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Sample records for alveolar soft-part sarcoma

  1. Cediranib for Metastatic Alveolar Soft Part Sarcoma

    PubMed Central

    Kummar, Shivaani; Allen, Deborah; Monks, Anne; Polley, Eric C.; Hose, Curtis D.; Ivy, S. Percy; Turkbey, Ismail B.; Lawrence, Scott; Kinders, Robert J.; Choyke, Peter; Simon, Richard; Steinberg, Seth M.; Doroshow, James H.; Helman, Lee

    2013-01-01

    Purpose Alveolar soft part sarcoma (ASPS) is a rare, highly vascular tumor, for which no effective standard systemic treatment exists for patients with unresectable disease. Cediranib is a potent, oral small-molecule inhibitor of all three vascular endothelial growth factor receptors (VEGFRs). Patients and Methods We conducted a phase II trial of once-daily cediranib (30 mg) given in 28-day cycles for patients with metastatic, unresectable ASPS to determine the objective response rate (ORR). We also compared gene expression profiles in pre- and post-treatment tumor biopsies and evaluated the effect of cediranib on tumor proliferation and angiogenesis using positron emission tomography and dynamic contrast-enhanced magnetic resonance imaging. Results Of 46 patients enrolled, 43 were evaluable for response at the time of analysis. The ORR was 35%, with 15 of 43 patients achieving a partial response. Twenty-six patients (60%) had stable disease as the best response, with a disease control rate (partial response + stable disease) at 24 weeks of 84%. Microarray analysis with validation by quantitative real-time polymerase chain reaction on paired tumor biopsies from eight patients demonstrated downregulation of genes related to vasculogenesis. Conclusion In this largest prospective trial to date of systemic therapy for metastatic ASPS, we observed that cediranib has substantial single-agent activity, producing an ORR of 35% and a disease control rate of 84% at 24 weeks. On the basis of these results, an open-label, multicenter, randomized phase II registration trial is currently being conducted for patients with metastatic ASPS comparing cediranib with another VEGFR inhibitor, sunitinib. PMID:23630200

  2. [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.

  3. Alveolar soft part sarcoma of the vagina. A case report.

    PubMed

    Chang, H C; Hsueh, S; Ho, Y S; Chang, M Y; Soong, Y K

    1994-02-01

    We present a case of alveolar soft part sarcoma of the vagina in an 18-year-old woman. The presenting symptoms were menometrorrhagia and a painless vaginal mass. The tumor was in the vaginal stroma with intact mucosa. Periodic acid-Schiff stain showed positive diastase-resistant crystals in some of the tumor cells. Electron microscopy demonstrated characteristic intracytoplasmic crystals. Formaldehyde-induced fluorescence stain showed no catecholamines, and Grimelius stain showed no argyrophilic granules. Black pigment was found within some tumor cells; it proved to be melanin pigment by Masson-Fontana stain. The tumor mass recurred four months after local excision. Following the second surgical removal, the patient received adjuvant radiotherapy to the whole pelvis, including the vagina. At this writing she was currently alive, without evidence of recurrence, five years after the primary diagnosis.

  4. A Case of Alveolar Soft Part Sarcoma of the Pleura

    PubMed Central

    Ju, Hyeong Uk; Seo, Kwang Won; Jegal, Yangjin; Ahn, Jong-Joon; Lee, Young Jik; Kim, Young Min; Oak, Chulho

    2013-01-01

    Alveolar soft part sarcoma (ASPS) is a rare malignant soft-tissue neoplasm of unknown histogenesis. The two main sites of occurrence are the lower extremities in adults and the head and neck in children. We report the first case of pleural ASPS occurring in a 58-yr-old man who presented with progressive dyspnea. A computed tomographic scan of the thorax revealed a large enhancing pleural mass with pleural effusion in the left hemithorax. Wide excision of the pleural mass was performed. Histologically, the tumor consisted of organoid nests of large polygonal cells, the cytoplasm of which had eosinophilic and D-PAS positive granules. Immunohistochemical staining showed that the tumor cell nuclei were positive for transcription factor 3 (TFE3). The pleural ASPS with multiple bone metastases recurred 1 yr after surgery and the patient died of acute pulmonary embolism 1.5 yr after diagnosis. PMID:23400231

  5. Alveolar soft-part sarcoma of the mediastinum: A case report

    PubMed Central

    Kameda, Yohei; Nishii, Teppei; Tsuboi, Masahiro; Arai, Hiromasa; Inui, Kenji; Kaneko, Takeshi; Kimura, Noriko; Naruse, Mitsuhide; Masuda, Munetaka

    2017-01-01

    We report a 53-year-old man with metastases of alveolar soft-part sarcoma originated from the mediastinum. He was hospitalized due to lower extremities’ paralysis. Computed tomography scan findings revealed multiple nodules of bilateral lungs, swollen mediastinal lymph nodes, and osteolysis of thoracic vertebrae. We performed spinal decompression and biopsy from vertebra. And, we finally diagnosed this case as metastases of mediastinal alveolar soft-part sarcoma which was removed 10 years ago. Alveolar soft-part sarcoma is rare tumor accounted for 0.5%–1.0% of soft tissue sarcoma that often occurs primarily in the lower extremities and trunk. It is difficult to distinguish between alveolar soft-part sarcoma and paraganglioma, renal cell carcinoma and granular cell tumor morphologically. Periodic acid–Schiff stain and immunohistochemical staining of ASPL-TFE3 are useful in making a definitive diagnosis of alveolar soft-part sarcoma. This case is a rare case of alveolar soft-part sarcoma originated in the mediastinum with local recurrence and distant metastases 10 years after the initial surgery. PMID:28321306

  6. Alveolar soft-part sarcoma of the mediastinum: A case report.

    PubMed

    Kameda, Yohei; Nishii, Teppei; Tsuboi, Masahiro; Arai, Hiromasa; Inui, Kenji; Kaneko, Takeshi; Kimura, Noriko; Naruse, Mitsuhide; Masuda, Munetaka

    2017-01-01

    We report a 53-year-old man with metastases of alveolar soft-part sarcoma originated from the mediastinum. He was hospitalized due to lower extremities' paralysis. Computed tomography scan findings revealed multiple nodules of bilateral lungs, swollen mediastinal lymph nodes, and osteolysis of thoracic vertebrae. We performed spinal decompression and biopsy from vertebra. And, we finally diagnosed this case as metastases of mediastinal alveolar soft-part sarcoma which was removed 10 years ago. Alveolar soft-part sarcoma is rare tumor accounted for 0.5%-1.0% of soft tissue sarcoma that often occurs primarily in the lower extremities and trunk. It is difficult to distinguish between alveolar soft-part sarcoma and paraganglioma, renal cell carcinoma and granular cell tumor morphologically. Periodic acid-Schiff stain and immunohistochemical staining of ASPL-TFE3 are useful in making a definitive diagnosis of alveolar soft-part sarcoma. This case is a rare case of alveolar soft-part sarcoma originated in the mediastinum with local recurrence and distant metastases 10 years after the initial surgery.

  7. Alveolar soft part sarcoma following radiotherapy for a spinal hemangioma. A case report

    SciTech Connect

    Wang, S.; Mirra, J.; Bhuta, S.

    1984-06-15

    A case of alveolar soft part sarcoma arising some 20 years later in a site previously irradiated as a treatment for a spinal cord hemangioma is described. This is the first known case of radiation-associated alveolar soft part sarcoma, and it fulfills the criteria for a tumor to be radiation-induced. The coincidental finding of ''viral-like'' particles within some of the tumor cells was noted.

  8. Lung Metastasis of Primary Alveolar Soft-Part Sarcoma Occurring 20 Years after Initial Treatment

    PubMed Central

    Falkenstern-Ge, R. F.; Kimmich, M.; Wohlleber, M.; Grabner, A.; Friedel, G.; Ott, G.; Leuschner, I.; Kohlhäufl, M.

    2013-01-01

    A 30-year old woman was referred to our center because of suspicion of a primary lung tumor of the right upper lobe. Histological examination of the lung lesion revealed lung metastasis of a previously treated alveolar soft part sarcoma of the musculus vastus medialis of the right femur, which was resected 20 years ago. Alveolar soft-part sarcoma is a rare malignant tumor that occurs most often in the soft tissue of lower limbs. It is a slow-growing malignant soft tissue tumor arising in muscle tissue, usually in young adults. Due to pleural and extensive mediastinal infiltration with bilateral lung metastases, a systemic treatment with chemotherapy doxorubicin and ifosfamide was initiated. Late metastases from previously treated alveolar part sarcoma should be considered in patients with suspicious lung lesions even if surgical treatment was performed a long time ago. PMID:24379977

  9. Metastatic Alveolar Soft Part Sarcoma Responsive to Pazopanib after Progression through Sunitinib and Bevacizumab: Two Cases

    PubMed Central

    Read, William L.; Williams, Felicia

    2016-01-01

    Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma with a propensity for lung metastases and indolent progression. ASPS is not responsive to chemotherapy, but there are case reports and small series describing benefit from drugs targeting the VEGF pathway. These drugs include sunitinib, cediranib and bevacizumab. There is no established second-line treatment for persons with ASPS progressing through first-line targeted therapy. We report two individuals with metastatic ASPS who obtained disease stabilization from sunitinib lasting over a year. After subsequent progression through sunitinib and second-line bevacizumab, both individuals again had disease response and subsequent stabilization from pazopanib. PMID:27920695

  10. Orbital alveolar soft-part sarcoma: clinico-pathological profiles, management and outcomes.

    PubMed

    Mulay, Kaustubh; Ali, Mohammad Javed; Honavar, Santosh G; Reddy, Vijay Anand P

    2014-01-01

    To study the clinico-radiological and clinic-pathological features of orbital alveolar soft-part sarcomas (ASPS). We retrieved 9 cases of orbital ASPS. Histopathological and immunohistochemical features and clinico-radiological findings were evaluated. Age at presentation varied from 1 to 31 years. Both, children and adults were affected. Proptosis was the most common clinical presentation. PAS-positive diastase-resistant crystals and granules were found in all cases. All patients received adjuvant radiotherapy. Two cases had positive surgical margins and were treated with additional adjuvant chemotherapy. None of the patients had recurrence or metastases till the last follow up. Early diagnosis with a multidisciplinary, multimodal approach of treatment is quintessential in achieving a long disease-free survival in patients with ASPS.

  11. Alveolar soft-part sarcoma of the tongue in a 17-month-old.

    PubMed

    Hillyer, Shahab; Vicens, Jose C; Levinson, Howard; Bhayani, Rajendra; Mesea, Lilia; Chaudhry, Rashid; Fayans, Edgar; Fogler, Richard

    2009-10-01

    Alveolar soft-part sarcoma (ASPS) is a rare and often fatal tumor. Overall survival rates have been reported to be 62% after 5 years, 42% after 10 years, and 18% after 20 years. ASPS accounts for 5% of all pediatric soft-tissue sarcomas other than rhabdomyosarcomas. In children, ASPS rarely occurs in the oral cavity, and to the best of our knowledge, only 12 cases of ASPS of the tongue occurring during the first decade of life have been previously reported in the literature. Because of the rarity of lingual ASPS in children, no specific treatment protocols have been developed, which makes its management difficult. We report a new case of lingual ASPS in a young child. Our patient was a 17-month-old girl whose tumor was located at the base of the tongue. She was successfully treated with surgical excision without adjuvant radiation or chemotherapy. Postoperatively, she has remained disease-free during 4 years of follow-up.

  12. 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

  13. Alveolar Soft Part Sarcoma-A Histological Surprise in a Male Patient who was Suspected to have Breast Cancer

    PubMed Central

    Varghese, Sunitha Susan; Sasidharan, Balukrishna; Kandasamy, Subramaniam; Manipadam, Marie Therese; Backianathan, Selvamani

    2013-01-01

    Alveolar Soft Part Sarcoma (ASPS) is a very rare type of soft tissue sarcoma. Its cell of origin is unclear. It usually presents in the second to fourth decade of life. The most common reported sites of ASPS are the lower extremities, the head and the neck. Because of the rarity of this disease, there is no standard treatment plan. Surgical excision with negative margins is considered as the treatment of choice. We are reporting a rare presentation of ASPS as a male breast lump. PMID:23730668

  14. Identification of target genes of cediranib in alveolar soft part sarcoma using a gene microarray.

    PubMed

    Jiang, Wenhua; Liu, Pengfei; Li, Xiaodong; Wang, Ping

    2017-04-01

    The aim of the present study was to identify the target genes of cediranib and the associated signaling pathways in alveolar soft part sarcoma (ASPS). A microarray dataset (GSE32569) was obtained from the Gene Expression Omnibus database. The R software package was used for data normalization and screening of differentially expressed genes (DEGs). The Database for Annotation, Visualization and Integrated Discovery was used to perform Gene Ontology analysis. Gene Set Enrichment Analysis was performed to obtain the up- and downregulated pathways in ASPS. The Distant Regulatory Elements of co-regulated genes database was used to identify the transcription factors (TFs) that were enriched in the signaling pathways. A protein-protein interaction (PPI) network was constructed using the Search Tool for the Retrieval of Interacting Genes/Proteins database and was visualized using Cytoscape software. A total of 71 DEGs, including 59 upregulated genes and 12 downregulated genes, were identified. Gene sets associated with ASPS were enriched primarily in four signaling pathways: The phenylalanine metabolism pathway, the mitogen-activated protein kinase (MAPK) signaling pathway, the taste transduction pathway and the intestinal immune network for the production of immunoglobulin A. Furthermore, 107 TFs were identified to be enriched in the MAPK signaling pathway. Certain genes, including those coding for Fms-like tyrosine kinase 1, kinase insert domain receptor, E-selectin and platelet-derived growth factor receptor D, that were associated with other genes in the PPI network, were identified. The present study identified certain potential target genes and the associated signaling pathways of cediranib action in ASPS, which may be helpful in understanding the efficacy of cediranib and the development of new targets for cediranib.

  15. The Precrystalline Cytoplasmic Granules of Alveolar Soft Part Sarcoma Contain Monocarboxylate Transporter 1 and CD147

    PubMed Central

    Ladanyi, Marc; Antonescu, Cristina R.; Drobnjak, Marija; Baren, Ann; Lui, Man Yee; Golde, David W.; Cordon-Cardo, Carlos

    2002-01-01

    Alveolar soft part sarcoma (ASPS) is an unusual tumor of young adults with the characteristic presence on ultrastructural analysis of rhomboid or rectangular cytoplasmic crystals. These membrane-bound crystals are known to form within specific PAS-diastase-resistant electron-dense cytoplasmic granules. The composition of these crystals and the dense granules from which they are derived has remained elusive. After the detection of strong discrete granular cytoplasmic immunoreactivity in ASPS for monocarboxylate transporter 1 (MCT1) in the course of a broad immunohistochemical characterization of an MCT1 antibody, we studied the expression of MCT1 and its interacting partner, CD147, in a panel of 10 ASPS cases using appropriate antibodies. MCT1 is one of a family of widely expressed proton-linked transporters for monocarboxylates such as lactate and pyruvate. In all normal and neoplastic tissues studied to date, MCT1 immunoreactivity is limited to the cell surface. We find that the periodic acid-Schiff-diastase-resistant cytoplasmic granules of ASPS are strongly immunoreactive for MCT1 and CD147. Specifically, intense cytoplasmic granular positivity for MCT1 and CD147 was found in 7 of 10 and 8 of 10 ASPSs, respectively. Ultrastructural immunohistochemistry with immunogold labeling confirmed that the MCT1 immunoreactivity localized to the cytoplasmic electron-dense granules in ASPS. Western blot analysis of several ASPS cases confirmed that the protein reactive with the MCT1 antibody and that reactive with the CD147 antibody both migrated at the size expected for MCT1 and CD147, respectively. Thus, ASPS cells seem to accumulate MCT1-CD147 complexes in the specific cytoplasmic granules known to undergo crystallization. The possible basis for the overproduction or impaired surface localization of these proteins in ASPS remains unclear. PMID:11943706

  16. Brain metastasis of crystal-deficient, CD68-positive alveolar soft part sarcoma: ultrastructural features and differential diagnosis

    PubMed Central

    Cykowski, Matthew D.; Hicks, John; Sandberg, David I.; Olar, Adriana; Bridge, Julia A.; Greipp, Patricia T.; Navarro, Patricia; Kolodziej, Steven; Bhattacharjee, Meenakshi B.

