with epithelial cysts and 1 example of lymphangioleiomyomatosis of the renal sinus. In all of the renal PEComas, cathepsin K was found to be constantly and strongly expressed and seems to be a more powerful marker than other commonly

  10. Combination capecitabine and bevacizumab in the treatment of metastatic hepatic epithelioid hemangioendothelioma.

    PubMed

    Lau, Augustine; Malangone, Steve; Green, Myke; Badari, Ambuga; Clarke, Kathryn; Elquza, Emad

    2015-07-01

    Hepatic epithelioid hemangioendothelioma (HEHE) is a rare, often misdiagnosed vascular neoplasm with clinical behaviors that range from indolent to highly aggressive. Even when the appropriate diagnosis is achieved, the best treatment for HEHE has not been defined or standardized, further complicating the care of these patients. We present a diagnostically challenging case of HEHE where we utilized capecitabine and bevacizumab as another novel treatment option.

  11. Improving recognition of hepatic perivascular epithelioid cell tumor: Case report and literature review

    PubMed Central

    Maebayashi, Toshiya; Abe, Katsumi; Aizawa, Takuya; Sakaguchi, Masakuni; Ishibashi, Naoya; Abe, Osamu; Takayama, Tadatoshi; Nakayama, Hisashi; Matsuoka, Shunichi; Nirei, Kazushige; Nakamura, Hitomi; Ogawa, Masahiro; Sugitani, Masahiko

    2015-01-01

    A 58-year-old man presented with the chief complaint of abdominal bloating and was incidentally found to have a liver tumor. As diagnostic imaging studies could not rule out malignancy, the patient underwent partial resection of segment 3 of the liver. The lesion pathologically showed eosinophilic proliferation, in addition to immunohistochemical positivity for human melanoma black 45 and Melan-A, thereby leading to the diagnosis of a hepatic perivascular epithelioid cell tumor (PEComa). A PEComa arising from the liver is relatively rare. Moreover, the name ‘PEComa’ has not yet been widely recognized, and the same disease entity has been called epithelioid angiomyolipoma (EAML), further diminishing the recognition of PEComa. In addition, PEComa imaging findings mimic those of malignant liver tumors, and clinically, this tumor tends to enlarge. Therefore, a PEComa is difficult to diagnose. We conducted a systematic review of PEComa and EAML cases and discuss the results, including findings useful for differentiating perivascular epithelioid cell tumors from malignant liver tumors. PMID:25954119

  12. Imaging features of soft tissue epithelioid angiosarcoma in the lower extremity: A case report

    PubMed Central

    LV, LULU; XU, PENG; SHI, YIBING; HAO, JINGMING; HU, CHUNAI; ZHAO, BAOZHONG

    2016-01-01

    Epithelioid angiosarcomas are extremely rare malignant tumors formed from endothelial cells. The majority of studies reporting these tumors have been concerned with the clinical and pathological aspects, with limited reporting of radiological diagnosis. The aim of the present study was to provide a reference to improve understanding of diagnosis, treatment choice and prognosis assessment of epithelioid angiosarcoma. The current study reports the case of a 44-year-old woman with epithelioid angiosarcoma located in the deep soft tissue of the lower extremities. Physical examination of the right thigh revealed a palpable hard mass and movement was clearly restricted and painful. X-ray, computed tomography (CT) scans and magnetic resonance imaging (MRI) were used to evaluate the imaging features of the tumor. Using X-ray and CT scanning, an inhomogenous tumor with osteolytic osseous destruction was observed. MRI revealed that the bordering skeletal muscles were infiltrated. The patient was treated with palliative surgery and chemotherapy, but succumbed to disease 1 year later. PMID:27123135

  13. Diagnosis and Management of Hereditary Sarcoma.

    PubMed

    Thomas, David M; Ballinger, Mandy L

    2016-01-01

    Sarcomas are rare and heterogeneous diseases that affect a younger population than most epithelial cancers. Epidemiologic studies suggest a strong genetic component to sarcomas, and many familial cancer syndromes have been described, in which sarcomas are a feature. The best known of these are the Li-Fraumeni and retinoblastoma syndromes, study of which has been pivotal to elucidating the molecular basis for the cell response to DNA damage and the cell division. Although much has been learnt about cancer biology from the study of sarcoma families, in general clinical management of increased sarcoma risk has lagged behind other cancer predisposition syndromes. With the advent of genomic tools for genetic testing, it is likely that a substantial fraction of sarcoma patients will be identified as carriers of known risk alleles. The translation of this knowledge into effective risk management programs and cancer treatments will be essential to changes in routine clinical practice. PMID:27075354

  14. Radiation-induced sarcoma of the thyroid

    SciTech Connect

    Griem, K.L.; Robb, P.K.; Caldarelli, D.D.; Templeton, A.C. )

    1989-08-01

    A 23-year-old white man presented with a thyroid mass 12 years after receiving high-dose radiotherapy for a T2 and N1 lymphoepithelioma of the nasopharynx. Following subtotal thyroidectomy, a histopathologic examination revealed liposarcoma of the thyroid gland. The relationship between sarcomas and irradiation is described and Cahan and colleagues' criteria for radiation-induced sarcomas are reviewed. To our knowledge, we are presenting the first such case of a radiation-induced sarcoma of the thyroid gland.

  15. [Breast hematoma masking a sarcoma].

    PubMed

    Relea Sarabia, A; Bahamonde Cabria, S; González Rebollo, M; Encinas Gaspar, M B

    2015-01-01

    Sarcomas account for less than 1% of malignant breast tumors. We present the case of a sarcoma (malignant fibrous histiocytoma) of the breast that debuted as a lump. The patient associated the lump with trauma, and the clinical, sonographic, and cytological findings were suggestive of a hematoma. The lump grew rapidly, and the follow-up sonogram one month later clearly revealed hypervascular solid poles. This atypical presentation is useful for reviewing the management of lesions suggestive of hematomas, which are often associated with nonspecific findings and occasionally with signs that raise suspicion of malignancy. The emergence of what appears to be a hematoma in the breast, even when accompanied by a history of trauma or a tendency toward bleeding, calls for prudence: very short-term follow-up and biopsy, even excisional biopsy, are recommended if the lesion does not evolve like a hematoma. PMID:26160042

  16. Ewing's sarcoma of proximal humeral epiphysis.

    PubMed

    Esmaili, Heydar Ali; Niknejad, Mohammad Taghi; Mohajeri, Shiva

    2015-02-01

    Ewing's sarcoma is one of the most common primary bone tumors of childhood. The tumor is almost always metaphyseal or diaphyseal, within long bones. In children, lesions of the epiphysis are often benign, with the most common diagnosis being chondroblastoma. Rarely, 1%-2% of Ewing sarcomas may involve epiphysis. We present a case of Ewing's sarcoma of the proximal humeral epiphysis in a 13-year-old boy. This case adds to previously reported cases of epiphyseal Ewing sarcoma and suggests that the diagnosis should be considered for pediatric epiphyseal lesions. PMID:25644805

  17. Ewing sarcoma: a chronicle of molecular pathogenesis.

    PubMed

    Kim, Sang Kyum; Park, Yong-Koo

    2016-09-01

    Sarcomas have traditionally been classified according to their chromosomal alterations regardless of whether they accompany simple or complex genetic changes. Ewing sarcoma, a classic small round cell bone tumor, is a well-known mesenchymal malignancy that results from simple sarcoma-specific genetic alterations. The genetic alterations are translocations between genes of the TET/FET family (TLS/FUS, EWSR1, and TAF15) and genes of the E26 transformation-specific (ETS) family. In this review, we intend to summarize a chronicle of molecular findings of Ewing sarcoma including recent advances and explain resultant molecular pathogenesis. PMID:27246176

  18. Primary pleuropulmonary synovial sarcoma: a case report.

    PubMed

    Yuan, Lianfang; Guan, Zhiyu; Dai, Xuan; Xu, Jie

    2015-01-01

    Pleuropulmonary synovial sarcoma (PPSS) is an extremely rare malignant tumor, which is increasingly recognized as a subtype of sarcoma with a distinctive chromosomal translocation specific to synovial sarcoma. It is often presents like any thoracic tumor with symptoms such as chest pain or cough. Here we report a case of PPSS in a 49-year-old woman presenting with cough, shortness of breath and chest pain. And who were found upon histologic examination of the resection specimen to have cystic primary pleuropulmonary synovial sarcoma. PMID:26823907

  19. [Molecular biology for sarcoma: useful or necessary?].

    PubMed

    Neuville, Agnès; Coindre, Jean-Michel; Chibon, Frédéric

    2015-01-01

    Sarcomas are a heterogeneous group of tumors. Their diagnosis is based on morphology and immunohistochemical profile, with categories of tumors according to the type of tissue that they resemble. Nevertheless, for several tumors, cellular origin is unknown. Molecular analysis performed in recent years allowed, combining histophenotype and genomics, better classifying such sarcomas, individualizing new entities and grouping some tumors. Simple and recurrent genetic alterations, such as translocation, mutation, amplification, can be identified in one of two sarcomas and appear as new diagnostic markers. Their identification in specialized laboratories in molecular pathology of sarcomas is often useful and sometimes necessary for a good diagnosis, leading to a heavy and multidisciplinary multi-step treatment.

  20. Synovial Sarcoma Mimicking Myositis Ossificans

    PubMed Central

    Erkut, Adem; Guvercin, Yılmaz; Bedir, Recep

    2016-01-01

    A calcification mass was incidentally found in the soft tissue of a patient who had a history of trauma to the extremity during examination. The patient had no symptom. The pathological analysis of the mass revealed it was an early-phase synovial sarcoma (SS). The diagnosis was made before the onset of symptoms and proper surgical intervention was performed. Therefore, in case of a <1 cm lesion clinically suspicious of myositis ossificans, SS should be taken into consideration as a possible diagnosis.

  1. Renal angiomyolipoma: clinicopathologic study of 194 cases with emphasis on the epithelioid histology and tuberous sclerosis association.

    PubMed

    Aydin, Hakan; Magi-Galluzzi, Cristina; Lane, Brian R; Sercia, Linda; Lopez, Jose I; Rini, Brian I; Zhou, Ming

    2009-02-01

    The majority of renal angiomyolipoma (AML) is sporadic and occasionally it occurs as part of tuberous sclerosis complex (TSC). Epithelioid AML (EAML), an uncommon variant, is considered potentially malignant based on anecdotal case reports. The prognostic significance of epithelioid component in an otherwise typical AML is uncertain. We studied 194 AMLs for the clinicopathologic features of epithelioid and TSC-associated AMLs. Epithelioid component was present in 15 cases (7.7%) with an average amount of 51% (range: 10% to 100%). Histologically, the epithelioid tumor cells were categorized into small, intermediate, and large cell type based on the cell size. Worrisome histologic features were seen in many EAMLs, including coagulative tumor necrosis in 27% (4/15), nuclear atypia in 93% (14/15), mitosis in 47% (7/15), and atypical mitosis in 1 case. All 15 EAML patients had a mean follow-up time of 5.1 years and none had local recurrence or distant metastasis. Sixteen (8.2%) AMLs occurred in patients with definitive TSC. Three histologic features, namely microscopic AML foci, epithelioid component, and epithelial cysts, were present in 10 (62.5%), 4 (25%), and 44% (7/16), respectively, of TSC-associated AMLs, compared with 11 (6.2%), 11 (6.2%), and 6 (3.4%), respectively, in non-TSC-associated AMLs (P value all <0.01). In summary, all 15 cases of EAMLs in our study had benign clinical outcomes despite adverse pathologic features. Epithelioid component, epithelial cysts, and microscopic AML foci are strongly associated with TSC and the presence of all 3 features should raise strong suspicion for TSC.

  2. Of Mice and Men: Opportunities to Use Genetically Engineered Mouse Models of Synovial Sarcoma for Preclinical Cancer Therapeutic Evaluation

    PubMed Central

    Jones, Kevin B.; Haldar, Malay; Schiffman, Joshua D.; Cannon-Albright, Lisa; Lessnick, Stephen L.; Sharma, Sunil; Capecchi, Mario R.; Randall, R. Lor

    2016-01-01

    Background Synovial sarcoma is a soft tissue malignancy with a predilection for adolescents and young adults. Despite recent improvements in the understanding of its character and etiology, few therapeutic advances have been made. The mortality rate is high among the young population it affects. The low incidence of most subtypes of sarcoma, such as synovial sarcoma, makes disease-specific trials difficult to organize. The biological differences between sarcoma subtypes make inclusion of multiple types in general trials unsatisfactory as well. Methods A review of the literature regarding targetable pathways in synovial sarcoma was undertaken. A strategy has been devised to utilize available technologies in order to prioritize drug trial planning. Results Cell culture and xenograft research with synovial sarcoma cell lines have identified some critical pathways that may be targetable. Promising therapeutic strategies include newer cytotoxic chemotherapies, antiangiogenic agents, anti-IGF1R pathway agents, anti-Bcl-2/proapoptotic agents, and histone deacetylase complex inhibitors. Conclusions We propose to prioritize potential therapeutic strategies via preclinical testing of agents in a genetic mouse model of synovial sarcoma. Preclinical optimization of treatment regimens can guide the development of more focused patient trials. PMID:21666582

  3. Common antigenic determinants on human melanoma, glioma, neuroblastoma, and sarcoma cells defined with monoclonal antibodies.

    PubMed

    Seeger, R C; Rosenblatt, H M; Imai, K; Ferrone, S

    1981-07-01

    Antigenic determinants that are common to melanomas, gliomas, neuroblastomas, and sarcomas but that are minimally or not detectably expressed by adult tissues were defined with monoclonal antibodies. Quantitative absorption of monoclonal antibody (Ab 165) with adult tissues followed by testing on antigen-positive UCLA-SO-M14 melanoma cells did not demonstrate antigenic determinant (Ag 165) in brain, lung, liver, kidney, intestine, adrenal, and muscle, Absorption of Ab 376 demonstrated Ag 376 in adult lung but minimal or no antigen in other tissues. Both antigens were associated with a variety of fetal tissues. Assessment of 28 human tumor cell lines with the 131I-staphylococcal Protein A-binding test demonstrated that Ab 165 reacted strongly with melanomas and gliomas and weakly with sarcomas. Ab 376 reacted strongly with melanomas, gliomas, neuroblastomas, and sarcomas. Neither of these antibodies reacted appreciably with carcinoma or teratoma cell lines. Absorption of Ab 165 and Ab 376 with noncultured tumors demonstrated that melanomas, sarcomas, and neuroblastomas can have greater quantities of these antigens in vivo than do normal adult tissues. Qualitative and quantitative antigenic heterogeneity within positive classes of tumors was demonstrated for both cultured and noncultured tumors. The differences in antigen expression in vivo between normal and neoplastic cells suggest potential value for these antibodies in immunodiagnosis and possibly immunotherapy.

  4. Differences in Activities of Daily Living Performance Between Long-Term Pediatric Sarcoma Survivors and a Matched Comparison Group on Standardized Testing

    PubMed Central

    Parks, Rebecca; Rasch, Elizabeth K.; Mansky, Patrick J.; Oakley, Frances

    2009-01-01

    BACKGROUND: In a cross-sectional study examining late effects of pediatric sarcoma therapy, long-term survivors were evaluated on their activities of daily living (ADL) performance. PROCEDURE: Thirty-two persons with Ewing sarcoma family of tumors, rhabdomyosarcoma, and non-rhabdomysarcoma-soft tissue sarcoma enrolled an average of 17 years after treatment. Participants were evaluated using the Assessment of Motor and Process Skills (AMPS) [1], a standardized observational evaluation of ADL task performance. Means and 95% confidence intervals for ADL motor and ADL process ability measures were calculated for four groups: 1) sarcoma survivors, 2) “well” adults matched for age and gender, 3) “well” adults matched for gender that were 10 years older; and 4) “well” adults matched for gender that were 20 years older. RESULTS: ADL motor ability was significantly lower for sarcoma survivors than for the age and gender matched comparison group (p<0.05). There was no significant difference between ADL motor ability of sarcoma survivors and the comparison group 10 years older, but sarcoma survivors had significantly better ADL motor ability (p<0.05) than the oldest comparison group (20 years older). Sarcoma survivors had significantly worse ADL process ability than the age matched group (p<0.05). There was no difference in ADL process ability between the sarcoma survivors and comparison groups that were 10 and 20 years older. CONCLUSIONS: This first report of a clinical evaluation of ADL limitation in pediatric sarcoma survivors treated with intensive multimodal cancer therapy suggests that influences on performance of daily life activities are more common than previously reported. PMID:19533662

  5. A case of osteoclast-like giant cell-rich epithelioid glioblastoma with BRAF V600E mutation.

    PubMed

    Funata, Nobuaki; Nobusawa, Sumihito; Yamada, Ryoji; Shinoura, Nobusada

    2016-01-01

    Epithelioid glioblastomas (E-GBMs) are rare, highly aggressive tumors consisting of closely packed tumor cells with smooth, round cell borders and abundant eosinophilic cytoplasm. They tend to affect younger patients compared with conventional GBM. BRAF V600E mutation is characteristically found in approximately 50% of all E-GBMs, compared with a low frequency of this mutation in conventional GBM. Here, we report an unusual case of glioma involving the right frontal lobe, basal ganglia and thalamus in an HIV-positive 30-year-old man on antiretroviral therapy. The lesion was composed of abundant discohesive, monotonous epithelioid cells with extensive necrosis, spindle and polyhedral cells, low-grade oligoastrocytoma-like areas, sarcomatous components, and numerous osteoclast-like giant cells (OLGCs) intermingled with epithelioid tumor cells. As the epithelioid cells accounted for more than one-third of the tumor, a pathological diagnosis of E-GBM was made. BRAF V600E mutation was detected in both oligoastrocytoma-like and epithelioid cell components. Similar to previously reported findings on E-GBM associated with low-grade glioma, this case suggested that low-grade astrocytic glioma with BRAF V600E mutation progressed to E-GBM. OLGCs are rarely observed in gliomas, and this is the first case report of E-GBM associated with abundant OLGC infiltration.

  6. Targeting epigenetic misregulation in synovial sarcoma.

    PubMed

    Waterfall, Joshua J; Meltzer, Paul S

    2012-03-20

    Like many sarcomas, synovial sarcoma is driven by a characteristic oncogenic transcription factor fusion, SS18-SSX. In this issue of Cancer Cell, Su et al. elucidate the protein partners necessary for target gene misregulation and demonstrate a direct effect of histone deacetylase inhibitors on the SS18-SSX complex composition, expression misregulation, and apoptosis.

  7. Ewing Sarcoma in the Right Ventricle

    PubMed Central

    Zhao, Bihong; Thangam, Manoj; Loyalka, Pranav; Buja, L. Maximilian; Kar, Biswajit; Gregoric, Igor D.

    2016-01-01

    Ewing sarcoma is the second most prevalent malignant primary bone tumor but constitutes only a small proportion of cardiac metastases. We present a case of asymptomatic Ewing sarcoma metastatic to the right ventricle. A 36-year-old man presented for evaluation and resection of a pedunculated right ventricular cardiac tumor. Three years before, he had been diagnosed with translocation-negative Ewing sarcoma, for which he had undergone chemotherapy and amputation of the left leg below the knee. We resected the right ventricular tumor. Analysis of the resected mass supported the diagnosis of metastatic Ewing sarcoma. Postoperative transthoracic echocardiograms showed normal biventricular size and function. One year later, the patient had no recurrence of the sarcoma. In addition to discussing this case, we review the relevant medical literature. PMID:27777536

  8. [Alveolar sarcoma. Report of a case].

    PubMed

    Devisme, L; Mensier, E; Bisiau, S; Bloget, F; Gosselin, B

    1996-01-01

    Alveolar soft part sarcoma occurs mostly in the deep soft tissues. An unusual case of primary pulmonary alveolar soft part sarcoma is reported. A 39-year-old woman presented with thoracic pain revealing the tumor. The left lower lobe was surgically resected. The microscopic features of this tumor, including characteristic alveolar pattern and the PAS-positive crystals were typical of alveolar soft part sarcoma. Immunohistochemically, granular cytoplasmic reactivities were observed with antibodies against vimentin, myoglobin, methionine-enkephalin, S100 protein and neuron-specific-enolase. Electron microscopic study demonstrated numerous crystallized structures in the tumor cell cytoplasm. This is the third case of pulmonary alveolar soft part sarcoma, one arising from the pulmonary vein. The histogenesis of alveolar soft part sarcoma is still debated. Our case does not allow distinction between myogenic or neural origin of this tumor.

  9. Monophasic Synovial Sarcoma of Prostatic Fascia: Case Report and Literature Review

    PubMed Central

    Benecchi, Luigi; Corti, Serena; Del Boca, Carlo; Ferrari, Matteo; Sergio, Pietro; Bercich, Luisa; Tanzi, Giulia

    2015-01-01

    Synovial sarcoma (SS) primarily occurs in the para-articular soft tissue of the lower extremities in young adults and it is extremely rare in the prostatic region. We report a case of a 46-year-old man who presented with urinary retention. Pelvic ultrasound (US) examination, computed tomography (CT), and magnetic resonance imaging (MRI) demonstrated an 8.5 cm mass that appeared to originate in the prostatic fascia of the right lobe. Preoperative prostatic ultrasound transrectal needle biopsy revealed mesenchymal neoplastic tissue. Patient underwent surgery. The final pathologic findings were consistent with the diagnosis of monophasic synovial sarcoma. PMID:26075135

  10. Extraskeletal Ewing’s sarcoma in a great toe of a young boy

    PubMed Central

    Cypel, Tatiana Karine Simon; Meilik, Benjamin; Zuker, Ronald Melvin

    2007-01-01

    Extraskeletal Ewing’s sarcoma (EES) is a rare, soft tissue, malignant neoplasm histologically similar to skeletal Ewing’s sarcoma. It occurs mainly in adolescents and young adults, and affects extremities in 36% of cases and central locations (commonly paravertebral regions) in the remainder. The differential diagnosis includes other small, blue, round cell tumours. A clinical case of EES involving a great toe in a young boy is reported. EES diagnosis was confirmed by features of histological analysis and immunohistochemistry, and by the presence of the t(11;22) chromosomal translocation. PMID:19554151

  11. Mediastinal epithelioid hemangioendothelioma with abundant spindle cells and osteoclast-like giant cells mimicking malignant fibrous histiocytoma

    PubMed Central

    2013-01-01

    Abstract Epithelioid hemangioendothelioma is a relatively uncommon lesion usually presenting in soft tissues. The occurrence in the mediastinum is exceptional rare. Histologically, this tumor is characterized by epithelioid cells with intracytoplasmic vacuoles in a hyalinized or mucinous stroma. Occasionally, spindle cells or osteoclast-like giant cells can be observed. Herein, we present a case of epithelioid hemangioendothelioma in a 38 year-old Chinese male. The tumor was predominantly composed of abundant spindle cells with marked atypia and scattered osteoclast-like giant cells reminiscent of malignant fibrous histiocytoma. The unusual histological appearance can pose a great diagnostic challenge. It may be easily misdiagnosed, especially if the specimen is limited or from fine-needle aspiration. Virtual slides http://www.diagnosticpathology.diagnomx.eu/vs/5804918529726307 PMID:23800015

  12. Renal epithelioid angiomyolipoma with epithelial cysts: demonstration of Melan A and HMB45 positivity in the cystic epithelial lining.

    PubMed

    Filho, José do Egypto Pereira; Meneses de Amorim, Daniele; Sweet, Gloria Maria Maranhão; Rodrigues de Freitas, Luiz Antonio; Athanazio, Paulo Roberto Fontes; Athanazio, Daniel Abensur

    2012-10-01

    Renal angiomyolipoma (AML) may present as rare variants such as epithelioid and AML with epithelial cysts posing difficulties for the diagnosis to the surgical pathologist. We report a case of a 46-year-old male patient presenting a 5-cm solid tumor in the lower pole of the left kidney, with cystic changes at cut surface. The tumor exhibited 95% of epithelioid cells with atypical nuclei. A small focus of typical AML was observed. The immunoprofile of tumor cells was classical of AML including expression of melanocytic markers such as HMB45 and Melan A. We report the immunohistochemical study of the cystic component in an epithelioid AML. In contrast to the immunoreactivity reported in typical AML, the present case shows obvious expression of melanocytic markers in the cystic epithelial lining. This is strong evidence that these cysts are neoplastic and derived from AML, rather than entrapped native collecting duct epithelium.

  13. Kaposi sarcoma in unusual locations

    PubMed Central

    Pantanowitz, Liron; Dezube, Bruce J

    2008-01-01

    Kaposi sarcoma (KS) is a multifocal, vascular lesion of low-grade malignant potential that presents most frequently in mucocutaneous sites. KS also commonly involves lymph nodes and visceral organs. This article deals with the manifestation of KS in unusual anatomic regions. Unusual locations of KS involvement include the musculoskeletal system, central and peripheral nervous system, larynx, eye, major salivary glands, endocrine organs, heart, thoracic duct, urinary system and breast. The development of KS within wounds and blood clots is also presented. KS in these atypical sites may prove difficult to diagnose, resulting in patient mismanagement. Theories to explain the rarity and development of KS in these unusual sites are discussed. PMID:18605999

  14. Synovial Sarcoma Mimicking Myositis Ossificans

    PubMed Central

    Erkut, Adem; Guvercin, Yılmaz; Bedir, Recep

    2016-01-01

    A calcification mass was incidentally found in the soft tissue of a patient who had a history of trauma to the extremity during examination. The patient had no symptom. The pathological analysis of the mass revealed it was an early-phase synovial sarcoma (SS). The diagnosis was made before the onset of symptoms and proper surgical intervention was performed. Therefore, in case of a <1 cm lesion clinically suspicious of myositis ossificans, SS should be taken into consideration as a possible diagnosis. PMID:27595081

  15. Synovial Sarcoma Mimicking Myositis Ossificans.

    PubMed

    Balik, Mehmet Sabri; Erkut, Adem; Guvercin, Yılmaz; Bedir, Recep

    2016-09-01

    A calcification mass was incidentally found in the soft tissue of a patient who had a history of trauma to the extremity during examination. The patient had no symptom. The pathological analysis of the mass revealed it was an early-phase synovial sarcoma (SS). The diagnosis was made before the onset of symptoms and proper surgical intervention was performed. Therefore, in case of a <1 cm lesion clinically suspicious of myositis ossificans, SS should be taken into consideration as a possible diagnosis. PMID:27595081

  16. Therapeutic Trial for Patients With Ewing Sarcoma Family of Tumor and Desmoplastic Small Round Cell Tumors

    ClinicalTrials.gov

    2015-12-01

    Desmoplastic Small Round Cell Tumor; Ewing Sarcoma of Bone or Soft Tissue; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  17. [Sclerosing epithelioid fibrosarcoma of the paravertebral column. Case report and literature review].

    PubMed

    Puerta Roldán, Patricia; Rodríguez Rodríguez, Rodrigo; Bagué Rossell, Silvia; de Juan Delago, Manel; Molet Teixidó, Joan

    2013-01-01

    Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of low-grade fibrosarcoma, with specific histological and immunohistochemical features and a poor prognosis. We report a case of SEF of the paravertebral column in a 49-year old male who presented a paraspinal mass with extension into the L4-L5 neural foramen and invasion of the L5 nerve root. Histology of the tumourectomy specimen and its immunohistochemical study led to the diagnosis of SEF. This case was particularly unusual due to its paravertebral column location and, despite its low grade, illustrates the malignant potential of SEF.

  18. An unusual presentation of malignant hepatic epithelioid haemangioendothelioma with left pleural and pulmonary localization.

    PubMed

    Caruso, Settimo; Miraglia, Roberto; Maruzzelli, Luigi; Spada, Marco; Vitulo, Patrizio; Minervini, Marta Ida; Luca, Angelo; Gridelli, Bruno

    2008-09-01

    Hepatic epithelioid haemangioendothelioma (HEHE) is extremely rare in children. We present an adolescent who presented with a scoliosis due to left thoracic pain. Multifocal HEHE was incidentally found during CT scan of the thorax, and confirmed with a multiphase CT scan of the abdomen, biopsy and immunochemistry. Left pleural and pulmonary involvement was suspected and later confirmed with biopsy and histopathological examination. The patient died 1 year after diagnosis. To the best of our knowledge, scoliosis has not previously been reported as the first symptom of EHE. PMID:18542941

  19. Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: A Rare and Lethal Entity.

    PubMed

    Celli, Romulo; Cai, Guoping

    2016-03-01

    Ewing sarcoma/primitive neuroectodermal tumor represents a spectrum of undifferentiated tumors with similar biology that together represent the second most common sarcoma in the pediatric-young adult age range. Very rarely, this tumor presents as a primary neoplasm of the kidney. The clinical presentation of this tumor is not specific, and other renal tumors may present with a similar histologic appearance. Establishing the correct diagnosis is critical because renal Ewing sarcoma/primitive neuroectodermal tumor carries a strikingly dismal prognosis and thus dictates a specific treatment strategy. A low threshold for the use of ancillary molecular tests is recommended, particularly in diagnostically problematic cases. Important considerations with regards to morphology, immunohistochemistry, and molecular alterations will be reviewed here and should be taken into account before rendering this rare and lethal diagnosis.

  20. TFE3 Translocation Associated Perivascular Epithelioid Cell Neoplasm (PEComa) of the Gynecologic Tract: Morphology, Immunophenotype, Differential Diagnosis

    PubMed Central

    Schoolmeester, J. Kenneth; Dao, Linda N.; Sukov, William R.; Park, Kay J.; Murali, Rajmohan; Hameed, Meera R.; Soslow, Robert A.

    2016-01-01

    TFE3 translocation associated PEComa is a distinct form of perivascular epithelioid cell neoplasm, the features of which are poorly defined owing to their general infrequency and limited prior reports with confirmed rearrangement or fusion totaling nine cases. Recent investigation has found a lack of TSC gene mutation in these tumors compared to their nonrearranged counterparts which underscores the importance of recognizing the translocated variant due to hypothetical ineffectiveness of targeted mTOR inhibitor therapy. Six cases were identified and TFE3 rearrangement was confirmed by FISH. Patient age ranged 46 to 66 years (median 50) and none had a history of tuberous sclerosis complex. Three cases arose in the uterine corpus, one in the vagina, and one pelvic tumor and one pulmonary tumor were likely a recurrence/metastasis from a probable uterine primary. Five cases had purely clear cell epithelioid morphology that showed a spectrum of atypia while one case had a mixture of clear cell epithelioid and spindle cells. A mostly consistent immunophenotype was observed in the purely clear cell epithelioid cases: each demonstrated diffuse TFE3, HMB45, CathepsinK labeling, either focal or no melanA staining and variably weak reactivity to smooth muscle markers. The mixed clear cell epithelioid and spindle cell case had a similar pattern in its epithelioid component, but strong muscle marker positivity in its spindle cell component. Follow up ranged 1 to 57 months. Three cases demonstrated aggressive behavior and three cases had no evidence of recurrence. Both GYN-specific and traditional sets of criteria for malignancy were evaluated. The GYN model showed improved inclusion and specificity in comparison to the traditional model. PMID:25517951

  1. Epithelioid hemangioendothelioma of the mandibular gingiva: A rare case of metastasis 4 years after radical excision and literature review

    PubMed Central

    Salgarelli, Attilio Carlo; Bellini, Pierantonio; Maccio, Livia; Setti, Giacomo

    2016-01-01

    Malignant epithelioid hemangioendothelioma (MEH), or high-risk epithelioid hemangioendothelioma, is a low- to intermediate-grade vascular malignancy. A few cases of MEH have been documented in the head and neck region, including the neck, thyroid gland, larynx and scalp. MEHs are extremely rare in the oral cavity. Only 31 cases of MEH in the oral cavity were described in English literature between 1975 and 2014. Further, only eleven cases were referred to MEH of the maxillary or mandibular gingiva. No gingival MEH metastases have been described in literature. We report a literature review and a case of MEH with a metastatic occurrence 4 years after surgical excision. PMID:27194877

  2. Uterine sarcomas: clinical presentation and MRI features

    PubMed Central

    Santos, Pedro; Cunha, Teresa Margarida

    2015-01-01

    Uterine sarcomas are a rare heterogeneous group of tumors of mesenchymal origin, accounting for approximately 8% of uterine malignancies. They comprise leiomyosarcoma, endometrial stromal sarcoma, undifferentiated endometrial sarcoma, and adenosarcoma. Compared with the more common endometrial carcinomas, uterine sarcomas behave more aggressively and are associated with a poorer prognosis. Due to their distinct clinical and biological behavior, the International Federation of Gynecology and Obstetrics introduced a new staging system for uterine sarcomas in 2009, categorizing uterine carcinosarcoma as a variant of endometrial carcinoma, rather than a pure sarcoma. Magnetic resonance imaging (MRI) has a developing role in the assessment of these malignancies. Features such as tumor localization, irregular or nodular margins, necrosis, rapid growth, intense contrast enhancement, and restriction at diffusion-weighted imaging can suggest the diagnosis and help differentiate from more common leiomyomas and endometrial carcinoma. MRI is therefore extremely useful in preoperative detection and staging and, consequently, in determination of appropriate management. This pictorial review aims to discuss the clinical features of uterine sarcomas, as well as their most common appearances and distinct characteristics in MRI. PMID:25347940

  3. Epithelioid myoepithelioma of the accessory parotid gland: pathological and magnetic resonance imaging findings.

    PubMed

    Iguchi, Hiroyoshi; Yamada, Kei; Yamane, Hideo; Hashimoto, Shigeo

    2014-05-01

    Tumors of the accessory parotid gland (APG) are rare, and pleomorphic adenoma (PA) is the most common benign APG tumor subtype. Myoepithelioma of the APG is much rarer than PA, and to date, only 5 cases have been sporadically reported in the English literature. We describe the clinicopathological and MRI findings of an epithelioid myoepithelioma of the APG that was treated in our hospital. The patient's only clinical symptom was a slow-growing and painless mid-cheek mass. The tumor was suspected to be PA before surgery based on the following MRI findings: (1) a well-circumscribed and lobulated contour, (2) isointensity and hyperintensity relative to the muscle on T1- and T2-weighted images (WIs), respectively, (3) good enhancement on contrast-enhanced T1-WIs, (4) peripheral hypointensity on T2-WIs, and (5) a gradual time-signal intensity curve enhancement pattern on gadolinium-enhanced dynamic MRI. The tumor was completely resected via a standard parotidectomy approach, and the postoperative pathological examination of the tumor, including immunohistochemistry, confirmed the diagnosis of epithelioid myoepithelioma. As it is hardly possible to distinguish myoepithelioma from PA and low-grade malignant tumors preoperatively, a pathological examination using frozen sections is helpful for surgical strategy-related decisions.

  4. Ziv-aflibercept in Treating Patients With Locally Advanced, Unresectable, or Metastatic Gynecologic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2015-12-03

    Fallopian Tube Cancer; Female Reproductive Cancer; Ovarian Carcinosarcoma; Ovarian Sarcoma; Recurrent Ovarian Epithelial Cancer; Recurrent Uterine Sarcoma; Stage III Ovarian Epithelial Cancer; Stage III Uterine Sarcoma; Stage IV Ovarian Epithelial Cancer; Stage IV Uterine Sarcoma; Uterine Carcinosarcoma; Uterine Leiomyosarcoma

  5. A non-comparative phase II study of dose intensive chemotherapy with doxorubicin and ifosfamide followed by high dose ICE consolidation with PBSCT in non-resectable, high grade, adult type soft tissue sarcomas.

    PubMed

    Hartmann, Jörg Thomas; Horger, M; Kluba, T; Königsrainer, A; de Zwart, P; von Weyhern, C Hann; Eckert, F; Budach, W; Bokemeyer, C

    2013-12-01

    The objective was to determine the role of dose intensive induction chemotherapy in patients with soft tissue sarcomas (STS) that were considered unresectable. Treatment consisted of 2-3 cycles of doxorubicin (Dox) and ifosfamide (Ifo) followed by high dose chemotherapy with ifosfamide, carboplatin, etoposide (HD-ICE) plus peripheral blood stem cell transplantation (PBSCT). 30 out of 631 consecutive patients, median age 46 years (21-62), with high grade STS were included. 29 patients completed at least 2 cycles of Dox/Ifo. HD-ICE was withheld because of progressive disease (PD) in 5 patients, neurotoxicity in 6 cases, insufficient peripheral blood stem cell (PBSC) mobilization, complete remission (CR) and refusal in 1 patient each. HD-ICE was associated with non-haematological grade III toxicity including emesis, mucositis, fever, neurotoxicity, and transaminase level elevation. Two additional patients attained a partial response after HD-ICE. Overall, 24 of 30 (80%) patients underwent surgery, with complete tumor resections in 19 patients (63% of all patients, 79% of the operated subgroup); however, 2 of these required amputation. After a median follow up period of 50 months in surviving patients (range, 26-120), 5-year PFS and OS rates were 39% and 48%, respectively. Induction chemotherapy plus consolidation HD-ICE is generally feasible, but is associated with significant neurotoxicity. The advantage of HD-ICE over conventional dose chemotherapy plus external beam radiation therapy (EBRT) in non-resectable disease remains unproven.

  6. [Grading of soft tissue and bone sarcomas].

    PubMed

    Petersen, I; Wardelmann, E

    2016-07-01

    Malignancy grading is an essential element in the classification of sarcomas. It correlates with the prognosis of the disease and the risk of metastasis. This article presents the grading schemes for soft tissue, bone and pediatric sarcomas. It summarizes the histological criteria of the Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) system and the Pediatric Oncology Group as well as the grading of bone tumors by the College of American Pathologists (CAP). Furthermore, the potential relevance of gene expression signatures, the complexity index in sarcoma (CINSARC) and single genetic alterations (p53, MDM2, p16, SWI/SNF, EWSR1 fusions and PAX3/PAX7-FOXO1 fusions) for the prognosis of sarcomas are discussed.

  7. [Grading of soft tissue and bone sarcomas].

    PubMed

    Petersen, I; Wardelmann, E

    2016-07-01

    Malignancy grading is an essential element in the classification of sarcomas. It correlates with the prognosis of the disease and the risk of metastasis. This article presents the grading schemes for soft tissue, bone and pediatric sarcomas. It summarizes the histological criteria of the Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) system and the Pediatric Oncology Group as well as the grading of bone tumors by the College of American Pathologists (CAP). Furthermore, the potential relevance of gene expression signatures, the complexity index in sarcoma (CINSARC) and single genetic alterations (p53, MDM2, p16, SWI/SNF, EWSR1 fusions and PAX3/PAX7-FOXO1 fusions) for the prognosis of sarcomas are discussed. PMID:27384333

  8. Penile Sarcoma: Report of a Rare Malignancy

    PubMed Central

    Kumar, Vijay; Chaturvedi, Arun; Vishnoi, Jeevan Ram; Dontula, Prashant

    2016-01-01

    Penile cancer is an uncommon malignancy. Squamous cell carcinoma constitutes approximately 95% of all histology. Non-squamous malignancies are rare in penis. Sarcomas of penis are rarer among them. Spindle cell sarcoma is one of the extremely rare sarcoma of penis. To best of our knowledge, only two cases have been reported so far, one in English literature and other in Japanese. We are presenting this uncommon case of spindle cell sarcoma of penis, which was diagnosed with microscopy with its characteristic immunohistochemistry. The disease had an aggressive course with multiple recurrences in a short duration despite margin negative resection. Disease responded poorly with the chemotherapy and patient succumbed to the disease. PMID:27630937

  9. Pulmonary Artery Intimal Sarcoma: A Case Report

    PubMed Central

    Kriz, Joseph P.; Munfakh, Nabil A.; King, Gregory S.; Carden, Juan O.