    2014-01-01

    We report a case of alveolar soft part sarcoma (ASPS) presenting as an isolated frontal lobe metastasis. The tumor demonstrated little or no immunoreactivity for a broad panel of antibodies yet strong, diffuse immunoreactivity with CD68. On electron microscopy, characteristic rectangular to rhomboid crystalline inclusions were not present. Electron-dense granules resembling peroxisomes were present, sometimes in association with elongated granular structures having a periodic, lattice-like arrangement. Metastatic ASPS was confirmed by demonstration of an ASPSCR1-TFE3 fusion and imaging studies that excluded metastatic Xp11.2 translocation renal cell carcinoma. The primary site was subsequently identified in the lower extremity. PMID:25268941

  17. Alveolar soft part sarcoma of the oral and maxillofacial region: clinical analysis in a series of 18 patients.

    PubMed

    Wang, Hong-Wei; Qin, Xing-Jun; Yang, Wen-Jun; Xu, Li-Qun; Ji, Tong; Zhang, Chen-Ping

    2015-04-01

    To summarize the clinical features, diagnosis, treatment strategies, and prognosis of alveolar soft part sarcoma (ASPS) of the oral and maxillofacial region. We performed a retrospective study in a consecutive series of 18 patients with ASPS of the oral and maxillofacial region between 1995 and 2013. Demographic characteristics, tumor sizes, sites, tumor metastasis, diagnosis, treatments, and overall follow-ups were documented. The 18 patients were diagnosed pathologically with primary tumor developed on the tongue (10), the cheek (5), the pharynx (1), and the gingiva (2) with an average tumor size of 4 cm. At the latest follow-up, 1 patient with lung metastases survived for 23 months; 1 died 3 months after the confirmation of local recurrence and multiple pulmonary metastases; the rest of the patients were disease free and remained in good health. ASPS of the oral and maxillofacial region appears to have special clinical characteristics. Copyright © 2015. Published by Elsevier Inc.

  18. Modeling alveolar soft part sarcomagenesis in the mouse: a role for lactate in the tumor microenvironment

    PubMed Central

    Goodwin, Matthew L.; Jin, Huifeng; Straessler, Krystal; Smith-Fry, Kyllie; Zhu, Ju-Fen; Monument, Michael J.; Grossmann, Allie; Randall, R. Lor; Capecchi, Mario R.; Jones, Kevin B.

    2014-01-01

    Summary Alveolar soft part sarcoma (ASPS), a deadly soft tissue malignancy with a predilection for adolescents and young adults, associates consistently with t(X;17) translocations that generate the fusion gene ASPSCR1-TFE3. We proved the oncogenic capacity of this fusion gene by driving sarcomagenesis in mice from conditional ASPSCR1-TFE3 expression. The completely penetrant tumors were indistinguishable from human ASPS by histology and gene expression. They formed preferentially in the anatomic environment highest in lactate--the cranial vault--, expressed high levels of lactate importers, harbored abundant mitochondria, metabolized lactate as a metabolic substrate and responded to the administration of exogenous lactate with tumor cell proliferation and angiogenesis. These data demonstrate lactate’s role as a driver of alveolar soft part sarcomagenesis. PMID:25453902

  19. Trial of Dasatinib in Advanced Sarcomas

    ClinicalTrials.gov

    2017-03-20

    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)

  20. T1-201 chloride scintigraphy for bone tumors and soft part sarcomas

    SciTech Connect

    Terui, S.; Oyamada, H.; Nishikawa, K.; Beppu, Y.; Fukuma, H.

    1984-01-01

    The author investigated T1-201 chloride as a tumor scanning agent of both tumors and soft part sarcomas. Six bone tumors (2 with Ewing sarcoma, 3 with osteosarcoma and 1 with giant cell tumor) and 3 soft part sarcoma (1 with liposarcoma and 2 with malignant fibrous histiocytoma (MFH)) were examined. All but one MFH were untreated primary cases. The diagnosis was determined from biopsy specimen. One patient with Ewing sarcoma had bone metastases. All cases were subsequently received chemotherpeutic agents. Surgery or local irradiation were also used in treatment. T1-201 scintigraphy were performed with intravenous administration of 2 mCi of T1-201 chloride before initiation of therapy. In addition, follow-up examinations were done in 4 patients (2 with Ewing sarcoma and 2 with osteosarcoma) to study the effect of chemotherapy on T1-201 uptake by the tumor. Tc-99m bone scans were available for comparison in 6 tumor. Ga-67 citrate scans were also examined for the 3 soft part sarcomas. The untreated tumors even in the metastatic lesions of Ewing sarcoma were distinctly visualized with T1-201 in all cases. The distribution of T1-201 in the tumors was sometimes different from that of Tc-99m and similar to that of Ga-67. Of 3 out of the 4 follow-up patients, the post-therapy scan showed reduction in T1-201 uptake more markedly than Tc-99m uptake during effective chemotherapy. The other one patient had not responded to the treatment so that the scan showed no changes in T1-201 uptake. These findings indicate that the tumor imaging with T1-201 is useful in the diagnosis of these malignant tumors and may be of value in assessing the response of bone tumors to chemotherapy.

  1. Detection of ASPL/TFE3 fusion transcripts and the TFE3 antigen in formalin-fixed, paraffin-embedded tissue in a series of 18 cases of alveolar soft part sarcoma: useful diagnostic tools in cases with unusual histological features.

    PubMed

    Williams, Ann; Bartle, Gillian; Sumathi, Vaiyapuri P; Meis, Jeanne M; Mangham, D Chas; Grimer, Rob J; Kindblom, Lars-Gunnar

    2011-03-01

    Alveolar soft part sarcoma (ASPS) is a rare malignancy; diagnostic problems may occur when cases present as a metastasis or with unusual morphologic features. In this study, a series of 18 cases with follow-up information were analysed with regard to the ASPL/TFE3 fusion transcripts and immuno-detection of TFE3 using archival formalin-fixed, paraffin-embedded tissues. Novel primers to detect ASPL/TFE3 fusion transcripts, type 1 and 2, were designed. The patients, ten female and eight male, ranged in age from 3 to 46 years; 16 involved soft tissues of the extremities (nine, lower; seven, upper), one involved the uterine cervix and one was a primary bone tumour of the foot. Seven ASPS had unusual morphologic features lacking the typical alveolar pattern. Seven had lung metastases at the time of diagnosis, and three developed lung and brain metastases later. Four patients died of disease (after 1-5 years); four are alive with metastases (after 2-15 years), and ten are alive and well (after 1-10 years). Vascular invasion correlated with metastatic disease. All 18 ASPS, four granular cell tumours (one of which was malignant) and one adrenal cortical carcinoma showed TFE3 immuno-positivity. The 18/18 ASPS showed ASPL/TFE3 fusion transcripts (nine, type 1; nine, type 2), four of which had a balanced translocation. ASPL/TFE3 fusion transcripts were not detected in 25 controls. We conclude that immuno-detection of TFE3 and RT-PCR-based identification of ASPL/TFE3 fusion transcripts in formalin-fixed, paraffin-embedded tissues are powerful tools in the diagnosis of ASPS, particularly in cases with unusual morphologic features.

  2. Clear cell sarcoma (malignant melanoma of soft parts) and sentinel lymph node biopsy.

    PubMed

    Picciotto, Franco; Zaccagna, Alessandro; Derosa, Giovanni; Pisacane, Alberto; Puiatti, Paolo; Colombo, Enrico; Dardano, Fabrizio; Ottinetti, Antonio

    2005-01-01

    Clear cell sarcoma of the tendons and aponeuroses is an aggressive, rare soft tissue tumour that occurs predominantly in the extremities of young adults. Although it appears to be histogenetically related to melanoma, its clinical behaviour resembles soft tissue sarcoma. Prognosis is reported to be poor due to the great propensity of regional and distant metastases. The risk of metastases to regional lymph nodes is very high. We describe a case of clear cell sarcoma of the hand and evaluate the feasibility of the sentinel lymph node biopsy (SLNB) technique in this kind of tumour. Up to now, there are no reports that describe the use of SLNB in clear cell sarcoma. The SLNB technique was carried out with success and the sentinel lymphnode was easily identified. Histological examination of the lymphnode allowed identification of metastatic cells and thus provide for radical lymphadenectomy. We maintain that SLNB can be successfully used for this type of tumour although this result should be evaluated and confirmed by larger case studies.

  3. 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

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

    ClinicalTrials.gov

    2017-03-16

    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 Malignant Solid Neoplasm; Recurrent Renal Cell Carcinoma; Recurrent Rhabdomyosarcoma; Renal Cell Carcinoma; Thyroid Gland Medullary Carcinoma; Wilms Tumor

  5. 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

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

    ClinicalTrials.gov

    2016-11-01

    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

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

    ClinicalTrials.gov

    2016-11-03

    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

  8. Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma

    ClinicalTrials.gov

    2016-11-14

    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 Undifferentiated Pleomorphic Sarcoma; Malignant Adult Hemangiopericytoma; Recurrent Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  9. MR appearance of clear cell sarcoma of tendons and aponeuroses (malignant melanoma of soft parts): radiologic-pathologic correlation.

    PubMed

    Hourani, Mukbil; Khoury, Nabil; Mourany, Bassem; Shabb, Nina Salem

    2005-09-01

    Clear cell sarcoma of tendons and aponeuroses (CCSTA) is a rare aggressive soft tissue tumor that frequently produces melanin. Its MR findings are rarely described in the literature. We report the case of a previously healthy 54-year-old man with clear cell sarcoma of the thigh who presented with a large painless mass of 1 year's duration. MR imaging showed the tumor to be of high signal intensity on fast spin-echo and STIR images. Both fine needle aspiration and excisional biopsy showed abundant melanin pigments. Histologic diagnosis was compatible with CCSTA.

  10. 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. Copyright © 2013 Elsevier Inc. All rights reserved.

  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. Patients with Advanced, Rare Sarcoma Respond to Cediranib | Center for Cancer Research

    Cancer.gov

    Alveolar soft part sarcomas (ASPS) are highly vascular tumors that usually affect adolescents and young adults. Comprising less than one percent of soft tissue sarcomas, ASPS can be cured with surgery. However, its tendency to metastasize and its lack of response to standard soft tissue sarcoma chemotherapy regimens makes ASPS a particularly lethal cancer with a five-year survival rate of 20 percent in patients with metastatic disease who are not candidates for surgery.

  13. 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

    2017-04-14

    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

  14. NY-ESO-1 expression in synovial sarcoma and other mesenchymal tumors: significance for NY-ESO-1-based targeted therapy and differential diagnosis.

    PubMed

    Lai, Jin-Ping; Robbins, Paul F; Raffeld, Mark; Aung, Phyu Phyu; Tsokos, Maria; Rosenberg, Steven A; Miettinen, Markku M; Lee, Chyi-Chia Richard

    2012-06-01

    A promising targeted therapy against NY-ESO-1 (CTAG 1B) using genetically modified T-cells in synovial sarcomas was recently demonstrated in a clinical trial at the NCI. To investigate the role of NY-ESO-1 immunohistochemistry in patient selection and gain better insight into the incidence of NY-ESO-1 expression in synovial sarcomas and other mesenchymal tumors, we evaluated NY-ESO-1 expression by immunohistochemistry in 417 tumors. This collection of samples included: 50 SS18/SSX1/2 fusion positive synovial sarcomas, 155 gastrointestinal stromal tumors (GIST), 135 other spindle cell sarcomas as well as 77 other sarcomas (chondrosarcoma, osteosarcoma, dedifferentiated liposarcoma, alveolar soft part sarcoma, rhabdomyosarcoma, angiosarcoma, malignant mesothelioma, and Ewing's sarcoma). We report that 76% of synovial sarcomas expressed NY-ESO-1 in a strong and diffuse pattern (2-3+, >50-70% of tumor cells). In contrast, only rare cases of other spindle cell mesenchymal tumor expressed NY-ESO-1 (GIST (2/155), malignant peripheral nerve sheath tumors (1/34), and dermatofibrosarcoma protuberans (2/20)). Individual cases of other sarcomas (angiosarcoma, malignant mesothelioma, chondrosarcoma, osteosarcoma, dedifferentiated liposarcoma, alveolar soft part sarcoma, and Ewing's sarcoma) were positive for NY-ESO-1. However, no positive cases were identified amongst our cohort of leiomyosarcomas (0/24), hemangiopericytoma/solitary fibrous tumors (0/40), and cellular schwannomas (0/17). In summary, we find that NY-ESO-1 is strongly and diffusely expressed in a majority of synovial sarcomas, but only rarely in other mesenchymal lesions. Beyond its role in patient selection for targeted therapy, immunohistochemistry for NY-ESO-1 may be diagnostically useful for the distinction of synovial sarcoma from other spindle cell neoplasms.

  15. Use of reverse transcriptase polymerase chain reaction for diagnosis and staging of alveolar rhabdomyosarcoma, Ewing sarcoma family of tumors, and desmoplastic small round cell tumor.

    PubMed

    Athale, U H; Shurtleff, S A; Jenkins, J J; Poquette, C A; Tan, M; Downing, J R; Pappo, A S

    2001-02-01

    To compare the use of reverse transcriptase polymerase chain reaction (RT-PCR) with that of morphology-based methods for diagnosis, staging, and detection of metastatic disease in pediatric alveolar rhabdomyosarcoma (ARMS), Ewing sarcoma family of tumors (ESFT), and desmoplastic small round cell tumors (DSRCT). RT-PCR assays for the EWS-FLII, EWS-ERG, PAX3-FKHR, PAX7-FKHR, and EWS-WTI fusion transcripts were performed on RNA extracted from the primary tumor tissue, bone marrow, and body fluids obtained at initial presentation and relapse. Molecular findings were compared with original histologic diagnoses and results of staging procedures. Eighty-eight samples from 47 patients with ARMS (n = 13), ESFT (n = 31), or DSRCT (n = 3) were analyzed. The detection rate of metastatic disease was significantly higher with RT-PCR (95%) as compared with the morphologic methods (70%) for the three pediatric sarcomas studied. In primary tumors with characteristic fusion transcript, RT-PCR was positive in all cases with morphologic evidence of metastatic disease. Moreover, in six patients (3 with ARMS, 2 with DSRCT, and 1 with ESFT) with metastatic disease, micrometastases in bone marrow (4) and other sites (2) were detected by RT-PCR alone. Importantly, none of the patients with localized disease diagnosed had micrometastases detected by RT-PCR in bone marrow. The high sensitivity and specificity of RT-PCR for the characteristic fusion transcripts of pediatric sarcomas make it an ideal method to aid in the routine staging of these patients. In addition, the 100% sensitivity of RT-PCR in detection of micrometastasis makes it useful for follow-up and detection of minimal residual disease. However, the clinical significance of molecularly-detectable disease remains unknown. Further studies should aim to elucidate the therapeutic and prognostic implications of micrometastases detected by RT-PCR alone.

  16. [Clinical analysis of 51 cases with rare childhood soft tissue sarcomas].

    PubMed

    Cai, M X; Pan, C; Ye, Q D; Zhou, M; Gao, Y J; Hu, W T; Tang, J Y

    2016-12-02

    Objective: To analyze the clinical characteristics and prognosis of rare soft tissue sarcomas. Method: Clinical data of 51 patients with rare soft tissue sarcomas including fibrosarcoma, synovial sarcoma, extrarenal rhabdoid tumor, alveolar soft part sarcoma, desmoplastic small round cell tumor and undifferentiated sarcoma in children and adolescents, diagnosed at Shanghai Children's Medical Center from June 1998 to December 2013, were retrospectively analyzed. All types were treated with the same strategy and chemotherapy regimens. Their clinical features, treatment and prognosis were discussed. Result: Seventeen patients with fibrosarcoma, 10 with synovial sarcoma, 9 with extrarenal rhabdoid tumor, 6 with alveolar soft part sarcoma, 3 with desmoplastic small round cell tumor and 6 with undifferentiated sarcoma were included. The mean age at initial diagnosis was 5 years(range from 1 month to 13.5 years). The most common primary site of tumors was limbs, followed by the thoracic and abdominal cavity, accounting for 41% and 24% respectively. Twelve cases presented distant tissue or organ involvement in which bone metastases occupied the first place. Seven cases(accounting for 14%)were at stage Ⅰ, 13 cases were at stage Ⅱ(accounting for 25%), 19 cases were at stage Ⅲ(accounting for 37%) and 12 cases were at stage Ⅳ(accounting for 24%). The median follow-up period was 36 months(range from 1 month to 123 months). Forty-four patients achieved complete remission and 3 patients achieved partial remission after initial treatment, the overall response rate was 92%. Subsequent follow-up showed 29 patients remained relapse-free while 13 patients had relapsed disease. Overall survival and event-free survival at 2 years were 88% and 57%.Postoperative surgical staging was the main prognostic factors. Patients with stage Ⅲ+ Ⅳ had poorer results than those with Ⅰ+ Ⅱ (χ(2)=4.909, P=0.027). Conclusion: These 6 types of soft tissue sarcomas are rare in children and

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

    PubMed

    Koelsche, Christian; Renner, Marcus; Hartmann, Wolfgang; Brandt, Regine; Lehner, Burkhard; Waldburger, Nina; Alldinger, Ingo; Schmitt, Thomas; Egerer, Gerlinde; Penzel, Roland; Wardelmann, Eva; Schirmacher, Peter; von Deimling, Andreas; Mechtersheimer, Gunhild

    2014-04-11

    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. 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. 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. TERT promoter mutations are frequent in MLSs including their round cell variants, representing

  18. 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

  19. Ewing sarcoma

    MedlinePlus

    Bone cancer - Ewing sarcoma; Ewing family of tumors; Primitive neuroectodermal tumors (PNET); Bone neoplasm - Ewing sarcoma ... Ewing sarcoma can occur anytime during childhood and young adulthood. But it usually develops during puberty, when bones ...

  20. Soft Tissue Sarcoma Across the Age Spectrum: A Population-Based Study from the Surveillance Epidemiology and End Results Database

    PubMed Central

    Ferrari, Andrea; Sultan, Iyad; Huang, Tseng Tien; -Galindo, Carlos Rodriguez; Shehadeh, Ahmad; Meazza, Cristina; Ness, Kirsten K.; Casanova, Michela; Spunt, Sheri L.

    2014-01-01

    Background Soft tissue sarcomas (STS) are a heterogeneous group of mesenchymal malignancies that occur throughout the lifespan. The impact of age on disease features and outcome is unclear. Methods We analyzed the clinical features and outcome of all STS cases registered between 1973 and 2006 in the SEER database. Results There were 48,012 cases that met the selection criteria. Individuals less than 20 years of age represented 5.6%, with rhabdomyosarcoma being the most common subtype. In adults, the most common types were Kaposi sarcoma, fibrohistiocytic tumors, and leiomyosarcoma. Rhabdomyosarcoma was the only entity with a median age < 20 years. Male predominance (male:female of 1.5:1) was noticed for almost all types of STS, except for alveolar soft part sarcoma and leiomyosarcoma. Tumor stage was similar across different age groups. Younger patients (<50 years) had significantly better survival than older patients (88.8%±0.2% vs. 40%±0.3%, P<0.001), but for most histologies the survival decline with advancing age was gradual and did not occur abruptly at the onset of adulthood. The decline in survival with advancing age was particularly significant for rhabdomyosarcoma. Conclusion With few exceptions, the clinical features of STS are similar in children and adults. However, individuals over 50 years of age have an inferior survival. PMID:21793180

  1. 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.

  2. Kaposi sarcoma

    MedlinePlus

    Kaposi's sarcoma; HIV - Kaposi; AIDS - Kaposi ... Before the HIV/AIDS epidemic, Kaposi sarcoma was seen mainly in older Italian and Jewish men, and rarely, in older women. Among this group, the tumors developed slowly. In ...

  3. Ossifying fibromyxoid tumor of soft parts. Cytogenetic findings.

    PubMed

    Nishio, Jun; Iwasaki, Hiroshi; Ohjimi, Yuko; Ishiguro, Masako; Isayama, Teruto; Naito, Masatoshi; Okabayashi, Hiroshi; Kaneko, Yasuhiko; Kikuchi, Masahiro

    2002-03-01

    Ossifying fibromyxoid tumor (OFMT) of soft parts is a recently described, rare but morphologically distinctive soft tissue tumor. The histogenesis of this lesion remains uncertain, although several immunohistochemical and ultrastructural features suggest that it is an unusual neural tumor, possibly of Schwann cell origin. We report here a case of a malignant variant of OFMT that occurred in the foot of a 52-year-old man. The karyotype of a pulmonary metastasis exhibited the following complex numeric and structural aberrations:72 approximately 74,XXY,-5,+6,+del(8)(p21),del(9)(p22),+10,der(11)t(3;11)(p21;p15),del(12) (q13),der(13)t(5;13)(q13;q34),+18,+19,+20,-22 [cp10]. A kidney metastasis exhibited the following karyotypic abnormalities: 46,XY,add(3)(p11),+der(3)t(3;?;11)(3qter-->3p11::?::11q13-->11qter), -5,del(8)(p21),add(9)(q22),del(9)(p22),der(11)t(3;11)(p21;p15),del(12)(q13),+der(13)t(5;13) (q13;q34),-22. To our knowledge, this is the first reported case of OFMT in which clonal chromosomal aberrations have been shown.

  4. Prognostic value of PAX-FKHR fusion status in alveolar rhabdomyosarcoma: a report from the cooperative soft tissue sarcoma study group (CWS).

    PubMed

    Stegmaier, Sabine; Poremba, Christopher; Schaefer, Karl-Ludwig; Leuschner, Ivo; Kazanowska, Bernarda; Békássy, Albert N; Bielack, Stefan S; Klingebiel, Thomas; Koscielniak, Ewa

    2011-09-01

    Alveolar Rhabdomyosarcomas (RMA) are characterized by chromosomal translocations, fusing the PAX3 or PAX7 gene with FKHR in about 85%. Previous studies have suggested that the fusion type is associated with prognosis. In order to investigate the predictive value of the PAX-FKHR fusion status on disease outcome of patients with RMA treated in the CWS trials we performed a retrospective analysis. Between 1986 and 2004, out of 446 patients with RMA treated in four consecutive CWS trials, tumor samples from 126 patients were available for RT-PCR analysis. Survival depending on fusion status in context with known clinical risk-factors was analyzed. Out of 126 samples, 121 had adequate quality for PAX-FKHR fusion status analysis. PAX-FKHR fusions were detected in 101 samples: 60% PAX3-FKHR and 24% PAX7-FKHR fusions, 17% were fusion-negative. There was no significant difference in survival between patients with PAX3-FKHR versus PAX7-FKHR positive tumors. The fusion transcript negative cohort showed a more favorable outcome than the fusion transcript positive cohort among patients with metastatic disease. From the established clinical risk-factors none was associated with a significantly higher risk of failure or death in a multivariate analysis. PAX-FKHR fusion type was not a significant predictor for survival in our analysis. More extensive molecular analyses are needed to identify features with prognostic relevance and useful therapeutic impact. Copyright © 2011 Wiley-Liss, Inc.

  5. Alveolar rhabdomyosarcoma of maxilla

    PubMed Central

    Ananthaneni, Anuradha; Kuberappa, Puneeth Horatti; Srinivas, G Vijay; Kiresur, Mohammad Asif

    2016-01-01

    Rhabdomyosarcoma (RMS), a malignant neoplasm of skeletal muscle origin, is the most common soft tissue sarcoma seen in childhood and adolescence. The most frequent site is the head and neck accounting for 40% of all cases and other involved sites are genitourinary tract, retroperitoneum, and to a lesser extent, the extremities. RMS is relatively uncommon in the oral cavity and the involvement of the jaws is extremely rare. Here, we report a case of 50-year-old female with oral RMS involving maxillary alveolar region with clinical, radiological, histopathological and immunohistochemical findings. PMID:27194887

  6. Adult soft tissue sarcoma

    MedlinePlus

    STS; Leiomyosarcoma; Hemangiosarcoma; Kaposi's sarcoma; Lymphangiosarcoma; Synovial sarcoma; Neurofibrosarcoma; Liposarcoma; Fibrosarcoma; Malignant fibrous histiocytoma; Dermatofibrosarcoma; Angiosarcoma

  7. Can Kaposi Sarcoma Be Prevented?

    MedlinePlus

    ... Kaposi Sarcoma Causes, Risk Factors, and Prevention Can Kaposi Sarcoma Be Prevented? Kaposi sarcoma (KS) is caused ... Sarcoma? Can Kaposi Sarcoma Be Prevented? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and ...

  8. 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

  9. Epidemic Kaposi Sarcoma

    MedlinePlus

    ... Kaposi sarcoma is found in patients who have acquired immunodeficiency syndrome (AIDS). Epidemic Kaposi sarcoma occurs in patients who have ... combines treatment for Kaposi sarcoma with treatment for AIDS. For the treatment of epidemic Kaposi sarcoma, combined ...

  10. What Is Kaposi Sarcoma?

    MedlinePlus

    ... Treatment? Kaposi Sarcoma About Kaposi Sarcoma What Is Kaposi Sarcoma? Cancer starts when cells in the body ... the lungs may cause trouble breathing. Types of Kaposi sarcoma The different types of KS are defined ...

  11. Uterine sarcoma

    MedlinePlus

    ... Churchill Livingstone; 2014:chap 88. Crum CP, Laury AR, Hirsch MS, Quick CM, Peters WA. Undifferentiated uterine sarcoma. In: Crum CP, Quick CM, Laury AR, Peters WA, Hirsch MS, eds. Gynecologic and Obstetric ...

  12. Surgery for Soft Tissue Sarcomas

    MedlinePlus

    ... Tissue Sarcomas Chemotherapy for Soft Tissue Sarcomas Targeted Therapy for Soft Tissue Sarcoma Treatment of Soft Tissue Sarcomas, by Stage ... Cancer Information Cancer Prevention & Detection Cancer Basics ...

  13. [Alveolar hemorrhage].

    PubMed

    Parrot, A; Fartoukh, M; Cadranel, J

    2015-04-01

    Alveolar hemorrhage occurs relatively rarely and is a therapeutic emergency because it can quickly lead to acute respiratory failure, which can be fatal. Hemoptysis associated with anemia and pulmonary infiltrates suggest the diagnosis of alveolar hemorrhage, but may be absent in one third of cases including patients in respiratory distress. The diagnosis of alveolar hemorrhage is based on the findings of a bronchoalveolar lavage. The causes are numerous. It is important to identify alveolar hemorrhage due to sepsis, then separate an autoimmune cause (vasculitis associated with antineutrophil cytoplasmic antibody, connective tissue disease and Goodpasture's syndrome) with the search for autoantibodies and biopsies from readily accessible organs, from a non-immune cause, performing echocardiography. Lung biopsy should be necessary only in exceptional cases. If the hemorrhage has an immune cause, treatment with steroids and cyclophosphamide may be started. The indications for treatment with rituximab are beginning to be established (forms that are not severe and refractory forms). The benefit of plasma exchange is unquestionable in Goodpasture's syndrome. In patients with an immune disease that can lead to an alveolar hemorrhage, removing any source of infection is the first priority.

  14. Kaposi sarcoma.

    PubMed

    Radu, Oana; Pantanowitz, Liron

    2013-02-01

    Kaposi sarcoma (KS) is a low-grade vascular tumor associated with Kaposi sarcoma herpesvirus/human herpesvirus 8 (KSHV/HHV8) infection. Kaposi sarcoma lesions predominantly present at mucocutaneous sites, but may involve all organs and anatomic locations. Recognized epidemiologic-clinical forms of KS include classic, African (endemic), AIDS-associated (epidemic), and iatrogenic KS. New clinical manifestations have been described, such as antiretroviral therapy-related KS regression or flares. Kaposi sarcoma lesions evolve from early (patch stage) macules into plaques (plaque stage) that grow into larger nodules (tumor stage). Newer histologic variants include anaplastic, hyperkeratotic, lymphangioma-like, bullous, telangiectatic, ecchymotic, keloidal, pyogenic granuloma-like, micronodular, intravascular, glomeruloid and pigmented KS, as well as KS with sarcoidlike granulomas and KS with myoid nodules. Latency-associated nuclear antigen (HHV8) is the most specific immunohistochemical marker available to help distinguish KS from its mimics. Since KS remains one of the most common AIDS-defining malignancies, it is important that pathologists be able to recognize KS and its contemporary manifestations.

  15. Orbital Sarcoma with Metastases at Diagnosis: A Report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group

    PubMed Central

    Huh, Winston W.; Anderson, James R.; Rodeberg, David; Teot, Lisa; Yock, Torunn; Raney, R. Beverly

    2011-01-01

    We reviewed clinicopathologic features and treatment outcomes in 7 patients diagnosed with Stage 4/Group IV orbital sarcoma and treated on IRSG protocols I-III. Three patients had embryonal rhabdomyosarcoma (RMS), and 2 patients each had alveolar RMS or unclassified sarcoma. Median age at diagnosis was 1.8 years (range 0.2 – 6.9 years). All patients had bone marrow involvement, including 6 with normal CBC at diagnosis. Cerebrospinal fluid (CSF) was normal in 6 patients. Three patients survived > 5 years, including one with local recurrence. Conclusion Further study is needed to determine necessity of bone marrow and CSF examination in orbital sarcoma patients. PMID:20162686

  16. Intra articular synovial sarcoma.

    PubMed

    Sistla, Radha; Tameem, Afroz; Vidyasagar, J V S

    2010-01-01

    Synovial sarcoma is a soft tissue neoplasm with a characteristic biphasic pattern. Incidence in soft tissues is 5-10%. Intra articularly synovial sarcoma is extremely rare. Fewer than 5% of all synovial sarcomas arise within the joint space. We report a case of intra articular synovial sarcoma in a young male who presented as internal derangement of the knee.

  17. General Information about Kaposi Sarcoma

    MedlinePlus

    ... Kaposi sarcoma is found in patients who have acquired immunodeficiency syndrome (AIDS). Epidemic Kaposi sarcoma occurs in patients who have ... combines treatment for Kaposi sarcoma with treatment for AIDS. For the treatment of epidemic Kaposi sarcoma, combined ...

  18. Stages of Adult Soft Tissue Sarcoma

    MedlinePlus

    ... soft tissue sarcomas: Childhood Soft Tissue Sarcoma Treatment Ewing Sarcoma Family of Tumors Treatment Gastrointestinal Stromal Tumors Treatment ... Sarcoma Home Page Childhood Soft Tissue Sarcoma Treatment Ewing Sarcoma Family of Tumors Treatment Gastrointestinal Stromal Tumors Treatment ...

  19. Treatment Options for Adult Soft Tissue Sarcoma

    MedlinePlus

    ... soft tissue sarcomas: Childhood Soft Tissue Sarcoma Treatment Ewing Sarcoma Family of Tumors Treatment Gastrointestinal Stromal Tumors Treatment ... Sarcoma Home Page Childhood Soft Tissue Sarcoma Treatment Ewing Sarcoma Family of Tumors Treatment Gastrointestinal Stromal Tumors Treatment ...

  20. Treatment Option Overview (Adult Soft Tissue Sarcoma)

    MedlinePlus

    ... soft tissue sarcomas: Childhood Soft Tissue Sarcoma Treatment Ewing Sarcoma Family of Tumors Treatment Gastrointestinal Stromal Tumors Treatment ... Sarcoma Home Page Childhood Soft Tissue Sarcoma Treatment Ewing Sarcoma Family of Tumors Treatment Gastrointestinal Stromal Tumors Treatment ...

  1. Do We Know What Causes Kaposi Sarcoma?

    MedlinePlus

    ... Factors, and Prevention Do We Know What Causes Kaposi Sarcoma? Kaposi sarcoma (KS) is caused by infection ... Sarcoma? Can Kaposi Sarcoma Be Prevented? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and ...