    2016-01-01

    Pulmonary artery intimal sarcomas are rare and lethal malignant tumors that typically affect larger vessels: the aorta, inferior vena cava, and pulmonary arteries. Since symptoms and imaging of pulmonary arterial intimal sarcomas mimic pulmonary thromboembolism, the differential diagnosis of a patient presenting with chest pain, dyspnea, and filling defect within the pulmonary arteries should include intimal sarcoma. Often right ventricular failure is observed due to pulmonary hypertension caused by the obstructive effect of the tumor and concomitant chronic thromboembolism. We report the case of a 72-year-old African-American male with arterial intimal sarcoma of the left and right pulmonary artery with extension through the right artery into the bronchus and right lung. PMID:27239183

  10. Soft Tissue Sarcomas and Agent Orange

    MedlinePlus

    ... survivors' benefits . Research on soft tissue sarcoma and herbicides The Health and Medicine Division (formally known as ... report " Veterans and Agent Orange: Health Effects of Herbicides Used in Vietnam " and other updates that there ...

  11. Solitary pulmonary nodule: pleuropulmonary synovial sarcoma.

    PubMed

    Ward, Robert C; Birnbaum, Ariel E; Aswad, Bassam I; Healey, Terrance T

    2014-05-01

    Pleuropulmonary synovial sarcoma (PPSS) is an extremely rare primary malignancy of the lung. We present a case of a middle-aged female with PPSS that was initially discovered as an incidental indeterminate nodule on chest radiograph. Following evaluation with computed tomography (CT), the patient went on to positron-emission tomography (PET)/CT for work-up of the solitary pulmonary nodule, which demonstrated mild FDG-avidity and no other evidence of FDG-avid disease. The patient then underwent thoracotomy and right upper lobectomy for definitive treatment. Only after evaluation of the gross pathology, histology, immunohistochemistry and cytogenetics was the diagnosis of synovial sarcoma made. Importantly, the preceding PET/CT, in addition to physical exam of the upper and lower extremities, helped exclude the more common extra-thoracic soft-tissue variety of synovial sarcoma, which frequently metastasizes to lung, carrying a worse prognosis. Discussion of synovial sarcoma and PPSS follows. PMID:24791267

  12. Immunosuppressive Therapy-Related Kaposi Sarcoma

    MedlinePlus

    ... therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. ... complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information ...

  13. Penile Sarcoma: Report of a Rare Malignancy.

    PubMed

    Rajan, Shiv; Kumar, Vijay; Chaturvedi, Arun; Vishnoi, Jeevan Ram; Dontula, Prashant

    2016-07-01

    Penile cancer is an uncommon malignancy. Squamous cell carcinoma constitutes approximately 95% of all histology. Non-squamous malignancies are rare in penis. Sarcomas of penis are rarer among them. Spindle cell sarcoma is one of the extremely rare sarcoma of penis. To best of our knowledge, only two cases have been reported so far, one in English literature and other in Japanese. We are presenting this uncommon case of spindle cell sarcoma of penis, which was diagnosed with microscopy with its characteristic immunohistochemistry. The disease had an aggressive course with multiple recurrences in a short duration despite margin negative resection. Disease responded poorly with the chemotherapy and patient succumbed to the disease. PMID:27630937

  14. Myeloid Sarcoma: Current Approach and Therapeutic Options

    PubMed Central

    Avni, Batia; Koren-Michowitz, Maya

    2011-01-01

    Myeloid sarcoma is a rare disease that can present as an isolated extramedullary leukemic tumor, concurrently with or at relapse of acute myeloid leukemia. Owing to the rarity of this disorder, most of the literature comprises small retrospective studies and case reports. The aim of this review is to summarize the current published data regarding the clinical presentation, morphological, cytogenetic and molecular features, prognosis and treatment of myeloid sarcoma. PMID:23556098

  15. Imaging spectrum in soft tissue sarcomas.

    PubMed

    Aga, Pallavi; Singh, Ragini; Parihar, Anit; Parashari, Umesh

    2011-12-01

    Imaging plays an important role in detection, diagnosis as well as pre and post operative management of patients with soft tissue sarcomas. Soft tissue sarcomas are generally a diagnostic dilemma needing the complimentary use of both radiology and pathology for their accurate diagnosis. In this review article, we have tried to highlight the important facts about the various imaging modalities available as well as the recent advances in the field of radiology. PMID:23204782

  16. Surgical management of soft tissue sarcomas

    SciTech Connect

    Arlen, M.; Marcove, R.C.

    1987-01-01

    This volume reflects the latest thinking in surgical and adjuvant forms of therapy that can be offered to the sarcoma patient. Based on their analysis of sarcoma patients, the authors stress management based on site of origin, and discuss tumors on and about the shoulder girdle, hip joint, extremity, retroperitoneum, etc. Coverage includes methods for limb preservation; techniques for regional node resection; indications and methods for arterial perfusion, cryosurgery and isotope implantation; pre- and post-operative immunotherapy chemotherapy and radiation therapy.

  17. Imaging in Soft Tissue Sarcomas: Current Updates.

    PubMed

    Jagannathan, Jyothi P; Tirumani, Sree Harsha; Ramaiya, Nikhil H

    2016-10-01

    Soft tissue sarcomas (STS) are heterogeneous malignant tumors that have nonspecific imaging features. A combination of clinical, demographic, and imaging characteristics can aid in the diagnosis. Imaging provides important information regarding the tumor extent, pretreatment planning, and surveillance of patients with STS. In this article, we illustrate the pertinent imaging characteristics of the commonly occurring STS and some uncommon sarcomas with unique imaging characteristics. PMID:27591491

  18. Kaposi's Sarcoma Herpesvirus Genome Persistence.

    PubMed

    Juillard, Franceline; Tan, Min; Li, Shijun; Kaye, Kenneth M

    2016-01-01

    Kaposi's sarcoma-associated herpesvirus (KSHV) has an etiologic role in Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman's disease. These diseases are most common in immunocompromised individuals, especially those with AIDS. Similar to all herpesviruses, KSHV infection is lifelong. KSHV infection in tumor cells is primarily latent, with only a small subset of cells undergoing lytic infection. During latency, the KSHV genome persists as a multiple copy, extrachromosomal episome in the nucleus. In order to persist in proliferating tumor cells, the viral genome replicates once per cell cycle and then segregates to daughter cell nuclei. KSHV only expresses several genes during latent infection. Prominent among these genes, is the latency-associated nuclear antigen (LANA). LANA is responsible for KSHV genome persistence and also exerts transcriptional regulatory effects. LANA mediates KSHV DNA replication and in addition, is responsible for segregation of replicated genomes to daughter nuclei. LANA serves as a molecular tether, bridging the viral genome to mitotic chromosomes to ensure that KSHV DNA reaches progeny nuclei. N-terminal LANA attaches to mitotic chromosomes by binding histones H2A/H2B at the surface of the nucleosome. C-terminal LANA binds specific KSHV DNA sequence and also has a role in chromosome attachment. In addition to the essential roles of N- and C-terminal LANA in genome persistence, internal LANA sequence is also critical for efficient episome maintenance. LANA's role as an essential mediator of virus persistence makes it an attractive target for inhibition in order to prevent or treat KSHV infection and disease. PMID:27570517

  19. Potential Therapeutic Targets in Uterine Sarcomas

    PubMed Central

    Cuppens, Tine; Tuyaerts, Sandra; Amant, Frédéric

    2015-01-01

    Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma. Based on clinical reports, promising approaches for uterine leiomyosarcoma patients include inhibition of VEGF and mTOR signaling, preferably in combination with other targeted or cytotoxic compounds. Currently, the only targeted therapy approved in leiomyosarcoma patients is pazopanib, a multitargeted inhibitor blocking VEGFR, PDGFR, FGFR, and c-KIT. Additionally, preclinical evidence suggests effect of the inhibition of histone deacetylases, tyrosine kinase receptors, and the mitotic checkpoint protein aurora kinase A. In low-grade endometrial stromal sarcomas, antihormonal therapies including aromatase inhibitors and progestins have proven activity. Other potential targets are PDGFR, VEGFR, and histone deacetylases. In high-grade ESS that carry the YWHAE/FAM22A/B fusion gene, the generated 14-3-3 oncoprotein is a putative target, next to c-KIT and the Wnt pathway. The observation of heterogeneity within uterine sarcoma subtypes warrants a personalized treatment approach. PMID:26576131

  20. Thymidine phosphorylase expression in Kaposi sarcoma.

    PubMed Central

    Dada, M A; Boshoff, C H; Comley, M A; Turley, H; Schneider, J W; Chetty, R; Gatter, K C

    1996-01-01

    AIMS: To examine the immunohistochemical distribution of thymidine phosphorylase (TP) in all clinicopathological subtypes of Kaposi sarcoma. METHODS: Thirty two biopsy specimens of Kaposi sarcoma (29 patients) were studied. Six of these patients represented classic, six endemic, eight HIV associated, seven post-immunosuppression/transplant related, and two unclassified variants of Kaposi sarcoma. The average age was 49 years (range 22-83 years) and the male: female ratio 24:5. Four samples of angiosarcoma and one of spindle cell haemangio-endothelioma were stained in parallel. All specimens were fixed in formalin, embedded in paraffin wax and processed routinely. Immunohistochemistry was carried out using an antibody directed against CD31 (JC70) and the monoclonal antibody P-GF.44C against TP. RESULTS: All biopsy specimens showed immunoexpression for TP. The spindle cell component stained more strongly than newly formed endothelium lined vessels and normal, resident vessels at a distance from the lesions. CONCLUSIONS: The strong immunoexpression of TP suggests up-regulation of TP and a role for TP in angiogensis in Kaposi sarcoma. The mechanism for the up-regulation of TP remains unknown, but viral infections may trigger it. The differential staining of the various cell components of Kaposi sarcoma also suggest that TP either plays a role in the differentiation and maturation of Kaposi sarcoma or is a reflection of such changes. Images PMID:8707955

  1. Use of negative pressure wound therapy as an adjunct to the treatment of extremity soft-tissue sarcoma with ulceration or impending ulceration

    PubMed Central

    CHEN, YU; XU, SONG-FENG; XU, MING; YU, XIU-CHUN

    2016-01-01

    Major wound complications of the extremities, following wide tumor resection and reconstruction for soft-tissue sarcomas (STSs), remain a challenge for limb-sparing surgery. Furthermore, STSs with ulceration or impending ulceration predispose patients to an increased risk of post-operative infection. The present study was conducted to assess the efficacy of negative pressure wound therapy (NPWT) in preventing wound complications associated with surgical treatment of STSs with ulceration or impending ulceration, in patients treated between February 2012 and January 2013. A total of 5 patients, with a mean age of 48 years (range, 24–68 years), were enrolled in the present study. The diagnoses consisted of undifferentiated pleomorphic sarcoma (n=2), leiomyosarcoma (n=1), synovial sarcoma (n=1) and epithelioid sarcoma (n=1). According to American Joint Committee on Cancer criteria, 3 cases were stage III tumors, and the remaining 2 cases were of stages IIA and IIB, respectively. A total of 3 patients exhibited ulceration at diagnosis, and the remaining patients demonstrated impending ulceration. The mean wound area following wide resection of the tumor was 73 cm2 (range, 45–110 cm2). A continuous suction mode, with pressures measuring −200 to −300 mmHg, was used for 7–10 days on the soft-tissue defects as preparation for wound closure. Soft-tissue reconstruction included muscle flaps (n=2) and skin grafts (n=5). No major wound complications occurred. Post-operative functional and cosmetic outcomes were acceptable. A single patient demonstrated local recurrence 12 months after surgery and re-excision of the tumor was performed. All patients remained alive at the conclusion of follow-up, with a mean follow-up time of 26 months (range, 12–36 months). The present study demonstrated that NPWT is effective and safe when used as an adjunct to wound closure following resection of extremity STS with ulceration/impending ulceration. PMID:27347212

  2. Identification of HHV8 in early Kaposi's sarcoma: implications for Kaposi's sarcoma pathogenesis.

    PubMed Central

    Kennedy, M M; Cooper, K; Howells, D D; Picton, S; Biddolph, S; Lucas, S B; McGee, J O; O'Leary, J J

    1998-01-01

    AIMS: Kaposi's sarcoma is a vascular tumour of uncertain pathogenesis possibly caused by an infectious agent, identified in high risk groups. Accumulating solution phase polymerase chain reaction (PCR) and seroepidemiological data suggest that a previously undescribed herpes DNA virus (human herpesvirus 8 (HHV8)) is the causative agent. Using a unique cohort of early Kaposi's sarcoma, the precise cell type infected with HHV8 in such lesions was identified to elucidate further the role of HHV8 in the pathobiology of Kaposi's sarcoma. METHODS: Sixteen cases of early Kaposi's sarcoma (derived from skin and lymph node) were assessed for the presence of HHV8 using both standard solution phase PCR and TaqMan PCR to the KS330 Bam region of HHV8. In situ amplification was also performed on a selected group in an attempt to identify the candidate infected cells. RESULTS: Using both conventional solution phase and TaqMan PCR, 87% of cases were positive. In addition, HHV8 amplicons were localised in situ to endothelial and spindle cell proliferations in early Kaposi's sarcoma. The HHV8 viral load varied from lesion to lesion. CONCLUSIONS: The presence of HHV8 in early lesions supports a role for HHV8 in the pathogenesis of Kaposi's sarcoma. Coupled with recent seroepidemiological studies, these results suggest that HHV8 is the aetiological agent of Kaposi's sarcoma. Its precise interaction with other factors known to be involved in the development of Kaposi's sarcoma, including cytokines and anti-apoptosis genes, requires elucidation. PMID:9624414

  3. Hepatic Epithelioid Hemangioendothelioma and the Danger of Misdiagnosis: Report of a Case

    PubMed Central

    Neofytou, Kyriakos; Chrysochos, Andreas; Charalambous, Nikolas; Dietis, Menelaos; Petridis, Christos; Andreou, Charalampos; Petrou, Athanasios

    2013-01-01

    Malignant hepatic epithelioid hemangioendothelioma (HEHE) is a rare malignant tumor of vascular origin. Nonspecific symptoms and the absence of experience of surgeons, radiologists, and histopathologists due to the rarity of HEHE make the diagnosis of this entity very challenging. Misdiagnosis is not a rare event, and the consequences of such an event are catastrophic. We report a case of a patient suffering from HEHE in which the initial diagnosis was hepatocellular carcinoma (HCC). The presence of normal laboratory values, liver function tests, tumor markers along with the absence of a chronic liver disease, or any other predisposing factors for HCC, was in contrast with the diagnosis of HCC. Clinical suspicion drove us to the repetition of a liver biopsy and the reevaluation of the sample by a more experience histopathology department in liver tumors. The last biopsy confirmed the diagnosis of HEHE, and the patient escaped any unnecessary treatment for a nonexisting HCC. PMID:23533870

  4. Hepatic epithelioid hemangioendothelioma and the danger of misdiagnosis: report of a case.

    PubMed

    Neofytou, Kyriakos; Chrysochos, Andreas; Charalambous, Nikolas; Dietis, Menelaos; Petridis, Christos; Andreou, Charalampos; Petrou, Athanasios

    2013-01-01

    Malignant hepatic epithelioid hemangioendothelioma (HEHE) is a rare malignant tumor of vascular origin. Nonspecific symptoms and the absence of experience of surgeons, radiologists, and histopathologists due to the rarity of HEHE make the diagnosis of this entity very challenging. Misdiagnosis is not a rare event, and the consequences of such an event are catastrophic. We report a case of a patient suffering from HEHE in which the initial diagnosis was hepatocellular carcinoma (HCC). The presence of normal laboratory values, liver function tests, tumor markers along with the absence of a chronic liver disease, or any other predisposing factors for HCC, was in contrast with the diagnosis of HCC. Clinical suspicion drove us to the repetition of a liver biopsy and the reevaluation of the sample by a more experience histopathology department in liver tumors. The last biopsy confirmed the diagnosis of HEHE, and the patient escaped any unnecessary treatment for a nonexisting HCC. PMID:23533870

  5. Treatment of Hepatic Epithelioid Hemangioendothelioma: Finding Uses for Thalidomide in a New Era of Medicine

    PubMed Central

    Soape, Matthew P.; Verma, Rashmi; Payne, J. Drew; Wachtel, Mitchell; Hardwicke, Fred; Cobos, Everardo

    2015-01-01

    Hepatic epithelioid hemangioendothelioma (HEH) is extremely rare, occurring in 1 to 2 per 100,000, with chemotherapy options not well defined. Our case involved a 49-year-old female who had hepatic masses and metastasis to the lungs with a liver biopsy revealing HEH. After developing a rash from sorafenib, thalidomide was started with the progression of disease stabilized. Resection is only an option in 10% of the cases; therefore, chemotherapy is the only line of treatment. Newer chemotherapy alternatives are targeting angiogenesis via the vascular endothelial growth factor. Thalidomide was first used as an antiemetic, but, sadly, soon linked to phocomelia birth defects. Given the mechanism of action against angiogenesis, thalidomide has a valid role in vascular tumors. In conclusion, the use of thalidomide as chemotherapy is novel and promising, especially in the setting of a rare vascular liver tumor such as HEH. PMID:26167310

  6. Treatment of Hepatic Epithelioid Hemangioendothelioma: Finding Uses for Thalidomide in a New Era of Medicine.

    PubMed

    Soape, Matthew P; Verma, Rashmi; Payne, J Drew; Wachtel, Mitchell; Hardwicke, Fred; Cobos, Everardo

    2015-01-01

    Hepatic epithelioid hemangioendothelioma (HEH) is extremely rare, occurring in 1 to 2 per 100,000, with chemotherapy options not well defined. Our case involved a 49-year-old female who had hepatic masses and metastasis to the lungs with a liver biopsy revealing HEH. After developing a rash from sorafenib, thalidomide was started with the progression of disease stabilized. Resection is only an option in 10% of the cases; therefore, chemotherapy is the only line of treatment. Newer chemotherapy alternatives are targeting angiogenesis via the vascular endothelial growth factor. Thalidomide was first used as an antiemetic, but, sadly, soon linked to phocomelia birth defects. Given the mechanism of action against angiogenesis, thalidomide has a valid role in vascular tumors. In conclusion, the use of thalidomide as chemotherapy is novel and promising, especially in the setting of a rare vascular liver tumor such as HEH.

  7. Pulmonary epithelioid hemangioendothelioma accompanied by bilateral multiple calcified nodules in lung

    PubMed Central

    2011-01-01

    Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor. It can present either as one solitary nodule or bilateral multiple nodules, usually without calcification. We describe here an unusual case of PEH in a 42-year-old female with a 6.0 cm dominant mass along with bilateral multiple calcified small nodules measuring 0.2-1.0 cm in diameter with a 25-year plus followup history. Overall histologic findings of the solitary tumor accorded with conventional PEH. While multiple calcified small nodules were composed predominantly of intra-alveolar homogeneously eosinophilic matrix, and only a few bland small cells were embedded in it. This lesion has never been reported in the literature. After comprehensive analysis of morphology, radiography, histochemistry, immunohistochemistry and differential diagnoses, PEH presenting multiple calcified small nodules was confirmed. PMID:21418612

  8. Sclerosing variant of perivascular epithelioid cell tumor in the female genital organs.

    PubMed

    Yamada, Yuichi; Yamamoto, Hidetaka; Ohishi, Yoshihiro; Nishiyama, Kenichi; Fukuhara, Masao; Saitou, Toshiaki; Tsuneyoshi, Masazumi; Oda, Yoshinao

    2011-12-01

    Perivascular epithelioid cell tumor (PEComas), other than angiomyolipoma, clear cell 'sugar' tumor of the lung, and lymphangioleiomyomatosis, is an uncommon mesenchymal neoplasm that arises in the soft tissue and visceral organs. We report herein two cases of sclerosing PEComa; a distinctive variant of PEComa, which is characterized by extensive stromal hyalinization, occurring in the uterus and broad ligament. The patients were 34- and 51-year-old females with no family history of tuberous sclerosis complex. Macroscopically, the tumors had white to gray cut surfaces and were microscopically composed of predominantly spindle- to polygon-shaped cells with clear to slightly eosinophilic cytoplasm and pleomorphic nuclei focally arranged in a perivascular pattern, accompanied by marked stromal hyalinization. These tumor cells were immunohistochemically positive for HMB45 and α-smooth muscle actin. Although this variant of PEComa is very rare, this entity should be considered as a potential primary neoplasm of the female genital organs.

  9. Perivascular epithelioid cell tumor of the liver coexisting with a gastrointestinal stromal tumor.

    PubMed

    Paiva, Carlos Eduardo; Moraes Neto, Francisco Alves; Agaimy, Abbas; Custodio Domingues, Maria Aparecida; Rogatto, Silvia Regina

    2008-02-01

    Approximately 10% of patients with gastrointestinal stromal tumors (GIST) develop other neoplasms, either synchronously or metachronously. In this report we describe coexistence of a gastrointestinal stromal tumor and a hepatic perivascular epithelioid cell tumor (PEComa) in a 51-year-old woman with no evidence of tuberous sclerosis. A subcapsular hepatic nodule (0.8 cm in diameter) was found during surgery for symptomatic gastric neoplasm (15 cm in diameter) arising from the lesser curvature. Both tumors revealed histomorphological and immunohistochemical features confirming a diagnosis of a small incidental hepatic PEComa and a high risky extramural gastric GIST, respectively. The patient remained disease-free 25 mo after surgery with no evidence of tumor recurrence or new neoplasms. To our knowledge, this is the first report of PEComa in a patient with GIST. Hepatic lesions detected synchronously or metachronously in patients with GISTs may represent histogenetically distinct lesions and should be sampled to confirm or exclude metastatic GISTs.

  10. Pathological characteristics of liver biopsies in eight patients with hepatic epithelioid hemangioendothelioma

    PubMed Central

    Dong, Kun; Wang, Xin-Xin; Feng, Ji-Liang; Liu, Hui; Zu, Ke-Jia; Chang, Jing; Lv, Fu-Dong

    2015-01-01

    We aim to investigate the pathological characteristics of liver biopsies and their implications for the prognosis of hepatic epithelioid hemangioendothelioma (HEHE). Clinical data of eight patients (5 male, 3 female) with HEHE were analyzed retrospectively. Expression of CD34, FVIII, AE1/AE3, Hepa-par1, GPC3, CK19 and the proliferation index marker Ki-67 were determined by immunohistochemical staining. The clinical pathological features and effects of treatment on prognosis were investigated. Among the eight patients, four did not exhibit significant symptoms, while four showed symptoms such as abdominal distension, aversion to greasy food and mild fever. Two patients had single liver lesions, while multiple lesions were observed in six cases, in which the tumor cells exhibited spindle, irregular or epithelioid morphology, with scattered, streaked and nested distribution. Individual luminal cells were also visible, containing red cells and accompanied by mucoid or fibrous stroma. All cases were CD34 positive, one case was FVIII factor negative, two cases were AE1/AE3 positive, Ki-67 staining exceeded 15% in two cases, and nuclear fission was visible in two cases. Patients with nuclear fission and Ki-67 > 15% died within 2 years after artery embolization, liver transplantation without relapse was observed in two cases and one case survived with the tumor. The other patients without cellular atypia, without nuclear fission and with Ki-67 < 10% did not relapse during the 2-5 years of follow-up. HEHE can be diagnosed according to hematoxylin and eosin morphology and immunohistochemical characteristics in biopsies before treatment allowing the selection of different treatment protocols based on pathological characteristics. PMID:26617819

  11. Pathological characteristics of liver biopsies in eight patients with hepatic epithelioid hemangioendothelioma.

    PubMed

    Dong, Kun; Wang, Xin-Xin; Feng, Ji-Liang; Liu, Hui; Zu, Ke-Jia; Chang, Jing; Lv, Fu-Dong

    2015-01-01

    We aim to investigate the pathological characteristics of liver biopsies and their implications for the prognosis of hepatic epithelioid hemangioendothelioma (HEHE). Clinical data of eight patients (5 male, 3 female) with HEHE were analyzed retrospectively. Expression of CD34, FVIII, AE1/AE3, Hepa-par1, GPC3, CK19 and the proliferation index marker Ki-67 were determined by immunohistochemical staining. The clinical pathological features and effects of treatment on prognosis were investigated. Among the eight patients, four did not exhibit significant symptoms, while four showed symptoms such as abdominal distension, aversion to greasy food and mild fever. Two patients had single liver lesions, while multiple lesions were observed in six cases, in which the tumor cells exhibited spindle, irregular or epithelioid morphology, with scattered, streaked and nested distribution. Individual luminal cells were also visible, containing red cells and accompanied by mucoid or fibrous stroma. All cases were CD34 positive, one case was FVIII factor negative, two cases were AE1/AE3 positive, Ki-67 staining exceeded 15% in two cases, and nuclear fission was visible in two cases. Patients with nuclear fission and Ki-67 > 15% died within 2 years after artery embolization, liver transplantation without relapse was observed in two cases and one case survived with the tumor. The other patients without cellular atypia, without nuclear fission and with Ki-67 < 10% did not relapse during the 2-5 years of follow-up. HEHE can be diagnosed according to hematoxylin and eosin morphology and immunohistochemical characteristics in biopsies before treatment allowing the selection of different treatment protocols based on pathological characteristics. PMID:26617819

  12. Explant culture of sarcoma patients' tissue.

    PubMed

    Muff, Roman; Botter, Sander M; Husmann, Knut; Tchinda, Joelle; Selvam, Philomina; Seeli-Maduz, Franziska; Fuchs, Bruno

    2016-07-01

    Human sarcomas comprise a heterogeneous group of rare tumors that affect soft tissues and bone. Due to the scarcity and heterogeneity of these diseases, patient-derived cells that can be used for preclinical research are limited. In this study, we investigated whether the tissue explant technique can be used to obtain sarcoma cell lines from fresh as well as viable frozen tissue obtained from 8 out of 12 soft tissue and 9 out of 13 bone tumor entities as defined by the World Health Organization. The success rate, defined as the percent of samples that yielded sufficient numbers of outgrowing cells to be frozen, and the time to freeze were determined for a total of 734 sarcoma tissue specimens. In 552 cases (75%) enough cells were obtained to be frozen at early passage. Success rates were higher in bone tumors (82%) compared with soft tissue tumors (68%), and the mean time to freezing was lower in bone tumors (65 days) compared with soft tissue tumors (84 days). Overall, from 40% of the tissues cells could be frozen at early passage within <2 month after tissue removal. Comparable results as with fresh tissue were obtained after explant of viable frozen patient-derived material. In a selected number of bone and soft tissue sarcoma entities, conventional karyotyping and/or FISH (fluorescence in situ hybridization) analysis revealed a high amount (>60%) of abnormal cells in 41% of analyzed samples, especially in bone sarcomas (osteosarcoma and Ewing sarcoma). In conclusion, the explant technique is well suited to establish patient-derived cell lines for a large majority of bone and soft tissue sarcoma entities with adequate speed. This procedure thus opens the possibility for molecular analysis and drug testing for therapeutic decision making even during patient treatment. PMID:27111283

  13. Intratumoral oxygen gradients mediate sarcoma cell invasion.

    PubMed

    Lewis, Daniel M; Park, Kyung Min; Tang, Vitor; Xu, Yu; Pak, Koreana; Eisinger-Mathason, T S Karin; Simon, M Celeste; Gerecht, Sharon

    2016-08-16

    Hypoxia is a critical factor in the progression and metastasis of many cancers, including soft tissue sarcomas. Frequently, oxygen (O2) gradients develop in tumors as they grow beyond their vascular supply, leading to heterogeneous areas of O2 depletion. Here, we report the impact of hypoxic O2 gradients on sarcoma cell invasion and migration. O2 gradient measurements showed that large sarcoma mouse tumors (>300 mm(3)) contain a severely hypoxic core [≤0.1% partial pressure of O2 (pO2)] whereas smaller tumors possessed hypoxic gradients throughout the tumor mass (0.1-6% pO2). To analyze tumor invasion, we used O2-controllable hydrogels to recreate the physiopathological O2 levels in vitro. Small tumor grafts encapsulated in the hydrogels revealed increased invasion that was both faster and extended over a longer distance in the hypoxic hydrogels compared with nonhypoxic hydrogels. To model the effect of the O2 gradient accurately, we examined individual sarcoma cells embedded in the O2-controllable hydrogel. We observed that hypoxic gradients guide sarcoma cell motility and matrix remodeling through hypoxia-inducible factor-1α (HIF-1α) activation. We further found that in the hypoxic gradient, individual cells migrate more quickly, across longer distances, and in the direction of increasing O2 tension. Treatment with minoxidil, an inhibitor of hypoxia-induced sarcoma metastasis, abrogated cell migration and matrix remodeling in the hypoxic gradient. Overall, we show that O2 acts as a 3D physicotactic agent during sarcoma tumor invasion and propose the O2-controllable hydrogels as a predictive system to study early stages of the metastatic process and therapeutic targets. PMID:27486245

  14. What's New in Kaposi Sarcoma Research and Treatment?

    MedlinePlus

    ... Next Topic Additional resources for Kaposi sarcoma What’s new in Kaposi sarcoma research and treatment? A great ... once it has developed. Treatment Researchers are studying new and different ways to treat KS. Imiquimod (Aldara) ...

  15. What's New in Uterine Sarcoma Research and Treatment?

    MedlinePlus

    ... Next Topic Additional resources for uterine sarcoma What`s new in uterine sarcoma research and treatment? Molecular pathology ... the chromosomes leads to the formation of a new gene, called JAZF1/JJAZ. This gene may help ...

  16. Pulmonary Kaposi's sarcoma in a female patient: Case report.

    PubMed

    Petribu, Natacha Calheiros de Lima; Cisneiros, Mayana Silva; Carvalho, Glauber Barbosa de; Baptista, Lucyana de Melo

    2016-01-01

    Kaposi's sarcoma (KS) is a multicentric lymphoproliferative malignancy. Most of the time this tumor is confined to the skin and subcutaneous tissue, but it can present with widespread visceral involvement, such as in the lung. Pulmonary KS is the most frequent form in young adult males, in a ratio of 15:1. The disease usually affects individuals with low CD4 lymphocyte counts (<150-200 cells/mm3). We report a case of a female patient aged 35 years, with the presence of skin lesions, self-limiting episodes of diarrhea and weight loss of 15 kg for nearly 9 months, progressing to persistent fever. AIDS was diagnosed and biopsy of the lesions revealed Kaposi's sarcoma. Computed tomography of the chest showed peribronchovascular thickening, areas of ground glass opacity, condensations with air bronchograms surrounded by ground glass opacity (halo sign) and bilateral pleural effusion. The diagnosis of pulmonary KS is still a challenge, especially due to the occurrence of other opportunistic diseases that may also occur concurrently. Therefore, suspecting this diagnosis based on clinical and laboratory manifestations, and even more with CT findings, is fundamental, especially in patients who already have the cutaneous form of the disease. PMID:27656847

  17. Targeted Therapy of Ewing's Sarcoma

    PubMed Central

    Subbiah, Vivek; Anderson, Pete

    2011-01-01

    Refractory and/or recurrent Ewing's sarcoma (EWS) remains a clinical challenge because the disease's resistance to therapy makes it difficult to achieve durable results with standard treatments that include chemotherapy, radiation, and surgery. Recently, insulin-like-growth-factor-1-receptor (IGF1R) antibodies have been shown to have a modest single-agent activity in EWS. Patient selection using biomarkers and understanding response and resistance mechanisms in relation to IGF1R and mammalian target of rapamycin pathways are areas of active research. Since EWS has a unique tumor-specific EWS-FLI1 t(11;22) translocation and oncogenic fusion protein, inhibition of EWS-FLI1 transcription, translation, and/or protein function may be key to eradicating EWS at the stem-cell level. Recently, a small molecule that blocks the protein-protein interaction of EWS-FLI1 with RNA helicase A has been shown in preclinical models to inhibit EWS growth. The successful application of this first-in-class protein-protein inhibitor in the clinic could become a model system for translocation-associated cancers such as EWS. PMID:21052545

  18. Spinal intradural extraosseous Ewing's sarcoma.

    PubMed

    Mateen, Farrah J; Nassar, Aziza; Bardia, Aditya; Jatoi, Aminah; Haddock, Michael G; Buckner, Jan C; Lachance, Daniel H

    2011-03-30

    Extraosseous Ewing's sarcoma (EES) involving the central nervous system is rare, but can be diagnosed and distinguished from other primitive neuroectodermal tumors (PNET) by identification of the chromosomal translocation (11;22)(q24;q12). We report EES arising from the spinal intradural extramedullary space, based on imaging, histopathological, and molecular data in two men, ages 50 and 60 years old and a review of the literature using PubMed (1970-2009). Reverse transcriptase polymerase chain reaction (RT-PCR) identified the fusion product FL1-EWS. Multimodal therapy, including radiation and alternating chemotherapy including vincristine, cyclophosphamide, doxorubicin and ifosfamide and etoposide led to local tumor control and an initial, favorable therapeutic response. No systemic involvement was seen from the time of diagnosis to the time of last follow-up (26 months) or death (4 years). This report confirms that EES is not confined to the earliest decades of life, and like its rare occurrence as an extra-axial meningeal based mass intracranially, can occasionally present as an intradural mass in the spinal canal without evidence of systemic tumor. Gross total resection followed by multimodal therapy may provide for extended progression free and overall survival.

  19. Uterine sarcoma Part II-Uterine endometrial stromal sarcoma: The TAG systematic review.

    PubMed

    Horng, Huann-Cheng; Wen, Kuo-Chang; Wang, Peng-Hui; Chen, Yi-Jen; Yen, Ming-Shyen; Ng, Heung-Tat

    2016-08-01

    Endometrial stromal tumors are rare uterine tumors (<1%). Four main categories include endometrial stromal nodule, low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and uterine undifferentiated sarcoma (UUS). This review is a series of articles discussing the uterine sarcomas. LG-ESS, a hormone-dependent tumor harboring chromosomal rearrangement, is an indolent tumor with a favorable prognosis, but characterized by late recurrences even in patients with Stage I disease, suggesting the requirement of a long-term follow-up. Patients with HG-ESS, based on the identification of YWHAE-NUTM2A/B (YWHAE-FAM22A/B) gene fusion, typically present with advanced stage diseases and frequently have recurrences, usually within a few years after initial surgery. UUS is, a high-grade sarcoma, extremely rare, lacking a specific line of differentiation, which is a diagnosis of exclusion (the wastebasket category, which fails to fulfill the morphological and immunohistochemical criteria of translocation-positive ESS). Surgery is the main strategy in the management of uterine sarcoma. Due to rarity, complex biological characteristics, and unknown etiology and risk factors of uterine sarcomas, the role of adjuvant therapy is not clear. Only LG-ESS might respond to progestins or aromatase inhibitors. PMID:27590366

  20. Current classification, treatment options, and new perspectives in the management of adipocytic sarcomas

    PubMed Central

    De Vita, Alessandro; Mercatali, Laura; Recine, Federica; Pieri, Federica; Riva, Nada; Bongiovanni, Alberto; Liverani, Chiara; Spadazzi, Chiara; Miserocchi, Giacomo; Amadori, Dino; Ibrahim, Toni

    2016-01-01

    Sarcomas are a heterogeneous group of mesenchymal tumors arising from soft tissue or bone, with an uncertain etiology and difficult classification. Soft tissue sarcomas (STSs) account for around 1% of all adult cancers. Till date, more than 50 histologic subtypes have been identified. Adipocyte sarcoma or liposarcoma (LPS) is one of the most common STS subtypes, accounting for 15% of all sarcomas, with an incidence of 24% of all extremity STSs and 45% of all retroperitoneal STSs. The new World Health Organization classification system has divided LPS into four different subgroups: atypical lipomatous tumor/well-differentiated LPS, dedifferentiated LPS, myxoid LPS, and pleomorphic LPS. These lesions can develop at any location and exhibit different aggressive potentials reflecting their morphologic diversity and clinical behavior. Patients affected by LPS should be managed in specialized multidisciplinary cancer centers. Whereas surgical resection is the mainstay of treatment for localized disease, the benefits of adjuvant and neoadjuvant chemotherapy are still unclear. Systemic treatment, particularly chemotherapy, is still limited in metastatic disease. Despite the efforts toward a better understanding of the biology of LPS, the outcome of advanced and metastatic patients remains poor. The advent of targeted therapies may lead to an improvement of treatment options and clinical outcomes. A larger patient enrollment into translational and clinical studies will help increase the knowledge of the biological behavior of LPSs, test new drugs, and introduce new methodological studies, that is, on treatment response. PMID:27785071

  1. Therapeutic opportunities in Ewing sarcoma: EWS-FLI inhibition via LSD1 targeting

    PubMed Central

    Theisen, Emily R.; Pishas, Kathleen I.; Saund, Ranajeet S.; Lessnick, Stephen L.

    2016-01-01

    Ewing sarcoma is an aggressive primary pediatric bone tumor, often diagnosed in adolescents and young adults. A pathognomonic reciprocal chromosomal translocation results in a fusion gene coding for a protein which derives its N-terminus from a FUS/EWS/TAF15 (FET) protein family member, commonly EWS, and C-terminus containing the DNA-binding domain of an ETS transcription factor, commonly FLI1. Nearly 85% of cases express the EWS-FLI protein which functions as a transcription factor and drives oncogenesis. As the primary genomic lesion and a protein which is not expressed in normal cells, disrupting EWS-FLI function is an attractive therapeutic strategy for Ewing sarcoma. However, transcription factors are notoriously difficult targets for the development of small molecules. Improved understanding of the oncogenic mechanisms employed by EWS-FLI to hijack normal cellular programming has uncovered potential novel approaches to pharmacologically block EWS-FLI function. In this review we examine targeting the chromatin regulatory enzymes recruited to conspire in oncogenesis with a focus on the histone lysine specific demethylase 1 (LSD1). LSD1 inhibitors are being aggressively investigated in acute myeloid leukemia and the results of early clinical trials will help inform the future use of LSD1 inhibitors in sarcoma. High LSD1 expression is observed in Ewing sarcoma patient samples and mechanistic and preclinical data suggest LSD1 inhibition globally disrupts the function of EWS-ETS proteins. PMID:26848860

  2. A Tumour in Disguise in the Right Palm- Monophasic Synovial Sarcoma.

    PubMed

    Arumugam, Vasugi Gramani; Sundaram, Sandhya; Ramesh, Anita; Duvuru, Prathiba; Rajappa, Srinivasa

    2016-05-01

    Synovial sarcoma is one of the poorly differentiated malignant soft tissue tumour occuring commonly among young adults in the extremities. We report a 50-year-old female presenting with a soft tissue mass in the right palm. On examination, a single firm and non tender swelling was noticed adjacent to the thenar muscles. Radiology suggested a benign soft tissue lesion. The swelling, clinically thought to be a lipoma, was excised and sent for histopathological examination. Microscopy showed a highly cellular tumour arranged in nests, cords and pseudo glandular pattern separated by dense fibrocollagenous tissue. An interesting and baffling finding was the presence of a distinct mucin vacuole in many of the tumour cells. A diagnosis of soft tissue sarcoma with epithelial features was considered and a panel of immunohistochemical stains done. Tumour cells showed strong positivity for cytokeratin 7, vimentin, EMA & Bcl2. CD 99 and S100 were focally positive. CD 34 and CEA were negative. In view of the above microscopic and immunohistochemical findings, a diagnosis of monophasic synovial sarcoma of epithelial type was rendered. This case is being documented for the rare morphological appearance of mucin vacuoles in a monophasic epithelial type synovial sarcoma. PMID:27437233

  3. A Tumour in Disguise in the Right Palm- Monophasic Synovial Sarcoma.

    PubMed

    Arumugam, Vasugi Gramani; Sundaram, Sandhya; Ramesh, Anita; Duvuru, Prathiba; Rajappa, Srinivasa

    2016-05-01

    Synovial sarcoma is one of the poorly differentiated malignant soft tissue tumour occuring commonly among young adults in the extremities. We report a 50-year-old female presenting with a soft tissue mass in the right palm. On examination, a single firm and non tender swelling was noticed adjacent to the thenar muscles. Radiology suggested a benign soft tissue lesion. The swelling, clinically thought to be a lipoma, was excised and sent for histopathological examination. Microscopy showed a highly cellular tumour arranged in nests, cords and pseudo glandular pattern separated by dense fibrocollagenous tissue. An interesting and baffling finding was the presence of a distinct mucin vacuole in many of the tumour cells. A diagnosis of soft tissue sarcoma with epithelial features was considered and a panel of immunohistochemical stains done. Tumour cells showed strong positivity for cytokeratin 7, vimentin, EMA & Bcl2. CD 99 and S100 were focally positive. CD 34 and CEA were negative. In view of the above microscopic and immunohistochemical findings, a diagnosis of monophasic synovial sarcoma of epithelial type was rendered. This case is being documented for the rare morphological appearance of mucin vacuoles in a monophasic epithelial type synovial sarcoma.

  4. A Tumour in Disguise in the Right Palm- Monophasic Synovial Sarcoma

    PubMed Central

    Arumugam, Vasugi Gramani; Ramesh, Anita; Duvuru, Prathiba; Rajappa, Srinivasa

    2016-01-01

    Synovial sarcoma is one of the poorly differentiated malignant soft tissue tumour occuring commonly among young adults in the extremities. We report a 50-year-old female presenting with a soft tissue mass in the right palm. On examination, a single firm and non tender swelling was noticed adjacent to the thenar muscles. Radiology suggested a benign soft tissue lesion. The swelling, clinically thought to be a lipoma, was excised and sent for histopathological examination. Microscopy showed a highly cellular tumour arranged in nests, cords and pseudo glandular pattern separated by dense fibrocollagenous tissue. An interesting and baffling finding was the presence of a distinct mucin vacuole in many of the tumour cells. A diagnosis of soft tissue sarcoma with epithelial features was considered and a panel of immunohistochemical stains done. Tumour cells showed strong positivity for cytokeratin 7, vimentin, EMA & Bcl2. CD 99 and S100 were focally positive. CD 34 and CEA were negative. In view of the above microscopic and immunohistochemical findings, a diagnosis of monophasic synovial sarcoma of epithelial type was rendered. This case is being documented for the rare morphological appearance of mucin vacuoles in a monophasic epithelial type synovial sarcoma. PMID:27437233

  5. A Radiological Review of Ewing's Sarcoma of Mandible: A Case Report with One Year Follow-up

    PubMed Central

    Thomas, Valsa; Kattoor, Jayasree; Kusumakumari, P

    2013-01-01

    ABSTRACT Ewing's sarcoma (ES) is an uncommon round cell tumor with an aggressive course affecting mainly children and young adults. Only 1% of cases is reported with jaw involvement and have mandibular predilection. Radiographic finding in ES reflect many destructive nature of the lesion, like osteolysis, cortical erosion, periostitis and soft tissue mass. A case of ES of the mandible is reported with special consideration to the radiological appearance. How to cite this article: Krishna KBB, Thomas V, Kattoor J, Kusumakumari P. A Radiological Review of Ewing's Sarcoma of Mandible: A Case Report with One Year Follow-up. Int J Clin Pediatr Dent 2013;6(2):109-114. PMID:25206203

  6. Primary pulmonary synovial sarcoma: a rare neoplasm

    PubMed Central

    Ramos, Montserrat Blanco; Fontán, Eva María García; Carretero, Miguel Ángel Cańizares; Pińeiro, Ana González

    2016-01-01

    Primary pulmonary synovial sarcoma is an extremely rare tumor with an unknown cause. The diagnosis is established after other primary lung malignancies or metastatic extrathoracic sarcoma have been excluded. We report the case of a 69-year-old man who presented with a well-defined mass in the right upper lobe on a chest X-ray. A video-assisted thoracoscopic surgery (VATS) right upper lobectomy was performed. Immunohistochemically, neoplastic cells were positive for vimentin, CD56 and Bcl-2, and focally positive for CD99, epithelial membrane antigen and cytokeratin 7 and 19. The cytogenetic study revealed a SYT genetic reassortment. So, the final pathological diagnosis was primary pulmonary synovial sarcoma. PMID:27516790

  7. Primary pulmonary synovial sarcoma: a rare neoplasm.

    PubMed

    García, José Soro; Ramos, Montserrat Blanco; Fontán, Eva María García; Carretero, Miguel Ángel Cańizares; Pińeiro, Ana González

    2016-06-01

    Primary pulmonary synovial sarcoma is an extremely rare tumor with an unknown cause. The diagnosis is established after other primary lung malignancies or metastatic extrathoracic sarcoma have been excluded. We report the case of a 69-year-old man who presented with a well-defined mass in the right upper lobe on a chest X-ray. A video-assisted thoracoscopic surgery (VATS) right upper lobectomy was performed. Immunohistochemically, neoplastic cells were positive for vimentin, CD56 and Bcl-2, and focally positive for CD99, epithelial membrane antigen and cytokeratin 7 and 19. The cytogenetic study revealed a SYT genetic reassortment. So, the final pathological diagnosis was primary pulmonary synovial sarcoma. PMID:27516790

  8. Osteosarcoma With Apparent Ewing Sarcoma Gene Rearrangement.

    PubMed

    Mathias, Melissa D; Chou, Alexander J; Meyers, Paul; Shukla, Neerav; Hameed, Meera; Agaram, Narasimhan; Wang, Lu; Berger, Michael F; Walsh, Michael; Kentsis, Alex

    2016-07-01

    Poorly differentiated round cell sarcomas present diagnostic challenges because of their variable morphology and lack of specific immunophenotypic markers. We present a case of a 15-year-old female with a tibial tumor that exhibited features of Ewing-like sarcoma, including apparent rearrangement of the EWSR1 gene. Hybridization capture-based next-generation DNA sequencing showed evidence of complex genomic rearrangements, absence of known pathogenic Ewing-like chromosome translocations, and deletions RB1, PTCH1, and ATRX, supporting the diagnosis of osteosarcoma. This illustrates the potential of clinical genomic profiling to improve diagnosis and enable specifically targeted therapies for cancers with complex pathologies. PMID:27352193

  9. [Sarcomas, example of a pathologist network organization].

    PubMed

    Neuville, Agnes; Coindre, Jean-Michel

    2013-12-01

    Sarcomas are rare and heterogeneous with many subtypes explaining the high level of diagnostic difficulty with frequent important therapeutic consequences. In 2009, a national network of pathologists has been set up with the main objective to perform a systematic histological review of every new sarcoma, gastro-intestinal stromal tumor (GIST) and desmoid tumor. We describe the network organization and report the results of the first two years of activity. These results clearly show the interest of this organization for the patients as well as for all pathologists. Moreover, data and material collect allows a better knowledge of these tumors and an improvement of the rules for their diagnostic management.

  10. Retroperitoneal Sarcoma: Fact, Opinion, and Controversy.

    PubMed

    Gladdy, Rebecca A; Gupta, Abha; Catton, Charles N

    2016-10-01

    After diagnosis of retroperitoneal sarcoma (RPS), detailed imaging and multidisciplinary discussion should guide treatment including surgical resection and in select cases, neoadjuvant therapy. Local recurrence is common in RPS and is associated with grade, histologic subtype, completeness of resection, and size. As guidelines to standardize RPS patient management emerge, expert pathologic assessment and management in centers of excellence are benchmarks of quality of care. The efficacy of current chemotherapy is limited and there is a critical need to understand the molecular basis of sarcoma so that new drug therapies are developed. Multicenter clinical trials are needed to limit opinion and controversy in this complex and challenging disease. PMID:27591493

  11. Systemic Therapy for Advanced Soft Tissue Sarcoma.

    PubMed

    Sheng, Jennifer Y; Movva, Sujana

    2016-10-01

    Soft tissue sarcomas are rare tumors that present with distant metastasis in up to 10% of patients. Survival has improved significantly because of advancements in histologic classification and improved management approaches. Older agents such as doxorubicin, ifosfamide, gemcitabine, and paclitaxel continue to demonstrate objective response rates from 18% to 25%. Newer agents such as trabectedin, eribulin, aldoxorubicin, and olaratumab have demonstrated improvements in progression-free survival, overall survival, or toxicity profiles. Future studies on treatment of advanced soft tissue sarcoma will continue to concentrate on reducing toxicity, personalization of therapy, and targeting novel pathways. PMID:27542647

  12. Primary cutaneous Ewing sarcoma--case report.

    PubMed

    Oliveira Filho, Jayme de; Tebet, Ana Carolina Franco; Oliveira, Anna Rita Ferrante Mitidieri de; Nasser, Kassila

    2014-01-01

    Ewing sarcoma is a primitive neuroectodermal tumor rarely occurs in the skin and sobcutaneous tissues. Generally Ewing's sarcoma is a primary bone tumor, but when present in soft tissues it characterizes an extremely uncommon clinical picture. It usually involves the deep subcutaneous tissue or muscles, and more rarely occurs like a primary skin cancer. Most patients are white, women, and in the second decade of life. The clinical features are a superficial mass, in average measuring 2-3 cm, of soft consistency, freely mobile and sometimes painful. The more affected locations are upper and lower extremities, trunk, head, neck or multiple lesions. The presence of metastases is very rare. PMID:24937829

  13. State-of-the-art approach for bone sarcomas.

    PubMed

    Mavrogenis, Andreas F; Angelini, Andrea; Vottis, Christos; Palmerini, Emanuela; Rimondi, Eugenio; Rossi, Giuseppe; Papagelopoulos, Panayiotis J; Ruggieri, Pietro

    2015-01-01

    Bone sarcomas are a variety of non-epithelial, malignant neoplasms of bone. The most common bone sarcomas are osteosarcoma, Ewing's sarcoma, and chondrosarcoma. The approach to a patient with a suspected bone sarcoma from initial examination to the histological diagnosis and classification is staging. Staging is of critical importance, in order to classify different treatment options and point out which combination of them is more suitable depending on the severity of the tumor in every individual patient. Staging should include medical history, physical and imaging examination, and biopsy. This article presents the current approach for staging, principles of biopsy, tumor classification, treatment, and follow-up of patients with bone sarcomas.

  14. Synovial sarcoma of kidney in a child: A rare presentation

    PubMed Central

    Radhakrishnan, Venkatraman; Dhanushkodi, Manikandan; Narayanswamy, Kathiresan; Raja, Anand; Sundersingh, Shirley; Sagar, Tenali

    2016-01-01

    There are no reported cases in the literature of primary renal synovial sarcoma in pediatric patients. The management of renal synovial sarcoma has been extrapolated from the management of soft tissue sarcomas at other sites. We present a 4-year-old female who was suspected to have Wilms’ tumor. The patient underwent guided biopsy as she did not respond to neoadjuvant chemotherapy for Wilms’ tumor. The biopsy was consistent with primary renal synovial sarcoma. The child was treated with change in her neoadjuvant chemotherapy regimen and surgery. The diagnosis of synovial sarcoma was confirmed by demonstrating the t (X, 18) translocation using polymerase chain reaction. PMID:27046979

  15. Head and Neck Soft Tissue Sarcomas Treated with Radiation Therapy

    PubMed Central

    Vitzthum, Lucas K.; Brown, Lindsay C.; Rooney, Jessica W.; Foote, Robert L.

    2016-01-01

    Head and neck soft tissue sarcomas (HNSTSs) are rare and heterogeneous cancers in which radiation therapy (RT) has an important role in local tumor control (LC). The purpose of this study was to evaluate outcomes and patterns of treatment failure in patients with HNSTS treated with RT. A retrospective review was performed of adult patients with HNSTS treated with RT from January 1, 1998, to December 31, 2012. LC, locoregional control (LRC), disease-free survival (DFS), overall survival (OS), and predictors thereof were assessed. Forty-eight patients with HNSTS were evaluated. Five-year Kaplan-Meier estimates of LC, LRC, DFS, and OS were 87, 73, 63, and 83%, respectively. Angiosarcomas were found to be associated with worse LC, LRC, DFS, and OS. Patients over the age of 60 had lower rates of DFS. HNSTSs comprise a diverse group of tumors that can be managed with various treatment regimens involving RT. Angiosarcomas have higher recurrence and mortality rates. PMID:27441072

  16. Hyperthermia in soft tissue sarcoma.

    PubMed

    Lindner, Lars H; Issels, Rolf D

    2011-03-01

    Patients with high-risk soft tissue sarcomas (STS)-FNCLCC grade 2-3, size >5 cm, deep to the fascia-are at risk for developing local recurrence and distant metastasis despite surgical tumor resection. Therefore, the management of high-risk STS requires a multidisciplinary approach. Besides surgery, radiotherapy, and chemotherapy, regional hyperthermia (RHT) has the potential to become the fourth standard treatment modality for the treatment of these patients. RHT means non-invasive selective heating of the tumor area to temperatures within the range of 40-43°C for 60 min by the use of an electromagnetic heating device. Thereby RHT is always applied in addition to radiotherapy or chemotherapy or both but is not effective as a single treatment. Beside direct cytotoxicity, RHT in combination with chemotherapy enhances the drug cytotoxicity mainly by increased chemical reaction and intratumoral drug accumulation. For the neoadjuvant setting, RHT in combination with a doxorubicin- and ifosfamide-based chemotherapy has been shown to dramatically improve the tumor response rate but also prevents from early disease progression as compared to chemotherapy alone. The addition of RHT to a multimodal treatment of high-risk STS consisting of surgery, radiotherapy, and chemotherapy either in the neoadjuvant setting but also after incomplete or marginal tumor resection has been shown to significantly improve local recurrence- and disease-free survival. Based on these results and in conjunction with the low RHT-related toxicity, RHT combined with preoperative or postoperative chemotherapy should be considered as an additional standard treatment option for the multidisciplinary treatment of locally advanced high-grade STS.

  17. A Biphasic Pleural Tumor with Features of an Epithelioid and Small Cell Mesothelioma: Morphologic and Molecular Findings

    PubMed Central

    2016-01-01

    Malignant mesotheliomas are generally classified into epithelioid, sarcomatoid, desmoplastic, and biphasic types with rare reports of a small cell form. These small cell variants display some morphologic overlap with desmoplastic small round cell tumors (DSRCTs) which generally occur within the abdominal cavity of young males and are defined by a characteristic t(11;22)(p13;q12) translocation. However, there are rare reports of DSRCTs lacking this translocation. We present a 78-year-old man with a pleura-based biphasic neoplasm with features of both epithelioid mesothelioma and a small cell blastema-like neoplasm. The epithelioid portion showed IHC reactivity for pan cytokeratin, CK5/6, D2-40, and calretinin and the small cell portion marked with CD99, pan cytokeratin, WT1, FLI1, S100, CD200, MyoD1, and CD15. Fluorescence in situ hybridization testing for the t(11;22)(p13;q12) translocation disclosed loss of the EWSR1 gene in 94% of tumor cell nuclei, but there was no evidence of the classic translocation. Array based-comparative genomic hybridization (a-CGH) confirmed the tumor had numerous chromosome copy number losses, including 11p15.5-p11.12 and 22q12.1-q13.33, with loss of the EWSR1 and WT1 gene regions. Herein, we report novel complex CGH findings in a biphasic tumor and review the molecular genetic alterations in both mesothelioma and DSRCTs. PMID:27403364

  18. High ALDH Activity Identifies Chemotherapy-Resistant Ewing's Sarcoma Stem Cells That Retain Sensitivity to EWS-FLI1 Inhibition

    PubMed Central

    Gul, Naheed; Katuri, Varalakshmi; O'Neill, Alison; Kong, Yali; Brown, Milton L.; Toretsky, Jeffrey A.; Loeb, David M.

    2010-01-01

    Background Cancer stem cells are a chemotherapy-resistant population capable of self-renewal and of regenerating the bulk tumor, thereby causing relapse and patient death. Ewing's sarcoma, the second most common form of bone tumor in adolescents and young adults, follows a clinical pattern consistent with the Cancer Stem Cell model – remission is easily achieved, even for patients with metastatic disease, but relapse remains frequent and is usually fatal. Methodology/Principal Findings We have isolated a subpopulation of Ewing's sarcoma cells, from both human cell lines and human xenografts grown in immune deficient mice, which express high aldehyde dehydrogenase (ALDHhigh) activity and are enriched for clonogenicity, sphere-formation, and tumor initiation. The ALDHhigh cells are resistant to chemotherapy in vitro, but this can be overcome by the ATP binding cassette transport protein inhibitor, verapamil. Importantly, these cells are not resistant to YK-4-279, a small molecule inhibitor of EWS-FLI1 that is selectively toxic to Ewing's sarcoma cells both in vitro and in vivo. Conclusions/Significance Ewing's sarcoma contains an ALDHhigh stem-like population of chemotherapy-resistant cells that retain sensitivity to EWS-FLI1 inhibition. Inhibiting the EWS-FLI1 oncoprotein may prove to be an effective means of improving patient outcomes by targeting Ewing's sarcoma stem cells that survive standard chemotherapy. PMID:21085683

  19. The epidemiology of classic, African, and immunosuppressed Kaposi's sarcoma.

    PubMed

    Wahman, A; Melnick, S L; Rhame, F S; Potter, J D

    1991-01-01

    The etiology of Kaposi's sarcoma remains somewhat obscure. While lesions of classic Kaposi's sarcoma, African Kaposi's sarcoma, and immunosuppressed Kaposi's sarcoma have been found to be indistinguishable from one another, the reasons for the variations in type and severity have not been established. The origin of the spindle cell is yet to be agreed on. Geographic variation does not seem as important as ethnic variation. The very young and the very old, perhaps two ages of weakened immunity, tend to have a higher incidence of Kaposi's sarcoma. Children and AIDS patients tend to develop more virulent disease. Males tend to get Kaposi's sarcoma at higher rates than do females. Jewish and Mediterranean males have the highest incidence of classic Kaposi's sarcoma, and African Bantu have the highest incidence of African Kaposi's sarcoma, classifications which do not apply to the Kaposi's sarcoma population in the United States. Male homosexuals have much higher incidence of Kaposi's sarcoma than do male heterosexuals, but since the early 1980s, its incidence as the presenting manifestation of AIDS has decreased dramatically. There is no unequivocal association with HLA haplotype (though DR5 carriers may be at especially high risk) or evidence of family clustering. There is an impressive but not always consistent association between Kaposi's sarcoma development and immunodeficiency. Environmental factors, such as nitrite use, immunosuppression, and repeated cytomegalovirus infection, are associated with Kaposi's sarcoma, but the exact mechanism is unclear and the associations remain inconsistent. Finally, it is still unclear if there is a causative infectious agent for Kaposi's sarcoma. While cytomegalovirus has been linked to Kaposi's sarcoma, there are weaknesses in its hypothetical role as an etiologic agent as is the case for HIV itself.(ABSTRACT TRUNCATED AT 400 WORDS)

  20. Spindle Cell Sarcoma Presenting as Pancoast Syndrome.

    PubMed

    Badshah, Aliena; Khan, Salman; Saeed, Usman

    2016-07-01

    This report describes a patient who presented with pancoast syndrome, secondary to spindle cell sarcoma of the lung. A 56-year man presented with dyspnea, engorged neck veins and bilateral upper limb pitting edema. The patient also had ptosis and miosis in the right eye. Right ulnar nerve palsy with atrophy of hand muscles was seen. His chest X-ray showed bilateral pleural effusion with an opacity involving the apex of the right lung along with mediastinal widening. Echocardiography revealed a pericardial effusion which was drained. The patient's CTscan of chest strongly suspected a malignant mass in right upper lobe with extensive mediastinal lymphadenopathy, pleural metastases and pericardial involvement. He was started on oxygen inhalation, dexamethasone, and clopidogrel. Bronchoscopic biopsy confirmed the diagnosis of spindle cell sarcoma. Meanwhile, he was advised radiotherapy. The tumour was not amenable to surgery. Spindle cell sarcoma is a rare connective tissue tumor that replicates rapidly. To the best of the authors' knowledge, it is hereby reported the first case of spindle cell sarcoma of the lung presenting as Pancoast syndrome. PMID:27504558

  1. Feline postvaccinal sarcoma: 20 years later

    PubMed Central

    Wilcock, Brian; Wilcock, Anne; Bottoms, Katherine

    2012-01-01

    Comparison of the annual prevalence of feline postvaccinal sarcomas among 11 609 feline skin mass submissions from 1992 to 2010 revealed no decrease in disease prevalence or increase in the age of affected cats in response to changes in vaccine formulation or recommended changes in feline vaccination protocols. PMID:23024394

  2. Instantánea del sarcoma

    Cancer.gov

    Información sobre las tendencias de incidencia, mortalidad y financiamiento del NCI sobre el sarcoma; así como ejemplos de actividades del NCI y adelantos en la investigación de este tipo de cáncer.

  3. ZFP36-FOSB Fusion Defines a Subset of Epithelioid Hemangioma with Atypical Features

    PubMed Central

    Antonescu, Cristina R; Chen, Hsiao-Wei; Zhang, Lei; Sung, Yun-Shao; Panicek, David; Agaram, Narasimhan P; Dickson, Brendan C; Krausz, Thomas; Fletcher, Christopher D

    2014-01-01

    Epithelioid hemangioma (EH) is a benign neoplasm with distinctive vasoformative features, which occasionally shows increased cellularity, cytologic atypia, and/or loco-regional aggressive growth, resulting in challenging differential diagnosis from malignant vascular neoplasms. Based on two intra-osseous EH index cases with worrisome histologic features, such as the presence of necrosis, RNA sequencing was applied for possible fusion gene discovery and potential subclassification of a novel atypical EH subset. A ZFP36-FOSB fusion was detected in one case, while a WWTR1-FOSB chimeric transcript in the other, both were further validated by FISH and RT-PCR. These abnormalities were then screened by FISH in 44 EH from different locations with 7 additional EH revealing FOSB gene rearrangements, all except one being fused to ZFP36. Interestingly, 4/6 penile EH studied showed FOSB abnormalities. Although certain atypical histologic features were observed in the FOSB-rearranged EH, including solid growth, increased cellularity, mild to moderate nuclear pleomorphism, and necrosis in 3/9 cases, no overt sarcomatous areas were discerned to objectively separate the lesions from the fusion-negative EH. No patient has developed recurrence to date, but the follow-up was relatively limited and short to draw definitive conclusions regarding behavior. Although FOSB-rearranged EH do not show significant morphologic overlap with SERPINE1-FOSB fusion-positive pseudomyogenic hemangioendothelioma, FOSB oncogenic activation is emerging as an important event in these benign and intermediate groups of vascular tumors. PMID:25043949

  4. Perivascular epithelioid cell tumour of the vagina in an 8 year old girl.

    PubMed

    Ong, Lin Yin; Hwang, Wei Sek; Wong, Adelina; Chan, Mei Yoke; Chui, Chan Hon

    2007-03-01

    Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor with an unpredictable natural history. We present the first reported case of PEComa of the vagina diagnosed in an 8-year-old girl. This was initially diagnosed on biopsy as an embryonal rhabdomyosarcoma, and the patient underwent 3 cycles of chemotherapy according to the Mesenchymal Malignant Tumour 1989 protocol. A repeat scan, however, revealed an increase in tumor size. Total resection was achieved via a transvaginal (vertical sleeve) partial vaginectomy. The resected tumor had the typical appearance of a PEComa. Immunostains showed positive staining of tumor cells for HMB45, neuron-specific enolase, and calponin but were negative for S100 protein, melaninA, chromogranin, smooth muscle actin, desmin, caldesmon, epithelial membrane antigen, AE1/3, and synaptophysin. Six months after surgery, she remains free of tumor and is on follow-up with regular local examinations under anesthesia and computed tomography scans. Our case highlights the importance of an accurate initial diagnosis, and we stress the importance of regular surveillance over a significant period. PMID:17336201

  5. Bone multicentric epithelioid hemangioendothelioma of the lower and upper extremities with pulmonary metastases: A case report

    PubMed Central

    ZHANG, HUA; FU, YANBIAO; YE, ZHAOMING

    2015-01-01

    The present study reports a rare case of bone multicentric epithelioid hemangioendothelioma (EH) involving the upper and lower extremities simultaneously, with visceral involvement of the lung. Osteolytic lesions were first identified in the right distal femur and proximal tibia. Slight increased radionuclide uptake was observed in the right shoulder joint on bone scintigraphy, however, this was ignored, as no clinical symptoms were present. The patient was initially misdiagnosed with multifocal chondroblastoma, and an extra-articular curettage of lesions was performed in the proximal tibia and medial femoral condyle, which was filled with bone cement. The histopathological diagnosis was corrected post-operatively following immunohistochemical analysis, which indicated EH, and subsequently, an amputation of the right leg at thigh level was performed. In addition, multiple lytic lesions in the right shoulder joint and pulmonary metastases were identified on whole-body radiological examination. Radiotherapy was administered to the right shoulder joint, however, the patient refused chemotherapy or further surgery. At 15 months after the initial surgery, the patient currently remains alive. This case indicates that an improved understanding with regard to the clinical features of this disease may prevent misdiagnosis and improve EH treatment. PMID:26137035

  6. Epithelioid hemangioendothelioma of the spine treated with RapidArc volumetric-modulated radiotherapy

    SciTech Connect

    Guy, Jean-Baptiste; Trone, Jane-Chloé; Chargari, Cyrus; Falk, Alexander Tuan; Khodri, Mustapha; Magné, Nicolas

    2014-10-01

    Radiotherapy for epithelioid hemangioendothelioma (EHE) using volumetric intensity-modulated arc radiotherapy (VMAT). A 48-year-old woman was referred for curative irradiation of a vertebral EHE after failure of surgery. A comparison between VMAT and conventional conformal tridimensional (3D) dosimetry was performed and potential advantage of VMAT for sparing critical organs from irradiation's side effects was discussed. The total delivered dose on the planning target volume was 54 Gy in 27 fractions. The patient was finally treated with VMAT. The tolerance was excellent. There was no acute toxicity, including no increase in pain. With a follow-up of 18 months, no delayed toxicity was reported. The clinical response consisted of a decrease in the dorsal pain. The D{sub max} for the spinal cord was reduced from 55 Gy (3D-radiotherapy [RT]) (which would be an unacceptable dose to the spine because of the risk of myelopathy) to 42.8 Gy (VMAT), which remains below the recommended dose threshold (45 Gy). The dose delivered to 20% of organ volume (D{sub 20}) was reduced from 47 Gy (3D-RT) to 3 Gy (VMAT) for the spinal cord. The study shows that VMAT allows the delivery of curative treatment for vertebral EHEs because of critical organ sparing.

  7. Diffuse hepatic epithelioid hemangioendothelioma developed in a patient with hepatitis C cirrhosis.

    PubMed

    Baron, Pedro W; Amankonah, Thomas; Cubas, Robert F; Kore, Arputharaj H; Elihu, Arvand; de Vera, Michael E; Perez, Mia C N

    2014-01-01

    Hepatic epithelioid hemangioendothelioma (HEHE) is an infrequent vascular tumor of endothelial origin that primarily occurs in women in the mid-fifth decade of life without underlying chronic liver disease or cirrhosis. Liver transplant should be the first-line of therapy in patients with large or diffuse unresectable tumors even in the presence of metastatic disease due to the favorable long-term outcome. We report the case of a 48-year-old female who complained of abdominal pain and weight loss. She has a history of cirrhosis secondary to chronic hepatitis C (HCV) and was treated with interferon and ribavirin with sustained virological response. Her work-up revealed multiple confluent infiltrating bilobar liver masses diagnosed as HEHE. She underwent a successful liver transplant without evidence of recurrent HCV infection. She developed cervical spine (C4-C6) HEHE metastases 4 years after transplant. She underwent surgical resection and local radiotherapy after resection with good clinical response. To the best of our knowledge, this is the first report of HEHE that developed in a patient with HCV cirrhosis successfully treated with antiviral therapy before transplant and liver transplant with good allograft function without evidence of recurrent liver tumor or HCV infection but developed metastases to the cervical spine 4 years after transplant. PMID:25276467

  8. Synchronous Hepatic Epithelioid Hemangioendothelioma and Hepatocellular Carcinoma: First Case Report in the Literature and Challenges.

    PubMed

    Athanasopoulos, Panagiotis G; Hadjittofi, Christopher; Luong, Tu Vinh; O'Beirne, James; Sharma, Dinesh

    2015-08-01

    We would like to report the first case in English literature, to the best of our knowledge, of a synchronous hepatic epithelioid hemangioendothelioma (HEHE) and hepatocellular carcinoma (HCC), as well as to address the current trends and challenges in the management of HEHE.An otherwise well 58-year-old man was referred to his local hepatology service with elevated serum γ-GT levels. Imaging revealed bilobar liver lesions consistent with HEHE, a discrete left lobe lesion suspected as HCC, and multiple pulmonary nodules. Biopsies confirmed HEHE with pulmonary metastases. After multidisciplinary team discussions, the patient was admitted under our team and underwent an uneventful laparoscopic left lateral hepatectomy for suspected HCC, which was confirmed histologically.As part of a watch-and-wait approach to metastatic HEHE, in the first follow-up (3 months postoperatively) the patient was clinically fine and the surveillance CT scan did not show recurrent disease.By presenting this case, we aim to raise awareness that this rare entity can coexist with others, potentially complicating their management. PMID:26313777

  9. Surgical Therapy for a Solitary Form of Hepatic Epithelioid Hemangioendothelioma: A Long-Term Survival Case.

    PubMed

    Oshima, Nobu; Terajima, Hiroaki; Hosotani, Ryo

    2009-01-01

    Hepatic epithelioid hemangioendothelioma (HEHE) is a rare neoplasm of vascular origin. The clinical presentation of HEHE is variable, and the therapeutic criteria are still unclear since its natural history is unpredictable. A 53-year-old woman was admitted to our hospital because she had a 2.5-cm diameter nodule in the segment V of the liver. She had undergone segmental hepatectomy for solitary HEHE in segment VII 10 years before. There had been no recurrence for the 10 years after the first operation. The tumor was diagnosed as a new lesion of HEHE by percutaneous needle liver biopsy, and thereafter repeated hepatectomy was performed. HEHE seems to be resistant to chemotherapy and radiotherapy. Either surgical resection or orthotopic liver transplantation is generally recommended as a curative treatment for this disease. However, HEHE tends to be detected in multiple lesions, and localized disease is rare. Therefore, the chance of resection is very low. Some reports do not recommend local resection because of early aggressive tumor spread even after curative resection. We herein demonstrate a rare case of HEHE in a patient who underwent repeated hepatectomy for a solitary lesion and who survived for 17 years. It is concluded that surgical resection is one of the most effective treatments for a solitary form of HEHE. PMID:21103278

  10. Diffuse Hepatic Epithelioid Hemangioendothelioma Developed in a Patient with Hepatitis C Cirrhosis

    PubMed Central

    Baron, Pedro W.; Amankonah, Thomas; Cubas, Robert F.; Kore, Arputharaj H.; Elihu, Arvand; de Vera, Michael E.; Perez, Mia C. N.

    2014-01-01

    Hepatic epithelioid hemangioendothelioma (HEHE) is an infrequent vascular tumor of endothelial origin that primarily occurs in women in the mid-fifth decade of life without underlying chronic liver disease or cirrhosis. Liver transplant should be the first-line of therapy in patients with large or diffuse unresectable tumors even in the presence of metastatic disease due to the favorable long-term outcome. We report the case of a 48-year-old female who complained of abdominal pain and weight loss. She has a history of cirrhosis secondary to chronic hepatitis C (HCV) and was treated with interferon and ribavirin with sustained virological response. Her work-up revealed multiple confluent infiltrating bilobar liver masses diagnosed as HEHE. She underwent a successful liver transplant without evidence of recurrent HCV infection. She developed cervical spine (C4-C6) HEHE metastases 4 years after transplant. She underwent surgical resection and local radiotherapy after resection with good clinical response. To the best of our knowledge, this is the first report of HEHE that developed in a patient with HCV cirrhosis successfully treated with antiviral therapy before transplant and liver transplant with good allograft function without evidence of recurrent liver tumor or HCV infection but developed metastases to the cervical spine 4 years after transplant. PMID:25276467

  11. Surgical Therapy for a Solitary Form of Hepatic Epithelioid Hemangioendothelioma: A Long-Term Survival Case

    PubMed Central

    Oshima, Nobu; Terajima, Hiroaki; Hosotani, Ryo

    2009-01-01

    Hepatic epithelioid hemangioendothelioma (HEHE) is a rare neoplasm of vascular origin. The clinical presentation of HEHE is variable, and the therapeutic criteria are still unclear since its natural history is unpredictable. A 53-year-old woman was admitted to our hospital because she had a 2.5-cm diameter nodule in the segment V of the liver. She had undergone segmental hepatectomy for solitary HEHE in segment VII 10 years before. There had been no recurrence for the 10 years after the first operation. The tumor was diagnosed as a new lesion of HEHE by percutaneous needle liver biopsy, and thereafter repeated hepatectomy was performed. HEHE seems to be resistant to chemotherapy and radiotherapy. Either surgical resection or orthotopic liver transplantation is generally recommended as a curative treatment for this disease. However, HEHE tends to be detected in multiple lesions, and localized disease is rare. Therefore, the chance of resection is very low. Some reports do not recommend local resection because of early aggressive tumor spread even after curative resection. We herein demonstrate a rare case of HEHE in a patient who underwent repeated hepatectomy for a solitary lesion and who survived for 17 years. It is concluded that surgical resection is one of the most effective treatments for a solitary form of HEHE. PMID:21103278

  12. Recurrence of Epithelioid Hemangioendothelioma during Pregnancy: Case Report and Systematic Review

    PubMed Central

    McCulloch, Michael; Russin, Michael; Nachat, Arian

    2016-01-01

    Introduction: Epithelioid hemangioendothelioma (EHE) is a family of blood vessel tumors originating in blood vessels, bone, brain, kidney, liver, and lung. EHE is more common in women, and chemotherapy, radiation, and surgery have brought few successes. Case presentation: We present a case of a 28-year-old woman whose EHE recurred during pregnancy, suggesting hormonal involvement. We conducted a systematic review to provide analysis and interpretation of the potential significance of her disease recurring, with fatal outcome, during pregnancy. Discussion: Very little research has explored the use of individual hormonal markers. Strongly positive expression of placenta growth factor (PlGF) and 17-beta estradiol receptors have been reported. Expression of PlGF is noteworthy in our case, in that our patient’s disease quickly and dramatically flared in the 25th week of pregnancy, near the peak in maternal PlGF production. PlGF binds to vascular endothelial growth factor-1 (VEGF-1), and PlGF may accelerate VEGF-induced angiogenesis. Taken together, these factors may explain our patient’s EHE recurrence and rapid flare-up during pregnancy. Treatment of EHE with VEGF inhibition, potentially in combination with other antiangiogenic and tumor-inhibiting therapies such as lenalidomide, thalidomide, sorafenib, and sunitinib, may also hold promise. PMID:27479949

  13. Monoclonality of multifocal epithelioid hemangioendothelioma of the liver by analysis of WWTR1-CAMTA1 breakpoints.

    PubMed

    Errani, Costantino; Sung, Yun Shao; Zhang, Lei; Healey, John H; Antonescu, Cristina R

    2012-01-01

    Similar to other vascular tumors, epithelioid hemangioendothelioma (EHE) can have multifocal presentation in up to 50% of cases. However, whether multifocal EHE represents an unusual pattern of metastasis or multiple separate primary tumors remains to be elucidated. Our recent identification of a WWTR1-CAMTA1 fusion as the genetic hallmark of EHE irrespective of anatomic location was used to clarify this question by comparing the similarity of translocation breakpoints. In our previous study, we found variability of the fusion transcripts of the t(1;3)(p36;q25) translocation among different patients with EHE. Thus, we undertook a molecular analysis of six samples from two patients with multicentric hepatic EHE to test our hypothesis that the presence of identical breakpoints in WWTR1 and CAMTA1 support the monoclonal nature of multifocal EHE. Using reverse transcription-polymerase chain reaction (RT-PCR) and subsequent sequencing, we confirmed an identical WWTR1-CAMTA1 fusion transcript product from different nodules in each patient. Our results confirm that multifocal EHE are monoclonal and thus represent metastatic implants of the same neoplastic clone rather than a "field-effect" or synchronous occurrence of multiple neoplastic clones. PMID:22429593

  14. Hepatic epithelioid hemangioendothelioma metastasized to the peritoneum, omentum and mesentery: a case report.

    PubMed

    Gurung, Sanita; Fu, Hua; Zhang, Wei Wen; Gu, Yong Hong

    2015-01-01

    Epithelioid hemangioendothelioma (EHAE) is a malignant vascular tumor derived from endothelial cell often misdiagnosed as Hepatic carcinoma on the basis of radiological features. Till now etiology of this rare curiosity is unknown but it is related with use of oral contraceptives pills (OCP), liver trauma, exposure to vinyl chloride and hepatitis. We herein report on a case which failed to be diagnosed by cytopathology, computed tomography (CT) and magnetic resonance imaging (MRI). Patient was a 46 yr old man presented with abdominal distension for a month. Initial liver function test (LFT) was increased whereas renal function test (RFT) and alpha-fetoprotein (AFP) were normal. His abdominal ultrasound revealed multiple hypoechoic nodules and multiple liver calcifications. Subsequently laparoscopic omental biopsy and Ultrasound guided liver biopsy was done showing the neoplastic cells scattered in fibrous stroma. The immunohistochemistry for endothelial tumor cells stained positive for Vimentin (+++), CD10 (+++), CD34 (++), CD31 (+), Factor VIII antigen (focal) (+) and low proliferative activity for ki-67. Our case is very interesting in which patient admitted with nonspecific symptoms of abdominal pain and diagnosed to be a Malignant Hepatic EHAE metastasized to the peritoneum, omentum and mesentery. The patient was on thalidomide 50 mg/day and increased to 100 mg/day. 5-Flurouracil (FU) intraperitoneal chemotherapy and other symptomatic and supportive treatment was given to the patient. Our case highlights on the importance of immunohistopathological diagnosis, compare the radiological findings of this disease and discuss the treatment strategy with review of available literature. PMID:26191313

  15. The Challenges of Detecting Circulating Tumor Cells in Sarcoma.

    PubMed

    Tellez-Gabriel, Marta; Brown, Hannah K; Young, Robin; Heymann, Marie-Françoise; Heymann, Dominique

    2016-01-01

    Sarcomas are a heterogeneous group of malignant neoplasms of mesenchymal origin, many of which have a propensity to develop distant metastases. Cancer cells that have escaped from the primary tumor are able to invade into surrounding tissues, to intravasate into the bloodstream to become circulating tumor cells (CTCs), and are responsible for the generation of distant metastases. Due to the rarity of these tumors and the absence of specific markers expressed by sarcoma tumor cells, the characterization of sarcoma CTCs has to date been relatively limited. Current techniques for isolating sarcoma CTCs are based on size criteria, the identification of circulating cells that express either common mesenchymal markers, sarcoma-specific markers, such as CD99, CD81, or PAX3, and chromosomal translocations found in certain sarcoma subtypes, such as EWS-FLI1 in Ewing's sarcoma, detection of osteoblast-related genes, or measurement of the activity of specific metabolic enzymes. Further studies are needed to improve the isolation and characterization of sarcoma CTCs, to demonstrate their clinical significance as predictive and/or prognostic biomarkers, and to utilize CTCs as a tool for investigating the metastatic process in sarcoma and to identify novel therapeutic targets. The present review provides a short overview of the most recent literature on CTCs in sarcoma. PMID:27656422

  16. The Challenges of Detecting Circulating Tumor Cells in Sarcoma

    PubMed Central

    Tellez-Gabriel, Marta; Brown, Hannah K.; Young, Robin; Heymann, Marie-Françoise; Heymann, Dominique

    2016-01-01

    Sarcomas are a heterogeneous group of malignant neoplasms of mesenchymal origin, many of which have a propensity to develop distant metastases. Cancer cells that have escaped from the primary tumor are able to invade into surrounding tissues, to intravasate into the bloodstream to become circulating tumor cells (CTCs), and are responsible for the generation of distant metastases. Due to the rarity of these tumors and the absence of specific markers expressed by sarcoma tumor cells, the characterization of sarcoma CTCs has to date been relatively limited. Current techniques for isolating sarcoma CTCs are based on size criteria, the identification of circulating cells that express either common mesenchymal markers, sarcoma-specific markers, such as CD99, CD81, or PAX3, and chromosomal translocations found in certain sarcoma subtypes, such as EWS-FLI1 in Ewing’s sarcoma, detection of osteoblast-related genes, or measurement of the activity of specific metabolic enzymes. Further studies are needed to improve the isolation and characterization of sarcoma CTCs, to demonstrate their clinical significance as predictive and/or prognostic biomarkers, and to utilize CTCs as a tool for investigating the metastatic process in sarcoma and to identify novel therapeutic targets. The present review provides a short overview of the most recent literature on CTCs in sarcoma. PMID:27656422

  17. The Challenges of Detecting Circulating Tumor Cells in Sarcoma

    PubMed Central

    Tellez-Gabriel, Marta; Brown, Hannah K.; Young, Robin; Heymann, Marie-Françoise; Heymann, Dominique

    2016-01-01

    Sarcomas are a heterogeneous group of malignant neoplasms of mesenchymal origin, many of which have a propensity to develop distant metastases. Cancer cells that have escaped from the primary tumor are able to invade into surrounding tissues, to intravasate into the bloodstream to become circulating tumor cells (CTCs), and are responsible for the generation of distant metastases. Due to the rarity of these tumors and the absence of specific markers expressed by sarcoma tumor cells, the characterization of sarcoma CTCs has to date been relatively limited. Current techniques for isolating sarcoma CTCs are based on size criteria, the identification of circulating cells that express either common mesenchymal markers, sarcoma-specific markers, such as CD99, CD81, or PAX3, and chromosomal translocations found in certain sarcoma subtypes, such as EWS-FLI1 in Ewing’s sarcoma, detection of osteoblast-related genes, or measurement of the activity of specific metabolic enzymes. Further studies are needed to improve the isolation and characterization of sarcoma CTCs, to demonstrate their clinical significance as predictive and/or prognostic biomarkers, and to utilize CTCs as a tool for investigating the metastatic process in sarcoma and to identify novel therapeutic targets. The present review provides a short overview of the most recent literature on CTCs in sarcoma.