  2. Soft Tissue Sarcoma

    MedlinePlus

    ... muscles, tendons, fat, and blood vessels. Soft tissue sarcoma is a cancer of these soft tissues. There ... have certain genetic diseases. Doctors diagnose soft tissue sarcomas with a biopsy. Treatments include surgery to remove ...

  3. Sarcoma Foundation of America

    MedlinePlus

    ... Make a Donation Matching Gifts Sarcoma Dedication Page Stocks and Securities Workplace Giving FAQ's Dedications Join Donate ... Make a Donation Matching Gifts Sarcoma Dedication Page Stocks and Securities Workplace Giving

  4. Soft tissue sarcomas in adolescents and young adults: a comparison with their paediatric and adult counterparts.

    PubMed

    van der Graaf, Winette T A; Orbach, Daniel; Judson, Ian R; Ferrari, Andrea

    2017-03-01

    Survival outcomes for adolescent and young adult patients with soft tissue sarcomas lag behind those of children diagnosed with histologically similar tumours. To help understand these differences in outcomes, we discuss the following issues with regard to the management of these patients with soft tissue sarcomas: delays in diagnosis, trial availability and participation, aspects of the organisation of care (with an emphasis on age-specific needs), national centralisation of sarcoma care, international consortia, and factors related to tumour biology. Improved understanding of the causes of the survival gap between adolescents and young adults with sarcomas will help drive new initiatives to improve final health outcomes in these populations. In this Review, we specifically focus on embryonal and alveolar rhabdomyosarcoma, synovial sarcoma, and adult soft tissue sarcomas diagnosed in adolescents and young adults, and discuss the age-specific needs of these patients. Copyright © 2017 Elsevier Ltd. All rights reserved.

  5. 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

  6. Can Soft Tissue Sarcomas Be Found Early?

    MedlinePlus

    ... Tissue Sarcoma Early Detection, Diagnosis, and Staging Can Soft Tissue Sarcomas Be Found Early? People who have ... Your Doctor About Soft Tissue Sarcomas? More In Soft Tissue Sarcoma About Soft Tissue Sarcoma Causes, Risk ...

  7. A technique for calculating particle systems containing rigid and soft parts

    NASA Astrophysics Data System (ADS)

    Nouri, Nima; Ziaei-Rad, Saeed

    2015-08-01

    In this paper, a method was proposed that can simulate the systems containing both rigid and soft parts with rigid body constraints. The idea was to consider the characteristics of rigid parts in their center of mass with three rotational degrees of freedom. In order to compute the systems containing both flexible and rigid parts, standard techniques in molecular dynamics were utilized for flexible parts. However, special procedures were proposed and formulated for rigid parts. Some details on the implementation of the proposed algorithm on GPU were also presented. Next, two case studies were solved. In the first example, a ball mill with the rigid particle of different shapes was considered and the performance of the proposed algorithm was checked and compared with the results obtained from others. In the second example, different self-assembly phases of a mixed rigid and non-rigid polymer molecule with Lennard-Jones pairwise interaction were studied. It was shown that the obtained self-assembly phases were identical to those reported by other researchers.

  8. 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.

  9. Cyclin D1 and Ewing's sarcoma/PNET: A microarray analysis.

    PubMed

    Fagone, Paolo; Nicoletti, Ferdinando; Salvatorelli, Lucia; Musumeci, Giuseppe; Magro, Gaetano

    2015-10-01

    Recent immunohistochemical analyses have showed that cyclin D1 is expressed in soft tissue Ewing's sarcoma/peripheral neuroectodermal tumor (PNET) of childhood and adolescents, while it is undetectable in both embryonal and alveolar rhabdomyosarcoma. In the present paper, microarray analysis provided evidence of a significant upregulation of cyclin D1 in Ewing's sarcoma as compared to normal tissues. In addition, we confirmed our previous findings of a significant over-expression of cyclin D1 in Ewing sarcoma as compared to rhabdomyosarcoma. Bioinformatic analysis also allowed to identify some other genes, strongly correlated to cyclin D1, which, although not previously studied in pediatric tumors, could represent novel markers for the diagnosis and prognosis of Ewing's sarcoma/PNET. The data herein provided support not only the use of cyclin D1 as a diagnostic marker of Ewing sarcoma/PNET but also the possibility of using drugs targeting cyclin D1 as potential therapeutic strategies.

  10. [Pulmonary artery intimal sarcoma].

    PubMed

    Bourry, N; Chabrot, P; Jeannin, G; Filaire, M; Charpy, C; Bay, J O; Kemeny, J L; Caillaud, D; Escande, G; Boyer, L

    2008-02-01

    Pulmonary artery sarcoma is a rare tumor. We present a case of intimal sarcoma arising from right pulmonary artery and left lower pulmonary vein observed in a 44-year-old man with a non-productive cough. Computed tomographic scans and magnetic resonance imaging showing filling defect enhancement contributed early, suggesting the diagnosis of primary vascular tumor, hypothesis confirmed by pathologist findings.

  11. The Epidemiology of Sarcoma

    PubMed Central

    2012-01-01

    Sarcomas account for over 20% of all pediatric solid malignant cancers and less than 1% of all adult solid malignant cancers. The vast majority of diagnosed sarcomas will be soft tissue sarcomas, while malignant bone tumors make up just over 10% of sarcomas. The risks for sarcoma are not well-understood. We evaluated the existing literature on the epidemiology and etiology of sarcoma. Risks for sarcoma development can be divided into environmental exposures, genetic susceptibility, and an interaction between the two. HIV-positive individuals are at an increased risk for Kaposi’s sarcoma, even though HHV8 is the causative virus. Radiation exposure from radiotherapy has been strongly associated with secondary sarcoma development in certain cancer patients. In fact, the risk of malignant bone tumors increases as the cumulative dose of radiation to the bone increases (p for trend <0.001). A recent meta-analysis reported that children with a history of hernias have a greater risk of developing Ewing’s sarcoma (adjusted OR 3.2, 95% CI 1.9, 5.7). Bone development during pubertal growth spurts has been associated with osteosarcoma development. Occupational factors such as job type, industry, and exposures to chemicals such as herbicides and chlorophenols have been suggested as risk factors for sarcomas. A case-control study found a significant increase in soft tissue sarcoma risk among gardeners (adjusted OR 4.1, 95% CI 1.00, 14.00), but not among those strictly involved in farming. A European-based study reported an increased risk in bone tumors among blacksmiths, toolmakers, or machine-tool operators (adjusted OR 2.14, 95% CI 1.08, 4.26). Maternal and paternal characteristics such as occupation, age, smoking status, and health conditions experienced during pregnancy also have been suggested as sarcoma risk factors and would be important to assess in future studies. The limited studies we identified demonstrate significant relationships with sarcoma risk, but many of

  12. A Silurian myodocope with preserved soft-parts: cautioning the interpretation of the shell-based ostracod record

    PubMed Central

    Siveter, David J.; Briggs, Derek E. G.; Siveter, Derek J.; Sutton, Mark D.; Joomun, Sarah C.

    2013-01-01

    Ostracod crustaceans are the most abundant fossil arthropods. The Silurian Pauline avibella gen. et sp. nov., from the Herefordshire Lagerstätte, UK, is an extremely rare Palaeozoic example with soft-part preservation. Based on its soft-part morphology, especially the exceptionally preserved limbs and presence of lateral eyes, it is assigned to the myodocopid myodocopes. The ostracod is very large, with an epipod on the fifth limb pair, as well as gills implying the presence of a heart and an integrated respiratory–circulatory system as in living cylindroleberidid myodocopids. Features of its shell morphology, however, recall halocyprid myodocopes and palaeocopes, encouraging caution in classifying ostracods based on the carapace alone and querying the interpretation of their shell-based fossil record, especially for the Palaeozoic, where some 500 genera are presently assigned to the Palaeocopida. PMID:23235709

  13. Pulmonary artery sarcoma.

    PubMed

    Blackmon, Shanda H; Reardon, Michael J

    2010-01-01

    Primary heart tumors are rare, and malignant primary heart tumors are only a small subset of these. Most primary malignant tumors are sarcomas arising from the cells of the structural elements of the heart such as blood vessels, muscle, connective tissue, fat and even bone. Unlike most malignancies, where cell type often dictates treatment choices and prognosis and is used for classification, the histology in primary cardiac sarcoma plays little role in determining therapeutic options or prognosis. We have found that anatomic location within the heart is the major determining factor in clinical presentation, treatment options and prognosis in cardiac sarcoma. Therefore, we accordingly classify primary cardiac sarcomas into right heart sarcomas, left heart sarcomas and pulmonary artery (PA) sarcomas. Since the first autopsy report of a primary PA sarcoma in 1923, there have been fewer than 250 cases reported in the English literature. Most of these reports have been single autopsy or case reports, and patient prognosis has generally been dismal. Since few institutions and even fewer individual physicians acquire much exposure to this disease, the diagnostic and treatment approaches have remained unresolved. Our cardiac sarcoma group working at the Methodist DeBakey Heart & Vascular Center and the MD Anderson Cancer Center has undertaken a systematic study of this disease, and operated on 9 patients using a radical resection with curative intent and multimodality approach. Based on this work, we have suggested a diagnostic strategy, treatment approach and staging system for primary PA sarcoma. A substantial improvement in patient survival over historical controls has also been demonstrated and will be discussed in this review.

  14. Primary CIC-DUX4 round cell sarcoma of the kidney: A treatment-refractory tumor with poor outcome.

    PubMed

    Bergerat, Sébastien; Barthelemy, Philippe; Mouracade, Pascal; Lang, Hervé; Saussine, Christian; Lindner, Véronique; Jacqmin, Didier

    2017-02-01

    The CIC-DUX4 sarcoma is a subset of the undifferentiated small round cell sarcoma family, presently recognized as a new clinicopathological entity. It is a rare and highly aggressive tumor usually arising in the soft parts of the limbs and the trunk. Only a very few cases of primitive visceral CIC-DUX4 have been hitherto described. We report the case of a 29 year-old male patient with a primary CIC-DUX4 sarcoma of the kidney with lung metastasis. The outcome of the disease was rapidly unfavorable. Despite radical nephrectomy, the patient experienced an early local retroperitoneal recurrence associated with lung and liver metastases. The tumor did not respond to four successive lines of chemotherapy nor to palliative radiotherapy. Due to partial morphologic and immunohistochemical overlap with Ewing sarcoma, CIC-DUX4 positive tumors have generally been considered as Ewing-like sarcomas and managed similarly. However, this tumor shows a high propensity to metastasize and is much less sensitive to chemotherapy than Ewing sarcomas. The management of this type of very aggressive sarcoma needs to be defined by comprehensive biological and clinical studies. Copyright © 2016 Elsevier GmbH. All rights reserved.

  15. Pulmonary Artery Sarcoma

    PubMed Central

    Shomaf, Maha; Obeidat, Nathir; Najjar, Saleh

    2014-01-01

    Pulmonary artery sarcomas (PAS) are extremely rare sarcomas of uncertain histogenesis that often mimic pulmonary thromboemboli. This is a report of a 60-year-old female patient who presented with recurrent chest pain and cough. The patient was first diagnosed with pulmonary embolism but she did not improve on anticoagulant therapy. Follow-up imaging studies revealed a mass in the left hilar region extending into the pulmonary trunk and branches of the left pulmonary artery. The tru-cut biopsy revealed an undifferentiated sarcoma. The patient died 10 months after her initial presentation. PMID:26425600

  16. 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. Copyright © 2016 Elsevier Inc. All rights reserved.

  17. Survival by Stage of Soft Tissue Sarcoma

    MedlinePlus

    ... Detection, Diagnosis, and Staging Survival by Stage of Soft Tissue Sarcoma Survival rates are often used by ... Your Doctor About Soft Tissue Sarcomas? More In Soft Tissue Sarcoma About Soft Tissue Sarcoma Causes, Risk ...

  18. Practical Issues for Retroperitoneal Sarcoma.

    PubMed

    Pham, Vicky; Henderson-Jackson, Evita; Doepker, Matthew P; Caracciolo, Jamie T; Gonzalez, Ricardo J; Druta, Mihaela; Ding, Yi; Bui, Marilyn M

    2016-07-01

    Retroperitoneal sarcoma is rare. Using initial specimens on biopsy, a definitive diagnosis of histological subtypes is ideal but not always achievable. A retrospective institutional review was performed for all cases of adult retroperitoneal sarcoma from 1996 to 2015. A review of the literature was also performed related to the distribution of retroperitoneal sarcoma subtypes. A meta-analysis was performed. Liposarcoma is the most common subtype (45%), followed by leiomyosarcoma (21%), not otherwise specified (8%), and undifferentiated pleomorphic sarcoma (6%) by literature review. Data from Moffitt Cancer Center demonstrate the same general distribution for subtypes of retroperitoneal sarcoma. A pathology-based algorithm for the diagnosis of retroperitoneal sarcoma is illustrated, and common pitfalls in the pathology of retroperitoneal sarcoma are discussed. An informative diagnosis of retroperitoneal sarcoma via specimens on biopsy is achievable and meaningful to guide effective therapy. A practical and multidisciplinary algorithm focused on the histopathology is helpful for the management of retroperitoneal sarcoma.

  19. CREATE: Cross-tumoral Phase 2 With Crizotinib

    ClinicalTrials.gov

    2017-08-04

    Locally Advanced and/or Metastatic Anaplastic Large Cell Lymphoma; Locally Advanced and/or Metastatic Inflammatory Myofibroblastic Tumor; Locally Advanced and/or Metastatic Papillary Renal Cell Carcinoma Type 1; Locally Advanced and/or Metastatic Alveolar Soft Part Sarcoma; Locally Advanced and/or Metastatic Clear Cell Sarcoma; Locally Advanced and/or Metastatic Alveolar Rhabdomyosarcoma

  20. Immune Therapy for Sarcomas.

    PubMed

    Anderson, Peter M

    2017-01-01

    Absolute lymphocyte count (ALC) recovery rapidly occurring at 14 days after start of chemotherapy for osteosarcoma and Ewing sarcoma is a good prognostic factor. Conversely, lymphopenia is associated with significantly decreased sarcoma survival. Clearly, the immune system can contribute towards better survival from sarcoma. This chapter will describe treatment and host factors that influence immune function and how effective local control and systemic interventions of sarcoma therapy can cause inflammation and/or immune suppression but are currently the standard of care. Preclinical and clinical efforts to enhance immune function against sarcoma will be reviewed. Interventions to enhance immune function against sarcoma have included regional therapy (surgery, cryoablation, radiofrequency ablation, electroporation, and radiotherapy), cytokines, macrophage activators (mifamurtide), vaccines, natural killer (NK) cells, T cell receptor (TCR) and chimeric antigen receptor (CAR) T cells, and efforts to decrease inflammation. The latter is particularly important because of new knowledge about factors influencing expression of checkpoint inhibitory molecules, PD1 and CTLA-4, in the tumor microenvironment. Since these molecules can now be blocked using anti-PD1 and anti-CTLA-4 antibodies, how to translate this knowledge into more effective immune therapies in the future as well as how to augment effectiveness of current interventions (e.g., radiotherapy) is a challenge. Barriers to implementing this knowledge include cost of agents that release immune checkpoint blockade and coordination of cost-effective outpatient sarcoma treatment. Information on how to research clinical trial eligibility criteria and how to access current immune therapy trials against sarcoma are shared, too.

  1. Bronchopulmonary Kaposi's sarcoma.

    PubMed

    Bashar, Nada; Innes, Nicholas; Orrell, Julian

    2015-01-01

    Kaposi's sarcoma (KS) is a highly vascular tumour, which was first described by the Hungarian dermatologist Moritz Kaposi Kohn before the discovery of the human immunodeficiency virus (HIV). Historically, KS has been linked to immunosuppression or to elderly male patients, especially in relation to diffuse cutaneous KS. We describe a case of Bronchopulmonary Kaposi's sarcoma in a patient with AIDS who was successfully treated with HAART and Liposomal Doxorubicin chemotherapy.