  18. Radiotherapy to Improve Local Control Regardless of Surgical Margin and Malignancy Grade in Extremity and Trunk Wall Soft Tissue Sarcoma: A Scandinavian Sarcoma Group Study

    SciTech Connect

    Jebsen, Nina L. Trovik, Clement S.; Bauer, Henrik C.F.; Rydholm, Anders; Monge, Odd R.; Hall, Kirsten Sundby; Alvegard, Thor; Bruland, Oyvind S.

    2008-07-15

    Purpose: Adjuvant radiotherapy has during the past decades become increasingly used in the treatment of localized soft tissue sarcoma. We evaluated the effect of radiotherapy (RT) on local recurrence rates (LRRs) in Scandinavia between 1986 and 2005. Methods and Materials: A total of 1,093 adult patients with extremity or trunk wall soft tissue sarcoma treated at four Scandinavian sarcoma centers were stratified according to the treatment period (1986-1991, 1992-1997, and 1998-2005). The use of adjuvant RT, quality of the surgical margin, interval between surgery and RT, and LRR were analyzed. The median follow-up was 5 years. Results: The use of RT (77% treated postoperatively) increased from 28% to 53%, and the 5-year LRR decreased from 27% to 15%. The rate of wide surgical margins did not increase. The risk factors for local recurrence were histologic high-grade malignancy (hazard ratio [HR], 5), an intralesional (HR, 6) or marginal (HR, 3) surgical margin, and no RT (HR, 3). The effect of RT on the LRR was also significant after a wide margin resection and in low-grade malignant tumors. The LRR was the same after preoperative and postoperative RT. The median interval from surgery to the start of RT was 7 weeks, and 98% started RT within 4 months. The LRR was the same in patients who started treatment before and after 7 weeks. Conclusion: The results of our study have shown that adjuvant RT effectively prevents local recurrence in soft tissue sarcoma, irrespective of the tumor depth, malignancy grade, and surgical margin status. The effect was most pronounced in deep-seated, high-grade tumors, even when removed with a wide surgical margin.

  19. Superficial paramucosal clear cell sarcoma of the soft parts resembling melanoma in a 13-year-old boy.

    PubMed

    Sidiropoulos, Michael; Busam, Klaus; Guitart, Joan; Laskin, William B; Wagner, Annette M; Gerami, Pedram

    2013-02-01

    Clear cell sarcoma (CCS) of tendons and aponeuroses, also known as melanoma of soft parts, represents an aggressive rare malignancy that is characterized by a nested or fascicular pattern of spindled cells and a pathognomonic reciprocal translocation, t(12;22)(q13;q12), that results in the fusion of EWSR1 and ATF1 genes. Numerous recent studies have recognized the importance of a cutaneous CCS variant that can mimic a broad spectrum of entities, including spindle cell melanoma, spindle cell squamous carcinoma, cutaneous leiomyosarcoma and atypical fibroxanthoma. We report a case of a 13-year-old boy with cutaneous CCS who presented with a few months history of an asymptomatic papule on the lower lip that was suggestive of a mucocele. Biopsy of the lesion showed a wedge shaped neoplasm arranged in nests and fascicles of epithelioid- to oval-shaped cells with pale cytoplasm, open chromatin and prominent nucleolus. The superficial component was closely opposed to the basal epithelium resembling the junctional nests of a melanocytic neoplasm. The process extended into and involved the striated muscle of the lip. The cells expressed S-100, CD99 and synaptophysin by immunohistochemistry, and there was focal HMB-45 and microphthalmia transcription factor (MiTF) positivity as well. Fluorescence in situ hybridization confirmed the presence of the t(12;22) (ESWR1-ATF1) translocation. PMID:23228147

  20. Fast neutron therapy in treatment of soft tissue sarcoma--the Berlin-Buch study.

    PubMed

    Steingräber, M; Lessel, A; Jahn, U

    1996-01-01

    From 1975-1994, 221 adult patients with a total of 232 radiation sites for soft tissue sarcomas were irradiated with fast neutrons with a mean energy of 6.2 MeV in Berlin-Buch/Dresden-Rossendorf. The tumour dose ranged between 6 and 12 Gy and was limited by the low dose penetration of the neutron beam. A local control rate of 66% was obtained. The local control was affected by the tumour differentiation, residual status and histological subtype. Severe fibrosis of the subcutaneous tissues occurred in 40% usually after 2 years. No serious general side effects occurred. To optimize neutron therapy, a high energy clinically-based cyclotron with a fully rotational gantry and a multileaf collimator should be utilized. It seems that patients with locally advanced and well differentiated sarcomas can benefit from this therapy.

  1. Production of Virus by Mammalian Cells Transformed by Rous Sarcoma and Murine Sarcoma Viruses

    PubMed Central

    Valentine, Artrice F.; Bader, John P.

    1968-01-01

    Cultured cells of mammalian tumors induced by ribonucleic acid (RNA)-containing oncogenic viruses were examined for production of virus. The cell lines were established from tumors induced in rats and hamsters with either Rous sarcoma virus (Schmidt-Ruppin or Bryan strains) or murine sarcoma virus (Moloney strain). When culture fluids from each of the cell lines were examined for transforming activity or production of progeny virus, none of the cell lines was found to be infectious. However, electron microscopic examination of the various cell lines revealed the presence of particles in the rat cells transformed by either Rous sarcoma virus or murine sarcoma virus. These particles, morphologically similar to those associated with murine leukemias, were found both in the extracellular fluid concentrates and in whole-cell preparations. In the latter, they were seen budding from the cell membranes or lying in the intercellular spaces. No viruslike particles were seen in preparations from hamster tumors. Exposure of the rat cells to 3H-uridine resulted in the appearance of labeled particles with densities in sucrose gradients typical of virus (1.16 g/ml.). RNA of high molecular weight was extracted from these particles, and double-labeling experiments showed that this RNA sedimented at the same rate as RNA extracted from Rous sarcoma virus. None of the hamster cell lines gave radioactive peaks in the virus density range, and no extractable high molecular weight RNA was found. These studies suggest that the murine sarcoma virus produces an infection analogous to certain “defective” strains of Rous sarcoma virus, in that particles produced by infected cells have a low efficiency of infection. The control of the host cell over the production and properties of the RNA-containing tumorigenic viruses is discussed. Images PMID:4316021

  2. Treatment of early uterine sarcomas: disentangling adjuvant modalities.

    PubMed

    Zagouri, Flora; Dimopoulos, Athanasios-Meletios; Fotiou, Stelios; Kouloulias, Vassilios; Papadimitriou, Christos A

    2009-01-01

    Uterine sarcomas are a rare group of neoplasms with aggressive clinical course and poor prognosis. They are classified into four main histological subtypes in order of decreasing incidence: carcinosarcomas, leiomyosarcomas, endometrial stromal sarcomas and "other" sarcomas. The pathological subtype demands a tailored approach. Surgical resection is regarded as the mainstay of treatment. Total abdominal hysterectomy and bilateral salpingo-oophorectomy represents the standard treatment of uterine sarcomas. Pelvic and para-aortic lymph node dissection in carcinosarcomas is recommended, given their high incidence of lymph node metastases, and may have a role in endometrial stromal sarcomas. Adjuvant radiation therapy has historically been of little survival value, but it appears to improve local control and may delay recurrence. Regarding adjuvant chemotherapy, there is little evidence in the literature supporting its use except for carcinosarcomas. However, more trials are needed to address these issues, especially, their sequential application. Patients with uterine sarcomas should be referred to large academic centers for participation in clinical trials.

  3. [Magnetic resonance imaging in the diagnosis of knee joint sarcomas].

    PubMed

    Shubkin, V N; Gunicheva, N V; Akhadov, T A; Puzhitskiĭ, L B; Keshishian, R A

    2007-01-01

    The purpose of the investigation was to study the potentialities of magnetic resonance imaging (MRI) in the diagnosis of knee joint sarcomas. The paper presents the results of examining 13 patients of different age, shows the potentialities of the technique in the identification of knee joint sarcomas, and describes the MRI semiotics of sarcomas in both the routine study and that using contrast enhancement in lesions of bone and soft tissue elements in the presence of regional metastases.

  4. A trimodality approach in the management of metastatic low-grade epithelioid hemangioendothelioma of the bone.

    PubMed

    Saste, Abhijit; Cabrera Fernandez, Diego Felipe; Gulati, Rohit; Gamalski, Steven

    2015-07-16

    A 29-year-old man presented with a 2-week history of severe pain in the left foot with no preceding history of trauma. A left foot radiograph demonstrated a cortical lucency in the mid-distal shaft of the third metatarsal bone. MRI of the left foot showed an expansile lesion in the same location. A staging bone scan showed a focal uptake in the known lucency in the left third metatarsal and in the proximal left femur. A subsequent left hip radiograph demonstrated a lucency in the intertrochanteric region. CT scan of the chest, abdomen and pelvis was unremarkable. A biopsy of the left third metatarsal expansile lesion performed during an incision and curettage procedure revealed an epithelioid haemangioma (EHE) of the bone. MRI of the left hip performed in response to the findings on the bone scan showed metastatic disease in the left intertrochanteric region. A prophylactic left hip fixation surgery with an interlocking intramedullary femoral nail was therefore undertaken to avoid a pathological fracture of the left hip from the metastatic disease. Simultaneously, a left hip biopsy was performed, which also revealed an EHE. The patient underwent external beam radiation to the left femoral head and neck. This was followed by fractionated radiosurgery to the left third metatarsal. Once the left foot wound had healed, the patient subsequently received four cycles of doxorubicin and ifosfamide. A restaging positron emission tomography CT carried out after completion of therapy showed no metabolic evidence of residual primary tumour or metastasis. More than 2 years after completing his trimodality therapy, the patient remains fully functional and symptom free.

  5. Patient with liver epithelioid hemangioendothelioma treated by transplantation: 3 years' observation.

    PubMed

    Mucha, K; Foroncewicz, B; Zieniewicz, K; Nyckowski, P; Krawczyk, M; Cyganek, A; Paczek, L

    2006-01-01

    Epithelioid hemangioendothelioma (EHE) is a rare neoplasm of vascular origin, but unknown etiology that occurs in the liver, lungs and other organs. Its hepatic form (HEHE) generally behaves as a low-grade malignant tumor with a slowly progressive phenotype. Surgical resection or liver transplantation (OLT) has been recommended after diagnosis. We present a 30-year-old woman with primary HEHE of the liver treated by OLT in 2002. Her medical history started 3 years prior when an abdominal ultrasound examination revealed multiple focal changes in the liver. The histopathological diagnosis from a needle biopsy was carcinoma cholangiogenes desmoplasticum. For 2 years the patient was treated with chemotherapy combinations. To explain the lack of efficacy of chemotherapy, a laparoscopic biopsy was performed and HEHE diagnosed. Immunohistochemistry revealed positive staining for the factor VIII-related antigens, CD34 and CD31, which have been previously described as HEHE markers. The patient underwent OLT in March 2002. In the first month after OLT, the thyroid stimulating hormone concentration was elevated but they continuously decreased from 11.4 to 2.4 uIU/mL in May 2002 and thereafter remains normal. After 3 years observation the patient presented with good liver function and no signs of tumor recurrence. We concluded that immunohistochemical staining for characteristic endothelial cell markers may facilitate the correct diagnosis of HEHE. After diagnosis, OLT followed by immunosuppressive therapy, consisting of basiliximab, corticoids, low doses of tacrolimus and temporary administration of rapamycin, may be safe and effective. Monitoring of thyroid-stimulating hormone concentrations should be performed in patients with HEHE. PMID:16504710

  6. Epithelioid hemangioendothelioma of the liver as a rare indication for liver transplantation

    PubMed Central

    Remiszewski, Piotr; Szczerba, Ewa; Kalinowski, Piotr; Gierej, Beata; Dudek, Krzysztof; Grodzicki, Mariusz; Kotulski, Marcin; Paluszkiewicz, Rafał; Patkowski, Waldemar; Zieniewicz, Krzysztof; Krawczyk, Marek

    2014-01-01

    AIM: To investigate the indications and outcomes of liver transplantation for hepatic epithelioid hemangioendothelioma (HEHE). METHODS: Between 1989 and August 2013, in the Department of General, Transplant, and Liver Surgery, Medical University of Warsaw, 1306 orthotopic liver transplantations (OLTx) were performed, including 72 retransplantations. Unresectable HEHE was an indication for OLTx in 10 patients (0.8% of primary OLTx), the mean age of the patients was 40.5 ± 13.3 years (range 23-65 years), and the male-to-female ratio was 2:8. Kaplan-Meier survival analysis in HEHE, hepatocellular carcinoma (HCC), and other OLTx recipients groups was performed. The differences in mortality were compared using the χ2 test. A P-value < 0.05 indicated statistical significance. RESULTS: No concomitant liver disease was found in any patient. There was no neoadjuvant chemotherapy or radiotherapy. Liver function test results were normal in most of the patients. The levels of alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19-9 were normal. In immunohistochemical staining, the neoplastic cells were positive for factor VIII-related antigen, CD31, and CD34, which are endothelial cell markers, and negative for cytokeratin 19, cytokeratin 7, and HepPar-1. Nine patients were alive without tumor recurrence. One patient died 2 mo after OLTx due to septic complications. No morbidity was observed. Maximum follow-up was 11.4 years, with a minimum of 1 mo. The cumulative survival rate at the end of follow-up in HEHE patients was 87.5% compared with 54.3% in the HCC group and 76.3% in the other OLTx recipients group (χ2 test = 1.784, df = 2, P = 0.409). CONCLUSION: Unresectable HEHE, without extrahepatic metastases is an excellent indication for liver transplantation. Long-term survival is very good and much better than in HCC patients and the entire group of OLTx patients. PMID:25170219

  7. Contrast-enhanced multiple-phase imaging features in hepatic epithelioid hemangioendothelioma

    PubMed Central

    Chen, Ying; Yu, Ri-Sheng; Qiu, Ling-Ling; Jiang, Ding-Yao; Tan, Yan-Bin; Fu, Yan-Biao

    2011-01-01

    AIM: To investigate and review the contrast-enhanced multiple-phase computed tomography (CEMP CT) and magnetic resonance imaging (MRI) findings in patients with pathologically confirmed hepatic epithelioid hemangioendothelioma (HEHE). METHODS: Findings from imaging examinations in 8 patients (5 women and 3 men) with pathologically confirmed HEHE were retrospectively reviewed (CT images obtained from 7 patients and MR images obtained from 6 patients). The age of presentation varied from 27 years to 60 years (average age 39.8 years). RESULTS: There were two types of HEHE: multifocal type (n = 7) and diffuse type (n = 1). In the multifocal-type cases, there were 74 lesions on CT and 28 lesions on MRI with 7 lesions found with diffusion weighted imaging; 18 (24.3%) of 74 lesions on plain CT and 26 (92.9%) of 28 lesions on pre-contrast MRI showed the target sign. On CEMP CT, 28 (37.8%) of 74 lesions appeared with the target sign and a progressive-enhancement rim and 9 (12.2%) of 74 lesions displayed progressive enhancement, maintaining a state of persistent enhancement. On CEMP MRI, 27 (96.4%) of 28 lesions appeared with the target sign with a progressive-enhancement rim and 28 (100%) of 28 lesions displayed progressive-enhancement, maintaining a state of persistent enhancement. In the diffuse-type cases, an enlarged liver was observed with a large nodule appearing with persistent enhancement on CEMP CT and MRI. CONCLUSION: The most important imaging features of HEHE are the target sign and/or progressive enhancement with persistent enhancement on CEMP CT and MRI. MRI is advantageous over CT in displaying these imaging features. PMID:21941423

  8. Epithelioid Hemangioendothelioma: An Overview and Update on a Rare Vascular Tumor

    PubMed Central

    Sardaro, Angela; Bardoscia, Lilia; Petruzzelli, Maria Fonte; Portaluri, Maurizio

    2014-01-01

    Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by Dail and Liebow as an aggressive bronchoalveolar cell carcinoma. The etiology is still a dilemma. Studies about suggestive hypothesis are ongoing. Most of the times it affects lung, liver and bones, although this kind of tumor may involve the head and neck area, breast, lymph nodes, mediastinum, brain and meninges, the spine, skin, abdomen and many other sites. Because of its heterogeneous presentation, as it represents less than 1% of all the vascular tumors, it is often misdiagnosed and not suitably treated, leading to a poor prognosis in some cases. Over 50-76% of the patients are asymptomatic. A small number of them complains respiratory symptoms. Bone metastases might cause pathological fractures or spine compression, if they arise in vertebrae. Imaging is necessary to determine morphological data, the involvement of surrounding tissues, and potentially the cleavage plan. It is important to recognize the expression of vascular markers (Fli-1 and CD31 are endothelial-specific markers), and the microscopic evidence of vascular differentiation to make a correct diagnosis, as many pulmonary diseases show multiple nodular lesions. Because of its rarity, there is no standard for treatment. We focused on radiotherapy as a good therapeutic option: despite the poor prognosis, evidence is in favor of radiotherapy which offers local pain control with good tolerance and better quality of life at least at a one-year follow-up in most of cases. Further studies are needed to establish the standard radiation dose to be used for locoregional control of such a complex and extremely rare disease. PMID:25992243

  9. Aortic intimal sarcoma with embolic metastases.

    PubMed

    Wright, E P; Glick, A D; Virmani, R; Page, D L

    1985-12-01

    A 46-year-old woman died from massive bowel infarction. At autopsy, a primary sarcoma was found growing along the intimal surface of the aorta at the level of the celiac axis. Tumor emboli were found in distal aortic branches and most abdominal organs. Immunoperoxidase for Factor VIII and electron microscopy (EM) did not support an endothelial origin. EM showed myofibroblastic differentiation. Review of the literature yields an array of diagnostic histologic terms for these tumors, hampering case comparison. The literature does suggest, however, that the clinical presentation of these rare neoplasms correlates nicely with the location and gross morphology of the lesion. We therefore propose a clinicopathologic classification, categorizing the lesions as intimal (obstructive and nonobstructive) and mural. The former are typically pleomorphic sarcomas and are probably of myofibroblastic origin, whereas the latter are usually leiomyosarcomas or fibrosarcomas that probably originate in the media or adventitia. PMID:3000205

  10. Recent advances in targeted therapy for Ewing sarcoma.

    PubMed

    Pishas, Kathleen I; Lessnick, Stephen L

    2016-01-01

    Ewing sarcoma is an aggressive, poorly differentiated neoplasm of solid bone that disproportionally afflicts the young. Despite intensive multi-modal therapy and valiant efforts, 70% of patients with relapsed and metastatic Ewing sarcoma will succumb to their disease. The persistent failure to improve overall survival for this subset of patients highlights the urgent need for rapid translation of novel therapeutic strategies. As Ewing sarcoma is associated with a paucity of mutations in readily targetable signal transduction pathways, targeting the key genetic aberration and master regulator of Ewing sarcoma, the EWS/ETS fusion, remains an important goal. PMID:27635231

  11. Recent advances in targeted therapy for Ewing sarcoma

    PubMed Central

    Pishas, Kathleen I.; Lessnick, Stephen L.

    2016-01-01

    Ewing sarcoma is an aggressive, poorly differentiated neoplasm of solid bone that disproportionally afflicts the young. Despite intensive multi-modal therapy and valiant efforts, 70% of patients with relapsed and metastatic Ewing sarcoma will succumb to their disease. The persistent failure to improve overall survival for this subset of patients highlights the urgent need for rapid translation of novel therapeutic strategies. As Ewing sarcoma is associated with a paucity of mutations in readily targetable signal transduction pathways, targeting the key genetic aberration and master regulator of Ewing sarcoma, the EWS/ETS fusion, remains an important goal. PMID:27635231

  12. Recent advances in targeted therapy for Ewing sarcoma

    PubMed Central

    Pishas, Kathleen I.; Lessnick, Stephen L.

    2016-01-01

    Ewing sarcoma is an aggressive, poorly differentiated neoplasm of solid bone that disproportionally afflicts the young. Despite intensive multi-modal therapy and valiant efforts, 70% of patients with relapsed and metastatic Ewing sarcoma will succumb to their disease. The persistent failure to improve overall survival for this subset of patients highlights the urgent need for rapid translation of novel therapeutic strategies. As Ewing sarcoma is associated with a paucity of mutations in readily targetable signal transduction pathways, targeting the key genetic aberration and master regulator of Ewing sarcoma, the EWS/ETS fusion, remains an important goal.

  13. Current Immunotherapies for Sarcoma: Clinical Trials and Rationale

    PubMed Central

    Mitsis, Demytra; Francescutti, Valerie

    2016-01-01

    Sarcoma tumors are rare and heterogeneous, yet they possess many characteristics that may facilitate immunotherapeutic responses. Both active strategies including vaccines and passive strategies involving cellular adoptive immunotherapy have been applied clinically. Results of these clinical trials indicate a distinct benefit for select patients. The recent breakthrough of immunologic checkpoint inhibition is being rapidly introduced to a variety of tumor types including sarcoma. It is anticipated that these emerging immunotherapies will exhibit clinical efficacy for a variety of sarcomas. The increasing ability to tailor immunologic therapies to sarcoma patients will undoubtedly generate further enthusiasm and clinical research for this treatment modality. PMID:27703409

  14. Primary Thyroid Sarcoma: A Systematic Review.

    PubMed

    Surov, Alexey; Gottschling, Sebastian; Wienke, Andreas; Meyer, Hans Jonas; Spielmann, Rolf Peter; Dralle, Henning

    2015-10-01

    Different types of malignant tumors can occur within the thyroid. Primary cancer is the most common type of thyroid malignancy. Non-epithelial malignancies can also arise within the thyroid. The aim of the present study was to analyze clinical and radiological characteristics of reported primary thyroid sarcomas (PTS), based on a large sample of cases. The PubMed database was screened for articles from between 1990 and 2014. Overall, 86 articles with 142 patients were identified. Ultrasound evaluation was reported for 36 patients. Data regarding computed tomography of the neck were available for 29 cases. Magnetic resonance imaging was performed for eight patients. The following data were retrieved for the identified sarcomas: localization, size, homogeneity, internal texture, and margin characteristics. In most cases, PTS occurred in patients over 40 years of age, with a peak incidence for the group aged 60-79 years. Angiosarcoma was diagnosed in 29 cases (20.4%), followed by malignant hemangioendothelioma (n=23, 16.3%), malignant fibrous histiocytoma (n=20, 14.1%), leiomyosarcoma (n=16, 11.3%), and fibrosarcoma (n=13, 9.2%). In most patients (n=113, 79.6%), PTS manifested clinically as a painless goiter. On ultrasound, PTS were predominantly mixed hypo-to-hyperechoic in comparison to the normal thyroid tissue. On non-contrast computed tomography, most sarcomas were inhomogeneous hypo-to-hyperdense. On post-contrast magnetic resonance images, most sarcomas showed marked non-homogenous enhancement. In 26.8%, infiltration of the adjacent organs was seen. The trachea or esophagus was affected more frequently in patients with malignant histiocytoma and liposarcoma. Different strategies were used in the treatment of PTS. Our analysis provides clinical and radiological characteristics of PTS. The described features should be taken into consideration in the differential diagnosis of thyroid tumors. PMID:26408676

  15. Multimodality Local Therapy for Retroperitoneal Sarcoma

    SciTech Connect

    Paryani, Nitesh N.; Zlotecki, Robert A.; Swanson, Erika L.; Morris, Christopher G.; Grobmyer, Stephen R.; Hochwald, Steven N.; Marcus, Robert B.; Indelicato, Daniel J.

    2012-03-01

    Purpose: Soft-tissue sarcomas of the retroperitoneum are rare tumors comprising less than 1% of all malignancies. Although surgery continues as the mainstay of treatment, the large size of these tumors coupled with their proximity to critical structures make resection with wide margins difficult to achieve. The role and timing of radiotherapy are controversial. This study updates our institutional experience using multimodality local therapy for resectable retroperitoneal sarcoma and identifies prognostic factors impacting disease control and survival. Methods and Materials: Between 1974 and 2007, 58 patients with nonmetastatic retroperitoneal sarcoma were treated with surgery and radiation at University of Florida. The median age at radiotherapy was 57 years old (range, 18-80 years). Forty-two patients received preoperative radiotherapy and 16 received postoperative radiotherapy. Nineteen patients received 1.8 Gy once daily and 39 patients received 1.2 Gy twice daily. Variables analyzed for prognostic value included age, grade, kidney involvement, histology, de novo versus recurrent presentation, tumor diameter, margin status, radiotherapy sequencing (preoperative vs. postoperative), total radiation dose, fractionation scheme, and treatment era. Results: The 5-year overall survival, cause-specific survival, and local control rates were 49%, 58%, and 62%, respectively. Nearly two-thirds of disease failures involved a component of local progression. On multivariate analysis, only margin status was significantly associated with improved 5-year local control (85%, negative margins; 63%, microscopic positive margins; 0%, gross positive margins; p < 0.0001) and 5-year overall survival (64%, negative margins; 56%, microscopic positive margins; 13%, gross positive margins; p = 0.0012). Thirty-one Grade 3 or greater toxicities were observed in 22 patients, including two treatment-related deaths (3%). Conclusion: For retroperitoneal sarcoma, local control remains a

  16. Primary pleuro-pulmonary synovial sarcoma.

    PubMed

    Shah, Unmil B; Joshi, S; Ghorpade, S V; Gaikwad, S N; Sundrani, R M

    2010-01-01

    Primary pleuro-pulmonary synovial sarcoma (PPSS) is a rare tumour and poses adiagnostic challenge particularly when unusual histological features are present. We report a case of a 30-year-old immunocompromised human immunodeficiency virus (HIV) sero-positive male who was referred to us with complaints of cough, breathlessness and left-sided chestpain for the past two months. The PPSS can be confirmed on tru-cut biopsy. PMID:20949738

  17. Extraskeletal Ewing's sarcoma mimicking traumatic hematoma.

    PubMed

    Ogose, A; Hotta, T; Yamamura, S; Shioya, Y; Yazawa, T

    1998-01-01

    We describe the clinical course of a 16-year-old baseball player with a history of recurrent hematoma of the thigh. The lesion was aspirated percutaneously several times and curetted under the diagnosis of traumatic hematoma. Microscopical examination revealed massive hemorrhage, necrosis, and a small number of Ewing's sarcoma cells. He died of multiple metastases. With recurrent hematoma in the soft tissue, neoplastic lesions should be ruled out.

  18. Granulocytic sarcoma with expression of CD30.

    PubMed Central

    Fickers, M; Theunissen, P

    1996-01-01

    A case of a young man with a spinal epidural tumour, initially diagnosed as large cell anaplastic malignant lymphoma, is reported. The tumour consisted of poorly differentiated cells showing immunoreactivity with antibodies directed against CD30 and CD45. Ten months later the patient developed acute myeloid leukaemia. The histological slides of the epidural tumour were reviewed, including additional enzymochemical and immunochemical stains. As the tumour showed immunoreactivity for myeloperoxidase and chloroacetate esterase, it was reclassified as a granulocytic sarcoma. Images PMID:9038763

  19. Management of soft tissue and bone sarcomas

    SciTech Connect

    Van Oosterom, A.T.; Van Unnik, J.A.M.

    1986-01-01

    This book contains 32 papers. Some of the titles are: Adjuvant Treatment for Osteosarcoma of the Limbs; Trial 20781 of the SIOP and the EORTC Radiotherapy/Chemotherapy; Application of Magnetic Resonance Imaging (MRI) in Diagnosis and Follow-up During Treatment of Bone Tumors; Radiological Assessment of Local Involvement in Bone Sarcomas; and Prevention of Lung Metastases by Irradiation Alone or Combined with Chemotherapy in an Animal Model.

  20. Titration patterns of a murine sarcoma-leukemia virus complex: evidence for existence of competent sarcoma virions.

    PubMed

    O'Connor, T E; Fischinger, P J

    1968-01-19

    Stocks of inurine sarcoma virus show titration patterns ranging from one-to two-hit kinetics. The comparison of various titrations of this virus, both with and without added helper virus, to theoretical model systems composed of defined constituents, suggests the existence of a sarcoma virus that does not need coinfectinig murine leukemia virus to be manifested as a focus-forming unit. The behavior of such nondefective particles is compatible with a postulated leukemia-sarcoma virus hybrid.

  1. Cutaneous myeloid sarcoma of the penile foreskin.

    PubMed

    Afrose, Ruquiya; Nebhnani, Deepa; Wadhwa, Neelam

    2015-01-01

    Myeloid sarcoma, considered to herald the onset of a blast crisis in the setting of chronic myeloproliferative neoplasm/dysplasia, typically presents during the course of the disorder. Cutaneous involvement is uncommon and lesions on genital skin are seldom seen. We present a case of a well-differentiated myeloid sarcoma in the penile foreskin in an apparently healthy 29-year-old male presenting with phimosis. The unusual composition of the inflammatory cell infiltrate, and characteristic sparing of dermal blood vessels, nerves and smooth muscle fibres led to the correct diagnosis. Absence of commonly observed changes in the circumcision skin like those of balanitis xerotica was also helpful. Detailed hematological work up revealed a previously undiagnosed chronic myeloid leukemia in chronic phase. The patient also had simultaneous priapism, another rare presentation of chronic myeloid leukemia. One year hence, the patient is in hematological remission with no evidence of extramedullary disease. Although priapism has been described as a rare presenting symptom in chronic myeloid leukemia, the present case is unique as this is the first time a cutaneous myeloid sarcoma has been documented in the penile foreskin. PMID:24913300

  2. Sarcoma Immunotherapy: Past Approaches and Future Directions

    PubMed Central

    D'Angelo, S. P.; Tap, W. D.; Schwartz, G. K.; Carvajal, R. D.

    2014-01-01

    Sarcomas are heterogeneous malignant tumors of mesenchymal origin characterized by more than 100 distinct subtypes. Unfortunately, 25–50% of patients treated with initial curative intent will develop metastatic disease. In the metastatic setting, chemotherapy rarely leads to complete and durable responses; therefore, there is a dire need for more effective therapies. Exploring immunotherapeutic strategies may be warranted. In the past, agents that stimulate the immune system such as interferon and interleukin-2 have been explored and there has been evidence of some clinical activity in selected patients. In addition, many cancer vaccines have been explored with suggestion of benefit in some patients. Building on the advancements made in other solid tumors as well as a better understanding of cancer immunology provides hope for the development of new and exciting therapies in the treatment of sarcoma. There remains promise with immunologic checkpoint blockade antibodies. Further, building on the success of autologous cell transfer in hematologic malignancies, designing chimeric antigen receptors that target antigens that are over-expressed in sarcoma provides a great deal of optimism. Exploring these avenues has the potential to make immunotherapy a real therapeutic option in this orphan disease. PMID:24778572

  3. Diagnostic triage for sarcoma: an effective model for reducing referrals to the sarcoma multidisciplinary team

    PubMed Central

    Botchu, R; Ashford, R U; Rennie, W J

    2015-01-01

    Objective: Soft-tissue lesions are common and often benign. Owing to the rarity of soft-tissue sarcomas (STSs), evidence has shown that patients are increasingly referred urgently onto the 2-week wait pathway, which may have a detrimental impact on the management of patients with a proven STS. Imaging plays a vital role in lesion characterization and can be used to triage referrals to reduce the caseload of a sarcoma multidisciplinary team (MDT). In our institution, we established a sarcoma diagnostic triage meeting (SDTM). This study aimed to determine the effectiveness of the SDTM in reducing non-sarcomatous referrals to the main sarcoma MDT. Methods: A retrospective review of the SDTM minutes from July 2011 to June 2012 was performed. Data collected for each case included details of referrer, referral modality and referral outcome. Results: 165 cases were reviewed. 58% of referrals underwent a core biopsy or surgical excision with 85% benign pathology, the commonest being lipoma. 15% of referrals were sarcomatous lesions and were referred onwards to the main MDT. Conclusion: A total of 82% of the patients referred urgently with a suspicious soft-tissue mass was managed by the SDTM and hence not referred onwards to the East Midlands Sarcoma Service MDT. A diagnostic triage is effective in reducing the caseload burden of the main MDT and allowing it to be more effective. Advances in knowledge: Referrals based on imaging can be prioritized by diagnostic triage. Diagnostic triage established in our institution reduced 82% of MDT referrals allowing a more focused MDT discussion on patients with a proven sarcoma. PMID:25697295

  4. Next generation sequencing in synovial sarcoma reveals novel gene mutations

    PubMed Central

    Vlenterie, Myrella; Hillebrandt-Roeffen, Melissa H.S.; Flucke, Uta E.; Groenen, Patricia J.T.A.; Tops, Bastiaan B.J.; Kamping, Eveline J.; Pfundt, Rolph; de Bruijn, Diederik R.H.; van Kessel, Ad H.M. Geurts; van Krieken, Han J.H.J.M.; van der Graaf, Winette T.A.; Versleijen-Jonkers, Yvonne M.H.

    2015-01-01

    Over 95% of all synovial sarcomas (SS) share a unique translocation, t(X;18), however, they show heterogeneous clinical behavior. We analyzed multiple SS to reveal additional genetic alterations besides the translocation. Twenty-six SS from 22 patients were sequenced for 409 cancer-related genes using the Comprehensive Cancer Panel (Life Technologies, USA) on an Ion Torrent platform. The detected variants were verified by Sanger sequencing and compared to matched normal DNAs. Copy number variation was assessed in six tumors using the Oncoscan array (Affymetrix, USA). In total, eight somatic mutations were detected in eight samples. These mutations have not been reported previously in SS. Two of these, in KRAS and CCND1, represent known oncogenic mutations in other malignancies. Additional mutations were detected in RNF213, SEPT9, KDR, CSMD3, MLH1 and ERBB4. DNA alterations occurred more often in adult tumors. A distinctive loss of 6q was found in a metastatic lesion progressing under pazopanib, but not in the responding lesion. Our results emphasize t(X;18) as a single initiating event in SS and as the main oncogenic driver. Our results also show the occurrence of additional genetic events, mutations or chromosomal aberrations, occurring more frequently in SS with an onset in adults. PMID:26415226

  5. Body protein status in Fischer 344 rats bearing the MCA sarcoma

    SciTech Connect

    Radcliffe, J.D.

    1986-03-05

    The effect of a methylcholanthrene (MCA) sarcoma on the protein status of adult, male, Fischer-344 rats was investigated. Three groups of ten rats were used. One group (TB) was inoculated with MCA sarcoma tissue, control (C) and pair-fed (PF) groups received saline only. Rats were fed a purified (20% casein, 20% fat, 45% sucrose) diet from day 7 post-transplant, when tumors became palpable. Food intake of TB was depressed relative to C at day 15 PT. Animals were killed at day 22 PT. In comparison to C, tumor growth was associated with a decrease in gastrocnemius muscle protein content and an increase in spleen weight and protein content. There was no effect of tumor growth on the weight or protein content of liver, heart, or kidneys. Data from PF animals suggested that decreased gastrocnemius muscle protein content was partly attributable to decreased food intake and partly to tumor growth; the increased spleen protein was due to tumor growth per se. Thus, growth of the MCA sarcoma affects host protein status. Some of these effects are caused partly by hypophagia and partly by tumor growth and others are due to tumor growth per se.

  6. Designing novel therapies against sarcomas in the era of personalized medicine and economic crisis.

    PubMed

    Manara, Maria Cristina; Garofalo, Cecilia; Ferrari, Stefano; Belfiore, Antonino; Scotlandi, Katia

    2013-01-01

    Drug "repurposing" is the process of finding new therapeutic indications for existing drugs, and can be considered as a more efficient and realistic strategy for the design of therapies against rare diseases than the current efforts to develop targeted-drugs. In this review, we explore the difficulties related to the identification and development of tailored therapies for individual patients with sarcomas, which are relatively rare diseases characterized by an extreme genetic and histologic variability. Overall, sarcomas comprise about 1% of all adult tumors and 10% of pediatric cancers. They are conventionally divided in bone and soft-tissue sarcomas, considering their site of origin. However, each group is highly heterogeneous and recent global characterization of their genetic alterations has clearly identified the existence of peculiarities that render these group of tumors even more "orphan" for pharmaceutical companies to develop and market specific- targeted drugs. The present review highlights key examples of molecular targets identification in bone sarcomas, reexamining the history of insulin-like growth factor receptor (IGF-IR) and its role in physiology and in cancer as well as developments regarding phase I to II clinical trials of agents directed against this receptor. The IGF system is quite complex, with many players in the field. Insulin receptor function in cancer cells has certainly been underestimated, but also little attention was paid to the type of ligands that are mainly involved in each tumor type. Strategies considering the system in its complex are encouraged and, in this context, drugs aimed at reducing circulating insulin levels, such as metformin, should receive attention as potential anti-cancer agents.

  7. Extrauterine epithelioid trophoblastic tumors presenting as primary lung carcinomas: morphologic and immunohistochemical features to resolve a diagnostic dilemma.

    PubMed

    Lewin, Sharyn N; Aghajanian, Carol; Moreira, Andre L; Soslow, Robert A

    2009-12-01

    Our objective was to describe the clinicopathologic features of epithelioid trophoblastic tumors (ETTs) in a series of patients who presented with elevated beta-human chorionic gonadotrophin (hCG) levels and extrauterine lesions resembling primary lung carcinomas. Clinical and pathologic materials were reviewed and Shih and Kurman's diagnostic criteria were applied. Three parous women (38, 49, and 34 y of age) with elevated beta-hCG levels had nondiagnostic gynecologic evaluations, including negative dilation and curettage specimens. Imaging revealed isolated pulmonary lesions, 2 to 8.5 cm in size, resembling primary lung carcinomas. Two patients received multiagent chemotherapy consisting of etoposide, methotrexate, dactinomycin, alternating with cisplatin and etoposide, and all underwent pulmonary resection. Histologically, the cytologic features, epithelioid growth pattern, and hyaline-like material simulated the appearance of nonsmall cell lung carcinoma, but overall, the histologic features along with the immunophenotype supported classification as ETT. Follow-up hysterectomy specimens were histologically normal. All 3 patients are alive and well. The rarity of ETT and its resemblance to squamous and pleomorphic carcinomas of lung have led to diagnostic difficulties. When reproductive-age women present with elevated beta-hCG levels, a pulmonary lesion, and no apparent intrauterine disease, primary pulmonary ETT should be considered. Correlating clinical indices with specific morphologic and immunohistochemical features can ensure diagnostic accuracy and appropriate treatment for favorable outcomes.

  8. Epithelioid trophoblastic tumor of the uterus in a postmenopausal woman: a case report and review of the literature.

    PubMed

    Coulson, L E; Kong, C S; Zaloudek, C

    2000-11-01

    We report an epithelioid trophoblastic tumor (ETT), a recently delineated type of gestational trophoblastic tumor (GTT), discovered in the uterus of a 66-year-old woman. She had been treated for a hydatidiform mole 17 years previously without chemotherapy. The resected uterus contained a solid/cystic tumor located entirely within the myometrium. Microscopically, there was an epithelial-like growth pattern. The tumor was circumscribed, with a pushing border, and the tumor cells grew in cords, nests, and sheets within which were aggregates of hyaline material and necrotic debris. Most tumor cells were mononuclear and had an epithelioid appearance with distinct cell borders, eosinophilic cytoplasm, and nuclei with occasional indistinct nucleoli. Scattered multinucleated cells consistent with syncytiotrophoblastic cells were also present. Immunohistochemical staining revealed strong diffuse reactivity for cytokeratins (CK7, AE1/AE3, CAM 5.2, CK18) and epidermal growth factor receptor, and focal reactivity, mainly in syncytiotrophoblastic cells, for beta-human chorionic gonadotropin, human placental lactogen, and inhibin-alpha. The histologic and immunohistochemical features were characteristic of ETT, and helped to distinguish the tumor from other trophoblastic tumors and squamous cell carcinoma. An unusual observation was a high mitotic count, reflected in a Ki-67 proliferative index of 68.6%. Our findings indicate that ETT, like other types of GTT, can occur in postmenopausal women, even years after a gestational event.

  9. Minute perivascular epithelioid cell (PEC) nests in the abdominal lymph nodes--a putative precursor of PEComa.

    PubMed

    Nagasaka, Toru; Murakami, Yoshiko; Sasaki, Eiichi; Hosoda, Waki; Nakanishi, Toru; Yatabe, Yasushi

    2015-04-01

    A perivascular epithelioid cell tumor (PEComa) is a peculiar growth defined as a mesenchymal tumor composed of histologically and immunohistochemically distinct perivascular epithelioid cells (PECs). Because neither normal counterparts nor precursor lesions of PEComa have been identified, we examined minute PEC nests, ranged from 0.8 mm to 10 mm, to investigate the possible origin of the PEComa. We examined a total of 80 677 para-aortic and pelvic lymph nodes that were systematically dissected from 1656 patients for gynecological malignancies. The identified lesions were confirmed immunohistochemically with multiple PEC markers, including smooth muscle actin, HMB45, melan-A, MiTF, ER and PgR. A total of 66 minute PEC nests were found in 21 patients (1.3% of the total population) with an average frequency of 3.1 lesions per patient. In cases of multiple involvement, 11 of 13 nests were located at the same level of multiple lymph node or on continuous levels. The lesions were preferentially distributed at the level of para-aortic and high pelvic lymph nodes. All nests were positive for actin and HMB45, whereas the other markers were positive with varying frequencies. The minute PEC nests may be associated with the possible normal counterpart of PEComas.

  10. BCOR-CCNB3 (Ewing-like) sarcoma: a clinicopathologic analysis of 10 cases, in comparison with conventional Ewing sarcoma.

    PubMed

    Puls, Florian; Niblett, Angela; Marland, Gillian; Gaston, Czar Louie L; Douis, Hassan; Mangham, D Chas; Sumathi, Vaiyapuri P; Kindblom, Lars-Gunnar

    2014-10-01

    BCOR-CCNB3 fusion transcripts resulting from an X-chromosomal paracentric inversion were recently identified in a series of unclassifiable soft tissue and bone sarcomas with Ewing sarcoma-like morphology. The morphologic and clinical features of these sarcomas are, as yet, not well characterized. Here we describe the clinicopathologic features of 10 cases of BCOR-CCNB3 sarcoma and compare their clinical course with typical Ewing sarcoma. Nine of 10 patients were male, and all were 11 to 18 years of age. Seven tumors were located in the bone and 3 in the deep soft tissues. The histomorphologic spectrum was quite wide, with 7 tumors predominately showing small primitive cell morphology with angulated nuclei simulating so-called atypical Ewing sarcoma and 3 predominately showing spindle cell morphology. Recurrent and metastatic lesions showed increased cellularity and marked pleomorphism. Immunohistochemistry showed expression of CCNB3 (100%), bcl2 (90%), CD99 (60%), and CD117 (60%). Reverse transcription polymerase chain reaction for BCOR-CCNB3 fusion transcripts was positive in all 9 cases, which yielded sufficient extracted RNA. Five- and 10-year survival rates were 75% and 56%, respectively. BCOR-CCNB3 sarcomas located in axial skeleton and soft tissues showed a significantly shorter survival. The Ewing sarcoma overall survival was not statistically different, although there was a trend for longer survival of patients with BCOR-CCNB3 sarcomas in the extremities. In conclusion, this study provides a detailed description of the histologic spectrum, immunohistochemical features, and clinical characteristic of BCOR-CCNB3 sarcoma justifying distinction from Ewing sarcoma with its typical EWS/FUS-ETS translocations. Ideally immunohistochemistry is used in combination with reverse transcription polymerase chain reaction for definitive diagnosis. PMID:24805859

  11. Synovial sarcoma presenting as iliotibial band friction syndrome.

    PubMed

    Mesiha, Mena; Bauer, Thomas; Andrish, Jack

    2009-10-01

    Iliotibial band friction syndrome is a common entity that is often quickly diagnosed in orthopedic clinics. However, synovial sarcoma is an elusive clinical entity that appears around many joints with variable presentations. This case report is an example of a patient with a classic presentation of iliotibial band friction syndrome that was diagnosed as a synovial sarcoma on further investigation.

  12. TAZ and YAP are frequently activated oncoproteins in sarcomas

    PubMed Central

    Fullenkamp, Colleen A.; Hall, Sarah L.; Jaber, Omar I.; Pakalniskis, Brittany L.; Savage, Erica C.; Savage, Johanna M.; Ofori-Amanfo, Georgina K.; Lambertz, Allyn M.; Ivins, Stephanie D.; Stipp, Christopher S.; Miller, Benjamin J.; Milhem, Mohammed M.; Tanas, Munir R.

    2016-01-01

    TAZ (WWTR1) and YAP are transcriptional coactivators and oncoproteins inhibited by the Hippo pathway. Herein we evaluate 159 sarcomas representing the most prevalent sarcoma types by immunohistochemistry for expression and activation (nuclear localization) of TAZ and YAP. We show that 50% of sarcomas demonstrate activation of YAP while 66% of sarcomas demonstrate activated TAZ. Differential activation of TAZ and YAP are identified in various sarcoma types. At an RNA level, expression of WWTR1 or YAP1 predicts overall survival in undifferentiated pleomorphic sarcoma and dedifferentiated liposarcoma. Immunohistochemistry demonstrates that TAZ and YAP expression and activation are positively correlated with grade in the well-differentiated liposarcoma to dedifferentiated liposarcoma tumor progression sequence as well as conventional chondrosarcomas. TAZ and YAP are constitutively activated oncoproteins in sarcoma cell lines. Knock-down of TAZ and YAP demonstrate differential activity for the two proteins. Verteporfin decreases colony formation in soft agar as well as CTGF expression in sarcoma cell lines harboring activated TAZ and YAP. PMID:27129148

  13. Postradiation sarcoma of bone: review of 78 Mayo Clinic cases.

    PubMed

    Weatherby, R P; Dahlin, D C; Ivins, J C

    1981-05-01

    Postradiation sarcoma of bone is an uncommon but serious sequela of radiation therapy. Seventy-eight Mayo Clinic patients have been treated for sarcomas arising in irradiated bones. They received their initial radiotherapy for a wide variety of nonneoplastic and neoplastic conditions, both benign and malignant. Thirty-five sarcomas arose in bone that was normal at the time of radiotherapy, and 43 arose in irradiated preexisting osseous lesions. The latent period between radiotherapy and diagnosis of sarcoma averaged 14.3 years. Ninety percent of the postradiation sarcomas were either osteosarcomas or fibrosarcomas; chondrosarcoma, malignant (fibrous) histiocytoma, malignant lymphoma, Ewing's tumor, and metastasizing chondroblastoma also occurred. Prompt radical surgery, when feasible, is usually the treatment of choice for the sarcoma. About 30% of patients with sarcomas of the extremities or craniofacial bones survived 5 years without recurrence; there were no disease-free survivors among patients with tumors of the vertebral column, pelvis, or shoulder girdle. The low risk of sarcoma following radiotherapy for the treatment of cancer should not be a contraindication to its use in these patients; however, radiation therapy for benign bone tumors should be reserved for lesions that are not amenable to surgical treatment. An unusual case is also reported herein in which a fibrosarcoma was discovered in the humerus of a patient who had received radiotherapy 55 years previously for a verified osteosarcoma in the same site. PMID:6939953

  14. Pazopanib in the management of advanced soft tissue sarcomas

    PubMed Central

    Cranmer, Lee D; Loggers, Elizabeth T; Pollack, Seth M

    2016-01-01

    Therapy of soft tissue sarcomas represents an area of significant unmet need in oncology. Angiogenesis has been explored as a potential target both preclinically and clinically, with suggestions of activity. Pazopanib is a multitargeted tyrosine kinase inhibitor with prominent antiangiogenic effects. In a Phase II study, pazopanib demonstrated activity in strata enrolling patients with leiomyosarcomas, synovial sarcomas, or other sarcomas but not those enrolling adipocytic sarcomas. PALETTE, the pivotal Phase III trial, demonstrated improved progression-free survival versus placebo in pazopanib-treated patients previously treated for advanced soft tissue sarcomas. No survival benefit was observed, and adipocytic sarcomas were excluded. Health-related quality-of-life assessments indicated significant decrements in several areas affected by pazopanib toxicities, but no global deterioration. Cost-effectiveness analyses indicate that pazopanib therapy may or may not be cost-effective in different geographic settings. Pazopanib provides important proof-of-concept for antiangiogenic therapy in soft tissue sarcomas. Its use can be improved by further biological studies of its activity profile in sarcomas, studies of biological rational combinations, and clinicopathologic/biological correlative studies of activity to allow better drug targeting. PMID:27354810

  15. Acute Myeloid Leukemia Presenting as Intracerebral Granulocytic Sarcoma.

    PubMed

    Dhandapani, E; Thirumavalavan; Sowrirajan

    2015-10-01

    The CNS involvement of acute myeloid leukemia (AML) is more commonly manifest as meningeal involvement. Rarely it may present as intravascular tumor aggregates called granulocytic sarcoma which presents as intracranial hemorrhage. We are presenting a case of intracranial, intra-parenchymal granulocytic sarcoma (other names: chloroma, extramedullary myeloblastoma), presenting as acute hemiplegia without cerebral hemorrhage. PMID:27608697

  16. An unusual pleomorphic sarcoma in a hybrid mallard

    USGS Publications Warehouse

    Roffe, Thomas J.

    1987-01-01

    An unusual pleomorphic sarcoma from a hybrid mallard (Anas platyrhynchos) is described. Rhabdomyosarcoma was considered in the original differential diagnoses but rejected due to lack of specific characteristics generally seen in these tumors. The histologic characteristics described are consistent with mammalian sarcomas recorded in the literature as malignant fibrous histiocytoma.

  17. Synovial sarcoma: a rare presentation of parapharyngeal mass.

    PubMed

    Shaariyah, Mohd Mokhtar; Mazita, Ami; Masaany, Mansor; Razif, Mohd Yunus; Isa, Mohamed Rose; Asma, Abdullah

    2010-06-01

    Synovial sarcoma is a rare soft tissue sarcoma of the head and neck region involving the parapharyngeal space. The diagnosis of synovial sarcoma can be very challenging to the pathologists. We present a rare case of parapharyngeal synovial sarcoma in a young female patient who had a two-month history of left cervical intumescent mass at level II. The fine needle aspiration cytology of the mass was proved inconclusive. Transcervical excision of the mass was performed and the first case of parapharyngeal sarcoma was identified in our center by fluorescence in situ hybridization (FISH) technique. Repeat imaging revealed residual tumor. The patient successfully underwent a second excision of the residual tumor and received adjuvant radiotherapy.

  18. Systemic therapy for endometrial stromal sarcomas: current treatment options.

    PubMed

    Serkies, Krystyna; Pawłowska, Ewa; Jassem, Jacek

    2016-01-01

    Uterine endometrial stromal sarcomas including true low-grade endometrial stromal sarcoma (LG-ESS) and high-grade (HG-ESS) or undifferentiated endometrial sarcoma (UES) constitute a group of rare, aggressive malignancies. Most LG-ESSs express steroid receptors. Surgery is the principal primary therapy for endometrial stromal sarcomas and should be considered in all cases. These malignancies are relatively radio- and chemoresistant. Chemotherapy is used in recurrent and advanced HG-ESS and UES. Currently, the combination of gemcitabine and docetaxel is considered the most effective regimen, but at the expense of substantial toxicity. In steroid receptor positive advanced LG-ESS hormonal therapy, mainly with progestins, allows in some patients for a long-term survival. Aromatase inhibitors seem to be equally effective as first- and subsequent-line of treatment, and are well tolerated. The role of molecular-targeted therapies in endometrial stromal sarcomas remains to be established. PMID:27629136

  19. Significance of Circulating Tumor Cells in Soft Tissue Sarcoma

    PubMed Central

    Nicolazzo, Chiara; Gradilone, Angela

    2015-01-01

    Circulating tumor cells can be detected from the peripheral blood of cancer patients. Their prognostic value has been established in the last 10 years for metastatic colorectal, breast, and prostate cancer. On the contrary their presence in patients affected by sarcomas has been poorly investigated. The discovery of EpCAM mRNA expression in different sarcoma cell lines and in a small cohort of metastatic sarcoma patients supports further investigations on these rare tumors to deepen the importance of CTC isolation. Although it is not clear whether EpCAM expression might be originally present on tumor sarcoma cells or acquired during the mesenchymal-epithelial transition, the discovery of EpCAM on circulating sarcoma cells opens a new scenario in CTC detection in patients affected by a rare mesenchymal tumor. PMID:26167450

  20. Retroperitoneal Sarcoma Target Volume and Organ at Risk Contour Delineation Agreement Among NRG Sarcoma Radiation Oncologists

    SciTech Connect

    Baldini, Elizabeth H.; Abrams, Ross A.; Bosch, Walter; Roberge, David; Haas, Rick L.M.; Catton, Charles N.; Indelicato, Daniel J.; Olsen, Jeffrey R.; Deville, Curtiland; Chen, Yen-Lin; Finkelstein, Steven E.; DeLaney, Thomas F.; Wang, Dian

    2015-08-01

    Purpose: The purpose of this study was to evaluate the variability in target volume and organ at risk (OAR) contour delineation for retroperitoneal sarcoma (RPS) among 12 sarcoma radiation oncologists. Methods and Materials: Radiation planning computed tomography (CT) scans for 2 cases of RPS were distributed among 12 sarcoma radiation oncologists with instructions for contouring gross tumor volume (GTV), clinical target volume (CTV), high-risk CTV (HR CTV: area judged to be at high risk of resulting in positive margins after resection), and OARs: bowel bag, small bowel, colon, stomach, and duodenum. Analysis of contour agreement was performed using the simultaneous truth and performance level estimation (STAPLE) algorithm and kappa statistics. Results: Ten radiation oncologists contoured both RPS cases, 1 contoured only RPS1, and 1 contoured only RPS2 such that each case was contoured by 11 radiation oncologists. The first case (RPS 1) was a patient with a de-differentiated (DD) liposarcoma (LPS) with a predominant well-differentiated (WD) component, and the second case (RPS 2) was a patient with DD LPS made up almost entirely of a DD component. Contouring agreement for GTV and CTV contours was high. However, the agreement for HR CTVs was only moderate. For OARs, agreement for stomach, bowel bag, small bowel, and colon was high, but agreement for duodenum (distorted by tumor in one of these cases) was fair to moderate. Conclusions: For preoperative treatment of RPS, sarcoma radiation oncologists contoured GTV, CTV, and most OARs with a high level of agreement. HR CTV contours were more variable. Further clarification of this volume with the help of sarcoma surgical oncologists is necessary to reach consensus. More attention to delineation of the duodenum is also needed.

  1. Pneumothorax as adverse event in patients with lung metastases of soft tissue sarcoma treated with pazopanib: a single reference centre case series

    PubMed Central

    2014-01-01

    Background Recently, the phase III PALETTE study introduced pazopanib (Votrient®) as treatment for adult patients with locally advanced or metastatic non-liposarcoma soft tissue sarcoma after prior treatment with doxorubicin and/or ifosfamide. Pneumothorax was reported as adverse event in 8 of 246 treated patients (3.3%) in that study. This case series presents the incidence and clinic of this complication in the Leiden University Medical Centre. Cases Forty-three patients were treated with pazopanib of which six patients (14.0%) developed a pneumothorax. These six patients were treated for malignant peripheral nerve sheath tumour, angiosarcoma, synovial sarcoma, fibromyxomatoid sarcoma, pleomorphic sarcoma and endometrial stromal sarcoma. All six patients had subpleural pulmonary or pleural metastases at the start of pazopanib and the pneumothorax developed during or shortly after treatment with pazopanib and was difficult to treat. Discussion The incidence reported by us is higher than the incidence in the PALETTE study. Trials with pazopanib in renal cell carcinoma, urothelial carcinoma and cervix carcinoma did not report pneumothorax as an adverse event, suggesting pneumothorax as a specific adverse event in soft tissue sarcoma patients treated with pazopanib. This may be related to the fact that there is often pleural metastatic involvement and cystic degeneration due to pazopanib treatment may add to the risk. Conclusion The risk of an, often difficult to treat, pneumothorax during pazopanib therapy should be discussed with the patient before initiation of treatment for a pulmonary metastasized sarcoma and physicians should be alert to the occurrence of such an event. PMID:25302110

  2. Further experience with Kaposi's sarcoma in Uganda.

    PubMed Central

    Serwadda, D.; Carswell, W.; Ayuko, W. O.; Wamukota, W.; Madda, P.; Downing, R. G.

    1986-01-01

    Four Ugandan patients (1 women, 3 men) with generalized Kaposi's sarcoma (KS) were seen in the Uganda Cancer Institute between October 1983 and December 1984. They presented with generalized lymphadenopathy, plaques/nodules on the body, general swelling of the head, oral and visceral involvement and respiratory distress. Initial responses to adriamycin as a single or a combination chemotherapy of actinomycin D, vincristine, adriamycin and imidazole carboxamide appeared to be favourable but no sustained response was obtained. Serological tests for human T-lymphotropic virus (HTLV-II) antibodies were positive in all 4 cases. Images Figure 1 Figure 2 PMID:3011052

  3. The radiotherapeutic management of chordoid sarcoma

    SciTech Connect

    Hitchon, H.; Nobler, M.P.; Wohl, M.; Levy, W. )

    1990-06-01

    Chordoid sarcoma is a rare neoplasm, with only 13 cases reported. In the past, the treatment of choice was excision or amputation, with radiation therapy reserved for metastases. This case report demonstrates the persistent nature and indolent course the tumor may have and its good response to high-dose irradiation. External beam irradiation, both photons and electrons, intraoperative radiation therapy, and 192-iridium implantation have all been successfully utilized for treatment of multiple recurrences in this patient. Doses of 6,000 cGy or greater were necessary to control the tumor in our patient, and this dose is recommended as adjuvant treatment following conservative (gross) tumor removal.

  4. Management of Sarcoma Metastases to the Lung.

    PubMed

    Digesu, Christopher S; Wiesel, Ory; Vaporciyan, Ara A; Colson, Yolonda L

    2016-10-01

    For decades, surgical resection of pulmonary metastases has been performed; despite limited randomized data, surgery is increasingly accepted as an integral part in the management of metastatic disease. Long-term results indicate resection is potentially curative with significantly improved survival following complete resection. Recurrence, however, is not uncommon with many patients undergoing repeat resection. With advancing surgical technique and adjuvant therapies, patients with high or recurrent tumor burden are increasingly afforded disease control and potential cure. In this review, the prognostic characteristics of pulmonary metastases from sarcoma, preoperative evaluation, operative technique, long-term outcomes, and management of complex patients are highlighted. PMID:27591495

  5. Targeting the DNA Repair Pathway in Ewing Sarcoma

    PubMed Central

    Stewart, Elizabeth; Goshorn, Ross; Bradley, Cori; Griffiths, Lyra M.; Benavente, Claudia; Twarog, Nathaniel R.; Miller, Gregory M.; Caufield, William; Freeman, Burgess B.; Bahrami, Armita; Pappo, Alberto; Wu, Jianrong; Loh, Amos; Karlström, Åsa; Calabrese, Chris; Gordon, Brittney; Tsurkan, Lyudmila; Hatfield, M. Jason; Potter, Philip M.; Snyder, Scott; Thiagarajan, Suresh; Shirinifard, Abbas; Sablauer, Andras; Shelat, Anang A.; Dyer, Michael A.

    2015-01-01

    Ewing sarcoma (EWS) is a tumor of the bone and soft-tissue that primarily affects adolescents and young adults. With current therapies, 70% of patients with localized disease survive, but patients with metastatic or recurrent disease have a poor outcome. We found that EWS cell lines are defective in DNA break repair and are sensitive to PARP inhibitors (PARPis). PARPi-induced cytotoxicity in EWS cells was 10- to 1,000-fold higher after administration of the DNA-damaging agents irinotecan or temozolomide. We developed an orthotopic EWS mouse model and performed pharmacokinetic and pharmacodynamic studies using 3 different PARPis that are in clinical development for pediatric cancer. Irinotecan administered on a low-dose, protracted schedule previously optimized for pediatric patients was an effective DNA-damaging agent when combined with PARPis; it was also better tolerated than combinations with temozolomide. Combining PARPis with irinotecan and temozolomide gave complete and durable responses in more than 80% of the mice. PMID:25437539

  6. Targeted Therapies for Advanced Ewing Sarcoma Family of Tumours

    PubMed Central

    Jiang, Yunyun; Ludwig, Joseph; Janku, Filip

    2015-01-01

    The prognosis of adolescent and young adult patients battling metastatic Ewing Sarcoma Family of Tumours (ESFT) remains less than 30% despite the development of systemic therapies. In the era of personalized medicine, novel molecular targets have been tested in preclinical or clinical settings in ESFT. In this review, we focus on early clinical and translational research that identified multiple molecular targets, including IGF-1R; mTOR; tyrosine kinase inhibitors; EWS-FLI1-related targets, and others. Overall, novel targeted therapies demonstrated modest efficacy; however pronounced and durable antineoplastic responses have been observed in small subsets of treated patients, for example with IGF-1R antibodies. Identifying outcome-predicting biomarkers and overcoming treatment resistance remain major challenges. Due to the rarity of ESFT, multi-institutional collaboration efforts of clinicians, basic and translational scientists are needed in order to understand biology of therapeutic response or resistance, which can lead to development of novel therapeutic methods and improved patient outcomes. PMID:25869102

  7. Prognostic factors in patients with jaw sarcomas.

    PubMed

    Vadillo, Rafael Morales; Contreras, Sonia Julia Sacsaquispe; Canales, Janet Ofelia Guevara

    2011-01-01

    The aim of this study was to identify the prognostic factors related to the survival of patients with sarcomas of the jaw treated in the Dr. Eduardo Caceres Graziani National Institute for Neoplastic Diseases, Lima, Peru. Age, gender, delay in consultation, diagnostic delay, therapeutic delay, tumor size, tumor location, facial asymmetry, pain, treatment type, and histopathological diagnosis were all evaluated as possible prognostic factors that would influence survival in those with jaw sarcomas. In the analysis, the following was used: mortality tables, Kaplan-Meier's product-limit method, log-rank, and Breslow and Tarone-Ware tests; for the prognostic factors, Cox's Regression Model was used. The overall survival rate, with the patient being free from disease at two years, was 55%, and that at five years was 45%. In the independent analysis of the prognostic factors, four variables were statistically significant in influencing survival: gender (p = 0.043), histopathologic diagnosis (p = 0.019), tumor location (p = 0.019), and treatment type (p = 0.030). According to Cox's Regression Model for the multivariate analysis, statistically significant prognostic factors were: gender (p = 0.086), tumor location (p = 0.020), and treatment type (p = 0.092). Thus, the variables of gender, tumor location, and treatment type were determined to be predictive factors for prognosis of survival.

  8. Primary fibro sarcoma of the heart.

    PubMed

    Kabashi, Serbeze; Hoxha, Naim; Gashi, Shkelzen; Ahmegjekaj, Ilir; Bejta, Ilir; Sadiku, Muharrem; Ymeri, Halit; Kabashi, Antigona; Bicaj, Xhavit; Mucaj, Sefedin

    2013-01-01

    Primary malignant heart tumors represent rare entities where fibro sarcoma represents about 3% of all. Introducing the patient: A 15 years old patient with cardiac insufficiency (heart failure) symptoms, such as weakness, cyanosis, palpitations and breathing difficulties; enlargement of upper mediastinum and pleural effusion. Through echocardiography a pericardial effusion and intracavitary thrombus in atrium was diagnosed. With computed tomography is diagnosed a tumoral mass in right atrium which is also spread in the right ventricle of the heart. Tumor is completely removed; pat histology result showed primary fibro sarcoma of the heart. At that time no metastasis was found. Conclusion. Primary malignant heart tumors may manifest like cardiac insufficiency or like systemic diseases. Fibrosarcomas are rare and have bad prognosis. On average patients can live around six months after initial symptoms appeared and diagnosis of the tumor was done. In the case of cardiac insufficiency with differential diagnosis we should also think of heart tumors, which could certainly be proved for or eliminated by echocardiography.

  9. Primary Fibro Sarcoma of the Heart

    PubMed Central

    Kabashi, Serbeze; Hoxha, Naim; Gashi, Shkelzen; Ahmegjekaj, Ilir; Bejta, Ilir; Sadiku, Muharrem; Ymeri, Halit; Kabashi, Antigona; Bicaj, Xhavit; Mucaj, Sefedin

    2013-01-01

    Primary malignant heart tumors represent rare entities where fibro sarcoma represents about 3% of all. Introducing the patient: A 15 years old patient with cardiac insufficiency (heart failure) symptoms, such as weakness, cyanosis, palpitations and breathing difficulties; enlargement of upper mediastinum and pleural effusion. Through echocardiography a pericardial effusion and intracavitary thrombus in atrium was diagnosed. With computed tomography is diagnosed a tumoral mass in right atrium which is also spread in the right ventricle of the heart. Tumor is completely removed; pat histology result showed primary fibro sarcoma of the heart. At that time no metastasis was found. Conclusion. Primary malignant heart tumors may manifest like cardiac insufficiency or like systemic diseases. Fibrosarcomas are rare and have bad prognosis. On average patients can live around six months after initial symptoms appeared and diagnosis of the tumor was done. In the case of cardiac insufficiency with differential diagnosis we should also think of heart tumors, which could certainly be proved for or eliminated by echocardiography. PMID:24167396

  10. Histiocytic sarcoma that mimics benign histiocytosis.

    PubMed

    Boisseau-Garsaud, A M; Vergier, B; Beylot-Barry, M; Nastasel-Menini, F; Dubus, P; de Mascarel, A; Eghbali, H; Beylot, C

    1996-06-01

    A 28-year-old man presented with a histiocytic sarcoma of a very uncommon origin, as it had developed for several years like a benign cutaneous histiocytosis resembling generalized eruptive histiocytoma before becoming acute, with nodal and massive pulmonary involvement. Despite various chemotherapies, the patient died within 8 months. Skin biopsies showed histiocytic proliferation in the dermis and node biopsies showed histiocytic proliferation with a sinusoidal pattern. Immunohistochemical analysis, performed on paraffin-embedded sections, demonstrated strong labeling of tumoral cells for CD68 and moderate labeling for CD3 and CD4. CD30 labeling was negative. S-100 protein was positive on a Langerhans' cell reactive subpopulation. Electron microscopy confirmed the histiocytic nature of malignant cells and showed cytoplasmic inclusions such as regularly laminated bodies, dense bodies and pleomorphic inclusions. No Birbeck granules were seen. A gene rearrangement study of T-cell receptor gamma and immunoglobulin heavy chain genes showed a germline configuration. Histiocytic sarcoma is an extremely rare true histiocytic malignancy, the existence of which has been recently debated since it has often been mistaken in the past for large cell lymphomas. Such a deceptive onset as benign cutaneous histiocytosis has not been described in the literature to our knowledge.

  11. Immunosuppression by Murine Sarcoma Virus (Moloney)

    PubMed Central

    Chan, S. P.; Hook, W. A.; Turner, W.; Chirigos, M. A.

    1970-01-01

    Infection of mice with the murine sarcoma virus (Moloney) markedly suppressed the humoral antibody response to sheep erythrocyte antigen injected 10 days after infection, when tumor size was maximal, and on day 26, when primary tumors had partially regressed. Humoral antibody response was also inhibited when antigen was injected at the time secondary tumors and metastases were evident. No significant suppression of humoral antibody was seen when mice were injected with sheep erythrocyte antigen 5 days after virus infection. Inhibition of the cellular immune response of murine sarcoma virus (Moloney)-infected mice, as measured by the increased survival time of skin grafts, was also determined. Mice that were infected 5 days prior to grafting demonstrated prolonged survival of grafts, suggesting a suppression of cellular immunity. These mice had a graft survival time 14 days greater than noninfected controls. No significant prolongation of graft survival was seen in mice grafted at the times of maximum primary tumor growth, of primary tumor regression, or when secondary tumors had appeared. PMID:16557730

  12. P53 mutations in Ewing's sarcoma.

    PubMed

    Park, Y K; Chi, S G; Kim, Y W; Park, H R; Unni, K K

    2001-01-01

    The p53 tumor suppressor gene is one of the most frequently altered genes in human malignancies. To explore the implication of p53 alteration in Ewing's sarcoma, we analyzed the deletion and sequence alterations of p53 and abnormal amplification of MDM2, which acts as a functional inhibitor of p53, in 35 tissue specimens. Quantitative genomic PCR analysis showed that 2 of 35 tumors have extremely low levels of the p53 gene, indicating a homozygous deletion of the gene. Mutational analysis of exons 4 to 9 of p53 by PCR-SSCP revealed that 3 of 35 tumors carry sequence alterations in exons 5 or 8, and DNA sequencing analysis identified missense point mutations at codon 132 (AAG-->ATG, lysine-->methionine) and codon 135 (TGC-->TCC, cystein-->serine) in exon 5, and codon 287 (GAG-->GTG, glutamic acid-->valine) in exon 8 from these tumors. No abnormal amplification of the MDM2 gene was recognized. Taken together, our data demonstrate that p53 is genetically altered in a small fraction of Ewing's sarcoma.

  13. Adjuvant chemotherapy for soft tissue sarcoma.

    PubMed

    Casali, Paolo G

    2015-01-01

    Adjuvant chemotherapy is not standard treatment in soft tissue sarcoma (STS). However, when the risk of relapse is high, it is an option for shared decision making with the patient in conditions of uncertainty. This is because available evidence is conflicting, even if several randomized clinical trials have been performed for 4 decades and also have been pooled into meta-analyses. Indeed, available meta-analyses point to a benefit in the 5% to 10% range in terms of survival and distant relapse rate. Some local benefit also was suggested by some trials. Placing chemotherapy in the preoperative setting may help gain a local advantage in terms of the quality of surgical margins or decreased sequelae. This may be done within a personalized approach according to the clinical presentation. Attempts to personalize treatment on the basis of the variegated pathology and molecular biology of STS subgroups are ongoing as well, according to what is done in the medical treatment of advanced STS. Thus, decision making for adjuvant and neoadjuvant indications deserves personalization in clinical research and in clinical practice, taking profit from all multidisciplinary clinical skills available at a sarcoma reference center, though with a degree of subjectivity because of the limitations of available evidence. PMID:25993233

  14. Kaposi sarcoma incidence in Mozambique: national and regional estimates.

    PubMed

    Meireles, Paula; Albuquerque, Gabriela; Vieira, Mariana; Foia, Severiano; Ferro, Josefo; Carrilho, Carla; Lunet, Nuno

    2015-11-01

    Kaposi sarcoma is expressed in four clinical variants, all associated with human herpes virus type 8 infection, namely, classic, endemic, immunosuppression-related and AIDS-related. The latter currently accounts for most of the burden of Kaposi sarcoma in sub-Saharan Africa, reflecting the frequency of HIV infection and its management. We aimed to estimate the incidence of Kaposi sarcoma in Mozambique and in its provinces. We estimated the number of incident cases of Kaposi sarcoma by adding up the expected number of endemic and AIDS-related cases. The former were estimated from the rates observed in Kyandondo, Uganda (1960-1971). The latter were computed from the number of AIDS-related deaths in each region, assuming that the ratio between the AIDS-related Kaposi sarcoma incident cases and the number of AIDS-related deaths observed in the city of Beira applies to all regions. A total of 3862 Kaposi sarcoma cases were estimated to have occurred in Mozambique in 2007, mostly AIDS-related, in the age group 25-49 years, and in provinces from South/Centre. The age-standardized incidence rates were 36.1/100 000 in men and 11.5/100 000 in women, with a more than three-fold variation across provinces. We estimated a high incidence of Kaposi sarcoma in Mozambique, along with large regional differences. These results can be used to improve disease management and to sustain political decisions on health policies.

  15. In vitro response of human gingival epithelioid S-G cells to minocycline.

    PubMed

    Babich, H; Tipton, D A

    2002-02-01

    Minocycline, a broad-spectrum antibiotic used in the treatment of acne and periodontal disease and to control inflammatory diseases such as rheumatoid arthritis, has recently been shown to induce a spectrum of adverse health effects. In the light of these contradictory data, this research was directed to provide basic information on the toxicology of minocycline, using in vitro cell culture models, and to evaluate its efficacy in periodontal therapies, particularly for wound healing. The human gingival epithelioid S-G cell line was used as the bioindicator. The greater toxicity of minocycline over doxycycline and tetracycline, related antimicrobial agents, probably correlated with its higher lipophilicity. The cytotoxicity of minocycline was unaffected by an S9 hepatic microsomal fraction, indicating that it is a direct-acting, rather than a metabolism-mediated, cytotoxicant. In comparative toxicity studies, much variation in the degree of sensitivity to minocycline was noted for different cell types. No correlation in the extent of sensitivity to minocycline and the physiologic state of the bioindicator cell (normal, transformed or malignant) was noted. The toxicity of minocycline to the S-G cells was dependent on its concentration and length of exposure. For a continuous 3-day exposure of the S-G cells to minocycline, the midpoint cytotoxicity (or, NR(50)) value, as quantified in the neutral red (NR) assay, was 204 microg/ml on day 1, 84 microg/ml on day 2, and 59 microg/ml on day 3. For a 1-h exposure of the S-G cells in phosphate buffered saline (PBS), the NR(50) value was 780 microg/ml minocycline. Although a 1-h exposure in PBS to 200 microg/ml minocycline exerted some toxicity, the S-G cells recovered on exposure to growth medium; irreversible, progressive damage occurred at 400 microg/ml minocycline and greater. Minocycline, at 50 microg/ml, enhanced attachment of the S-G cells to a gelatin-coated surface and cell migration towards an immobilized

  16. Targeted Chemotherapy in Bone and Soft-Tissue Sarcoma.

    PubMed

    Harwood, Jared L; Alexander, John H; Mayerson, Joel L; Scharschmidt, Thomas J

    2015-10-01

    Historically surgical intervention has been the mainstay of therapy for bone and soft-tissue sarcomas, augmented with adjuvant radiation for local control. Although cytotoxic chemotherapy revolutionized the treatment of many sarcomas, classic treatment regimens are fraught with side effects while outcomes have plateaued. However, since the approval of imatinib in 2002, research into targeted chemotherapy has increased exponentially. With targeted therapies comes the potential for decreased side effects and more potent, personalized treatment options. This article reviews the evolution of medical knowledge regarding sarcoma, the basic science of sarcomatogenesis, and the major targets and pathways now being studied.

  17. A rare case of myeloid sarcoma presenting as anal fissure

    PubMed Central

    VECCHIO, R.; INTAGLIATA, E.; FIUMARA, P.F.; VILLARI, L.; MARCHESE, S.; CACCIOLA, E.

    2015-01-01

    Myeloid sarcoma is a tumor composed of myeloblasts occurring at an extramedullary site. It may develop in patients with acute myeloid leukemia, myeloproliferative or myelodysplastic syndrome, sometimes preceding onset of the systemic disease. Frequent sites of myeloid sarcoma are bones or various soft tissues. Gastrointestinal involvement is very rare. We report a unique case of myeloid sarcoma presenting as a painful anal fissure, in a patient with a history of acute myeloid leukemia. The diagnosis was achieved by a surgical excisional biopsy and immunoistochemical staining. PMID:26712260

  18. Primary Intimal Sarcoma of Thoracic Aorta Presenting as Hypertensive Crisis

    PubMed Central

    Lin, Shu-I; Su, Min-I; Tsai, Cheng-Ting

    2015-01-01

    We report a 45-year-old woman who presented to our facility in a hypertensive crisis. Computed tomography (CT) revealed a thoracic aortic tumor, and tissues obtained via endovascular biopsy revealed undifferentiated sarcoma. A final diagnosis of intimal sarcoma was made by intra-operative pathological examination. Despite undergoing surgical resection followed by adjuvant chemotherapy, the patient died from progressive multiple metastasis and severe sepsis. Although aortic sarcoma is rarely diagnosed, it should be considered a possible etiology of hypertensive crisis. PMID:27122923

  19. Pseudo-Kaposi sarcoma (acroangiodermatitis): occurring after bullous erysipelas.

    PubMed

    Kutlubay, Zekayi; Yardimci, Gürkan; Engin, Burhan; Demirkesen, Cuyan; Aydin, Övgü; Khatib, Rashid; Tuzun, Yalçın

    2015-05-01

    Pseudo-Kaposi sarcoma is a benign reactive vascular proliferative disorder, which can be seen at any age. It occurs when the chronic venous pressure changes result in vascular proliferation in the upper and mid dermis. This disease is divided into two subtypes: the most frequent subtype is the Mali type and seen in early ages. The Mali type is seen in chronic venous insufficiency and in those patients with arteriovenous shunts. The rare subtype is the Stewart-Bluefarb type. This disease must be distinguished from Kaposi sarcoma because of their clinical resemblance. Herein, we present a patient with pseudo-Kaposi sarcoma, which developed after bullous erysipelas.