  2. Bronchopulmonary Kaposi's sarcoma

    PubMed Central

    Bashar, Nada; Innes, Nicholas; Orrell, Julian

    2015-01-01

    Kaposi's sarcoma (KS) is a highly vascular tumour, which was first described by the Hungarian dermatologist Moritz Kaposi Kohn before the discovery of the human immunodeficiency virus (HIV). Historically, KS has been linked to immunosuppression or to elderly male patients, especially in relation to diffuse cutaneous KS. We describe a case of Bronchopulmonary Kaposi's sarcoma in a patient with AIDS who was successfully treated with HAART and Liposomal Doxorubicin chemotherapy. PMID:26236600

  3. HUMAN SARCOMAS IN CULTURE

    PubMed Central

    Giraldo, Gaetano; Beth, Elke; Hirshaut, Yashar; Aoki, Tadao; Old, Lloyd J.; Boyse, Edward A.; Chopra, Harish C.

    1971-01-01

    In a study of human sarcomas maintained in culture for periods up to two years, the following observations were made. The most prominent cell type in serially cultured osteosarcomas was fibroblastic in appearance. After 16–20 wk in culture some lines spontaneously developed foci of altered cells resembling the foci produced in monolayer cultures by oncogenic viruses. The presence of these foci in the sarcoma cultures was transient, and usually they did not reappear; but in one instance they recurred with a characteristic periodicity of several weeks. From one of the sarcoma lines, in which foci appeared after 5 months in culture, two subcultures were established from stored frozen cells and these both exhibited foci after approximately the same lapse of time. The same phenomenon has been seen with another line, suggesting that the time of appearance of foci is characteristic for particular sarcomas. Foci of similar type could sometimes be induced in monolayer cultures of human fibroblasts by filtered medium from cultured sarcomas; this bore no relation to the presence or absence of foci in the sarcoma cultures at the time the filtrate was prepared. Electron microscopy of the spontaneous and induced foci, and of the sarcoma cultures, revealed no demonstrable virus. 12 out of 15 sarcoma cultures contained an antigen (S) demonstrable by indirect immunofluorescence with human sera. It was not present in any of the original sarcoma specimens, nor in any culture lines other than sarcomas. At least 3–4 wk in culture appear to be required for its demonstration. The antigen was cytoplasmic, occurred in only a small proportion of the cells, and was unpredictably variable in its expression, even in the same culture line. It could be induced in monolayer cultures of human fibroblasts by filtrates of medium from sarcoma cultures. As with the foci, the induction of S antigen in indicator cultures was not dependent upon the expression of antigen in the sarcoma line from which

  4. How Is Uterine Sarcoma Staged?

    MedlinePlus

    ... The American Cancer Society medical and editorial content team Our team is made up of doctors and master’s-prepared ... Sarcoma About Uterine Sarcoma Causes, Risk Factors, and Prevention Early Detection, Diagnosis, and Staging Treatment After Treatment ...

  5. Orbital granulocytic sarcoma

    PubMed Central

    Stockl, F.; Dolmetsch, A.; Saornil, M; Font, R.; Burnier, M.

    1997-01-01

    AIM—Orbital granulocytic sarcoma is a localised tumour composed of cells of myeloid origin. Histological diagnosis can be difficult in patients with poorly differentiated orbital tumours and no evidence of systemic leukaemia. The naphthol AS-D chloracetate esterase (Leder stain) and immunohistochemical stains for lysozyme and MAC387 were used to determine the staining characteristics of these tumours. A case series of seven patients with orbital granulocytic sarcoma is presented.
METHODS—Seven patients with orbital granulocytic sarcoma were studied. Haematoxylin and eosin, Leder, and lysozyme stained sections were available in seven cases. Unstained formalin fixed paraffin embedded sections of seven cases were available for immunohistochemical evaluation using the avidin-biotin-complex technique for MAC387.
RESULTS—The mean age of presentation of the orbital tumour was 8.8 years. Four patients presented with an orbital tumour before any systemic manifestations of leukaemia. In two cases the diagnosis of the orbital tumour and systemic leukaemia was made simultaneously. There was one case of established systemic myeloid leukaemia in remission with the subsequent development of orbital granulocytic sarcoma. Six of seven cases (86%) were positive for the Leder stain. Five of seven cases (71%) showed positive immunoreactivity with lysozyme. The immunohistochemical stain for MAC387 was positive in all seven cases (100%) including one case that was negative for both lysozyme and Leder stains.
CONCLUSIONS—Orbital granulocytic sarcoma is a tumour that affects children and can present with rapidly progressive proptosis. This tumour may develop before, during, or after the occurrence of systemic leukaemia. The combination of Leder and lysozyme stains is useful in the diagnosis of orbital granulocytic sarcoma. MAC387 may be a more reliable marker for orbital granulocytic sarcoma.

 PMID:9497470

  6. Soft-part anatomy of the Early Cambrian bivalved arthropods Kunyangella and Kunmingella: significance for the phylogenetic relationships of Bradoriida

    PubMed Central

    Hou, Xianguang; Williams, Mark; Siveter, David J.; Siveter, Derek J.; Aldridge, Richard J.; Sansom, Robert S.

    2010-01-01

    Bradoriids are small bivalved marine arthropods that are widespread in rocks of Cambrian to Early Ordovician age. They comprise seven families and about 70 genera based on shield (‘carapace’) morphology. New bradoriid specimens with preserved soft-part anatomy of Kunmingella douvillei (Kunmingellidae) are reported from the Early Cambrian Chengjiang Lagerstätte of China together with, for the first time to our knowledge, a second bradoriid species with preserved soft parts, Kunyangella cheni (Comptalutidae). Kunmingella douvillei has a 10-segmented limb-bearing body with uniramous ninth and tenth appendages and a series of homogeneous, apparently (proximal parts not preserved) unspecialized post-antennal biramous limbs with setose leaf-shaped exopods. Each endopod consists of five podomeres. A presumed penultimate instar of Ky. cheni preserves remnants of three head and two trunk appendages, and the adult is reconstructed as having four head appendages. This material allows testing of the affinity of the Bradoriida. Kunmingella is identified as a stem crustacean in character-based analyses, through both morphological comparisons and cladistic reconstructions. Global parsimony analysis recovers a monophyletic Bradoriida as the sister group to crown crustaceans. PMID:20181565

  7. INTRAOCULAR SYNOVIAL SARCOMA.

    PubMed

    Richards, Nikisha Q; Kofler, Julia K; Chu, Charleen T; Stefko, S Tonya

    2017-01-01

    To describe the first reported case of intraocular synovial sarcoma. A 29-year-old man was enucleated for a blind, painful eye. Pathologic examination revealed an unexpected intraocular spindle cell tumor. Immunohistochemical characterization revealed diffuse reactivity of the tumor cells for vimentin and focal positivity for epithelial markers pankeratin and epithelial membrane antigen. Melanoma markers were negative. Fluorescent in situ hybridization studies identified a t(X;18) (p11.2;q11.2) translocation, establishing a final diagnosis of synovial sarcoma. There was no evidence of extraocular extension as the resected margin of the optic nerve was free of tumor. Further imaging studies revealed no extraocular primary site or metastasis. The incidental discovery of an intraocular malignancy in this case underscores the importance of routine histopathologic analysis of all enucleated globes. To the authors' knowledge, this is the first reported case of an intraocular synovial sarcoma, either as metastasis or as primary site.

  8. [Sarcoma of the breast].

    PubMed

    Haberthür, F; Almendral, A C; Feichter, G; Torhorst, J K

    1992-05-01

    Sarcoma of the breast represents only 0.2-1% of all mammary malignancies. This study reports 5 such cases, including 2 osteosarcomas, 1 fibro-, 1 lipo-, and 1 malignant fibrous sarcoma. The treatment used was mastectomy in 3 cases with excision of axillary lymph nodes. The remaining 2 patients were treated by simple mastectomy whereby 1 of these received a immediate reconstruction with a prosthesis. 1 patient demonstrated local recurrence and died. The remaining 4 patients did not develop neither metastases nor local recurrence and are still alive after an observing period between 12 months up to 17 years. Today, first-line treatment is wide local excision or simple mastectomy. Excision of the axillary lymphatics, adjuvant radiotherapy, and chemotherapy have been disappointing in the treatment of breast sarcoma.

  9. [Moritz Kaposi and his sarcoma].

    PubMed

    van Kessel, Anne; Quint, Koen D

    2011-01-01

    Nowadays, Kaposi sarcoma is a multidisciplinary condition, not only observed by dermatologists. Since the HIV epidemic in the 80s and 90s of the last century, more insight into the aetiology of Kaposi sarcoma has been acquired. However, this sarcoma had already been described in 1872 by a Hungarian dermatologist named Moritz Kaposi (1832-1902). Kaposi described the entity as 'idiopathic multiple pigmented sarcoma of the skin'. This entity was an extraordinary diagnosis at that time, mostly observed in Jewish or Mediterranean men. In 1912, 10 years after the death of Moritz Kaposi, the entity name was changed to Kaposi sarcoma.

  10. Systemic therapy for cardiac sarcomas.

    PubMed

    Ravi, Vinod; Benjamin, Robert S

    2010-01-01

    Cardiac sarcomas create 2 risks: local problems and metastatic disease. Most frequently, the histologies are angiosarcoma and high-grade pleomorphic unclassified sarcoma (formerly called MFH or malignant fibrous histiocytoma). There is also a clinical-pathological entity without distinctive histological features of tumors that originate in the pulmonary artery and are referred to as pulmonary artery sarcomas or intimal sarcomas of the pulmonary artery. Conventional wisdom indicates that soft-tissue sarcomas are poorly responsive to chemotherapy. Luckily, that is not the case. Attempts to concentrate on the local problem only with therapies up to and including cardiac transplantation have been unsuccessful due to the high rate of fatal metastatic disease.

  11. Microenvironmental Targets in Sarcoma

    PubMed Central

    Ehnman, Monika; Larsson, Olle

    2015-01-01

    Sarcomas are rare malignant tumors affecting all age groups. They are typically classified according to their resemblance to corresponding normal tissue. Their heterogeneous features, for example, in terms of disease-driving genetic aberrations and body location, complicate both disease classification and development of novel treatment regimens. Many years of failure of improved patient outcome in clinical trials has led to the conclusion that novel targeted therapies are likely needed in combination with current multimodality regimens. Sarcomas have not, in contrast to the common carcinomas, been the subject of larger systematic studies on how tumor behavior relates to characteristics of the tumor microenvironment. There is consequently an urgent need for identifying suitable molecular targets, not only in tumor cells but also in the tumor microenvironment. This review discusses preclinical and clinical data about potential molecular targets in sarcomas. Studies on targeted therapies involving the tumor microenvironment are prioritized. A greater understanding of the biological context is expected to facilitate more successful design of future clinical trials in sarcoma. PMID:26583076

  12. Leukosis/Sarcoma Group

    USDA-ARS?s Scientific Manuscript database

    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 ...

  13. 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. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. Pulmonary Alveolar Microlithiasis.

    PubMed

    Saito, Atsushi; McCormack, Francis X

    2016-09-01

    Pulmonary alveolar microlithiasis (PAM) is a genetic lung disorder that is characterized by the accumulation of calcium phosphate deposits in the alveolar spaces of the lung. Mutations in the type II sodium phosphate cotransporter, NPT2b, have been reported in patients with PAM. PAM progresses gradually, often producing incremental dyspnea on exertion, desaturation in young adulthood, and respiratory insufficiency by late middle age. Treatment remains supportive, including supplemental oxygen therapy. For patients with end-stage disease, lung transplantation is available as a last resort. The recent development of a laboratory animal model has revealed several promising treatment approaches for future trials. Published by Elsevier Inc.

  15. Immunotherapy of Childhood Sarcomas

    PubMed Central

    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. PMID:26301204

  16. What Happens After Treatment for Soft Tissue Sarcomas?

    MedlinePlus

    ... Sarcoma After Treatment What Happens After Treatment for Soft Tissue Sarcomas? For some people with soft tissue ... for Soft Tissue Sarcoma Stops Working More In Soft Tissue Sarcoma About Soft Tissue Sarcoma Causes, Risk ...

  17. Alveolar rhabdomyosarcoma. Demonstration of the muscle type of intermediate filament protein, desmin, as a diagnostic aid.

    PubMed Central

    Miettinen, M.; Lehto, V. P.; Badley, R. A.; Virtanen, I.

    1982-01-01

    Three cases of soft-tissue sarcomas with the characteristic histologic features of alveolar rhabdomyosarcoma, but lacking cytoplasmic cross-striations, were studied ultrastructurally and immunohistochemically to confirm the diagnosis and evaluate the histogenesis. The results showed that it was not possible to judge the skeletal muscle derivation of the cells at the ultrastructural level. However, immunohistochemically, the results of every case were positive for desmin-the muscle type of the intermediate filament protein. The results suggest that demonstration of desmin may be a helpful adjunct tool in the diagnosis of poorly differentiated alveolar rhabdomyosarcomas. Images Figure 4 Figure 5 Figure 1 Figure 2 Figure 3 PMID:6765734

  18. Doxorubicin With Upfront Dexrazoxane Plus Olaratumab for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2017-08-29

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

  19. Pericytes in sarcomas of bone.

    PubMed

    Chang, Le; Nguyen, Vi; Nguyen, Alan; Scott, Michelle A; James, Aaron W

    2015-07-01

    Pericytes are mesenchymal cells that closely enwrap small blood vessels, lying in intimate association with the endothelium. Pericytes have recently gained attention as an important mediator of vascular biology and angiogenesis in cancer. Although better studied in carcinoma, pericytes have known interaction with sarcomas of bone, including Ewing's sarcoma, osteosarcoma, and chondrosarcoma. Best studied is Ewing's sarcoma (ES), which displays a prominent perivascular growth pattern. Signaling pathways of known importance in intratumoral pericytes in ES include Notch, PDGF/PDGFR-β, and VEGF signaling. In summary, pericytes serve important functions in the tumor microenvironment. Improved understanding of pericyte biology may hold significant implications for the development of new therapies in sarcoma.

  20. [Brachytherapy for sarcomas].

    PubMed

    Ducassou, A; Haie-Méder, C; Delannes, M

    2016-10-01

    The standard of care for local treatment for extremities soft tissue sarcomas relies on conservative surgery combined with external beam radiotherapy. Brachytherapy can be realized instead of external beam radiotherapy in selected cases, or more often used as a boost dose on a limited volume on the area at major risk of relapse, especially if a microscopic positive resection is expected. Close interaction and communication between radiation oncologists and surgeons are mandatory at the time of implantation to limit the risk of side effects. Long-term results are available for low-dose rate brachytherapy. Nowadays, pulsed dose rate or high-dose-rate brachytherapy are more often used. Brachytherapy for paediatric sarcomas is rare, and has to be managed in reference centres.

  1. Primary pulmonary artery sarcoma.

    PubMed

    Jin, Tao; Zhang, Chong; Feng, Zhiying; Ni, Yiming

    2008-08-01

    Primary pulmonary artery sarcoma is an uncommon tumor. We report a case of a 73-year-old male patient with a two-week history of palpitations and shortness of breath, aggravated for two days and was believed to be pulmonary hypertension. Emergency heart ultrasound after admission presented a massive pulmonary embolism in the pulmonary artery. The patient's condition was successfully managed with urgent pulmonary artery embolectomy. The patient demonstrated improvement in hemodynamics after the operation. Histologic and immunohistochemical assays were performed and a diagnosis was made as primary pulmonary artery sarcoma arising from the left pulmonary artery. Resection of the tumor is recommended for the treatment of this rare malignant tumor. The corresponding chemotherapy, follow-up and prognosis are described as well in this case report.

  2. Molecular biology of sarcomas.

    PubMed

    Gebhardt, M C

    1996-07-01

    There has been a virtual explosion of information relating to the biology of sarcomas with which we as orthopaedists deal. Much more is yet to be learned. These findings will teach us more about the etiology of these tumors. More important, the findings will alter the way in which these tumors are treated. It is unlikely that we will continue to treat osteosarcoma or Ewing's sarcoma patients with currently available drug regimens and surgery or make treatment decisions based on the histologic classification of tumors we know today. If we are to remain active in the management of these patients we must be aware of the findings as they occur. That will ensure both that we remain the primary caretakers of these patients, and that we will continue to be stimulated intellectually by these intriguing scientific investigations.