  20. Ewing Sarcoma in a Patient With Cowden Syndrome.

    PubMed

    Chandhanayingyong, Mod C; Bernthal, Nicholas M; Ungarreevittaya, Piti; Nelson, Scott D; Chawla, Sant P; Singh, Arun S

    2015-11-01

    A 47-year-old woman, initially diagnosed in 1996 with Cowden syndrome (CS), PTEN-mutant bilateral breast cancer, a thyroid nodule, and uterine fibroids, presented to UCLA in 2013 with Ewing sarcoma of the pelvic bone. Her treatment course included mastectomies, hysterectomy/oophorectomy, and total thyroid resection, and chemotherapy, radiation, and hemipelvectomy for Ewing sarcoma. This case report illustrates the unusual presentation of Ewing sarcoma in a patient with PTEN-mutant CS, the probable underlying molecular pathogenesis, long-term management, and therapeutic considerations. PMID:26553762

  1. Pulmonary Artery Sarcoma Masquerading as Chronic Pulmonary Thromboembolism

    PubMed Central

    Coskun, Ugur; Calpar, Ilknur; Yildizeli, Bedrettin; Yanartas, Mehmet; Filinte, Deniz; Kucukoglu, Mehmet Serdar

    2014-01-01

    We describe the case of a 60-year-old woman who presented with pulmonary artery sarcoma, a very rare tumor of the cardiovascular system. Her tumor was initially misdiagnosed as chronic pulmonary thromboembolism, and she underwent pulmonary endarterectomy. Early diagnosis of primary pulmonary artery sarcoma is crucial. That alternative should always be considered before settling on a diagnosis of pulmonary embolism. Suspicion should be aroused by the failure of anticoagulant treatment to alleviate pulmonary perfusion abnormalities and systemic symptoms. Surgical resection of the tumor—preferably by pulmonary endarterectomy, followed by reconstruction as needed—is currently the most promising treatment for pulmonary artery sarcoma. PMID:25425987

  2. [Epidural extraskeletal Ewing sarcoma. Case report and literature review].

    PubMed

    García-Moreno, Rafael; Bernal-García, Luis Miguel; Pineda-Palomo, Manuel; Botana-Fernández, Marcos; Gilete-Tejero, Ignacio Javier; Cabezudo-Artero, José Manuel

    2015-01-01

    Ewing sarcoma is a malignant tumour of the bone that sometimes presents extraskeletal involvement, with the epidural location being rare. We report the case of a 45-year-old woman with paresthesia, paresis and urinary retention. Magnetic resonance imaging showed an epidural mass from C6 to D3. Laminectomy from C7 to D2 and partial resection of the lesion was performed. Pathological analysis was consistent with Ewing sarcoma. The patient received chemotherapy and radiotherapy, without evidence of disease at 8 months follow-up. A review of the literature on all published cases of extraskeletal Ewing sarcoma with epidural involvement is presented. PMID:25497289

  3. Malignant hepatic epithelioid hemangioendothelioma with high-output heart failure: successful management of heart failure with transcatheter arterial chemoembolization.

    PubMed

    Hsu, Chien-Yi; Liu, Yao-Chung; Li, Chung-Pin; Huang, Po-Hsun; Lin, Chin-Hsuan; Chao, Yee

    2014-06-01

    A 73-year-old woman was admitted to hospital because of progressive dyspnea on exertion. Computed tomography revealed a large hepatic tumor, which was proved to be a hepatic epithelioid hemangioendothelioma (EHE). Echocardiography demonstrated high cardiac output, for which the tumor was considered to be the leading cause. A transcatheter arterial chemoembolization (TACE) was performed sequentially at 1-month intervals to reduce the size of the hepatic tumor, and this temporarily improved the patient's cardiac condition and quality of life. In this case, we successfully used TACE in the treatment of hepatic EHE with high-output heart failure. TACE is a reasonable choice of treatment both for managing malignant hepatic tumors and resolving low systemic vascular resistance by embolization of the abnormal neoangiogenic vessels. Nevertheless, clinicians should be aware of the potential adverse effect of hepatic decompensation induced by TACE, especially when the tumor involvement is widespread and poorly preserved hepatic function is encountered.

  4. Epithelioid angiosarcoma of the skin: a study of 18 cases with emphasis on its clinicopathologic spectrum and unusual morphologic features.

    PubMed

    Bacchi, Carlos E; Silva, Tacio R; Zambrano, Eduardo; Plaza, José; Suster, Saul; Luzar, Bostjan; Lamovec, Janez; Pizzolitto, Stefano; Falconieri, Giovanni

    2010-09-01

    We report 18 cases of cutaneous angiosarcoma with predominant or exclusive epithelioid morphology. Both sexes were similarly affected. Patients' ages ranged from 2 to 97 years, median 77.5 years; 2 were pediatric patients. In elderly patients scalp or facial lesions and cutaneous lesions arising within irradiated breast skin predominated. Limb lesions were seen in younger patients. Microscopically, the tumors were composed of packed polygonal cells with focal evidence of endothelial differentiation. Diverging phenotypes included syncytial growth of large cells with clear nuclei and prominent nucleoli, micronodules of tumor cells scattered in dermis, predominance of discohesive plasmacytoid polygonal cells with abundant bright eosinophilic cytoplasm, sheets of clear cells with coarse granular cytoplasm, trabecular and cord arrangement of tumor cells splaying the dermal collagen, or a pseudoglandular appearance owing to clear cell tubular arrangement with open lumina. These cases posed further diagnostic challenges simulating lymphoma, melanoma, lymphoepithelioma-like carcinoma, adnexal carcinoma, and neuroendocrine carcinoma. Immunohistochemical studies showed positivity for CD31 and CD34; no immunoreactivity was documented for other tested antigens including cytokeratins, S100 protein, melanocytic antigens, leukocyte common antigen, and desmin. Therapeutic modalities included combined local excision, chemotherapy, and radiotherapy, depending on patient clinical status. Of the 9 patients available for follow-up, 5 were alive and apparently well, 2 had recurrent disease, and 2 had died of tumor. Our data show that epithelioid cutaneous angiosarcoma may have a broad morphological spectrum, raising interpretive challenges on microscopy. In addition, its clinical presentation seems to differ in nonelderly patients, with lesions likely related to lymphedema or vascular malformations.

  5. Gastrointestinal sarcomas. Analysis of prognostic factors.

    PubMed Central

    McGrath, P C; Neifeld, J P; Lawrence, W; Kay, S; Horsley, J S; Parker, G A

    1987-01-01

    Clinical and pathologic data from 51 patients with primary sarcomas of the gastrointestinal tract treated from 1951 through 1984 were reviewed to determine clinical presentation, histologic features, treatment, and prognostic factors. The most common signs and symptoms were abdominal pain (62%), gastrointestinal bleeding (40%), and/or abdominal mass (38%). The primary site was stomach in 50%, small bowel in 30%, colorectum in 15%, and esophagus in 5%. Virtually all the sarcomas were leiomyosarcomas. Distribution was uniform among the three histologic grades; although 88% of Grade 1 tumors could be completely excised, only 35% of Grade 3 tumors could be completely resected. The 5-year survival rate was 75% for Grade 1 tumors, 16% for Grade 2 tumors, and 28% for Grade 3 tumors (p = 0.0013, Grade 1 vs. 2 and 3). Thirty of the 51 patients (59%) had curative resection with an operative morbidity rate of 24% and an operative mortality rate of 12%; at 5 years the disease-free survival rate was 58% and the overall survival rate was 63% (48% at 10 years). Eleven patients (42%) had recurrent disease develop at a median interval of 2 years after complete tumor excision. Twenty-one patients (41%) had partial excision or biopsy only of their tumors with an operative morbidity rate of 28%, operative mortality rate of 8%, and median survival of only 9 months. Overall, patients whose tumors were confined to the site of origin had a 58% 5-year survival rate compared with 20% for those whose tumors had invaded adjacent organs (p less than 0.05). If the tumor was less than 10 cm in size, the 5-year survival rate was 78%, significantly better than the 38% for tumors greater than 10 cm (p = 0.03). These data suggest that histologic grade, local invasiveness, size, and extent of resection are the most important prognostic factors for patients with primary gastrointestinal sarcomas. Patients who have resection of all gross tumor, especially if it is well differentiated and localized

  6. Multimodality Treatment in Ewing's Sarcoma Family Tumors of the Maxilla and Maxillary Sinus: Review of the Literature

    PubMed Central

    Mamot, Christoph; Krasniqi, Fatime; Metternich, Frank

    2016-01-01

    The Ewing sarcoma family of tumors (ESFT) encompasses a group of highly aggressive, morphologically similar, malignant neoplasms sharing a common spontaneous genetic translocation that affect mostly children and young adults. These predominantly characteristic, small round-cell tumors include Ewing's sarcoma of the bone and soft tissue, as well as primitive neuroectodermal tumors (PNETs) involving the bone, soft tissue, and thoracopulmonary region (Askin's tumor). Extraosseous ESFTs are extremely rare, especially in the head and neck region, where literature to date consists of sporadic case reports and very small series. We hereby present a review of the literature published on ESFTs reported in the maxilla and maxillary sinus region from 1968 to 2016. PMID:27413360

  7. Acute necrotising ulcerative gingivitis in an immunocompromised young adult.

    PubMed

    Hu, Jessie; Kent, Paul; Lennon, Joshua M; Logan, Latania K

    2015-01-01

    Acute necrotising ulcerative gingivitis is an acute onset disease characterised by ulceration, necrosis, pain and bleeding in gingival surfaces. It is predominantly seen in severely malnourished children and young adults with advanced HIV infection. We present a unique presentation in a young adult with high-grade osteogenic sarcoma.

  8. Classic Kaposi's sarcoma in Arabs--widening ethnic involvement.

    PubMed

    Kumar, Pramod

    2011-01-01

    Kaposi's sarcoma is a tumor caused by human herpes virus 8 also known as Kaposi sarcoma-associated herpes virus. Originally described by Kaposi in 1872, this tumor is recognized as an AIDS-defining illness. Classic Kaposi's sarcoma (CKS) is a relatively indolent disease affecting elderly men from the Mediterranean region or of eastern European descent, besides Jews in whom it is the most common. It has been also reported in the Arab population living in Israel. Kaposi's sarcoma has been reported in Arabs after kidney transplantation; however, there are no reports of CKS occurring in non-Israeli Arabs. This is first such article reporting two Arab patients who presented with CKS thus widening the ethnic and geographic area of involvement with this condition.

  9. Sarcomas of the fallopian tube: disentangling a rare entity.

    PubMed

    Zagouri, Flora; Dimopoulos, Meletios-Athanassios; Thomakos, Nikolaos; Chrysikos, Dimosthenis; Papadimitriou, Christos A

    2011-01-01

    Sarcomas of the fallopian tube are exceedingly rare malignancies. They have been considered the most lethal of all gynaecological malignancies with high metastatic potential, frequent recurrences and cancer-related deaths. The reported pathological types of the fallopian tube sarcomas are malignant mixed mullerian (mesodermal) tumours or carcinosarcomas, leiomyosarcomas, rhabdomyosarcomas, liposarcomas, and synovial sarcomas. The rarity of these sarcomas and their often aggressive clinical course has resulted in a relatively limited amount of literature. Thus a single hospital or specialist cannot gain sufficient experience with these tumours. This review article tries to elucidate this uncommon malignancy, in a systematic way, focusing on the different pathological types, epidemiology, risk factors, diagnosis, survival, and different therapeutic modalities (surgery, chemotherapy, and radiotherapy).

  10. What Are the Risk Factors for Soft Tissue Sarcoma?

    MedlinePlus

    ... soft tissue sarcoma. Exposure to dioxin and to herbicides that contain phenoxyacetic acid at high doses (such ... known for certain. There is no evidence that herbicides (weed killers) or insecticides, at levels encountered by ...

  11. What Are the Risk Factors for Uterine Sarcoma?

    MedlinePlus

    ... a uterine sarcoma. Pelvic radiation therapy High-energy (ionizing) radiation used to treat some cancers can damage cells’ DNA, sometimes increasing the risk of developing a second type of cancer . If ...

  12. Sarcoma of the breast: an update on a rare entity.

    PubMed

    Lim, Sue Zann; Ong, Kong Wee; Tan, Benita Kiat Tee; Selvarajan, Sathiyamoorthy; Tan, Puay Hoon

    2016-05-01

    Breast sarcoma is a rare condition. It consists of a heterogeneous group of non-epithelial tumours arising from the mesenchymal tissue of the breast. It has a distinctly different natural history, treatment response and prognosis as compared with carcinoma of the breast. A different diagnostic approach and treatment strategy have to be defined for this group of tumours. Due to its rarity, the current understanding on breast sarcoma is limited and is mostly based on small retrospective case series or case reports. Hence, the management generally follows the algorithms derived from randomised control trials of soft tissue sarcomas in the extremities and chest wall. Through this review, we discuss the results of major retrospective studies on breast sarcomas including data on epidemiology, aetiology, diagnostic approach, treatment strategies and outcomes of this challenging and potentially aggressive condition.

  13. [Immunohistochemistry in the diagnosis of sarcomas].

    PubMed

    Decouvelaere, Anne-Valérie

    2015-01-01

    Immunohistochemistry (IHC) is essential in the diagnosis of soft tissue tumor and must rely on good quality technic. Among useful antibodies, it is important to distinguish those with a poor specificity required in order to establish the broad lineage, from those with high specificity, which may lead straightforward towards the entity. Diagnostically useful antibodies such as myogenin, ALK1 and DOG1 have been recently completed by MUC4 and STAT6 which show good sensitivity and specificity in the diagnosis of low-grade fibromyxoid sarcoma and solitary fibrous tumor respectively. ERG is also an interesting antibody. However, it is not completely specific of vascular tumors. Moreover, available material is often limited because of the increase of microbiopsy specimens. Therefore, it is mandatory to optimize this precious tissue by using these new antibodies, especially because molecular technics are increasingly performed in addition to IHC.

  14. Ewing's sarcoma of the vertebral column

    SciTech Connect

    Pilepich, M.V.; Vietti, T.J.; Nesbit, M.E.; Tefft, M.; Kissane, J.; Burgert, O.; Pritchard, D.; Gehan, E.A.

    1981-01-01

    Twenty-two patients with vertebral primaries were registered in the Intergroup Ewing's Sarcoma Study between 1973 and 1977. The radiation doses to the primary tumors ranged between 3800 and 6200 rad. All patients received intensive combination chemotherapy. After a followup ranging between 14 and 62 months, 14 patients remained disease-free. All patients with primary tumor of the cervical and dorsal spine remained disease-free. Of eight patients with lesions in the distal spine, (sacrococcygeal region) six developed recurrence, in three a local recurrence was observed despite doses of 6000 rad or higher. Doses of 5000 rad or less (in addition to combination chemotherapy as used in the Intergroup Ewing's Study) appear adequate in controlling the primary tumors of the proximal segments of the spinal column.

  15. Cytogenetics findings in a histiocytic sarcoma case.

    PubMed

    Alonso-Dominguez, J M; Calbacho, M; Talavera, M; Villalon, C; Abalo, L; Garcia-Gutierrez, J V; Lozano, S; Tenorio, M; Villarrubia, J; Lopez-Jimenez, J; Ferro, M T

    2012-01-01

    Histiocytic sarcoma (HS) is a neoplasm derived from histiocytes. Its diagnosis was not clear until its immunohistochemistry profile was correctly established. Not much is known about its genetic properties. We report a case of a 48-year-old male patient whose bone marrow was almost completely occupied by monomorphic medium size neoplastic cellularity. Its immunohistochemical profile was CD68(+), CD4(+), CD45(+) with negativity of other dendritic cells, and other lineage markers. Cytogenetic study showed 4 related clones: one with trisomy 8 and extra material on the short arms of chromosome 4; a second line with tetrasomy of chromosome 8, add(4)(p16); the third clone had the same alterations as the previous and deletion of chromosome 3 at q11; the fourth line had tetrasomy 8 and translocation t(3;5)(q25;q35). To our knowledge this is the first HS case showing chromosome 8 trisomy and tetrasomy and the other described alterations. PMID:22937328

  16. Osteogenic Sarcoma: A 21st Century Review.

    PubMed

    Osasan, Stephen; Zhang, Mingyong; Shen, Fan; Paul, Paulose J; Persad, Sujata; Sergi, Consolato

    2016-09-01

    Compared to other bone tumors, bone osteogenic sarcoma (BOS) continues to confer a much grimmer prognosis as the survival benefit of traditional chemotherapy treatment regimens is still unsatisfactory. Chemotherapy was demonstrated to be effective in eradicating both primary tumor and pulmonary metastases in the last century, with effective agents used in various combination regimens having changed the survival rate from less than 10% to 75%. The most common primary bone cancer, BOS is conventionally a primary intramedullary high-grade malignant tumor characterized by malignant cells forming immature bone or osteoid. BOS is a disease with diverse morphological presentations. The treatment of all morphological variants seem to have been the same for over 30 years. The introduction of antiproliferative agents such as insulin growth factor-binding protein 3 hold promise of a potentially veritable therapeutic target. In this review, we highlight recent data on osteosarcoma to consolidate a platform able to connect bench and bedside. PMID:27630274

  17. A Case of Myeloid Sarcoma of Intestine.

    PubMed

    Lim, Sung Won; Lee, Hang Lak; Lee, Kang Nyeong; Jun, Dae Won; Kim, In Young; Kim, Eunjin; Ahn, Hyein; Park, Chan Kum

    2016-09-25

    Myeloid sarcoma (MS) is an extramedullary involvement of immature myeloid proliferation. An isolated MS is defined as a myeloblastic tumor when it arises without any concomitant circulating disease. A diagnosis of MS is established using pathologic features including infiltration of myeloblasts and strong myeloperoxidase expression with negative cytokeratin immunohistochemical staining. We report a rare case of colonic MS without any peripheral blood abnormality. If the affected patient were left untreated, the MS could evolve into acute myeloid leukemia (AML) within one year. Several studies recommend the same regimens of chemotherapy as used for circulating AML to treat isolated MS. We focused on the diagnosis of MS in this study. The correct diagnosis of MS is important for adequate treatment. In conclusion, MS should be considered in the differential diagnosis of intestinal tumor. PMID:27646584

  18. Primary extraosseous Ewing sarcoma of the orbit.

    PubMed

    Alio, Jorge L; Sales-Sanz, Marco; Vaz, Maria A; Barrancos, Constanza; Reguero, Maria E; Diamantopoulus, Jorge; Poveda, Pedro

    2013-01-01

    A 40-year-old man presented with painless, progressive vision loss and mild proptosis of the OD. CT revealed a right intraconal mass with slight penetration of the optic canal not contiguous with any bony structure. Incisional biopsy through a transfrontal orbitotomy revealed a diffuse growth of homogeneous, small, round cells. Immunohistochemical stains were positive for vimentin and MIC2 (CD99), and the translocation at EWS gene (22q12) was detected. Metastatic workup and a full-body bone scan were negative, confirming primary orbital extraosseous Ewing sarcoma. The patient received neoadjuvant chemotherapy and an orbital exenteration with preservation of eyelids and conjunctiva. He also received adjuvant chemotherapy and local radiotherapy, and he has remained disease-free for almost 3 years.

  19. Ewing Sarcoma of the External Ear Canal.

    PubMed

    Binnetoglu, Adem; Baglam, Tekin; Tokuc, Gulnur; Kecelioglu Binnetoglu, Kiymet; Gerin, Fatma; Sari, Murat

    2016-01-01

    Background. Ewing sarcoma (ES) is a high-grade malignant tumor that has skeletal and extraskeletal forms and consists of small round cells. In the head and neck region, reported localization of extraskeletal ES includes the larynx, thyroid gland, submandibular gland, nasal fossa, pharynx, skin, and parotid gland, but not the external ear canal. Methods. We present the unique case of a 2-year-old boy with extraskeletal ES arising from the external ear canal, mimicking auricular hematoma. Results. Surgery was performed and a VAC/IE (vincristine, adriamycin, cyclophosphamide alternating with ifosfamide, and etoposide) regimen was used for adjuvant chemotherapy for 12 months. Conclusion. The clinician should consider extraskeletal ES when diagnosing tumors localized in the head and neck region because it may be manifested by a nonspecific clinical picture mimicking common otorhinolaryngologic disorders. PMID:27313930

  20. Contemporary Management of Retroperitoneal Soft Tissue Sarcomas.

    PubMed

    Olimpiadi, Yuliya; Song, Suisui; Hu, James S; Matcuk, George R; Chopra, Shefali; Eisenberg, Burton L; Sener, Stephen F; Tseng, William W

    2015-08-01

    Management of retroperitoneal soft tissue sarcomas (RP STS) can be very challenging. In contrast to the more common extremity STS, the two predominant histologic subtypes encountered in the retroperitoneum are well-differentiated/dedifferentiated liposarcoma and leiomyosarcoma. Surgery remains the mainstay of treatment for RP STS. Preoperative planning and anticipation of the need for resection of adjacent organs/structures are critical. The extent of surgery, including the role of compartmental resection, is still controversial. Radiation therapy may be an important adjunct to surgery to provide locoregional disease control; this is currently being evaluated in the preoperative setting in the EORTC STRASS trial. Systemic therapy, tailored to the specific histologic subtype, may also be of benefit for the management of RP STS. Further investigation of novel therapies (e.g., targeted therapies, immunotherapy) is needed. Overall, multi-institutional collaboration is important moving forward, to continue to better understand and optimize management of this disease.

  1. Orbital alveolar soft part sarcoma: Histopathologic report of two cases

    PubMed Central

    Alkatan, Hind; Al-Shedoukhy, Ahlam A.; Chaudhry, Imtiaz A.; Al-Ayoubi, Ayman

    2010-01-01

    Alveolar soft part sarcoma is considered as a distinct histopathological entity with rare cases reported from the orbit area. Two cases of alveolar soft part sarcomas occurring in the orbit of two patients along with their histopathologic findings are reported herewith. In both cases, the patients presented with eyelid swelling and proptosis. The diagnosis was made by incisional biopsies and histopathology. The literature is reviewed regarding occurrence of this tumor, its diagnosis and management. PMID:23960876

  2. Male urethral sarcoma: a case report and literature review

    PubMed Central

    Nogueira, Magno Almeida; dos Santos, Guilherme Campelo Lopes; Lopes, Roberto Iglesias; Campos, Octavio Henrique Arcos; Dall’Oglio, Marcos Francisco; Sant’Anna, Alexandre Crippa

    2016-01-01

    ABSTRACT Urethral tumors are rare and aggressive. They usually affect men (2:1) and occur more commonly in white (85% of cases). Soft tissue sarcomas are a heterogeneous group of tumors that arise from embryonic mesoderm. It represents 1% of all cases of urinary tract malignancies and rarely primary affect the ureter. We report a case of male urethral sarcoma. To date, only two similar cases have been published in literature. PMID:26398363

  3. Kaposi’s Sarcoma Herpesvirus Genome Persistence

    PubMed Central

    Juillard, Franceline; Tan, Min; Li, Shijun; Kaye, Kenneth M.

    2016-01-01

    Kaposi’s sarcoma-associated herpesvirus (KSHV) has an etiologic role in Kaposi’s sarcoma, primary effusion lymphoma, and multicentric Castleman’s disease. These diseases are most common in immunocompromised individuals, especially those with AIDS. Similar to all herpesviruses, KSHV infection is lifelong. KSHV infection in tumor cells is primarily latent, with only a small subset of cells undergoing lytic infection. During latency, the KSHV genome persists as a multiple copy, extrachromosomal episome in the nucleus. In order to persist in proliferating tumor cells, the viral genome replicates once per cell cycle and then segregates to daughter cell nuclei. KSHV only expresses several genes during latent infection. Prominent among these genes, is the latency-associated nuclear antigen (LANA). LANA is responsible for KSHV genome persistence and also exerts transcriptional regulatory effects. LANA mediates KSHV DNA replication and in addition, is responsible for segregation of replicated genomes to daughter nuclei. LANA serves as a molecular tether, bridging the viral genome to mitotic chromosomes to ensure that KSHV DNA reaches progeny nuclei. N-terminal LANA attaches to mitotic chromosomes by binding histones H2A/H2B at the surface of the nucleosome. C-terminal LANA binds specific KSHV DNA sequence and also has a role in chromosome attachment. In addition to the essential roles of N- and C-terminal LANA in genome persistence, internal LANA sequence is also critical for efficient episome maintenance. LANA’s role as an essential mediator of virus persistence makes it an attractive target for inhibition in order to prevent or treat KSHV infection and disease. PMID:27570517

  4. Soft tissue sarcoma and occupational exposures

    SciTech Connect

    Wingren, G.; Fredrikson, M.; Brage, H.N.; Nordenskjoeld, B.A.; Axelson, O. )

    1990-08-15

    The associations between soft tissue sarcoma (STS) and occupational exposures were studied in a case-referent study in the southeast of Sweden. Exposure information was obtained through mailed questionnaires to 96 cases, 450 randomly selected population referents, and 200 cancer referents. Odds ratios (OR), were calculated for various occupational groups, and particularly, for occupations with potential exposure to chlorinated phenoxy herbicides and chlorophenols. In the analyses based on population referents, increased risks for soft tissue sarcoma were seen for especially gardeners (OR = 4.1), but also railroad workers (OR = 3.1); construction workers with exposure to impregnating agents (OR = 2.3), asbestos (OR = 1.8), or pressure impregnating agents (OR = 1.7); and unspecified chemical workers with potential exposure to phenoxy herbicides and/or chlorophenols (OR = 1.6). A similar pattern appeared when cancer referents were used although the numerical values of the odds ratios became different. A grouping of jobs resulted in Mantel-Haensel OR from 1.5 to 1.9 for farmers and forestry workers, dependent on referents used and even more increased OR for railroad workers and unspecified chemical workers with potential exposure to phenoxy herbicides and chlorophenols. The results of the study confirm rather than refute that phenoxy herbicides and chlorophenols could be of etiologic importance for STS; the high risk for gardeners, although based on a small number of individuals, was unexpected and remains unclear. Also, since other cancers were used as referents, no definite problems of recall bias should obtain in this material. None of the exposed groups had a higher proportion of smokers than the unexposed group.

  5. Langerhans cell sarcoma: an unusual microscopic presentation.

    PubMed

    Gagnon, A L; Daniel, S; Greer, K; Patterson, J W; Tchernev, G; Chokoeva, A A; Wollina, U; Lotti, T; Fioranelli, M; Roccia, M G; Guarneri, C; Aguilera, N

    2016-01-01

    A 70-year-old Caucasian male presented to our clinic for a pruritic eruption progressing over several months. He complained of fatigue with a 20-pound weight loss over the past year. On presentation, the patient had browny-yellow to violaceous, purpuric, macular and papular lesions on the legs, arms, lower abdomen and back. Initial biopsy showed an angiocentric infiltrate with a suggestion of intraluminal proliferation; CD31 and Fli-1 positivity suggested either reactive angioendotheliomatosis or an unusual intravascular histiocytosis. Further excisional biopsies demonstrated perivascular collections of cells with ample cytoplasm, prominent nuclear pleomorphism and mitotic activity. The nuclei demonstrated nuclear folding, grooves and indentations. The atypical cells were S100, CD1a and CD56 positive with immunohistochemistry. A diagnosis of Langerhans cell sarcoma (LCS) was made. LCS is a rare, aggressive malignancy that can involve multiple organs including the skin, lymph nodes, lung, bone marrow, spleen, heart, and brain. The skin and lymph nodes are commonly involved, and the cutaneous presentation varies greatly. Immunohistochemistry characteristically shows CD1a and S100 positivity. CD56 expression is uncommon and often portends a poor prognosis. There is no established treatment of LCS due to its rarity. Surgery, radiation, and chemotherapy have been used with varied outcomes. Our patient was treated with prednisone with improvement of cutaneous disease. He did not develop systemic involvement, but died 1.5 years later from complications associated with heart failure. Langerhans cell sarcoma should be considered when faced with an unusual angiocentric infiltrate in which initial immunohistochemical staining results may be misleading. PMID:27373133

  6. CD10 immunoreactivity in sarcomatoid carcinomas: comparison with true sarcomas.

    PubMed

    Vennapusa, Bharathi; Fischer, Edgar G; Wick, Mark R; Cerilli, Lisa A

    2011-10-01

    CD10 is a cell surface-related neutral endopeptidase that is involved in cleaving cytokine peptides; it may also play a role in the proliferation of tumor cells and their acquisition of invasiveness. On account of its association with other overtly epithelial neoplasms, we hypothesized that CD10 might be preferentially expressed in the sarcoma-like components of sarcomatoid carcinomas as compared with true sarcomas. Immunohistochemical labeling for CD10 was assessed in various sarcomas and sarcomatoid carcinomas. An aggregate score was generated using both the intensity and extent of staining throughout the neoplasms. Overall, CD10 was expressed more often in true sarcomas (27 of 33 cases) as contrasted with sarcomatoid carcinomas (22 of 34 cases), but with no statistically significant difference between the 2 groups. Uterine "carcinosarcomas" expressed CD10 with accentuation in periglandular tumor cells. The sarcoma-like components in squamous cell carcinoma of the respiratory tract (larynx and lung), tongue, bladder, skin, and penis also expressed CD10 consistently. In the final analysis, there was no difference in CD10 expression between sarcomatoid carcinomas and true sarcomas. This marker seems to have little, if any, differential diagnostic value in the specified histopathologic context.

  7. Radiation-induced sarcomas of the chest wall

    SciTech Connect

    Souba, W.W.; McKenna, R.J. Jr.; Meis, J.; Benjamin, R.; Raymond, A.K.; Mountain, C.F.

    1986-02-01

    Sixteen patients are presented who had sarcomas of the chest wall at a site where a prior malignancy had been irradiated. The first malignancies included breast cancer (ten cases), Hodgkin's disease (four cases), and others (two cases). Radiation doses varied from 4200 to 5500 R (mean, 4900 R). The latency period ranged from 5 to 28 years (mean, 13 years). The histologic types of the radiation-induced sarcomas were as follows: malignant fibrous histiocytoma, nine cases; osteosarcoma, six cases; and malignant mesenchymoma, one case. The only long-term survivor is alive and well 12 years after resection of a clavicular chondroblastic osteosarcoma. Three cases were recently diagnosed. Despite aggressive multimodality treatment, the remaining 13 patients have all died from their sarcomas (mean survival, 13.5 months). All patients have apparently been cured of their first malignancies. Chemotherapy was ineffective. No treatment, including forequarter amputation, appeared to palliate the patients with supraclavicular soft tissue sarcomas. Major chest wall resection offered good palliation for seven of eight patients with sarcomas arising in the sternum or lateral chest wall. Close follow-up is needed to detect signs of these sarcomas in the ever-increasing number of patients receiving therapeutic irradiation.

  8. Cyclin D1 expression and HHV8 in Kaposi sarcoma.

    PubMed Central

    Kennedy, M M; Biddolph, S; Lucas, S B; Howells, D D; Picton, S; McGee, J O; Silva, I; Uhlmann, V; Luttich, K; O'Leary, J J

    1999-01-01

    BACKGROUND: Human herpesvirus 8 (HHV8) appears to be the agent responsible for Kaposi sarcoma. The mechanism remains undetermined but may involve cell cycle regulating genes including D type cyclins which are pivotal in cell cycle progression. Recent HHV8 genetic analysis has revealed the presence of a v-cyclin which is homologous to D type cyclins. AIMS: First, to assess whether there is an independent relation between endogenous cyclin D1 expression in Kaposi sarcoma and HHV8 status; second to determine whether v-cyclin mRNA expression varies with Kaposi sarcoma stage. METHODS: Cyclin D1 immunohistochemistry was performed on 17 paraffin embedded Kaposi sarcoma samples from 16 patients. HHV8 status was assessed in 15 of these using nested polymerase chain reaction (PCR) to ORF 26 and the newly described technique of TaqMan PCR. An additional 10 fresh Kaposi sarcoma samples (early and nodular) were examined for HHV8 v-cyclin RNA. RESULTS: One case, which did not contain amplifiable HHV8, showed strong cyclin D1 staining. The remaining cases were negative or weakly staining; v-cyclin transcript load was higher in early Kaposi sarcoma. CONCLUSIONS: While endogenous cyclin D1 expression is independent of HHV8 status, v-cyclin transcription is higher in early lesions, supporting the "viral hit" hypothesis. Images PMID:10645225

  9. Immunoprecipitation of membrane proteins of cultured human sarcoma cells.

    PubMed

    Grófová, M; Forchhammer, J; Lizonová, A; Popovic, M

    1981-01-01

    Human sarcoma associated antigens (HSAA) have previously been identified by indirect immune fluorescence in human sarcoma cells in culture using sera from patients bearing different types of sarcoma. To further characterize these HSAA, surface proteins of cultured cells were labeled with 125Iodine, [3H]-glucosamine and [35S]-methionine and solubilized. After immunoprecipitation labeled proteins were detected in immune complexes by SDS polyacrylamide gel electrophoresis and autoradiography, which allowed comparison with antigens described by other groups. A surface protein (Mr 96 000) was precipitated with sera from sarcoma bearing patients, and two glycoproteins (Mr 115 000 and 85 000) were preferentially precipitated with antisera from rabbits immunized with membranes from two human sarcoma cell lines. At least two of these proteins were found in each of five human sarcoma cell lines studied (U-4SS, U-3930S, U-20S, B-5GT and B-6FS). None of the proteins were precipitated with three human control sera, and only occasionally a faint band was observed in immunoprecipitates from control cells (B-25F, B-41B, B-42FC, U-2S, and U-393S with the immune sera. These proteins are probably some of the antigens responsible for the immune fluorescence observed in determination of HSAA. However, purification of the proteins and competition experiments are needed before this can be finally established.

  10. The role of radiology in paediatric soft tissue sarcomas

    PubMed Central

    van Rijn, R.; McHugh, K.

    2008-01-01

    Abstract Paediatric soft tissue sarcomas (STS) are a group of malignant tumours that originate from primitive mesenchymal tissue and account for 7% of all childhood tumours. Rhabdomyosarcomas (RMS) and undifferentiated sarcomas account for approximately 50% of soft tissue sarcomas in children and non-rhabdomyomatous soft tissue sarcomas (NRSTS) the remainder. The prognosis and biology of STS tumours vary greatly depending on the age of the patient, the primary site, tumour size, tumour invasiveness, histologic grade, depth of invasion, and extent of disease at diagnosis. Over recent years, there has been a marked improvement in survival rates in children and adolescents with soft tissue sarcoma and ongoing international studies continue to aim to improve these survival rates whilst attempting to reduce the morbidity associated with treatment. Radiology plays a crucial role in the initial diagnosis and staging of STS, in the long term follow-up and in the assessment of many treatment related complications. We review the epidemiology, histology, clinical presentation, staging and prognosis of soft tissue sarcomas and discuss the role of radiology in their management. PMID:18442956

  11. The Genomic Landscape of the Ewing Sarcoma Family of Tumors Reveals Recurrent STAG2 Mutation

    PubMed Central

    Brohl, Andrew S.; Solomon, David A.; Chang, Wendy; Wang, Jianjun; Song, Young; Sindiri, Sivasish; Patidar, Rajesh; Hurd, Laura; Chen, Li; Shern, Jack F.; Liao, Hongling; Wen, Xinyu; Gerard, Julia; Kim, Jung-Sik; Lopez Guerrero, Jose Antonio; Machado, Isidro; Wai, Daniel H.; Picci, Piero; Triche, Timothy; Horvai, Andrew E.; Miettinen, Markku; Wei, Jun S.; Catchpool, Daniel; Llombart-Bosch, Antonio; Waldman, Todd; Khan, Javed

    2014-01-01

    The Ewing sarcoma family of tumors (EFT) is a group of highly malignant small round blue cell tumors occurring in children and young adults. We report here the largest genomic survey to date of 101 EFT (65 tumors and 36 cell lines). Using a combination of whole genome sequencing and targeted sequencing approaches, we discover that EFT has a very low mutational burden (0.15 mutations/Mb) but frequent deleterious mutations in the cohesin complex subunit STAG2 (21.5% tumors, 44.4% cell lines), homozygous deletion of CDKN2A (13.8% and 50%) and mutations of TP53 (6.2% and 71.9%). We additionally note an increased prevalence of the BRCA2 K3326X polymorphism in EFT patient samples (7.3%) compared to population data (OR 7.1, p = 0.006). Using whole transcriptome sequencing, we find that 11% of tumors pathologically diagnosed as EFT lack a typical EWSR1 fusion oncogene and that these tumors do not have a characteristic Ewing sarcoma gene expression signature. We identify samples harboring novel fusion genes including FUS-NCATc2 and CIC-FOXO4 that may represent distinct small round blue cell tumor variants. In an independent EFT tissue microarray cohort, we show that STAG2 loss as detected by immunohistochemistry may be associated with more advanced disease (p = 0.15) and a modest decrease in overall survival (p = 0.10). These results significantly advance our understanding of the genomic and molecular underpinnings of Ewing sarcoma and provide a foundation towards further efforts to improve diagnosis, prognosis, and precision therapeutics testing. PMID:25010205

  12. Dasatinib inhibits migration and invasion in diverse human sarcoma cell lines and induces apoptosis in bone sarcoma cells dependent on SRC kinase for survival.

    PubMed

    Shor, Audrey C; Keschman, Elizabeth A; Lee, Francis Y; Muro-Cacho, Carlos; Letson, G Douglas; Trent, Jonathan C; Pledger, W Jack; Jove, Richard

    2007-03-15

    Sarcomas are rare malignant mesenchymal tumors for which there are limited treatment options. One potential molecular target for sarcoma treatment is the Src tyrosine kinase. Dasatinib (BMS-354825), a small-molecule inhibitor of Src kinase activity, is a promising cancer therapeutic agent with p.o. bioavailability. Dasatinib exhibits antitumor effects in cultured human cell lines derived from epithelial tumors, including prostate and lung carcinomas. However, the action of dasatinib in mesenchymally derived tumors has yet to be shown. Based on our previous findings of Src activation in human sarcomas, we evaluated the effects of dasatinib in 12 cultured human sarcoma cell lines derived from bone and soft tissue sarcomas. Dasatinib inhibited Src kinase activity at nanomolar concentrations in these sarcoma cell lines. Downstream components of Src signaling, including focal adhesion kinase and Crk-associated substrate (p130(CAS)), were also inhibited at similar concentrations. This inhibition of Src signaling was accompanied by blockade of cell migration and invasion. Moreover, apoptosis was induced in the osteosarcoma and Ewing's subset of bone sarcomas at nanomolar concentrations of dasatinib. Inhibition of Src protein expression by small interfering RNA also induced apoptosis, indicating that these bone sarcoma cell lines are dependent on Src activity for survival. These results show that dasatinib inhibits migration and invasion of diverse sarcoma cell types and selectively blocks the survival of bone sarcoma cells. Therefore, dasatinib may provide therapeutic benefit by preventing the growth and metastasis of sarcomas in patients.