  3. Giant retroperitoneal sarcomas.

    PubMed

    Ianoşi, G; Neagoe, Daniela; Buteică, Elena; Ianoşi, Simona; Drighiciu, Corina; Stănoiu, B; Burada, F; Mercuţ, D

    2007-01-01

    Retroperitoneal sarcomas are rare malignant tumors, which are developing from mesenchymal stem cells residing in muscle, fat, and connective tissues. Underlying the rarity of this kind of tumors in general population, the aim of this paper is to present three cases of retroperitoneal sarcomas operated in Surgical Department of Military Hospital of Craiova (a retroperitoneal liposarcoma, a dedifferentiated liposarcoma and a malignant fibrous histiocytoma). From clinical point of view, we note the poverty of symptoms and non-specificity of these and a great tolerability of retroperitoneal space that offers the possibility for a great development of the tumor. CT-scan and MRI are the best investigations for diagnosis but surgical exploration is the best way for a good evaluation of these tumors. From histological point of view, we try to present new features about these kinds of tumors in order to classify them. Results of surgery correlated with complementary therapies were good without per-operative mortality or postoperative morbidity but we noted a recidive of one tumor (with different histological pattern) 21 months after the surgical intervention. The rarity of retroperitoneal sarcomas, combined with the vast array of histologic subtypes, has complicated our understanding of these tumors and impeded the development of effective therapies.

  4. Clinical effect of molecular methods in sarcoma diagnosis (GENSARC): a prospective, multicentre, observational study.

    PubMed

    Italiano, Antoine; Di Mauro, Ilaria; Rapp, Jocelyn; Pierron, Gaëlle; Auger, Nathalie; Alberti, Laurent; Chibon, Frédéric; Escande, Fabienne; Voegeli, Anne-Claire; Ghnassia, Jean-Pierre; Keslair, Frédérique; Laé, Marick; Ranchère-Vince, Dominique; Terrier, Philippe; Baffert, Sandrine; Coindre, Jean-Michel; Pedeutour, Florence

    2016-04-01

    Advances in molecular genetics of sarcoma have enabled the identification of type-specific aberrations. We aimed to assess the clinical effect of systematic implementation of molecular assays to improve sarcoma misdiagnosis. In this multicentre, observational study, we recruited patients from 32 centres of the French Sarcoma Group/Reference Network in Pathology of Sarcomas. Eligibility criteria included: biopsy or surgical resection; suspicion of: dermatofibrosarcoma protuberans (cohort 1), dedifferentiated liposarcoma (cohort 2), Ewing's sarcoma family of tumours (cohort 3), synovial sarcoma (cohort 4), alveolar rhabdomyosarcoma (cohort 5), and myxoid or round cell liposarcoma (cohort 6); review by one sarcoma-expert pathologist; availability of frozen material (except for cohort 1 of patients with dermatofibrosarcoma protuberans because anti-CD34 immunohistochemistry is performed on paraffin-embedded tissue); and patient information. For each case, the pathologist made one primary diagnosis followed by up to two differential diagnoses, based on histological characteristics only. Each diagnosis was classified as certain, probable, or possible. For each case to determine the molecular classification, we did fluorescence in-situ hybridisation on paraffin-embedded samples. We also did comparative genomic hybridisation and quantitative PCR (cohort 2) or reverse transcriptase PCR (cohorts 3-6) on frozen and paraffin-embedded samples. We made a final diagnosis based on the molecular results. The clinical effect of diagnosis correction was assessed by a board of experts. Between June 22, 2009, and Oct 30, 2012, 395 patients were enrolled in the study, of which 384 were eligible for inclusion. The diagnosis was eventually modified by molecular genetics for 53 patients: eight (16%) of 50 patients with dermatofibrosarcoma (cohort 1), seven (23%) of 30 patients with dedifferentiated liposarcoma (cohort 2), 13 (12%) of 112 with Ewing's sarcoma family of tumours (cohort 3), 16

  5. Routes to diagnosis for sarcoma - Describing the sarcoma patient journey.

    PubMed

    Gerrand, C; Francis, M; Dennis, N; Charman, J; Lawrence, G; Evans, T; Grimer, R

    2015-10-01

    Understanding the route to diagnosis for patients with cancer is important to improve the diagnostic pathway and therefore outcomes. We aimed to investigate routes to diagnosis for patients with sarcoma in England. National patient level datasets relating to 7716 soft tissue and 1240 bone sarcoma patients diagnosed between 2006 and 2008 were analysed. Routes to diagnosis were defined as: "Two Week Wait Referral", "GP Referral", "Other Outpatient", "Inpatient Elective", "Emergency Presentation", "Death Certificate Only" and "Unknown". Patients with sarcoma are most likely to be diagnosed after "GP Referral" or "Emergency Presentation" and are less likely to be referred under a two week wait compared with other malignancies. Patients with bone sarcoma under 10 or over 80 years of age were more likely to present by emergency routes, as were patients with vertebral column, pelvis or unspecified site tumours and those with Ewing's sarcoma or sarcoma NOS. Patients with soft tissue sarcoma under 19 or over 80 years of age and patients with GI tract tumours were more likely to present by emergency routes. Patients presenting by emergency routes more often had metastases and had lower 1 year survival. Patients from least deprived quintiles more often presented by unknown routes: those from more deprived quintiles more often presented by emergency routes. Routes to diagnosis for sarcoma patients differ from other cancers. Interventions to improve the diagnostic experience should consider the very young and elderly, tumours in abdominal, pelvic or spinal locations and on reducing emergency presentations. Crown Copyright © 2015. Published by Elsevier Ltd. All rights reserved.

  6. Taking Aim at Important Targets in Sarcoma | Center for Cancer Research

    Cancer.gov

    Rhabdomyosarcoma (RMS) is the most common childhood soft tissue sarcoma, a cancer of the body’s connective or supportive tissues such as muscle, cartilage, or fat. The two major classifications of RMS include the embryonal subtype, which accounts for approximately three-quarters of children diagnosed with RMS, and the more aggressive alveolar (ARMS) subtype, which has a five-year survival rate of less than 30 percent.

  7. Alveolar Rhabdomyosarcoma in a 69-Year-Old Woman Receiving Glucagon-Like Peptide-2 Therapy

    PubMed Central

    Zyczynski, Laura E.; McHugh, Jonathan B.; Gribbin, Thomas E.; Schuetze, Scott M.

    2015-01-01

    A 69-year-old woman was diagnosed with alveolar rhabdomyosarcoma (ARMS) of the nasopharynx. She has a history of catastrophic thromboembolic event in the abdomen that caused short-gut syndrome and dependence on total parenteral nutrition (TPN) twelve hours per day. She was treated for short-gut syndrome with teduglutide, a glucagon-like peptide-2 (GLP-2) analog, which led to reduction of TPN requirements. However, a few months later, she developed metastatic alveolar rhabdomyosarcoma. Though a causative relationship is unlikely between the peptide and ARMS due to the brief time course between teduglutide therapy and sarcoma diagnosis, neoplastic growth may have been accelerated by the GLP-2 analog, causing release of IGF-1. The transmembrane receptor for IGF-1 is frequently overexpressed in ARMS and is implicated in cell proliferation and metastatic behavior. This case describes a rare incidence of metastatic alveolar rhabdomyosarcoma in a sexagenarian and possibly the first case reported associated with the use of teduglutide. Teduglutide was discontinued due to a potential theoretical risk of acceleration of sarcoma growth, and the patient's rhabdomyosarcoma is in remission following sarcoma chemotherapy. PMID:26266067

  8. Alveolar Rhabdomyosarcoma in a 69-Year-Old Woman Receiving Glucagon-Like Peptide-2 Therapy.

    PubMed

    Zyczynski, Laura E; McHugh, Jonathan B; Gribbin, Thomas E; Schuetze, Scott M

    2015-01-01

    A 69-year-old woman was diagnosed with alveolar rhabdomyosarcoma (ARMS) of the nasopharynx. She has a history of catastrophic thromboembolic event in the abdomen that caused short-gut syndrome and dependence on total parenteral nutrition (TPN) twelve hours per day. She was treated for short-gut syndrome with teduglutide, a glucagon-like peptide-2 (GLP-2) analog, which led to reduction of TPN requirements. However, a few months later, she developed metastatic alveolar rhabdomyosarcoma. Though a causative relationship is unlikely between the peptide and ARMS due to the brief time course between teduglutide therapy and sarcoma diagnosis, neoplastic growth may have been accelerated by the GLP-2 analog, causing release of IGF-1. The transmembrane receptor for IGF-1 is frequently overexpressed in ARMS and is implicated in cell proliferation and metastatic behavior. This case describes a rare incidence of metastatic alveolar rhabdomyosarcoma in a sexagenarian and possibly the first case reported associated with the use of teduglutide. Teduglutide was discontinued due to a potential theoretical risk of acceleration of sarcoma growth, and the patient's rhabdomyosarcoma is in remission following sarcoma chemotherapy.

  9. Alveolar bone grafting.

    PubMed

    Semb, Gunvor

    2012-01-01

    In the 1970s, Boyne and Sands published reports on a new technique for alveolar bone grafting. They recommended that only cancellous bone be used and that the procedure be undertaken in the mixed dentition prior to canine eruption. Alveolar bone grafting prior to canine eruption soon became a routine part of the protocol for 90% of European and North American cleft teams. Several uncertainties remain however, such as the specifics of the surgical and orthodontic procedures, type of bone and donor site, and the best way to manage the space in the dental arch. Probably the commonest timing of the bone graft falls between 8 and 11 years, however there has been a trend in some centres to graft earlier in the hope of better outcome for the unerupted incisors. The influence on maxillary growth of earlier grafting has not been ascertained. A wide range of donor sites has been use but iliac crest remains the most popular. Many teams perform orthodontics prior to grafting to correct severe segment displacement or align incisors to improve surgical access. Following grafting, absence of the lateral incisor may be managed with orthodontic space closure, placement of an implant or bridgework. The introduction of alveolar bone grafting probably represents one of the most significant clinical innovations in cleft care. Hopefully, advances in tissue engineering will replace the need for transplantation of autogenous bone, or will provide an in-situ biological solution to the generation of a continuous bone fill across the alveolar cleft. Copyright © 2012 S. Karger AG, Basel.

  10. Surgical treatment of uterine sarcoma.

    PubMed

    Nam, Joo-Hyun

    2011-12-01

    Uterine sarcomas are rare, heterogeneous malignant tumours of several histologic types originating from mesenchymal tissues of the uterus. The most common histologic types are carcinosarcoma, leiomyosarcoma, and endometrial stromal sarcoma, accounting for 90% of uterine sarcomas. To date, no effective treatment has been found to achieve a high rate of cure or prolong survival. Although complete surgical excision of the tumour is the only curative treatment modality, the rarity of these tumours and their diversity of histologic types have precluded the development of standard surgical strategies. Surgery may also be optimal for recurrent uterine sarcomas, but indications for secondary surgical treatment have not been established. Here, we describe recent changes in, and updates of, the surgical treatment of the three most common types of malignant uterine sarcomas.

  11. IGF1R- and ROR1-Specific CAR T Cells as a Potential Therapy for High Risk Sarcomas.

    PubMed

    Huang, Xin; Park, Haein; Greene, Joseph; Pao, James; Mulvey, Erin; Zhou, Sophia X; Albert, Catherine M; Moy, Fred; Sachdev, Deepali; Yee, Douglas; Rader, Christoph; Hamby, Carl V; Loeb, David M; Cairo, Mitchell S; Zhou, Xianzheng

    2015-01-01

    Patients with metastatic or recurrent and refractory sarcomas have a dismal prognosis. Therefore, new targeted therapies are urgently needed. This study was designed to evaluate chimeric antigen receptor (CAR) T cells targeting the type I insulin-like growth factor receptor (IGF1R) or tyrosine kinase-like orphan receptor 1 (ROR1) molecules for their therapeutic potential against sarcomas. Here, we report that IGF1R (15/15) and ROR1 (11/15) were highly expressed in sarcoma cell lines including Ewing sarcoma, osteosarcoma, alveolar or embryonal rhabdomyosarcoma, and fibrosarcoma. IGF1R and ROR1 CAR T cells derived from eight healthy donors using the Sleeping Beauty (SB) transposon system were cytotoxic against sarcoma cells and produced high levels of IFN-γ, TNF-α and IL-13 in an antigen-specific manner. IGF1R and ROR1 CAR T cells generated from three sarcoma patients released significant amounts of IFN-γ in response to sarcoma stimulation. The adoptive transfer of IGF1R and ROR1 CAR T cells derived from a sarcoma patient significantly reduced tumor growth in pre-established, systemically disseminated and localized osteosarcoma xenograft models in NSG mice. Infusion of IGF1R and ROR1 CAR T cells also prolonged animal survival in a localized sarcoma model using NOD/scid mice. Our data indicate that both IGF1R and ROR1 can be effectively targeted by SB modified CAR T cells and that such CAR T cells may be useful in the treatment of high risk sarcoma patients.

  12. IGF1R- and ROR1-Specific CAR T Cells as a Potential Therapy for High Risk Sarcomas

    PubMed Central

    Huang, Xin; Park, Haein; Greene, Joseph; Zhou, Sophia X.; Albert, Catherine M.; Moy, Fred; Sachdev, Deepali; Yee, Douglas; Rader, Christoph; Hamby, Carl V.; Loeb, David M.; Cairo, Mitchell S.; Zhou, Xianzheng

    2015-01-01

    Patients with metastatic or recurrent and refractory sarcomas have a dismal prognosis. Therefore, new targeted therapies are urgently needed. This study was designed to evaluate chimeric antigen receptor (CAR) T cells targeting the type I insulin-like growth factor receptor (IGF1R) or tyrosine kinase-like orphan receptor 1 (ROR1) molecules for their therapeutic potential against sarcomas. Here, we report that IGF1R (15/15) and ROR1 (11/15) were highly expressed in sarcoma cell lines including Ewing sarcoma, osteosarcoma, alveolar or embryonal rhabdomyosarcoma, and fibrosarcoma. IGF1R and ROR1 CAR T cells derived from eight healthy donors using the Sleeping Beauty (SB) transposon system were cytotoxic against sarcoma cells and produced high levels of IFN-γ, TNF-α and IL-13 in an antigen-specific manner. IGF1R and ROR1 CAR T cells generated from three sarcoma patients released significant amounts of IFN-γ in response to sarcoma stimulation. The adoptive transfer of IGF1R and ROR1 CAR T cells derived from a sarcoma patient significantly reduced tumor growth in pre-established, systemically disseminated and localized osteosarcoma xenograft models in NSG mice. Infusion of IGF1R and ROR1 CAR T cells also prolonged animal survival in a localized sarcoma model using NOD/scid mice. Our data indicate that both IGF1R and ROR1 can be effectively targeted by SB modified CAR T cells and that such CAR T cells may be useful in the treatment of high risk sarcoma patients. PMID:26173023

  13. 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

  14. 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

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

    ClinicalTrials.gov

    2016-11-21

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

  16. Pulmonary Alveolar Microlithiasis

    PubMed Central

    Mehta, Kevan; Dell, Sharon; Birken, Catherine; Al-Saleh, Suhail

    2016-01-01

    Pulmonary alveolar microlithiasis (PAM) is a rare autosomal recessive condition that is often asymptomatic despite significant changes in chest imaging. Diagnosis is often made when patients become symptomatic in adulthood. There are still no proven treatments, but earlier diagnosis may allow for evaluation of preventative strategies that could improve outcome. It is an important diagnosis to consider in children who have marked radiographic findings with no or very mild symptoms or physical findings. Diagnosis can be made with imaging alone but may necessitate lung biopsy for definitive diagnosis. PMID:27445543

  17. Kaposi sarcoma herpesvirus pathogenesis

    PubMed Central

    Koch, Sandra; Schulz, Thomas F.