  13. A European project on incidence, treatment, and outcome of sarcoma

    PubMed Central

    2010-01-01

    Background Sarcomas are rare tumors (1-2% of all cancers) of mesenchymal origin that may develop in soft tissues and viscera. Since the International Classification of Disease (ICD) attributes visceral sarcomas (VS) to the organ of origin, the incidence of sarcoma is grossly underestimated. The rarity of the disease and the variety of histological types (more than 70) or locations account for the difficulty in acquiring sufficient personal experience. In view of the above the European Commission funded the project called Connective Tissues Cancers Network (CONTICANET), to improve the prognosis of sarcoma patients by increasing the level of standardization of diagnostic and therapeutic procedures through a multicentre collaboration. Methods/Design Two protocols of epidemiological researches are here presented. The first investigation aims to build the population-based incidence of sarcoma in a two-year period, using the new 2002 WHO classification and the "second opinion" given by an expert regional pathologist on the initial diagnosis by a local pathologist. A three to five year survival rate will also be determined. Pathology reports and clinical records will be the sources of information. The second study aims to compare the effects on survival or relapse-free period - allowing for histological subtypes, clinical stage, primary site, age and gender - when the disease was treated or not according to the clinical practice guidelines (CPGs). Discussion Within CONTICANET, each group was asked to design a particular study on a specific objective, the partners of the network being free to accept or not the proposed protocol. The first protocol was accepted by the other researchers, therefore the incidence of sarcoma will be assessed in three European regions, Rhone-Alpes and Aquitaine (France) and Veneto (Italy), where the geographic distribution of sarcoma will be compared after taking into account age and gender. The conformity of the clinical practice with the

  14. Immunostimulation by OX40 Ligand Transgenic Ewing Sarcoma Cells.

    PubMed

    Reuter, Dajana; Staege, Martin S; Kühnöl, Caspar D; Föll, Jürgen

    2015-01-01

    Interleukin-2 (IL-2) transgenic Ewing sarcoma cells can induce tumor specific T and NK cell responses and reduce tumor growth in vivo and in vitro. Nevertheless, the efficiency of this stimulation is not high enough to inhibit tumor growth completely. In addition to recognition of the cognate antigen, optimal T-cell stimulation requires signals from so-called co-stimulatory molecules. Several members of the tumor necrosis factor superfamily have been identified as co-stimulatory molecules that can augment antitumor immune responses. OX40 (CD134) and OX40 ligand (OX40L = CD252; also known as tumor necrosis factor ligand family member 4) is one example of such receptor/ligand pair with co-stimulatory function. In the present investigation, we generated OX40L transgenic Ewing sarcoma cells and tested their immunostimulatory activity in vitro. OX40L transgenic Ewing sarcoma cells showed preserved expression of Ewing sarcoma-associated (anti)gens including lipase member I, cyclin D1 (CCND1), cytochrome P450 family member 26B1 (CYP26B1), and the Ewing sarcoma breakpoint region 1-friend leukemia virus integration 1 (EWSR1-FLI1) oncogene. OX40L-expressing tumor cells showed a trend for enhanced immune stimulation against Ewing sarcoma cells in combination with IL-2 and stimulation of CD137. Our data suggest that inclusion of the OX40/OX40L pathway of co-stimulation might improve immunotherapy strategies for the treatment of Ewing sarcoma. PMID:26579494

  15. Sarcoma Cell Line Screen of Oncology Drugs and Investigational Agents Identifies Patterns Associated with Gene and microRNA Expression.

    PubMed

    Teicher, Beverly A; Polley, Eric; Kunkel, Mark; Evans, David; Silvers, Thomas; Delosh, Rene; Laudeman, Julie; Ogle, Chad; Reinhart, Russell; Selby, Michael; Connelly, John; Harris, Erik; Monks, Anne; Morris, Joel

    2015-11-01

    The diversity in sarcoma phenotype and genotype make treatment of this family of diseases exceptionally challenging. Sixty-three human adult and pediatric sarcoma lines were screened with 100 FDA-approved oncology agents and 345 investigational agents. The investigational agents' library enabled comparison of several compounds targeting the same molecular entity allowing comparison of target specificity and heterogeneity of cell line response. Gene expression was derived from exon array data and microRNA expression was derived from direct digital detection assays. The compounds were screened against each cell line at nine concentrations in triplicate with an exposure time of 96 hours using Alamar blue as the endpoint. Results are presented for inhibitors of the following targets: aurora kinase, IGF-1R, MEK, BET bromodomain, and PARP1. Chemical structures, IC50 heat maps, concentration response curves, gene expression, and miR expression heat maps are presented for selected examples. In addition, two cases of exceptional responders are presented. The drug and compound response, gene expression, and microRNA expression data are publicly available at http://sarcoma.cancer.gov. These data provide a unique resource to the cancer research community. PMID:26351324

  16. Targeting the epigenetic readers in Ewing Sarcoma inhibits the oncogenic transcription factor EWS/Fli1

    PubMed Central

    Jacques, Camille; Lamoureux, François; Baud’huin, Marc; Calleja, Lidia Rodriguez; Quillard, Thibaut; Amiaud, Jérôme; Tirode, Franck; Rédini, Françoise; Bradner, James E.; Heymann, Dominique; Ory, Benjamin

    2016-01-01

    Ewing Sarcoma is a rare bone and soft tissue malignancy affecting children and young adults. Chromosomal translocations in this cancer produce fusion oncogenes as characteristic molecular signatures of the disease. The most common case is the translocation t (11; 22) (q24;q12) which yields the EWS-Fli1 chimeric transcription factor. Finding a way to directly target EWS-Fli1 remains a central therapeutic approach to eradicate this aggressive cancer. Here we demonstrate that treating Ewing Sarcoma cells with JQ1(+), a BET bromodomain inhibitor, represses directly EWS-Fli1 transcription as well as its transcriptional program. Moreover, the Chromatin Immuno Precipitation experiments demonstrate for the first time that these results are a consequence of the depletion of BRD4, one of the BET bromodomains protein from the EWS-Fli1 promoter. In vitro, JQ1(+) treatment reduces the cell viability, impairs the cell clonogenic and the migratory abilities, and induces a G1-phase blockage as well as a time- and a dose-dependent apoptosis. Furthermore, in our in vivo model, we observed a tumor burden delay, an inhibition of the global vascularization and an increase of the mice overall survival. Taken together, our data indicate that inhibiting the BET bromodomains interferes with EWS-FLi1 transcription and could be a promising strategy in the Ewing tumors context. PMID:27006472

  17. Targeting the epigenetic readers in Ewing sarcoma inhibits the oncogenic transcription factor EWS/Fli1.

    PubMed

    Jacques, Camille; Lamoureux, François; Baud'huin, Marc; Rodriguez Calleja, Lidia; Quillard, Thibaut; Amiaud, Jérôme; Tirode, Franck; Rédini, Françoise; Bradner, James E; Heymann, Dominique; Ory, Benjamin

    2016-04-26

    Ewing Sarcoma is a rare bone and soft tissue malignancy affecting children and young adults. Chromosomal translocations in this cancer produce fusion oncogenes as characteristic molecular signatures of the disease. The most common case is the translocation t (11; 22) (q24;q12) which yields the EWS-Fli1 chimeric transcription factor. Finding a way to directly target EWS-Fli1 remains a central therapeutic approach to eradicate this aggressive cancer. Here we demonstrate that treating Ewing Sarcoma cells with JQ1(+), a BET bromodomain inhibitor, represses directly EWS-Fli1 transcription as well as its transcriptional program. Moreover, the Chromatin Immuno Precipitation experiments demonstrate for the first time that these results are a consequence of the depletion of BRD4, one of the BET bromodomains protein from the EWS-Fli1 promoter. In vitro, JQ1(+) treatment reduces the cell viability, impairs the cell clonogenic and the migratory abilities, and induces a G1-phase blockage as well as a time- and a dose-dependent apoptosis. Furthermore, in our in vivo model, we observed a tumor burden delay, an inhibition of the global vascularization and an increase of the mice overall survival. Taken together, our data indicate that inhibiting the BET bromodomains interferes with EWS-FLi1 transcription and could be a promising strategy in the Ewing tumors context. PMID:27006472

  18. Molecular Characterization of Synovial Sarcoma in Children and Adolescents: Evidence of Akt Activation1

    PubMed Central

    Bozzi, Fabio; Ferrari, Andrea; Negri, Tiziana; Conca, Elena; Luca, Da Riva; Losa, Marco; Casieri, Paola; Orsenigo, Marta; Lampis, Andrea; Meazza, Cristina; Casanova, Michela; Pierotti, Marco A; Tamborini, Elena; Pilotti, Silvana

    2008-01-01

    Synovial sarcoma (SS) is the most frequent nonrhabdomyosarcomatous soft tissue sarcoma encountered in adolescents and young adults, and despite advances in the treatment of local disease, metastases remain the main cause of death. The aim of this study was to characterize a single-center series of pediatric SS molecularly to seek any biomarkers or pathways that might make suitable targets for new agents. Seventeen cases of pediatric SS showing the SYT-SSX fusion transcript were screened immunohistochemically, biochemically, molecularly, and cytogenetically (depending on the available material) to investigate any expression/activation of epidermal growth factor receptor, platelet-derived growth factor receptor alpha (PDGFRα), PDGFRβ, Akt, and deregulated Wnt pathway. The most relevant outcome was the finding of activated epidermal growth factor receptor, PDGFRα, and PDGFRβ, which activated Akt in both the monophasic and biphasic histologic subtypes. Consistently, Akt activation was completely abolished in an SS cell line assay when stimulated by PDGF-AA and treated with the phosphatidylinositol 3-kinase inhibitor LY294002. Our results also showed the nuclear localization of β-catenin and cyclin D1 gene products in monophasic SS and the movement of β-catenin into the cytoplasm in the glandular component of the biphasic subtype. Although they need to be confirmed in larger series, these preliminary data suggest that therapeutic strategies including specific inhibitors of the phosphatidylinositol 3-kinase/Akt pathway might be exploited in SS. PMID:18633459

  19. C11orf95-RELA fusion present in a primary supratentorial ependymoma and recurrent sarcoma.

    PubMed

    Cachia, David; Wani, Khalida; Penas-Prado, Marta; Olar, Adriana; McCutcheon, Ian E; Benjamin, Robert S; Armstrong, Terri S; Gilbert, Mark R; Aldape, Kenneth D

    2015-04-01

    Ependymomas are rare glial tumors of the central nervous system that arise from the cells lining the ventricles and central canal within the spinal cord. The distribution of these tumors along the neuroaxis varies by age, most commonly involving the spinal cord in adults and the posterior fossa in children. It is becoming evident that ependymomas of infratentorial, supratentorial, and spinal cord location are genetically distinct which may explain the differences in clinical outcomes. A novel oncogenic fusion involving the C11orf95 and RELA genes was recently described in supratentorial ependymomas that results in constitutive aberrant activation of the nuclear factor-kB signaling pathway. Ependymosarcomas are rare neoplasms in which a malignant mesenchymal component arises within an ependymoma. We here describe a case of a sarcoma developing in a patient previously treated with chemotherapy and radiation whose original ependymoma and recurrent sarcoma were both shown to carry the type 1 C11orf95-RELA fusion transcript indicating a monoclonal origin for both tumors. PMID:25388523

  20. Blue Cell Tumour at Unusual Site: Retropritoneal Ewings Sarcoma.

    PubMed

    Javalgi, Anita P; Karigoudar, Mahesh H; Palur, Katyayani

    2016-04-01

    Ewing's sarcoma is a highly malignant tumour of osseous or non-osseous origin, tremed as extra-skeletal Ewings sarcoma if arising from soft tissue. It is rare occurrence tumor most commonly occurring in paravertebral area, chest wall, head & neck and retroperitoneum. Reporting an interesting case of retroperitoneal Ewing's sarcoma in 39 years old female. Patient had complains of abdominal discomfort & vague pain since 2 months, following weakness in lower limb and loss of weight. On detail history and examination she was further referred to detail pathological and radiological investigations. Haematological profile, renal function test and liver function test were in normal limits. USG abdomen was normal, MRI showed a mass in pelvis retroperitoneum measuring 10x10cms, bilateral ovaries and tubes were normal. Because of retroperitoneal nature of tumor and suspicion of uterine sarcoma, laparotomy was performed. The large retroperitoneal mass adherent to posterior of uterus was excised and send for histopathological diagnosis. On gross and microscopy examination the diagnosis of blue cell tumor with PAS positivity, possibility of extraskeletal Ewing's sarcoma/primitive neuro-ectodermal tumor was made which was further confirmed by immunohistochemistry, positive for S100, Vementin and CD99 and negative for desmin and CK. Confirmed diagnosis help in accurate management and improves survival rate. PMID:27190820

  1. Synovial Sarcoma in Head and Neck: A Case Report

    PubMed Central

    Fonseca, Adriano Santana; Azevedo, Amanda Canário Andrade; Magalhães, Fabíola Moreira; Andrade, Nilvano Alves de

    2013-01-01

    Introduction Synovial sarcoma is a malignant tumor of mesenchymal pluripotent cells. Objectives We present a case of synovial sarcoma in the posterolateral wall of the oropharynx. Resumed report The patient, a 23-year-old woman, was admitted with a history of dysphagia and difficulty in breathing for 8 months, resulting in progressive deterioration and onset of snoring, muffled voice, and local pain. An oropharyngeal tumor in the left posterolateral wall touched the base of the ipsilateral tongue. The patient underwent endoscopic pharyngectomy to remove the lesion. Pathologic examination revealed synovial sarcoma with positive margins, and Mohs technique was proposed for margin control. The margins were disease-free, without the need for total laryngectomy. The pharynx was reconstructed with a microvascular forearm flap. The patient developed postoperative stability. Conclusion Despite its name, synovial sarcoma is rarely sourced directly from synovial membranes. It is most commonly found in the vicinity of large joints. The location at the head and neck, a location poor in synovial tissue, is unusual. Synovial sarcoma in the head and neck has an aggressive nature and poor prognosis. Resection with negative margins remains the foundation of therapy, which is not so easily achieved in the head and neck. It is important for the otorhinolaryngologist and head and neck surgeon to be familiar with this aggressive tumor, which carries high mortality and morbidity. The appropriate diagnosis and treatment can improve prognosis and patient survival. PMID:25992071

  2. Immediate versus Delayed Sarcoma Reconstruction: Impact on Outcomes

    PubMed Central

    Sanniec, Kyle J.; Zhang, Nan; III, William J. Casey

    2016-01-01

    Background. Sarcoma is a rare malignancy, and more recent management algorithms emphasize a multidisciplinary approach and limb salvage, which has resulted in an increase in overall survival and limb preservation. However, limb salvage has resulted in a higher rate of wound complications. Objective. To compare the complications between immediate and delayed (>three weeks) reconstruction in the multidisciplinary limb salvage sarcoma patient population. Methods. A ten-year retrospective review of patients who underwent sarcoma resection was performed. The outcome of interest was wound complication in the postoperative period based on timing of reconstruction. We defined infection as any infection requiring intravenous antibiotics, partial flap failure as any flap requiring a debridement or revision, hematoma/seroma as any hematoma/seroma requiring drainage, and wound dehiscence as a wound that was not completely intact by three weeks postoperatively. Results. 70 (17 delayed, 53 immediate) patients who underwent sarcoma resection and reconstruction met the inclusion criteria. Delayed reconstruction significantly increased the incidence of postoperative wound infection and wound dehiscence. There was no difference in partial or total flap loss, hematoma, or seroma between the two groups. Discussion and Conclusion. Immediate reconstruction results in decreased wound complications may reduce the morbidity associated with multidisciplinary treatment in the limb salvage sarcoma patient. PMID:27478403

  3. Sarcomas arising after radiotherapy for peptic ulcer disease

    SciTech Connect

    Lieber, M.R.; Winans, C.S.; Griem, M.L.; Moossa, R.; Elner, V.M.; Franklin, W.A.

    1985-06-01

    Therapeutic gastric irradiation has been used to reduce peptic juice secretion in patients with peptic ulcer disease. Between 1937 and 1968 a total of 2049 patients received such therapy at the University of Chicago. Three of these patients are known to have developed sarcomas in the field of radiation. Two gastric leiomyosarcomas of the stomach were diagnosed 26 and 14 years after treatment and a malignant fibrous histiocytoma of the anterior chest wall was removed six years after gastric irradiation. Of 743 peptic ulcer patients treated without irradiation and constituted as a control group for the study of therapeutic gastric radiation, none is known to have developed sarcoma. As the incidence of sarcoma in these patient groups is known only from the tumor registry of the University of Chicago, other cases of sarcoma may exist in the groups. While an increased incidence of sarcoma has not been proven to occur in patients who received therapeutic gastric irradiation for peptic ulcer disease, the possibility of such a risk should be borne in mind by physicians caring for such patients.

  4. Targeting the p53 Pathway in Ewing Sarcoma

    PubMed Central

    Neilsen, Paul M.; Pishas, Kathleen I.; Callen, David F.; Thomas, David M.

    2011-01-01

    The p53 tumour suppressor plays a pivotal role in the prevention of oncogenic transformation. Cancers frequently evade the potent antitumour surveillance mechanisms of p53 through mutation of the TP53 gene, with approximately 50% of all human malignancies expressing dysfunctional, mutated p53 proteins. Interestingly, genetic lesions in the TP53 gene are only observed in 10% of Ewing Sarcomas, with the majority of these sarcomas expressing a functional wild-type p53. In addition, the p53 downstream signaling pathways and DNA-damage cell cycle checkpoints remain functionally intact in these sarcomas. This paper summarizes recent insights into the functional capabilities and regulation of p53 in Ewing Sarcoma, with a particular focus on the cross-talk between p53 and the EWS-FLI1 gene rearrangement frequently associated with this disease. The development of several activators of p53 is discussed, with recent evidence demonstrating the potential of small molecule p53 activators as a promising systemic therapeutic approach for the treatment of Ewing Sarcomas with wild-type p53. PMID:21197471

  5. Kaposi's sarcoma in renal transplant recipients.

    PubMed

    Zmonarski, Sławomir C; Boratyńska, Maria; Puziewicz-Zmonarska, Anna; Kazimierczak, Krzysztof; Klinger, Marian

    2005-01-01

    Kaposi's sarcoma (KS) is a spindle-shaped vascular cell tumor that occurs in the skin, lymphoid, respiratory and gastrointestinal tissues. It may resemble aggressive malignant neoplasm in HIV-related or in post-transplant types but classic form may behave as benign, potentially controllable and reversible hyperplasia. KS lesions from the onset are dispersed and multicentric. KS probability increases in solid organ transplant recipients (approximately 3/1000 patients). KS occurrence is associated with: type and dose of immunosuppression, chronic stimulation by foreign allograft antigens, viral infections (Herpes virus 8), anti rejection and induction therapy, etc. 90% of KS cases appear as dark blue or purplish macular lesions that may form nodular tumors. Histological picture shows networks of spindle shaped cells and vascular spaces surrounded by an endothelial cell layer. There is no uniform schema of KS treatment in renal transplant recipients. Immunosuppression must be reduced to the lowest levels which preserve allograft function. CsA should be converted to mofetil mycophenolate or mTOR-inhibitors. After conversion to MMF regression of KS was observed, although low therapeutic MMF doses seem to be appropriate. Sirolimus seems to inhibit the growth of established vascularized tumors and this effect is best realized with relatively low immunosuppressive doses of drug. PMID:16218035

  6. Current and future options in the management and treatment of uterine sarcoma

    PubMed Central

    du Bois, Andreas; Heitz, Florian; Kurzeder, Christian; Sehouli, Jalid; Harter, Philipp

    2014-01-01

    Uterine sarcomas are rare aggressive mesenchymal tumours with limited prognosis. They encompass various histological subtypes such leiomyosarcoma, endometrial stromal sarcoma and undifferentiated sarcomas with different surgical and medical strategies. Current evidence of surgery, adjuvant and palliative therapy is reported. PMID:24381658

  7. Epithelioid osteoblastoma of maxilla: A rare and aggressive variant of a benign neoplasm at an uncommon site

    PubMed Central

    Rana, Vandana; Saxena, Vivek; Sahai, Kavita; Singh, Giriraj

    2016-01-01

    Osteoblastoma (OB) is an uncommon benign bone-forming tumor accounting for <1% of all bone neoplasms. Unlike conventional OB, its small subset variant “Epithelioid osteoblastoma (EO)” is characterized by its propensity for local invasion and recurrent behavior. This rare variant of an uncommon tumor when occurs in an atypical site can lead to diagnostic problems more so due to ambiguous clinico-radiologic presentation. This was what faced in the present case of 18-year-old female with a swelling in upper jaw. OB is usually more common in males and involves primarily the posterior element of the spine and the sacrum (40–55%). Less frequently, long bones of limbs are involved. Clinical, radiological and histopathological correlation in this case guided us to reach at right diagnosis of EO which helped the patient in getting correct treatment which involves surgical excision over conventional curettage. The purpose behind this case presentation is to improve the awareness about this recurrent tumor variant which has many close differentials including well-differentiated osteoblastic osteosarcoma. PMID:27601840

  8. Epithelioid osteoblastoma of maxilla: A rare and aggressive variant of a benign neoplasm at an uncommon site.

    PubMed

    Rana, Vandana; Saxena, Vivek; Sahai, Kavita; Singh, Giriraj

    2016-01-01

    Osteoblastoma (OB) is an uncommon benign bone-forming tumor accounting for <1% of all bone neoplasms. Unlike conventional OB, its small subset variant "Epithelioid osteoblastoma (EO)" is characterized by its propensity for local invasion and recurrent behavior. This rare variant of an uncommon tumor when occurs in an atypical site can lead to diagnostic problems more so due to ambiguous clinico-radiologic presentation. This was what faced in the present case of 18-year-old female with a swelling in upper jaw. OB is usually more common in males and involves primarily the posterior element of the spine and the sacrum (40-55%). Less frequently, long bones of limbs are involved. Clinical, radiological and histopathological correlation in this case guided us to reach at right diagnosis of EO which helped the patient in getting correct treatment which involves surgical excision over conventional curettage. The purpose behind this case presentation is to improve the awareness about this recurrent tumor variant which has many close differentials including well-differentiated osteoblastic osteosarcoma. PMID:27601840

  9. Epithelioid osteoblastoma of maxilla: A rare and aggressive variant of a benign neoplasm at an uncommon site

    PubMed Central

    Rana, Vandana; Saxena, Vivek; Sahai, Kavita; Singh, Giriraj

    2016-01-01

    Osteoblastoma (OB) is an uncommon benign bone-forming tumor accounting for <1% of all bone neoplasms. Unlike conventional OB, its small subset variant “Epithelioid osteoblastoma (EO)” is characterized by its propensity for local invasion and recurrent behavior. This rare variant of an uncommon tumor when occurs in an atypical site can lead to diagnostic problems more so due to ambiguous clinico-radiologic presentation. This was what faced in the present case of 18-year-old female with a swelling in upper jaw. OB is usually more common in males and involves primarily the posterior element of the spine and the sacrum (40–55%). Less frequently, long bones of limbs are involved. Clinical, radiological and histopathological correlation in this case guided us to reach at right diagnosis of EO which helped the patient in getting correct treatment which involves surgical excision over conventional curettage. The purpose behind this case presentation is to improve the awareness about this recurrent tumor variant which has many close differentials including well-differentiated osteoblastic osteosarcoma.

  10. Synovial Sarcoma of the Tongue: Report of a Case.

    PubMed

    Basile, Lauren E; Hoch, Benjamin; Dillon, Jasjit K

    2016-01-01

    This report outlines the workup and management of a 55-year-old woman with a synovial sarcoma of the lateral border of the tongue that was initially diagnosed as a glomus tumor. A review was performed of the literature on synovial sarcomas of the oral cavity and current National Comprehensive Cancer Network guidelines. Synovial sarcomas of the tongue are rare neoplasms, with variable morphologic microscopic types and immunohistochemical profiles. Fluorescence in situ hybridization analysis of the known gene translocation also can be used in diagnosis. According to the literature, resection of the tumor is the current treatment of choice; however, owing to the rarity of this entity, diagnosis and management prove challenging for the oral and maxillofacial surgeon.

  11. [Alveolar soft part sarcoma of the larynx: a case report].

    PubMed

    Xiao, Hao; Yang, Hui; Wang, Huan

    2015-07-01

    Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcomas, this sarcoma occurs rarely in the larynx. Herein, we describe an unusual case of ASPS occurring in the larynx. The patient was a 46-year-old woman who presented with a more than 2-year history of right pyriform fossa mass and progressive hoarseness for three months. The endoscopic examination of the larynx revealed a mass in the right pyriform fossa with smooth surface and well-defined margin. Computed tomography demonstrated a heterogeneous tumor in the right pyriform fossa, and involving the paralaryngeal space, measuring approximately 2.1 cm x 1.7 cm x 2.6 cm, which was resected via lateral neck approach. Histopathology and immunohistochemistry revealed an ASPS. To ours knowledge, this is the first reported case of ASPS arising in pyriform fossa.

  12. Dose Titration of Walleye Dermal Sarcoma (WDS) Tumor Filtrate.

    PubMed

    Getchell, R G; Wooster, G A; Sutton, C A; Casey, J W; Bowser, P R

    2006-12-01

    Walleyes Stizostedion vitreum were challenged with a topical application of a dilution series of cell-free dermal sarcoma tumor filtrates to determine the minimum dose of virus needed to induce these walleye tumors. A series of six 10-fold dilutions of the filtrate were applied to the side of the fish, which were allowed to develop grossly visible tumors at 15°C for 20 weeks. Quantification of the virus in the filtrates was accomplished by quantitative (real-time) reverse transcriptase-polymerase chain reaction. We determined that there are approximately 10(10) viral RNA copies in 100 μL of walleye dermal sarcoma inoculum. The minimum dose of walleye dermal sarcoma virus that could induce tumors by the topical challenge method was the 1,000-fold dilution of this 10(10) inoculum, or approximately 10(7) viral RNA copies.

  13. Stewart-Treves syndrome following idiopathic leg lymphoedema: remember sarcoma.

    PubMed

    Harrison, W D; Chandrasekar, C R

    2015-06-01

    Patients with skin complaints secondary to oedema are commonly encountered and can be a diagnostic challenge. Here, we present the case of a 78-year-old lady with Stewart-Treves syndrome, a rare cutaneous angiosarcoma. The histology showed angiocutaneous sarcoma with poorly defined margins. The prognosis remains extremely poor. A successful transfemoral amputation reduced the massive tumour burden and cleared a source of sepsis from this fungating tumour. Our aim is to highlight this rare but high-grade sarcoma which results from chronic lymphoedema. The red flag signs of sarcoma are masses greater than 5 cm, tumours which are rapidly growing, deep to fascia or painful, and tumours that recur after previous surgery. Non-healing or progressive ulceration in the background of chronic lymphoedema should raise the possibility of underlying malignancy. We need a low threshold for biopsy and early referral to an appropriate multidisciplinary team for the optimum management of the patient. PMID:26075513

  14. A rare cause of mechanical obstruction: Intestinal myeloid sarcoma

    PubMed Central

    Yoldaş, Tayfun; Erol, Varlık; Demir, Batuhan; Hoşcoşkun, Cüneyt

    2014-01-01

    Myeloid sarcoma is a rare aggressive tumour that originates from immature extramedullary myeloid cells. It can be seen as a relapse in patients with acute myeloid leukaemia. Sometimes it can be seen in the form of a solid tumour without any evidence of leukaemia. A case of a 44-year-old male patient who was admitted with symptoms and signs of mechanical intestinal bowel obstruction was operated on. The operation findings showed small bowel obstruction due to a mass. The mass was then resected with end-to-end intestinal anastomosis. The resected mass pathology results were consistent with myeloid sarcoma. The post-operative period was uneventful and adjuvant therapy was applied. In this case report we aimed to evaluate the clinical signs and treatment modalities of small intestinal myeloid sarcoma. PMID:25931908

  15. Delayed phlegmon with gallstone fragments masquerading as soft tissue sarcoma

    PubMed Central

    Goodman, Laura F.; Bateni, Cyrus P.; Bishop, John W.; Canter, Robert J.

    2016-01-01

    Complications from lost gallstones after cholecystectomy are rare but varied from simple perihepatic abscess to empyema and expectoration of gallstones. Gallstone complications have been reported in nearly every organ system, although reports of malignant masquerade of retained gallstones are few. We present the case of an 87-year-old woman with a flank soft tissue tumor 4 years after laparoscopic cholecystectomy. The initial clinical, radiographic and biopsy findings were consistent with soft tissue sarcoma (STS), but careful review of her case in multidisciplinary conference raised the suspicion for retained gallstones rather than STS. The patient was treated with incisional biopsy/drainage of the mass, and gallstones were retrieved. The patient recovered completely without an extensive resectional procedure, emphasizing the importance of multidisciplinary sarcoma care to optimize outcomes for potential sarcoma patients. PMID:27333918

  16. [Ewing sarcoma located in the mandible: A case report].

    PubMed

    Hernandez, M; Droz, D; Mansuy, L; Simon, E; Chastagner, P

    2015-06-01

    Ewing sarcoma is the second most common primary malignant bone cancer in children and adolescents. Clinical presentation is usually dominated by local pain and a palpable mass. These symptoms justify imaging investigations: the first one, when an osseous lesion is suspected, is usually a conventional radiograph in two planes. Ewing sarcoma appears as a poorly defined osteolytic lesion that may frequently be associated with cortical erosion or laminar periosteal response ("onion skin"). However, this aspect is not pathognomonic and the definitive diagnosis is made by biopsy. Absence of pain or an unusual localization can lead to misdiagnosis. We report the case of a 7-year-old boy with Ewing sarcoma located in the mandible with a clinical picture including progressive mandibular swelling but no pain. PMID:25896628

  17. Intracranial granulocytic sarcoma: two cases and literature review

    PubMed Central

    Wang, Huanyu; Wang, Hong; Ma, Quanfeng; Chen, Yiyang

    2015-01-01

    Intracranial granulocytic sarcoma was a relatively rare tumor composed of myeloid blasts and/or immature myeloid cells in an extramedullary site which is associated with acute/chronic myeloid leukemia. In this paper, two cases of intracranial granulocytic sarcoma, one male aged 36 and one 28-year-old female, were reported to improve the diagnosis and treatment of such diseases. Diagnostic and treatment procedures for them were retrospectively summarized and relevant literature reviews were combined. Pathological biopsy was conducted to validate the diagnosis. Surgical resections in combination with chemotherapy were performed. The differential diagnosis of intracranial granulocytic sarcoma from malignant lymphomas and alternative small round cell malignancy was confirmed by biopsy and immunohistochemistry. PMID:26770615

  18. Extraosseous Ewing Sarcoma: Diagnosis, Prognosis and Optimal Management.

    PubMed

    Galyfos, George; Karantzikos, Georgios A; Kavouras, Nikolaos; Sianou, Argiri; Palogos, Konstantinos; Filis, Konstantinos

    2016-02-01

    Extraosseous Ewing sarcomas (EESs) are rare tumours originating from soft tissues. Their clinical picture depends mainly on the primary site of the sarcoma. Patient characteristics and outcomes seem to be different in EES compared to patients with skeletal Ewing sarcoma, with implications for patient care and prognosis. However, multimodality therapeutic strategies are recommended for all types of the Ewing tumour family. The available diagnostic tools include ultrasonographic evaluation and computed tomography (CT) or magnetic resonance imaging as well as histopathologic and immunohistochemical tissue examination. Several histologic and genetic biomarkers have been established, although their utilization needs to be further tested by larger prospective studies. Regarding localized disease, the recommended treatment remains surgery. However, chemotherapy can be added to achieve improved survival, with neoadjuvant regimens showing more promising results than adjuvant regimens. Radiotherapy is an option to obtain local control, although its complications have reduced its utilization. In metastatic or recurrent disease, systematic chemotherapy improves survival. PMID:27186040

  19. Primary Ewing's sarcoma of the squamous part of temporal bone in a young girl treated with adjuvant volumetric arc therapy.

    PubMed

    Nandi, Moujhuri; Bhattacharya, Jibak; Goswami, Suchanda; Goswami, Chanchal

    2015-01-01

    Ewing's sarcoma (ES)/peripheral primitive neuroectodermal tumors usually arise in the long bones of children and young adults. Primary ES of the cranium is unusual. Treatment involves multi-modality therapy incorporating surgery, radiotherapy and chemotherapy; outcomes are similar to those arising from long bones. We report a case of Primary ES of the squamous part of temporal bone with intracranial extension in a 9-year-old girl who was treated with surgery, chemotherapy followed by adjuvant radiotherapy by volumetric arc therapy. Post 1-year of treatment the girl is performing well in her classes.

  20. Primary Ewing's sarcoma of the squamous part of temporal bone in a young girl treated with adjuvant volumetric arc therapy.

    PubMed

    Nandi, Moujhuri; Bhattacharya, Jibak; Goswami, Suchanda; Goswami, Chanchal

    2015-01-01

    Ewing's sarcoma (ES)/peripheral primitive neuroectodermal tumors usually arise in the long bones of children and young adults. Primary ES of the cranium is unusual. Treatment involves multi-modality therapy incorporating surgery, radiotherapy and chemotherapy; outcomes are similar to those arising from long bones. We report a case of Primary ES of the squamous part of temporal bone with intracranial extension in a 9-year-old girl who was treated with surgery, chemotherapy followed by adjuvant radiotherapy by volumetric arc therapy. Post 1-year of treatment the girl is performing well in her classes. PMID:26881573

  1. Extremity Soft Tissue Sarcoma: Tailoring Resection to Histologic Subtype.

    PubMed

    Cable, Matthew G; Randall, R Lor

    2016-10-01

    Soft tissue sarcomas comprise tumors originating from mesenchymal or connective tissue. Histologic grade is integral to prognosis. Because sarcoma management is multimodal, histologic subtype should inform optimum treatment. Appropriate biopsy and communication between surgeon and pathologist can help ensure a correct diagnosis. Treatment often involves surgical excision with wide margins and adjuvant radiotherapy. There is no consensus on what constitutes an adequate margin for histologic subtypes. An appreciation of how histology corresponds with tumor biology and surgical anatomic constraints is needed for management of this disease. Even with the surgical goal of wide resection being obtained, many patients do not outlive their disease. PMID:27591492

  2. [Molecular targeted drugs for soft tissue sarcoma and neuroendocrine tumor].

    PubMed

    Kato, Shunsuke

    2015-08-01

    Both the soft tissue sarcomas and the neuroendocrine tumors are rare diseases. Therefore the recruiting of these patients was more difficult than other cancer species, and the development of the new therapy for these diseases did not readily advance. However, the identification of driver molecules for each sub-type enabled us to the development of the molecular targeted drugs. As for the GIST, several TKIs are used, but in late years it is found that susceptibility of TKIs varies according to difference in second mutation. In this chapter, the molecular target drug for the soft tissue sarcoma and the neuroendocrine tumor is reviewed. PMID:26281696

  3. Extremity Soft Tissue Sarcoma: Tailoring Resection to Histologic Subtype.

    PubMed

    Cable, Matthew G; Randall, R Lor

    2016-10-01

    Soft tissue sarcomas comprise tumors originating from mesenchymal or connective tissue. Histologic grade is integral to prognosis. Because sarcoma management is multimodal, histologic subtype should inform optimum treatment. Appropriate biopsy and communication between surgeon and pathologist can help ensure a correct diagnosis. Treatment often involves surgical excision with wide margins and adjuvant radiotherapy. There is no consensus on what constitutes an adequate margin for histologic subtypes. An appreciation of how histology corresponds with tumor biology and surgical anatomic constraints is needed for management of this disease. Even with the surgical goal of wide resection being obtained, many patients do not outlive their disease.

  4. Hepatic Involvement of Histiocytic Sarcoma: CT and MRI Findings.

    PubMed

    Kubo, Takatoshi; Kiryu, Shigeru; Akai, Hiroyuki; Ota, Yasunori; Tojo, Arinobu; Yoshida, Hideo; Kato, Naoya; Nakano, Yoshiyasu; Ohtomo, Kuni

    2016-01-01

    Histiocytic sarcoma in the liver is an extremely rare hematological malignancy. Herein, we reported the case of a 68-year-old woman who presented with characteristic wedge-shaped abnormality bounded by hepatic veins on computed tomography and magnetic resonance imaging of the liver. In the wedge-shaped area, decreased portal flow and the deposition of iron were observed. These imaging findings are consistent with intrasinusoidal tumor cell infiltration. A liver biopsy was performed, and histiocytic sarcoma was confirmed histopathologically. PMID:27587965

  5. Primary Cutaneous Ewing Sarcoma: Report of a Case.

    PubMed

    Yuste, Veronica; Sierra, Elena; Ruano, David; Llamas-Velasco, Mar; Conde, Esther; Azorin, Daniel

    2015-01-01

    Primary cutaneous Ewing's sarcoma is a rare entity. Although the diagnosis may be very difficult, it can be confirmed through molecular biology. We present the case of a 13-years old male with a lesion in the sole of the right foot, characterized by a monomorphous proliferation of small, round and blue cells. The histology and molecular biology allowed us to perform the diagnosis of cutaneous Ewing's sarcoma. This neoplasm must be distinguished from other round cell tumors with cutaneous involvement. The prognosis and treatment of this rare disease will also be discussed. PMID:26207391

  6. Kaposi's Sarcoma in Uganda: Geographic and Ethnic Distribution

    PubMed Central

    Taylor, J. F.; Smith, P. G.; Bull, Diana; Pike, M. C.

    1972-01-01

    Over the quinquennium 1964-68 the crude annual incidence of Kaposi's sarcoma in Uganda per million of the population was 7·9 overall, 14·6 for males and 1·1 for females. Statistical analysis indicates that the disease is most prevalent in highland areas to the west and among the indigenous Bantu tribes. There was no correlation with the distribution of squamous cell carcinoma of the lower leg, and Kaposi's sarcoma was not seen in an Indian or European during the period under review. PMID:4647399

  7. Hepatic Involvement of Histiocytic Sarcoma: CT and MRI Findings

    PubMed Central

    Kubo, Takatoshi; Akai, Hiroyuki; Ota, Yasunori; Tojo, Arinobu; Yoshida, Hideo; Kato, Naoya; Nakano, Yoshiyasu; Ohtomo, Kuni

    2016-01-01

    Histiocytic sarcoma in the liver is an extremely rare hematological malignancy. Herein, we reported the case of a 68-year-old woman who presented with characteristic wedge-shaped abnormality bounded by hepatic veins on computed tomography and magnetic resonance imaging of the liver. In the wedge-shaped area, decreased portal flow and the deposition of iron were observed. These imaging findings are consistent with intrasinusoidal tumor cell infiltration. A liver biopsy was performed, and histiocytic sarcoma was confirmed histopathologically. PMID:27587965

  8. SARC: Development and Support of a Sarcoma Research Consortium Infrastructure

    SciTech Connect

    Arkison, Jim

    2007-10-29

    SARC is a non-for-profit organization whose mission and vision is to advocate for the collaboration on the design of clinical trials on sarcoma, to further the knowledge regarding the diagnosis and treatment of sarcoma and provide accurate and up to date information to physicians, patients and families. The objectives are to assist in the development of the infrastructure for the continued growth and spectrum of clinical research, to facilitate biannual meeting of investigators, and to develop a preclinical research base that would design and conduct research that would improve the process of drug treatments selected for clinical research trials.