    2017-01-01

    Kaposi sarcoma herpesvirus (KSHV), taxonomical name human gammaherpesvirus 8, is a phylogenetically old human virus that co-evolved with human populations, but is now only common (seroprevalence greater than 10%) in sub-Saharan Africa, around the Mediterranean Sea, parts of South America and in a few ethnic communities. KSHV causes three human malignancies, Kaposi sarcoma, primary effusion lymphoma, and many cases of the plasmablastic form of multicentric Castleman's disease (MCD) as well as occasional cases of plasmablastic lymphoma arising from MCD; it has also been linked to rare cases of bone marrow failure and hepatitis. As it has colonized humans physiologically for many thousand years, cofactors are needed to allow it to unfold its pathogenic potential. In most cases, these include immune defects of genetic, iatrogenic or infectious origin, and inflammation appears to play an important role in disease development. Our much improved understanding of its life cycle and its role in pathogenesis should now allow us to develop new therapeutic strategies directed against key viral proteins or intracellular pathways that are crucial for virus replication or persistence. Likewise, its limited (for a herpesvirus) distribution and transmission should offer an opportunity for the development and use of a vaccine to prevent transmission. This article is part of the themed issue ‘Human oncogenic viruses’. PMID:28893942

  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. Diffuse Alveolar Hemorrhage

    PubMed Central

    2013-01-01

    Diffuse alveolar hemorrhage (DAH) is a life-threatening and medical emergency that can be caused by numerous disorders and presents with hemoptysis, anemia, and diffuse alveolar infiltrates. Early bronchoscopy with bronchoalveolar lavage is usually required to confirm the diagnosis and rule out infection. Most cases of DAH are caused by capillaritis associated with systemic autoimmune diseases such as anti-neutrophil cytoplasmic antibody-associated vasculitis, anti-glomerular basement membrane disease, and systemic lupus erythematosus, but DAH may also result from coagulation disorders, drugs, inhaled toxins, or transplantation. The diagnosis of DAH relies on clinical suspicion combined with laboratory, radiologic, and pathologic findings. Early recognition is crucial, because prompt diagnosis and treatment is necessary for survival. Corticosteroids and immunosuppressive agents remain the gold standard. In patients with DAH, biopsy of involved sites can help to identify the cause and to direct therapy. This article aims to provide a general review of the causes and clinical presentation of DAH and to recommend a diagnostic approach and a management plan for the most common causes. PMID:23678356

  20. What Should You Ask Your Doctor about Kaposi Sarcoma?

    MedlinePlus

    ... What Should You Ask Your Doctor About Kaposi Sarcoma? Kaposi Sarcoma Early Detection, Diagnosis, and Staging What Should You Ask Your Doctor About Kaposi Sarcoma? As you cope with Kaposi sarcoma (KS) and ...

  1. Alisertib in Treating Patients With Advanced or Metastatic Sarcoma

    ClinicalTrials.gov

    2016-11-01

    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

  2. Ewing's sarcoma of the patella.

    PubMed

    Gorelik, Natalia; Dickson, Brendan C; Wunder, Jay S; Bleakney, Robert

    2013-05-01

    Ewing's sarcoma is a relatively rare malignancy, occurring mainly between 4 and 25 years of age. It usually arises from the pelvis, followed by the femur, tibia, and remainder of both the long bones of the extremities and flat bones of the axial skeleton. To the best of our knowledge, Ewing's sarcoma of the patella has never been reported previously. Patellar tumors occur infrequently and represent an uncommon etiology of anterior knee pain. We describe the rare case of a 41-year-old man who presented with a 3-4 month history of escalating right anterior knee pain and swelling. Imaging demonstrated an aggressive patellar tumor with an adjacent soft tissue mass. The diagnosis of Ewing's sarcoma was confirmed by pathology. Physicians should be aware of atypical locations for Ewing's sarcoma and, conversely, of rare tumors arising in the patella and accounting for anterior knee pain. Early recognition of such malignancies allows prompt initiation of treatment, hence improving prognosis.

  3. 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.

  4. Promiscuous partnerships in Ewing's sarcoma.

    PubMed

    Sankar, Savita; Lessnick, Stephen L

    2011-07-01

    Ewing's sarcoma is a highly aggressive bone and soft tissue tumor of children and young adults. At the molecular genetic level Ewing's sarcoma is characterized by a balanced reciprocal translocation, t(11;22)(q24;q12), which encodes an oncogenic fusion protein and transcription factor EWS/FLI. This tumor-specific chimeric fusion retains the amino terminus of EWS, a member of the TET (TLS/EWS/TAF15) family of RNA-binding proteins, and the carboxy terminus of FLI, a member of the ETS family of transcription factors. In addition to EWS/FLI, variant translocation fusions belonging to the TET/ETS family have been identified in Ewing's sarcoma. These studies solidified the importance of TET/ETS fusions in the pathogenesis of Ewing's sarcoma and have since been used as diagnostic markers for the disease. EWS fusions with non-ETS transcription factor family members have been described in sarcomas that are clearly distinct from Ewing's sarcoma. However, in recent years there have been reports of rare fusions in "Ewing's-like tumors" that harbor the amino-terminus of EWS fused to the carboxy-terminal DNA or chromatin-interacting domains contributed by non-ETS proteins. This review aims to summarize the growing list of fusion oncogenes that characterize Ewing's sarcoma and Ewing's-like tumors and highlights important questions that need to be answered to further support the existing concept that Ewing's sarcoma is strictly a "TET/ETS" fusion-driven malignancy. Understanding the molecular mechanisms of action of the various different fusion oncogenes will provide better insights into the biology underlying this rare but important solid tumor.

  5. Synovial sarcoma in childhood

    SciTech Connect

    Israels, S.J.; Chan, H.S.L.; Daneman, A.; Weitzman, S.S.

    1984-04-01

    The clinical and radiologic findings in seven children with synovial sarcoma are described. The five boys and two girls had a mean age at presentation of 4.4 years. All seven had the lesion situated in an extremity. Plain radiographs in four revealed the presence of a soft-tissue mass with no calcification or bone and joint involvement. In two patients studied with computed tomography (CT), the primary lesions had peripheral irregular areas of enhancement with central areas of poor enhancement, reflecting the necrotic, cystic, and hemorrhagic changes found in the centers of these tumors. Although the exact margins of these lesions were difficult to define accurately even with intravenous contrast enhancement, CT is still recommended as the best imaging method for assessing the local extent of the primary tumor and is a useful tool in the planning of appropriate therapy as well as the gauging of the tumor response to ongoing treatment.

  6. [Radiotherapy for retroperitoneal sarcomas].

    PubMed

    Sargos, P; Stoeckle, E; Henriques de Figueiredo, B; Antoine, M; Delannes, M; Mervoyer, A; Kantor, G

    2016-10-01

    The management of retroperitoneal sarcoma can be very challenging, and the quality of initial treatment strategy appears to be a crucial prognostic factor. En bloc surgery is currently the standard of care for these rare tumours and perioperative treatments such as chemotherapy or radiotherapy have not been validated yet. However, local-regional relapse constitutes the most common disease course. While adjuvant radiotherapy is less and less common due to gastrointestinal toxicities, preoperative radiation therapy offers numerous advantages and is being evaluated as part of a national multicentre phase II study (TOMOREP trial) and is the subject of a European randomized phase III study (STRASS trial). The objective of this article is to present data on preoperative irradiation in terms of dose, volumes and optimal radiotherapy techniques for the treatment of this rare disease.

  7. Myeloid Sarcoma in the Orbit.

    PubMed

    Qian, Xiaoxiao; Gigantelli, James W; Abromowitch, Minnie; Morgan, Linda A; Suh, Donny W

    2016-12-08

    The authors describe a case of myeloid sarcoma of the orbit in a pediatric patient. An 8-month-old male infant presented to the ophthalmology clinic with a left orbital mass, which had been increasing in size over the previous 2 months. The mass was initially diagnosed at another clinic as an infantile hemangioma, and had been treated with a topical formulation of timolol. In the ophthalmology clinic, orbital magnetic resonance imaging showed a solid enhancing mass. A biopsy was performed, and histopathology revealed myeloid sarcoma. The disease responded well to a standard chemotherapy regimen. Myeloid sarcoma is a rare, extra-medullary presentation that can occur as an isolated tumor, concurrently with or at relapse of acute myeloid leukemia. Because few cases of myeloid sarcoma in the orbit have been reported, this case report aids in the management of myeloid sarcoma in pediatric patients. The report describes an 8-month-old male infant, the youngest patient to develop myeloid sarcoma without preexisting acute myeloid leukemia. [J Pediatr Ophthalmol Strabismus. 2016;53:e64-e68.].

  8. Soft-tissue sarcomas of the extremities in patients of pediatric age.

    PubMed

    Casanova, Michela; Meazza, Cristina; Gronchi, Alessandro; Fiore, Marco; Zaffignani, Elena; Podda, Marta; Collini, Paola; Gandola, Lorenza; Ferrari, Andrea

    2007-09-01

    The extremity site is a peculiar location for soft-tissue sarcomas (STS) of children and adolescents. Here we report the experience of the Pediatric Oncology Unit of the Istituto Nazionale Tumori of Milan, Italy, concerning 204 patients with STS of the limbs treated between 1977 and 2006. The study series included 52 patients with rhabdomyosarcoma (RMS)(65% of which were of the alveolar subtype), nine with extraosseous Ewing sarcoma and 143 with non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS), 38% of which were synovial sarcoma. Patients were treated with a multimodality approach including surgery, chemotherapy, and radiotherapy. For the RMS patients, the 5-year event-free survival (EFS) rate was 37.1%, with distant metastases being the main cause of treatment failure. The outcome was particularly poor for patients with large invasive tumors, hand/foot involvement and/or nodal or distant metastases, and for patients who were not given radiotherapy. For the extraosseous Ewing sarcoma cases, 5-year EFS was 74%. For the NRSTS cases, the 5-year EFS was 72.6%: tumor size and local invasiveness, tumor grade, malignant peripheral nerve sheath tumor (MPNST) histology, and distant metastases were the main prognostic factors. While the limbs are the most common sites of NRSTS and are often characterized by a more favorable prognosis than for axial tumors, the clinical features of extremity RMS often differ from those of RMS of other sites, with a higher incidence of unfavorable prognostic factors (e.g., alveolar subtype) and consequently unsatisfactory treatment results. The treatment of these patients is complex and necessarily multidisciplinary, and it demands not only adequate experience of treating children and adolescents in clinical trials, but also particular skills in the field of orthopedic surgery.

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

    ClinicalTrials.gov

    2017-01-31

    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

  10. 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

  11. 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 ...

  12. What Is a Soft Tissue Sarcoma?

    MedlinePlus

    ... know has just been diagnosed with sarcoma, this short, simple guide can help. Downloadable PDFs Download free PDFs of our soft tissue sarcoma information About and ... Read More Latest Cancer News Read More Stories ...

  13. Drugs Approved for Soft Tissue Sarcoma

    MedlinePlus

    ... Your Treatment Research Drugs Approved for Soft Tissue Sarcoma This page lists cancer drugs approved by the ... not listed here. Drugs Approved for Soft Tissue Sarcoma Cosmegen (Dactinomycin) Dactinomycin Doxorubicin Hydrochloride Eribulin Mesylate Gleevec ( ...

  14. [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.

  15. Emergency management of alveolar osteitis.

    PubMed

    Summers, Anthony

    2011-12-01

    Patients with urgent dental problems who present to emergency departments (EDs) during weekday office hours are usually referred to their dentists, often after being provided with analgesia. Outside these hours, however, ED professionals may have to provide treatment before referral. One dental emergency with which patients may present but of which ED staff are unlikely to have experience is alveolar osteitis, a painful condition that occurs usually after tooth extraction. This article defines alveolar osteitis and describes management in an ED.

  16. Trabectedin in soft tissue sarcomas.

    PubMed

    Petek, Bradley J; Loggers, Elizabeth T; Pollack, Seth M; Jones, Robin L

    2015-02-12

    Soft tissue sarcomas are a group of rare tumors derived from mesenchymal tissue, accounting for about 1% of adult cancers. There are over 60 different histological subtypes, each with their own unique biological behavior and response to systemic therapy. The outcome for patients with metastatic soft tissue sarcoma is poor with few available systemic treatment options. For decades, the mainstay of management has consisted of doxorubicin with or without ifosfamide. Trabectedin is a synthetic agent derived from the Caribbean tunicate, Ecteinascidia turbinata. This drug has a number of potential mechanisms of action, including binding the DNA minor groove, interfering with DNA repair pathways and the cell cycle, as well as interacting with transcription factors. Several phase II trials have shown that trabectedin has activity in anthracycline and alkylating agent-resistant soft tissue sarcoma and suggest use in the second- and third-line setting. More recently, trabectedin has shown similar progression-free survival to doxorubicin in the first-line setting and significant activity in liposarcoma and leiomyosarcoma subtypes. Trabectedin has shown a favorable toxicity profile and has been approved in over 70 countries for the treatment of metastatic soft tissue sarcoma. This manuscript will review the development of trabectedin in soft tissue sarcomas.

  17. 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. © The Author(s) 2016.

  18. Skin Ultrasound in Kaposi Sarcoma.

    PubMed

    Carrascosa, R; Alfageme, F; Roustán, G; Suarez, M D

    2016-05-01

    The use of ultrasound imaging has recently been increasing in numerous dermatologic diseases. This noninvasive technique provides additional details on the structure and vascularization of skin lesions. Kaposi sarcoma is a vascular tumor that typically arises in the skin and mucosas. It can spread to lymph nodes and internal organs. We performed B-mode and color Doppler ultrasound studies in 3 patients with a clinical diagnosis of Kaposi sarcoma confirmed by histological examination. We found differences in the ultrasound pattern between nodular and plaque lesions, in both B-mode and color Doppler. We believe that skin ultrasound imaging could be a useful technique for studying cutaneous Kaposi sarcoma, providing additional information on the structural and vascular characteristics of the lesion.

  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. Epithelioid sarcoma in FNAB smears.

    PubMed

    Pohar-Marinsek, Z; Zidar, A

    1994-12-01

    FNAB smears of eleven epithelioid sarcomas were reviewed and analysed. Ten cases had a very similar cytomorphologic picture composed predominantly of dissociated epithelioid-like cells with eccentrically placed nuclei. These tumors were clearly malignant but difficult to differentiate morphologically from melanoma, epithelioid leiomiosarcoma, and Schwannoma or adenocarcinoma. One case was composed of spindle cells and was reminiscent of a fibrohistiocytic tumor. Immunocytochemical reactions to vimentin and cytokeratin were performed in six cases on the Papanicolaou stained smears. The reactions to both antigens were positive in all six cases. Ultrastructural characteristics of eight of the tumors are also described. It seems that epithelioid sarcoma has a rather distinct cytomorphologic picture. Taking into consideration clinical data and using also immunocytochemistry, a definitive diagnosis of epithelioid sarcoma can probably be given from FNAB smears.

  1. Reticular schwannoma mimicking myxoid sarcoma.

    PubMed

    Chaurasia, Jai Kumar; Afroz, Nishat; Sahoo, Biswajit; Naim, Mohammed

    2014-02-20

    Reticular/microcystic schwannoma is a recently described rare distinctive variant of schwannoma with a predilection for gastrointestinal tract. Its occurrence in soft tissue is extremely rare. We report a case of reticular/microcystic variant of schwannoma in a 55-year-old Indian woman who presented with a painless slow-growing swelling in the right forearm for the past 6 months. MRI findings suggested myxoid sarcoma. However, histological and immunohistochemical findings in the excised specimen were consistent with reticular/microcystic variant of schwannoma. This case report emphasises that the diagnosis of reticular/microcystic schwannoma should always be considered in cases where myxoid sarcomas are suspected as it can mimic malignant myxoid sarcomas, clinically and radiologically, thereby avoiding aggressive intervention and overtreatment.

  2. [Therapeutic options for synovial sarcoma].

    PubMed

    Deme, Dániel; Telekes, András

    2015-05-31

    Synovial sarcomas account for approximately 5 to 10% of soft tissue sarcomas and 0.05 to 0.1% of all malignant neoplasms. They predominantly affect the extremities but can occur in any part of the body. More than 50% of the patients are expected to develop metastatic disease within 3-5 years. In some patients disease recurrence may develop after 20 years. The 5-year overall survival rate is 10% for patients with metastatic disease and 76% for patients with localized one. Age, tumour size, histological subtype, and adjuvant radiotherapy influence prognosis. The role of adjuvant chemotherapy has not been proven yet. There are several ongoing clinical trials to determine the efficacy of active agents used for therapy of locally advanced, relapsed/refractory or metastatic disease. Better understanding of the biological behaviour of synovial sarcomas would provide the future way for the targeted therapy in combination with conventional treatments.