  9. Isolated Gastric Myeloid Sarcoma: A Case Report and Review of the Literature

    PubMed Central

    Bose, Prithviraj

    2014-01-01

    Myeloid sarcoma represents the proliferation of myeloblasts of acute myeloid leukemia (AML) at extramedullary sites. While extramedullary involvement in AML is uncommon in itself, isolated myeloid sarcomas, that is, myeloid sarcomas without any bone marrow involvement, are extremely rare and pose a diagnostic and therapeutic challenge. Here, we present the case of a middle-aged woman with isolated myeloid sarcoma in the stomach—an organ seldom involved by this disease. Additionally, the literature on the epidemiology, diagnosis, pathology, prognosis, and therapeutic options in myeloid sarcomas has been reviewed. PMID:25105036

  10. Undifferentiated Pleomorphic Sarcoma Presenting as Abdominal Pain with a Pulsatile Mass.

    PubMed

    Moradi, Arash; Afsharfard, Abolfazl; Atqiaee, Khashayar

    2016-01-01

    Malignant fibrous histiocytoma (MFH) is a rare tumor that mostly involves adults aged 50 to 70. The most common anatomic location is the lower extremities. MFH of the retroperitoneum usually manifests late in its course and may be initially mistaken with other more common diagnosis. Here, the authors describe a 60-year-old man that was brought to the emergency department with a chief complaint of periumbilical abdominal pain. Our patient presented with symptoms consistent with a symptomatic aortic aneurysm, but a mass was encountered during surgery. In such circumstances the diagnosis of malignant sarcoma must be kept in mind and attempts at full resection with tumor-free margins are necessary. PMID:27563479

  11. Undifferentiated Pleomorphic Sarcoma Presenting as Abdominal Pain with a Pulsatile Mass

    PubMed Central

    Afsharfard, Abolfazl

    2016-01-01

    Malignant fibrous histiocytoma (MFH) is a rare tumor that mostly involves adults aged 50 to 70. The most common anatomic location is the lower extremities. MFH of the retroperitoneum usually manifests late in its course and may be initially mistaken with other more common diagnosis. Here, the authors describe a 60-year-old man that was brought to the emergency department with a chief complaint of periumbilical abdominal pain. Our patient presented with symptoms consistent with a symptomatic aortic aneurysm, but a mass was encountered during surgery. In such circumstances the diagnosis of malignant sarcoma must be kept in mind and attempts at full resection with tumor-free margins are necessary. PMID:27563479

  12. Low-Grade Myofibroblastic Sarcoma in the Mandibular Canal: A Case Report.

    PubMed

    Yu, Yueyuan; Xiao, Jin; Wang, Lan; Yang, Guiqiang

    2016-07-01

    Low-grade myofibroblastic sarcoma (LGMS) represents an atypical myofibroblastic tumor characterized by a diffusely infiltrating pattern of spindle-shaped tumor cells. It was classified as a distinct soft tissue tumor by the World Health Organization in 2002. LGMS occurs mostly in adult patients and has a predilection for the head and neck region. So far, only a few cases of LGMS located in the mandible have been reported. Aggressive surgical resection with clear margins is the primary treatment for LGMS. Because of its rarity, reports of radiation therapy are limited, and the therapeutic effect is still controversial. We present the case of an 8-year-old girl with LGMS of the mandibular canal to highlight the clinical features and rarity and to improve the understanding of the therapeutic effect of radiotherapy on LGMS. PMID:27020841

  13. Giant Intramuscular Nodular Fasciitis Masquerading as Soft Tissue Sarcoma with Neural Involvement - A Case Report

    PubMed Central

    Ramakrishnaiah, Vishnu Prasad Nelamangala; Rajan, Susan; Ganesh, Rajesh Nachiappa

    2016-01-01

    Nodular Fasciitis (NF) is described as a benign reactive proliferation of myofibroblasts that occurs predominantly in the subcutaneous location. Most commonly presents as rapidly growing swelling in the young adults, with usual size of less than 4cm and located commonly in the extremities. We hereby report a case of giant nodular fasciitis of 10cm diameter which presented in an unusual location (gluteal region) and rare plane of origin (intramuscular) with unusual symptom of sciatic nerve involvement. Because of the size, location and neural involvement a clinical diagnosis of soft tissue sarcoma was made. After investigating with trucut biopsy and magnetic resonance imaging, benign nature of the lesion confirmed and treated with complete excision, carefully preserving the sciatic nerve. This case is presented for its rarity in size, location, plane of origin and neural compression. To the best of our knowledge this is the first case of nodular fasciitis of 10cm size and in the gluteal muscles. PMID:27504350

  14. Radiation Therapy for Chloroma (Granulocytic Sarcoma)

    SciTech Connect

    Bakst, Richard; Wolden, Suzanne; Yahalom, Joachim

    2012-04-01

    Objectives: Chloroma (granulocytic sarcoma) is a rare, extramedullary tumor of immature myeloid cells related to acute nonlymphocytic leukemia or myelodysplastic syndrome. Radiation therapy (RT) is often used in the treatment of chloromas; however, modern studies of RT are lacking. We reviewed our experience to analyze treatment response, disease control, and toxicity associated with RT to develop treatment algorithm recommendations for patients with chloroma. Patients and Methods: Thirty-eight patients who underwent treatment for chloromas at our institution between February 1990 and June 2010 were identified and their medical records were reviewed and analyzed. Results: The majority of patients that presented with chloroma at the time of initial leukemia diagnosis (78%) have not received RT because it regressed after initial chemotherapy. Yet most patients that relapsed or remained with chloroma after chemotherapy are in the RT cohort (90%). Thirty-three courses of RT were administered to 22 patients. Radiation subsite breakdown was: 39% head and neck, 24% extremity, 9% spine, 9% brain, 6% genitourinary, 6% breast, 3% pelvis, and 3% genitourinary. Median dose was 20 (6-36) Gy. Kaplan-Meier estimates of progression-free survival and overall survival in the RT cohort were 39% and 43%, respectively, at 5 years. At a median follow-up of 11 months since RT, only 1 patient developed progressive disease at the irradiated site and 4 patients developed chloromas at other sites. RT was well tolerated without significant acute or late effects and provided symptom relief in 95% of cases. Conclusions: The majority of patients with chloromas were referred for RT when there was extramedullary progression, marrow relapse, or rapid symptom relief required. RT resulted in excellent local disease control and palliation of symptoms without significant toxicity. We recommend irradiating chloromas to at least 20 Gy, and propose 24 Gy in 12 fractions as an appropriate regimen.

  15. Indian data on bone and soft tissue sarcomas: A summary of published study results

    PubMed Central

    Ramaswamy, Anant; Rekhi, Bharat; Bakhshi, Sameer; Hingmire, Sachin; Agarwal, Manish

    2016-01-01

    Bone sarcomas are rare tumors, approximating 0.2% of all cancers, with osteosarcoma (OGS), chondrosarcoma, and Ewing sarcoma being the most common cancers in this subset. The formation of disease management groups/clinics focused on sarcomas has resulted in better understanding and management of these uncommon tumors. Multiple large-scale retrospective data from Tata Memorial Hospital (TMH) and All India Institute of Medical Sciences have reported outcomes comparable to Western data in the field of OGS and Ewing sarcoma, with interesting prognostic factors identified for further evaluation. Soft tissue sarcomas are a rare heterogeneous group of tumors, more than 50 different tumor entities. The common subtypes identified in India include Ewing sarcoma and synovial sarcoma. Valuable work regarding brachytherapy has been done by radiation oncologists from the TMH, especially in pediatric patients. PMID:27606300

  16. Indian data on bone and soft tissue sarcomas: A summary of published study results

    PubMed Central

    Ramaswamy, Anant; Rekhi, Bharat; Bakhshi, Sameer; Hingmire, Sachin; Agarwal, Manish

    2016-01-01

    Bone sarcomas are rare tumors, approximating 0.2% of all cancers, with osteosarcoma (OGS), chondrosarcoma, and Ewing sarcoma being the most common cancers in this subset. The formation of disease management groups/clinics focused on sarcomas has resulted in better understanding and management of these uncommon tumors. Multiple large-scale retrospective data from Tata Memorial Hospital (TMH) and All India Institute of Medical Sciences have reported outcomes comparable to Western data in the field of OGS and Ewing sarcoma, with interesting prognostic factors identified for further evaluation. Soft tissue sarcomas are a rare heterogeneous group of tumors, more than 50 different tumor entities. The common subtypes identified in India include Ewing sarcoma and synovial sarcoma. Valuable work regarding brachytherapy has been done by radiation oncologists from the TMH, especially in pediatric patients.

  17. Indian data on bone and soft tissue sarcomas: A summary of published study results.

    PubMed

    Ramaswamy, Anant; Rekhi, Bharat; Bakhshi, Sameer; Hingmire, Sachin; Agarwal, Manish

    2016-01-01

    Bone sarcomas are rare tumors, approximating 0.2% of all cancers, with osteosarcoma (OGS), chondrosarcoma, and Ewing sarcoma being the most common cancers in this subset. The formation of disease management groups/clinics focused on sarcomas has resulted in better understanding and management of these uncommon tumors. Multiple large-scale retrospective data from Tata Memorial Hospital (TMH) and All India Institute of Medical Sciences have reported outcomes comparable to Western data in the field of OGS and Ewing sarcoma, with interesting prognostic factors identified for further evaluation. Soft tissue sarcomas are a rare heterogeneous group of tumors, more than 50 different tumor entities. The common subtypes identified in India include Ewing sarcoma and synovial sarcoma. Valuable work regarding brachytherapy has been done by radiation oncologists from the TMH, especially in pediatric patients. PMID:27606300

  18. Lysyl Oxidase Is Downregulated by the EWS/FLI1 Oncoprotein and Its Propeptide Domain Displays Tumor Supressor Activities in Ewing Sarcoma Cells

    PubMed Central

    García-García, Laura; de la Parra, Juan; Alonso, Javier

    2013-01-01

    Ewing sarcoma is the second most common bone malignancy in children and young adults. It is driven by oncogenic fusion proteins (i.e. EWS/FLI1) acting as aberrant transcription factors that upregulate and downregulate target genes, leading to cellular transformation. Thus, identificating these target genes and understanding their contribution to Ewing sarcoma tumorigenesis are key for the development of new therapeutic strategies. In this study we show that lysyl oxidase (LOX), an enzyme involved in maintaining structural integrity of the extracellular matrix, is downregulated by the EWS/FLI1 oncoprotein and in consequence it is not expressed in Ewing sarcoma cells and primary tumors. Using a doxycycline inducible system to restore LOX expression in an Ewing sarcoma derived cell line, we showed that LOX displays tumor suppressor activities. Interestingly, we showed that the tumor suppressor activity resides in the propeptide domain of LOX (LOX-PP), an N-terminal domain produced by proteolytic cleavage during the physiological processing of LOX. Expression of LOX-PP reduced cell proliferation, cell migration, anchorage-independent growth in soft agar and formation of tumors in immunodeficient mice. By contrast, the C-terminal domain of LOX, which contains the enzymatic activity, had the opposite effects, corroborating that the tumor suppressor activity of LOX is mediated exclusively by its propeptide domain. Finally, we showed that LOX-PP inhibits ERK/MAPK signalling pathway, and that many pathways involved in cell cycle progression were significantly deregulated by LOX-PP, providing a mechanistic explanation to the cell proliferation inhibition observed upon LOX-PP expression. In summary, our observations indicate that deregulation of the LOX gene participates in Ewing sarcoma development and identify LOX-PP as a new therapeutic target for one of the most aggressive paediatric malignancies. These findings suggest that therapeutic strategies based on the

  19. Lysyl oxidase is downregulated by the EWS/FLI1 oncoprotein and its propeptide domain displays tumor supressor activities in Ewing sarcoma cells.

    PubMed

    Agra, Noelia; Cidre, Florencia; García-García, Laura; de la Parra, Juan; Alonso, Javier

    2013-01-01

    Ewing sarcoma is the second most common bone malignancy in children and young adults. It is driven by oncogenic fusion proteins (i.e. EWS/FLI1) acting as aberrant transcription factors that upregulate and downregulate target genes, leading to cellular transformation. Thus, identificating these target genes and understanding their contribution to Ewing sarcoma tumorigenesis are key for the development of new therapeutic strategies. In this study we show that lysyl oxidase (LOX), an enzyme involved in maintaining structural integrity of the extracellular matrix, is downregulated by the EWS/FLI1 oncoprotein and in consequence it is not expressed in Ewing sarcoma cells and primary tumors. Using a doxycycline inducible system to restore LOX expression in an Ewing sarcoma derived cell line, we showed that LOX displays tumor suppressor activities. Interestingly, we showed that the tumor suppressor activity resides in the propeptide domain of LOX (LOX-PP), an N-terminal domain produced by proteolytic cleavage during the physiological processing of LOX. Expression of LOX-PP reduced cell proliferation, cell migration, anchorage-independent growth in soft agar and formation of tumors in immunodeficient mice. By contrast, the C-terminal domain of LOX, which contains the enzymatic activity, had the opposite effects, corroborating that the tumor suppressor activity of LOX is mediated exclusively by its propeptide domain. Finally, we showed that LOX-PP inhibits ERK/MAPK signalling pathway, and that many pathways involved in cell cycle progression were significantly deregulated by LOX-PP, providing a mechanistic explanation to the cell proliferation inhibition observed upon LOX-PP expression. In summary, our observations indicate that deregulation of the LOX gene participates in Ewing sarcoma development and identify LOX-PP as a new therapeutic target for one of the most aggressive paediatric malignancies. These findings suggest that therapeutic strategies based on the

  20. Hydrogen sulfide activates TRPA1 and releases 5-HT from epithelioid cells of the chicken thoracic aorta.

    PubMed

    Delgermurun, Dugar; Yamaguchi, Soichiro; Ichii, Osamu; Kon, Yasuhiro; Ito, Shigeo; Otsuguro, Ken-Ichi

    2016-09-01

    Epithelioid cells in the chicken thoracic aorta are chemoreceptor cells that release 5-HT in response to hypoxia. It is likely that these cells play a role in chemoreception similar to that of glomus cells in the carotid bodies of mammals. Recently, H2S was reported to be a key mediator of carotid glomus cell responses to hypoxia. The aim of the present study was to reveal the mechanism of action of H2S on 5-HT outflow from chemoreceptor cells in the chicken thoracic aorta. The 5-HT outflow induced by NaHS, an H2S donor, and Na2S3, a polysulfide, was measured by using a HPLC equipped with an electrochemical detector. NaHS (0.3-3mM) caused a concentration-dependent increase in 5-HT outflow, which was significantly inhibited by the removal of extracellular Ca(2+). 5-HT outflow induced by NaHS (0.3mM) was also significantly inhibited by voltage-dependent L- and N-type Ca(2+) channel blockers and a selective TRPA1 channel blocker. Cinnamaldehyde, a TRPA1 agonist, mimicked the secretory response to H2S. 5-HT outflow induced by Na2S3 (10μM) was also inhibited by the TRPA1 channel blocker. Furthermore, the expression of TRPA1 was localized to 5-HT-containing chemoreceptor cells in the aortic wall. These findings suggest that the activation of TRPA1 and voltage-dependent Ca(2+) channels is involved in H2S-evoked 5-HT release from chemoreceptor cells in the chicken aorta.

  1. Cardiac dysfunction among soft tissue sarcoma patients in Denmark

    PubMed Central

    Shantakumar, Sumitra; Olsen, Morten; Vo, Thao T; Nørgaard, Mette; Pedersen, Lars

    2016-01-01

    Purpose Soft tissue sarcoma (STS) patients may experience post-treatment cardiotoxicity, yet no population-based data exist. We examined the incidence of left ventricular ejection fraction (LVEF) decline, heart failure, and cardiac death following STS diagnosis among adults, using Danish patient registries and medical record review. Patients and methods LVEF decline was examined in a regional cohort of STS patients diagnosed during 1997–2011 in Western Denmark for whom cardiac imaging data were available. LVEF decline was defined as an absolute decline from baseline to follow-up of 10% or more, or, where baseline imaging was not available, a decline below the lower limit of normal (or 40%) for a follow-up LVEF. Heart failure and cardiac death were investigated in a national Danish cohort of all STS patients diagnosed from 2000 to 2009. We followed patients from STS diagnosis until heart failure, cardiac death, emigration or December 31, 2012 (whichever occurred first). Results The incidence rate of LVEF decline for the regional cohort with follow-up data (N=100, five events) or baseline and follow-up measurements (N=75, 19 events) was 16.8 (95% confidence interval [CI]: 7.0–40.3) and 108 (95% CI: 69–170), respectively, per 1,000 person-years. In the national cohort (N=1,187), the incidence of heart failure (40 events) and cardiac death (15 events) was 7.3 (95% CI: 5.4–10.0) and 2.7 (95% CI: 1.6–4.5), respectively, per 1,000 person-years. The strongest predictors of heart failure were doxorubicin treatment (hazard ratio [HR] =2.2, 95% CI: 0.5–10.2) and pre-existing cardiovascular disease (HR=6.3, 95% CI: 0.98–40.6). Conclusion LVEF decline occurred more frequently compared to heart failure or cardiac death in a nationally representative cohort of Danish STS patients. PMID:27186077

  2. Ewing's sarcoma in mandibular similar to dental abscess.

    PubMed

    Keshani, Forouz; Jahanshahi, Gholamreza; Attar, Bijan Movahedian; Kalantari, Mahsa; Razavi, Seyed Mohammad; Hashemzade, Zahra; Tavakoli, Payam

    2014-01-01

    Ewing's sarcoma is a rare malignant neoplasm that comprises approximately 4-6% of primary bone tumors. In most cases, femur and pelvis are affected, and less commonly the head and neck areas (in the jaws, usually the mandible). These tumors have been reported more frequently in males, mostly aged 5-20 years old. Systemic symptoms and signs such as fever, weight loss, anemia, leukocytosis, and elevated erythrocyte sedimentation rate (ESR) may be the first signs in oral Ewing's sarcoma. Such signs and symptoms are also seen in odontogenic infections and abscess. In one case, the patient went to a dentist with pain, swelling, and abscess similar to odontogenic infection and patient's tooth was pulled due to misdiagnosis. This tumor has an aggressive clinical behavior and is identified with rapid growth and high probability of metastasis at diagnosis. Thus, it is necessary to differentiate it from a dental abscess. As for the treatment of Ewing's sarcoma, first the tumor must undergo chemotherapy to reduce its size and, eventually, it undergoes extensive surgery. This case report deals with a 16-year-old patient wrongly diagnosed with odontogenic infection and abscess, and hospitalized. As the symptoms did not remit, biopsy was carried out and the patient was operated on with Ewing's sarcoma diagnosis. PMID:24627870

  3. Primary extraskeletal Ewing's sarcoma/primitive neuroectodermal tumor of breast

    PubMed Central

    Srivastava, Smita; Arora, Jyoti; Parakh, Anushri; Goel, Ruchika Kumar

    2016-01-01

    Extraskeletal Ewing's sarcoma (EES) is a rare soft tissue tumor that is morphologically indistinguishable from skeletal ES. We report a case of a 25-year-old female with recurrent EES/primitive neuroectodermal tumor of right breast with imaging findings on mammogram, ultrasound, magnetic resonance imaging breast, and positron emission tomography–computed tomography. PMID:27413270

  4. Transponder-induced sarcoma in the heterozygous p53+/- mouse.

    PubMed

    Blanchard, K T; Barthel, C; French, J E; Holden, H E; Moretz, R; Pack, F D; Tennant, R W; Stoll, R E

    1999-01-01

    Heterozygous p53+/- transgenic mice are being studied for utility as a short-term alternative model to the 2-yr rodent carcinogenicity bioassay. During a 26-wk study to assess the potential carcinogenicity of oxymetholone using p-cresidine as a positive control, glass/polypropylene microchips (radio transponder identification devices) were subcutaneously implanted into male and female p53+/- mice. During week 15, the first palpable mass was clinically observed at an implant site. This rapidly growing mass virtually quadrupled in size by week 25. Microscopic examination of all implant sites revealed that 18 of 177 animals had a subcutaneous histologically malignant sarcoma. The neoplasms were characterized as undifferentiated sarcomas unrelated to drug treatment, as indicated by the relatively even distribution among dose groups, including controls. An unusual preneoplastic mesenchymal change characterized by the term "mesenchymal dysplasia" was present in most groups and was considered to be a prodromal change to sarcoma development. The tumors were observed to arise from dysplastic mesenchymal tissue that developed within the tissue capsule surrounding the transponder. The preneoplastic changes, including mesenchymal dysplasia, appeared to arise at the transponder's plastic anchoring barb and then progressed as a neoplasm to eventually surround the entire microchip. Capsule membrane endothelialization, inflammation, mesenchymal basophilia and dysplasia, and sarcoma were considered unequivocal preneoplastic/neoplastic responses to the transponder and were not related to treatment with either oxymetholone or p-cresidine.

  5. Kaposi’s sarcoma concurrent with granulomatosis polyangiitis

    PubMed Central

    Kılıç, Diler Taş; Özkan, Selma Karaahmetoğlu; Canbakan, Başol; Dündar, Nesrin

    2016-01-01

    Kaposi’s sarcoma (KS), a malignant vascular tumor, can develop in patients who receive corticosteroids or immunosuppressive therapy. We report a patient with KS and granulomatosis polyangiitis (GPA). To the best of our knowledge, this is the first report regarding the co-occurrence of these two diseases. The physician must be aware of the risk of cancer in patients with rheumatism.

  6. JAZF1/SUZ12 gene fusion in endometrial stromal sarcomas.

    PubMed

    Hrzenjak, Andelko

    2016-01-01

    Endometrial stromal sarcomas (ESSs) belong to the rarest uterine malignancies (prevalence category <1-9/1,000,000). According to the new 2014 World Health Organisation (WHO) classification, they are separated into four categories; benign endometrial stromal nodules (ESNs), low grade endometrial stromal sarcomas (LG-ESSs), high-grade endometrial stromal sarcomas (HG-ESSs) and undifferentiated uterine sarcomas (UUSs). Due to heterogeneous histopathologic appearance these tumors still represent diagnostic challenge, even for experienced pathologists. ESSs are genetically very heterogeneous and several chromosomal translocations and gene fusions have so far been identified in these malignancies. To date the JAZF1/SUZ12 gene fusion is by far the most frequent and seems to be the cytogenetic hallmark of ESN and LG-ESS. Based on present literature data this gene fusion is present in approximately 75% of ESN, 50% of LG-ESS and 15% of HG-ESS cases. The frequency of JAZF1/SUZ12 appearance varies between classic ESS and different morphologic variants. This gene fusion is suggested to become a specific diagnostic tool, especially in difficult borderline cases. In combination with the recently described YWHAE/FAM22 gene fusion the JAZF1/SUZ12 fusion could be used to differentiate between LG-ESS and HG-ESS. The purpose of this review is to summarize literature data published in last two and a half decades about this gene fusion, as a contribution to our understanding of ESS genetics and pathogenesis. PMID:26879382

  7. Sorafenib in advanced, heavily pretreated patients with soft tissue sarcomas.

    PubMed

    Brämswig, Kira; Ploner, Ferdinand; Martel, Alexandra; Bauernhofer, Thomas; Hilbe, Wolfgang; Kühr, Thomas; Leitgeb, Clemens; Mlineritsch, Brigitte; Petzer, Andreas; Seebacher, Veronika; Stöger, Herbert; Girschikofsky, Michael; Hochreiner, Gerhard; Ressler, Sigrun; Romeder, Franz; Wöll, Ewald; Brodowicz, Thomas

    2014-08-01

    Therapeutic options for patients with advanced pretreated soft tissue sarcomas are limited. However, in this setting, sorafenib has shown promising results. We reviewed the data of 33 patients with soft tissue sarcoma treated with sorafenib within a named patient program in Austria. Twelve physicians from eight different hospitals provided records for the analysis of data. Among the 33 patients, the predominant histological subtype of sarcoma was leiomyosarcoma (n=18, 55%). Other subtypes were represented by only one or two cases. Fifteen patients presented with metastases at the time of diagnosis. Another 17 patients developed metastases later in the course of the disease (data on one patient are missing). Most of the 33 patients had undergone resection of the primary (n=29, 88%) and half of the patients had received radiotherapy (n=17, 52%). Chemotherapy for metastatic disease had been administered to 30 patients (91%). The majority had received two or more regimens of chemotherapy (n=25, 76%) before sorafenib treatment. The use of sorafenib resulted in a median time to treatment failure of 92 days in patients with leiomyosarcoma and 45 days in patients with other histological subtypes. One-third of the patients derived benefits from treatment: four patients were documented with partial response and six with stabilized disease. In terms of treatment-related toxicity, skin problems of various degrees and gastrointestinal disturbances were frequently reported. In this retrospective analysis of heavily pretreated patients with advanced soft tissue sarcomas, sorafenib was associated with some antitumor activity and an acceptable toxicity profile. PMID:24667659

  8. Sarcoma de tejido blando—Versión para pacientes

    Cancer.gov

    Información del Instituto Nacional del Cáncer sobre el tratamiento del sarcoma de tejido blando, así como referencias a estudios clínicos, investigación, estadísticas y otros temas relacionados con este tipo de cáncer.

  9. Myeloid sarcoma of the Gingiva with myelodysplastic syndrome

    PubMed Central

    Jin, Seong-Ho; Park, Gyeongsin; Ko, Youngkyung; Park, Jun-Beom

    2016-01-01

    Abstract The purpose of this report is to present a case of myeloid sarcoma of the gingiva with myelodysplastic syndrome. A 52-year-old male diagnosed with myelodysplastic syndrome with skin lesions presented with gingival swelling and gingival redness involving the maxillary left second premolar and the maxillary left first molar. The patient was referred from the Department of Hematology for a biopsy of the lesion. Full-thickness flaps were elevated and inflamed, and neoplastic soft tissue was removed from a lesion and the samples sent for histopathologic analysis. Histopathologic results showed leukemic cell infiltration beneath the oral epithelium, and the specimen was positive for the leukocyte marker. The diagnosis was myeloid sarcoma. Uneventful healing was observed at 2-week follow-up, but relapse of the lesions with the hyperplastic and neoplastic tissue was noted at 4-week follow-up. Further follow-up or treatment could not be performed because the patient did not visit at the next follow-up. In conclusion, myeloid sarcoma should be a diagnosis option for gingival growth because it can involve intraoral lesion. In this report, a biopsy was performed due to referral considering the patient's medical history. Although myeloid sarcoma in the oral cavity is extremely rare, a small biopsy and consultation with a hematologist may be beneficial for patients and may provide a differential diagnosis. PMID:27310987

  10. A psychosocial intervention for patients with soft tissue sarcoma.

    PubMed

    Payne, D K; Lundberg, J C; Brennan, M F; Holland, J C

    1997-03-01

    Support groups have increasingly been demonstrated to be an effective intervention in reducing psychological difficulties and emotional problems in patients with cancer. This report describes a pilot support group intervention based upon a modified version of Cain and colleagues' (1986) thematic counseling model for patients who have been treated for soft tissue sarcoma and are free of disease. The modified thematic counseling model consisted of eight group sessions that focused on providing information about soft tissue sarcoma, management of stress, relaxation techniques, and coping skills. In addition, time was set aside for general discussion of personal concerns. Common themes reported by patients were: communication with family, friends and physicians, anxiety about lack of information about soft tissue sarcoma, and major financial disruption because of their illness. At the conclusion of the eighth session, patients were given an informal survey and asked to rate the value of different aspects of the program for them. They reported that feelings of isolation, anger, depression, and anxiety significantly decreased; and their level of self-confidence increased dramatically. This pilot support group intervention is recommended as a model for enhancing the quality of life of patients with soft tissue sarcoma. PMID:9126716

  11. Instantánea del sarcoma de Kaposi

    Cancer.gov

    Información sobre las tendencias de incidencia, mortalidad y financiamiento del NCI sobre el sarcoma de Kaposi; así como ejemplos de actividades del NCI y adelantos en la investigación de este tipo de cáncer.

  12. Dose-response relationships for radium-induced bone sarcomas

    SciTech Connect

    Rowland, R.E.; Stehney, A.F.; Lucas, H.F. Jr.

    1981-01-01

    The incidence of bone sarcomas among 3055 female radium-dial workers who entered the dial industry before 1950 was used to determine dose-response relationships for the induction of bone sarcomas by radium. Two subpopulations were analyzed: all measured cases who survived at last five years after the start of employment and all cases who survived at least two years after first measurement. The first constituted a group based on year of entry; it contained 1468 women who experienced 42 bone sarcomas; the expected number was 0.4. The second comprised a group based on first measurement; it contained 1257 women who experienced 13 bone sarcomas; the expected number was 0.2. The dose-response function, I = (C + ..cap alpha..D + ..beta..D/sup 2/)e/sup -..gamma..D/, and simplifications of this general form, were fit to each data set. Two functions, I = (C + ..cap alpha..D + ..beta..D/sup 2/)e/sup -..gamma..D/ and I = (C + ..beta..D/sup 2/)e/sup -..gamma..D/, fit the data for year of entry (p greater than or equal to 0.05); both these functions and I = (C + ..cap alpha..D) fit the data for first measurement. The function I = (C + ..beta..D/sup 2/)e/sup -..gamma..D/ was used to predict the number of bone sarcomas in all other pre-1950 radium cases (medical, laboratory, and other exposure); fewer were actually observed than the fit of this function to the female dial workers predicted.

  13. Biologic Activity of Autologous, Granulocyte-Macrophage Colony Stimulating Factor Secreting Alveolar Soft Parts Sarcoma and Clear Cell Sarcoma Vaccines

    PubMed Central

    Goldberg, John; Fisher, David E.; Demetri, George D.; Neuberg, Donna; Allsop, Stephen A.; Fonseca, Catia; Nakazaki, Yukoh; Nemer, David; Raut, Chandrajit P.; George, Suzanne; Morgan, Jeffrey A.; Wagner, Andrew J.; Freeman, Gordon J.; Ritz, Jerome; Lezcano, Cecilia; Mihm, Martin; Canning, Christine; Hodi, F. Stephen; Dranoff, Glenn

    2015-01-01

    Purpose Alveolar soft parts sarcoma (ASPS) and clear cell sarcoma (CCS) are rare mesenchymal malignancies driven by chromosomal translocations that activate members of the microphthalmia transcription factor (MITF) family. However, in contrast to malignant melanoma, little is known about their immunogenicity. To learn more about the host response to ASPS and CCS, we conducted a phase I clinical trial of vaccination with irradiated, autologous sarcoma cells engineered by adenoviral mediated gene transfer to secrete granulocyte-macrophage colony stimulating factor (GM-CSF). Experimental Design Metastatic tumors from ASPS and CCS patients were resected, processed to single cell suspensions, transduced with a replication defective adenoviral vector encoding GM-CSF, and irradiated. Immunizations were administered subcutaneously and intradermally weekly times three and then every other week. Results Vaccines were successfully manufactured for 11 of the 12 enrolled patients. Eleven subjects received from 3 to 13 immunizations. Toxicities were restricted to grade 1–2 skin reactions at inoculation sites. Vaccination elicited local dendritic cell infiltrates and stimulated T cell mediated delayed type-hypersensitivity reactions to irradiated, autologous tumor cells. Antibody responses to tissue-type plasminogen activator (tTPA) and angiopoietins-1/2 were detected. Tumor biopsies showed programmed death-1 (PD-1) positive CD8+ T cells in association with PD ligand-1 (PD-L1) expressing sarcoma cells. No tumor regressions were observed. Conclusions Vaccination with irradiated, GM-CSF secreting autologous sarcoma cell vaccines is feasible, safe, and biologically active. Concurrent targeting of angiogenic cytokines and antagonism of the PD-1 negative regulatory pathway might intensify immune-mediated tumor destruction. PMID:25805798

  14. Differential Expression of Viral Bcl-2 Encoded by Kaposi's Sarcoma-Associated Herpesvirus and Human Bcl-2 in Primary Effusion Lymphoma Cells and Kaposi's Sarcoma Lesions

    PubMed Central

    Widmer, Isabelle; Wernli, Marion; Bachmann, Felix; Gudat, Fred; Cathomas, Gieri; Erb, Peter

    2002-01-01

    Expression of human herpesvirus 8 viral Bcl-2 protein was demonstrated in spindle cells of late-stage Kaposi's sarcoma lesions but not in primary effusion lymphoma cell lines. In contrast, strong expression of human Bcl-2 was found in stimulated primary effusion lymphoma cells, whereas in Kaposi's sarcoma lesions preferential mononuclear cells, and to a lesser extent spindle cells, stained positive. PMID:11836434

  15. "Malignant" uterine perivascular epithelioid cell tumor, pelvic lymph node lymphangioleiomyomatosis, and gynecological pecomatosis in a patient with tuberous sclerosis: a case report and review of the literature.

    PubMed

    Liang, Sharon X; Pearl, Michael; Liu, Jingxuan; Hwang, Sonia; Tornos, Carmen

    2008-01-01

    We report a case of uterine perivascular epithelioid cell tumor (PEComa) with malignant histological features in a 59-year-old woman with tuberous sclerosis (TBS). The patient also had extrapulmonary lymphangioleiomyomatosis involving pelvic lymph nodes, myometrium, cervix, and ovary ("pecomatosis"). The uterine tumor measured 2.6 cm and had marked nuclear pleomorphism, necrosis, and 2 mitoses per 50 high-powered field, with an occasional atypical mitosis and infiltrative borders. The nonneoplastic myometrium, the cervical wall, and the hilum of the ovary had multiple clusters of bland-looking epithelioid clear cells that ranged from 1 to 5 mm (pecomatosis). The uterine tumor cells were positive for HMB-45 (90%), Melan-A (70%), smooth muscle actin (50%), and estrogen receptor (30%). Of the 16 pelvic lymph nodes excised, 3 were involved with lymphangioleiomyomatosis that was positive for HMB-45 and estrogen receptor. This is only the second reported PEComa associated with pecomatosis and the fourth PEComa described in a patient with TBS. The clinical significance of pecomatosis is still uncertain but seems to be seen only in patients with TBS.

  16. Hepatic epithelioid hemangioendothelioma: A comparison of Western and Chinese methods with respect to diagnosis, treatment and outcome.

    PubMed

    Zhao, Xin Yan; Rakhda, Mohmedmohsin Ismail Ahmed; Habib, Sohail; Bihi, Ali; Muhammad, Abdullah; Wang, Tai Ling; Jia, Ji-Dong

    2014-04-01

    Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor of vascular origin. Whether HEHE in Chinese patients exhibits similar characteristics compared with Western patients is not well known. The aim of the present study was to summarize the characteristics of HEHE in Chinese patients and identify its prognostic factors. In total, six patients diagnosed with HEHE at the Beijing Friendship Hospital between 2000 and 2012 were combined with 44 previously reported cases in China, retrieved from the literature between 1989 and mid-2012. These 50 cases from China were compared with 402 patients from Western populations. Prognostic factors were identified by the χ(2) test and Cox regression analysis. The male to female ratio of the Chinese patients was 1:2.1 with the mean age of 44.2 years (range, 22-86 years). The percentage of asymptomatic Chinese patients was significantly higher than in the Western patients (40.0 vs. 24.8%; P=0.026), and that of extrahepatic metastasis (16.0 vs. 36.6%; P=0.005) was significantly lower in Chinese patients. On imaging study, capsular retraction (59.5%) and calcification (26.0%), as well as positivity of CD34 (93.5%) and CD31 (80.6%), were more frequently found in the Chinese patients. Management for the Chinese patients included liver resection (LRx; 45.7%), liver transplantation (LTx; 5.7%), trans-catheter arterial chemoembolization (14.3%) and palliative treatment (34.3%). Chinese patients with larger-sized tumor nodules [relative risk (RR), 1.58; 95% confidence interval (CI), 1.032-2.422; P=0.035) and diffuse type (RR, 12.17; 95% CI, 1.595-92.979; P=0.016) exhibited unfavorable outcomes. In contrast to Western patients with HEHE, a larger number of Chinese patients were asymptomatic with less extrahepatic metastasis. In China, LRx is widely adopted rather than LTx. Chinese patients with large tumor size or diffuse type may encounter a poorer prognosis. PMID:24944653

  17. Hepatic epithelioid hemangioendothelioma: A comparison of Western and Chinese methods with respect to diagnosis, treatment and outcome

    PubMed Central

    ZHAO, XIN YAN; RAKHDA, MOHMEDMOHSIN ISMAIL AHMED; HABIB, SOHAIL; BIHI, ALI; MUHAMMAD, ABDULLAH; WANG, TAI LING; JIA, JI-DONG

    2014-01-01

    Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor of vascular origin. Whether HEHE in Chinese patients exhibits similar characteristics compared with Western patients is not well known. The aim of the present study was to summarize the characteristics of HEHE in Chinese patients and identify its prognostic factors. In total, six patients diagnosed with HEHE at the Beijing Friendship Hospital between 2000 and 2012 were combined with 44 previously reported cases in China, retrieved from the literature between 1989 and mid-2012. These 50 cases from China were compared with 402 patients from Western populations. Prognostic factors were identified by the χ2 test and Cox regression analysis. The male to female ratio of the Chinese patients was 1:2.1 with the mean age of 44.2 years (range, 22–86 years). The percentage of asymptomatic Chinese patients was significantly higher than in the Western patients (40.0 vs. 24.8%; P=0.026), and that of extrahepatic metastasis (16.0 vs. 36.6%; P=0.005) was significantly lower in Chinese patients. On imaging study, capsular retraction (59.5%) and calcification (26.0%), as well as positivity of CD34 (93.5%) and CD31 (80.6%), were more frequently found in the Chinese patients. Management for the Chinese patients included liver resection (LRx; 45.7%), liver transplantation (LTx; 5.7%), trans-catheter arterial chemoembolization (14.3%) and palliative treatment (34.3%). Chinese patients with larger-sized tumor nodules [relative risk (RR), 1.58; 95% confidence interval (CI), 1.032–2.422; P=0.035) and diffuse type (RR, 12.17; 95% CI, 1.595–92.979; P=0.016) exhibited unfavorable outcomes. In contrast to Western patients with HEHE, a larger number of Chinese patients were asymptomatic with less extrahepatic metastasis. In China, LRx is widely adopted rather than LTx. Chinese patients with large tumor size or diffuse type may encounter a poorer prognosis. PMID:24944653

  18. Large cervicothoracic myxoinflammatory fibroblastic sarcoma with brachial plexus invasion: A case report and literature review

    PubMed Central

    Jia, Xiaotian; Yang, Jianyun; Chen, Lin; Yu, Cong

    2016-01-01

    Myxoinflammatory fibroblastic sarcoma is a rare sarcoma that develops in patients of all ages, which usually presents as a slow-growing painless mass in the distal extremities. To date, myxoinflammatory fibroblastic sarcoma with invasion of the brachial plexus has rarely been reported in the literature. In this study, a case of large cervicothoracic sarcoma, which invaded the brachial plexus, is presented. The patient reported no sensory disturbance or dyskinesia. The tumor was completely resected without injury of the brachial plexus. The postoperative histological diagnosis was myxoinflammatory fibroblastic sarcoma. Follow-up examination performed 24 months after surgery revealed no tumor recurrence and no sensory disturbance or dyskinesia was reported. This study presents a rare case of large myxoinflammatory fibroblastic sarcoma with brachial plexus invasion that was successfully managed by surgery. PMID:27588121

  19. Molecular Pathogenesis and Diagnostic, Prognostic and Predictive Molecular Markers in Sarcoma.

    PubMed

    Mariño-Enríquez, Adrián; Bovée, Judith V M G

    2016-09-01

    Sarcomas are infrequent mesenchymal neoplasms characterized by notable morphological and molecular heterogeneity. Molecular studies in sarcoma provide refinements to morphologic classification, and contribute diagnostic information (frequently), prognostic stratification (rarely) and predict therapeutic response (occasionally). Herein, we summarize the major molecular mechanisms underlying sarcoma pathogenesis and present clinically useful diagnostic, prognostic and predictive molecular markers for sarcoma. Five major molecular alterations are discussed, illustrated with representative sarcoma types, including 1. the presence of chimeric transcription factors, in vascular tumors; 2. abnormal kinase signaling, in gastrointestinal stromal tumor; 3. epigenetic deregulation, in chondrosarcoma, chondroblastoma, and other tumors; 4. deregulated cell survival and proliferation, due to focal copy number alterations, in dedifferentiated liposarcoma; 5. extreme genomic instability, in conventional osteosarcoma as a representative example of sarcomas with highly complex karyotype. PMID:27523972

  20. Ewing's Sarcoma as a Second Malignancy in Long-Term Survivors of Childhood Hematologic Malignancies

    PubMed Central

    Grotzer, Michael A.; Niggli, Felix; Zimmermann, Dieter; Rushing, Elisabeth

    2016-01-01

    Modern multimodal treatment has significantly increased survival for patients affected by hematologic malignancies, especially in childhood. Following remission, however, the risk of developing a further malignancy is an important issue. The long-term estimated risk of developing a sarcoma as a secondary malignancy is increased severalfold in comparison to the general population. Ewing's sarcoma family encompasses a group of highly aggressive, undifferentiated, intra- and extraosseous, mesenchymal tumors, caused by several types of translocations usually involving the EWSR1 gene. Translocation associated sarcomas, such as Ewing sarcoma, are only rarely encountered as therapy associated secondary tumors. We describe the clinical course and management of three patients from a single institution with Ewing's sarcoma that followed successfully treated lymphoblastic T-cell leukemia or non-Hodgkin lymphoma. The literature on secondary Ewing's sarcoma is summarized and possible pathogenic mechanisms are critically discussed. PMID:27524931