  3. Sessile alveolar macrophages communicate with alveolar epithelium to modulate immunity

    NASA Astrophysics Data System (ADS)

    Westphalen, Kristin; Gusarova, Galina A.; Islam, Mohammad N.; Subramanian, Manikandan; Cohen, Taylor S.; Prince, Alice S.; Bhattacharya, Jahar

    2014-02-01

    The tissue-resident macrophages of barrier organs constitute the first line of defence against pathogens at the systemic interface with the ambient environment. In the lung, resident alveolar macrophages (AMs) provide a sentinel function against inhaled pathogens. Bacterial constituents ligate Toll-like receptors (TLRs) on AMs, causing AMs to secrete proinflammatory cytokines that activate alveolar epithelial receptors, leading to recruitment of neutrophils that engulf pathogens. Because the AM-induced response could itself cause tissue injury, it is unclear how AMs modulate the response to prevent injury. Here, using real-time alveolar imaging in situ, we show that a subset of AMs attached to the alveolar wall form connexin 43 (Cx43)-containing gap junction channels with the epithelium. During lipopolysaccharide-induced inflammation, the AMs remained sessile and attached to the alveoli, and they established intercommunication through synchronized Ca2+ waves, using the epithelium as the conducting pathway. The intercommunication was immunosuppressive, involving Ca2+-dependent activation of Akt, because AM-specific knockout of Cx43 enhanced alveolar neutrophil recruitment and secretion of proinflammatory cytokines in the bronchoalveolar lavage. A picture emerges of a novel immunomodulatory process in which a subset of alveolus-attached AMs intercommunicates immunosuppressive signals to reduce endotoxin-induced lung inflammation.

  4. 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.

  5. Epidemiology and therapies for metastatic sarcoma

    PubMed Central

    Amankwah, Ernest K; Conley, Anthony P; Reed, Damon R

    2013-01-01

    Sarcomas are cancers arising from the mesenchymal layer that affect children, adolescents, young adults, and adults. Although most sarcomas are localized, many display a remarkable predilection for metastasis to the lungs, liver, bones, subcutaneous tissue, and lymph nodes. Additionally, many sarcoma patients presenting initially with localized disease may relapse at metastatic sites. While localized sarcomas can often be cured through surgery and often radiation, controversies exist over optimal management of patients with metastatic sarcoma. Combinations of chemotherapy are the most effective in many settings, and many promising new agents are under active investigation or are being explored in preclinical models. Metastatic sarcomas are excellent candidates for novel approaches with additional agents as they have demonstrated chemosensitivity and affect a portion of the population that is motivated toward curative therapy. In this paper, we provide an overview on the common sarcomas of childhood (rhabdomyosarcoma), adolescence, and young adults (osteosarcoma, Ewing sarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumor) and older adults (leiomyosarcoma, liposarcoma, and undifferentiated high grade sarcoma) in terms of the epidemiology, current therapy, promising therapeutic directions and outcome with a focus on metastatic disease. Potential advances in terms of promising therapy and biologic insights may lead to more effective and safer therapies; however, more clinical trials and research are needed for patients with metastatic sarcoma. PMID:23700373

  6. 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

  7. 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

  8. Kaposi sarcoma associated with lipoedema.

    PubMed

    Ekmekci, T R; Ayabakan, O; Sakiz, D; Koslu, A

    2005-05-01

    Lipoedema is a form of lipodistrophy, which consists of abnormal accumulation of fat in subcutaneous tissue of the lower limbs. It does not cause any disease and it has not been reported association with malignity. We describe a 63-year-old woman occurring of Kaposi sarcoma on the lipoedema base.

  9. Postradiation sarcoma involving the spine

    SciTech Connect

    Sundaresan, N.; Huvos, A.G.; Krol, G.; Hughes, J.E.; Cahan, W.G.

    1986-06-01

    Postradiation sarcomas arising many years after treatment of cancer are long term sequelae of therapy. We describe the clinical features, radiographic findings, and results of treatment in 13 patients with such sarcomas encountered over a 6-year period. Of these patients, 9 had bone sarcomas and the remaining 4 had paraspinal tumors arising from adjacent soft tissue and nerve. The primary cancer for which radiation was given included Hodgkin's disease (4 patients), breast cancer (2 patients), cervix cancer (2 patients), and a variety of others (5 patients). The latent interval to the occurrence of the second neoplasm varied from 6 to 30 years (median, 10 years) after treatment of the original tumor. Despite aggressive treatment, the overall prognosis was poor. The median survival was 8 months, with only 3 surviving more than 2 years. Although rare, postradiation sarcoma should be considered in the differential diagnosis of patients presenting with late onset of spinal pain or neurological symptoms after clinical remission of an original cancer.

  10. Pulmonary Artery Sarcoma - Multimodality Imaging.

    PubMed

    Jeong, Nari; Seol, Sang-Hoon; Kim, Il Hwan; Kim, Ji Yeon

    2016-01-01

    Pulmonary artery sarcoma (PAS) is a rare and fatal disease. PAS can often be misdiagnosed as pulmonary thromboembolism. Moreover, the correct diagnosis is frequently delayed due to nonspecific signs and symptoms. The prognosis of patients with PAS is poor. We report a case of a woman with a primary PAS who was initially diagnosed with pulmonary thromboembolism.

  11. Pulmonary Artery Sarcoma - Multimodality Imaging

    PubMed Central

    Jeong, Nari; Seol, Sang-Hoon; Kim, Il Hwan; Kim, Ji Yeon

    2016-01-01

    Pulmonary artery sarcoma (PAS) is a rare and fatal disease. PAS can often be misdiagnosed as pulmonary thromboembolism. Moreover, the correct diagnosis is frequently delayed due to nonspecific signs and symptoms. The prognosis of patients with PAS is poor. We report a case of a woman with a primary PAS who was initially diagnosed with pulmonary thromboembolism. PMID:27833785

  12. The Biology of Ewing Sarcoma

    PubMed Central

    Ross, Keir A.; Smyth, Niall A.; Murawski, Christopher D.; Kennedy, John G.

    2013-01-01

    Objective. The goal of this study was to review the current literature on the biology of Ewing's sarcoma, including current treatments and the means by which an understanding of biological mechanisms could impact future treatments. Methods. A search of PubMed and The Cochrane Collaboration was performed. Both preclinical and clinical evidence was considered, but specific case reports were not. Primary research articles and reviews were analyzed with an emphasis on recent publications. Results. Ewing sarcoma is associated with specific chromosomal translocations and the resulting transcripts/proteins. Knowledge of the biology of Ewing sarcoma has been growing but has yet to significantly impact or produce new treatments. Localized cases have seen improvements in survival rates, but the same cannot be said of metastatic and recurrent cases. Standard surgical, radiation, and chemotherapy treatments are reaching their efficacy limits. Conclusion. Improving prognosis likely lies in advancing biomarkers and early diagnosis, determining a cell(s) of origin, and developing effective molecular therapeutics and antiangiogenic agents. Preclinical evidence suggests the utility of molecular therapies for Ewing sarcoma. Early clinical results also reveal potential for novel treatments but require further development and evaluation before widespread use can be advocated. PMID:23346417

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

    ClinicalTrials.gov

    2017-10-12

    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

  14. CCI-779 in Treating Patients With Soft Tissue Sarcoma or Gastrointestinal Stromal Tumor

    ClinicalTrials.gov

    2013-06-03

    Gastrointestinal Stromal Tumor; Recurrent Adult 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

  15. What Are the Key Statistics about Kaposi Sarcoma?

    MedlinePlus

    ... Sarcoma About Kaposi Sarcoma What Are the Key Statistics About Kaposi Sarcoma? Before the AIDS epidemic, Kaposi ... and children. Visit the American Cancer Society’s Cancer Statistics Center for more key statistics. Written by References ...

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

    MedlinePlus

    ... and Treatment? Kaposi Sarcoma About 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) ...

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

    MedlinePlus

    ... and Treatment? Uterine Sarcoma About 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 ...

  18. What Are the Key Statistics about Uterine Sarcoma?

    MedlinePlus

    ... Sarcoma About Uterine Sarcoma What Are the Key Statistics About Uterine Sarcoma? The American Cancer Society's estimates ... uterine corpus. Visit the American Cancer Society’s Cancer Statistics Center for more key statistics. Written by References ...

  19. Ribociclib and Doxorubicin in Treating Patients With Metastatic or Advanced Soft Tissue Sarcomas That Cannot Be Removed by Surgery

    ClinicalTrials.gov

    2017-04-10

    Metastatic Angiosarcoma; Metastatic Epithelioid Sarcoma; Metastatic Fibrosarcoma; Metastatic Leiomyosarcoma; Metastatic Liposarcoma; Metastatic Malignant Peripheral Nerve Sheath Tumor; Metastatic Synovial Sarcoma; Metastatic Undifferentiated Pleomorphic Sarcoma; Myxofibrosarcoma; Pleomorphic Rhabdomyosarcoma; Stage III Soft Tissue Sarcoma; Stage IV Soft Tissue Sarcoma; Undifferentiated (Embryonal) Sarcoma

  20. Alveolar bone and the bisphosphonates.

    PubMed

    Cheng, A; Daly, C G; Logan, R M; Stein, B; Goss, A N

    2009-09-01

    Bisphosphonate associated osteonecrosis of the jaws (ONJ) usually commences at the alveolus. Comparison is made between the structure and function of long bones and alveolar bone and the differing susceptibilities of the bisphosphonates at these different sites are explored. Current concepts of the causation of ONJ are discussed. The clinical implications of these findings to dentists managing periodontal conditions are presented.

  1. Primary diffuse alveolar septal amyloidosis.

    PubMed Central

    Poh, S C; Tjia, T S; Seah, H C

    1975-01-01

    The case is reported of a 61-year-old man with primary diffuse alveolar septal pulmonary amyloidosis. Amyloid infiltration of the heart and other organs was also observed. The clinical findings and laboratory investigations reveal features characteristic of defective gas transfer with pulmonary oedema due to left ventricular failure from myocardial involvement. Images PMID:1179316

  2. Noncoding RNA in drug resistant sarcoma

    PubMed Central

    Li, Xiaoyang; Shen, Jacson K.; Hornicek, Francis J.; Xiao, Tao; Duan, Zhenfeng

    2017-01-01

    Sarcomas are a group of malignant tumors that arise from mesenchymal origin. Despite significant development of multidisciplinary treatments for sarcoma, survival rates have reached a plateau. Chemotherapy has been extensively used for sarcoma treatment; however, the development of drug resistance is a major obstacle limiting the success of many anticancer agents. Sarcoma biology has traditionally focused on genomic and epigenomic deregulation of protein-coding genes to identify the therapeutic potential for reversing drug resistance. New and more creative approaches have found the involvement of noncoding RNAs, including microRNAs and long noncoding RNAs in drug resistant sarcoma. In this review, we discuss the current knowledge of noncoding RNAs characteristics and the regulated genes involved in drug resistant sarcoma, and focus on their therapeutic potential in the future.

  3. Sarcomas related to the heart and vasculature.

    PubMed

    Raaf, H N; Raaf, J H

    1994-01-01

    Soft tissue sarcoma is the most common malignant neoplasm of the heart, pericardium, and great vessels. Its presentation is infrequent, nonspecific, and subtle. For example, emboli from these tumors to the lungs or peripheral arteries may mimic thrombotic embolic disease. New noninvasive techniques such as echocardiography and magnetic resonance imaging (MRI) aid in diagnosis and preoperative assessment. Angiosarcoma, the most common cardiac sarcoma, is aggressive and usually arises in the right atrium. Kaposi's sarcoma of the heart has been found in patients with AIDS and in immunosuppressed organ transplant recipients. Most primary sarcomas of the aorta and pulmonary artery (the elastic arteries) show minimal differentiation and are classified as "intimal, sarcomas," whereas leiomyosarcomas predominate in the muscular arteries and great veins. Surgical resection of any sarcoma of the vasculature, when feasible, is technically challenging but may result in cure or palliation. Adjuvant chemotherapy and radiation therapy can also relieve symptoms and prolong survival.

  4. Soft Tissue Sarcomas of the Kidney

    PubMed Central

    Köhle, Olivia; Abt, Dominik; Rothermundt, Christian; Öhlschlegel, Christian; Brugnolaro, Christiane; Schmid, Hans-Peter

    2015-01-01

    Soft tissue sarcomas are rare mesenchymal tumors. Amongst others, primitive neuroectodermal tumors (PNET) of the kidney and synovial sarcoma of the kidney belong to the group of soft tissue sarcomas. Synovial sarcomas can occur almost anywhere in the body, most frequently, however, in the lower (62%) or upper extremities (21%). Metastases occur in 50-70% of cases, and thus the prognosis is poor. PNETs are rare, highly aggressive neoplastic lesions which mainly occur in the torso or axial skeleton in young adults. The prognosis is poor with a 5-year disease-free survival rate of 45-55%. The primary therapeutic approach is surgical resection. Most randomized studies assessing adjuvant chemotherapy for all types of localized soft tissue sarcomas did not show statistically significantly better overall survival times after chemotherapy, although they did show longer progression-free survival. We report on two cases of primary renal synovial sarcoma and one case of PNET of the kidney. PMID:25918607

  5. 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.

  6. Kaposi sarcoma: review and medical management update.

    PubMed

    Fatahzadeh, Mahnaz

    2012-01-01

    Despite recent advances in our understanding of pathogenic mechanisms involved, the true nature of Kaposi sarcoma remains an enigma. Four clinical variants have been described for the disease, differing in natural history, site of predilection, and prognosis. All forms of Kaposi sarcoma may manifest in the oral cavity and Kaposi sarcoma-associated virus appears essential to development of all clinical variants. The spectrum of therapeutic strategies is broad and selection of appropriate intervention mandates a thorough understanding of disease spread and the patient's symptomatology, as well as risks and benefits of therapy. This article provides an overview of epidemiology, subtypes, clinical course, pathogenesis, and management strategies for Kaposi sarcoma.

  7. Intimal sarcoma of the pulmonary valve.

    PubMed

    Scheidl, Stefan; Taghavi, Shahrokh; Reiter, Ursula; Tröster, Natascha; Kovacs, Gabor; Rienmüller, Rainer; Lang, Susanna; Klepetko, Walter; Olschewski, Horst

    2010-04-01

    Pulmonary artery intimal sarcoma is a rare tumor of the cardiovascular system. Intimal sarcoma of the pulmonary valve itself has not been described. Embolization into pulmonary arteries originating from the pulmonary valve intimal sarcoma can mimic chronic thromboembolic pulmonary hypertension and mislead the diagnosis. We present and discuss a patient initially diagnosed as chronic thromboembolic pulmonary hypertension, treated by pulmonary endarterectomy. After 24 months, a tumor of the pulmonary valve was detected by echocardiography. The patient underwent removal and replacement of the pulmonary valve. Histology revealed pulmonary valve intimal sarcoma.

  8. Myeloid sarcoma causing airway obstruction

    PubMed Central

    Krause, John R.

    2017-01-01

    Myeloid sarcoma is an extramedullary collection of blasts of the myeloid series that partially or totally effaces the architecture of the tissue in which it is found. These tumors have been described in many sites of the body, but the skin, lymph nodes, gastrointestinal tract, bone, soft tissue, and testes are most common. They can arise in a patient following the diagnosis of acute myeloid leukemia, but they may also be precursors of leukemia and should be considered diagnostic for acute myeloid leukemia. The differential diagnosis of this neoplasm includes malignant lymphoma, with which it is often mistaken, leading to diagnostic and therapeutic delays. We present the case of an 84-year-old African American man with a history of renal disease secondary to hypertension and coronary artery disease without any prior history of malignancies who presented with airway obstruction. He was diagnosed with a myeloid sarcoma of the mediastinum compressing his trachea. PMID:28405080

  9. Olaratumab for soft tissue sarcoma.

    PubMed

    Teyssonneau, Diego; Italiano, Antoine

    2017-08-01

    Soft tissue sarcomas (STS) are rare malignant tumors. Unfortunately, the first-line doxorubicin-based treatment has not been improved since the 1970s. Platelet-derived growth factor (PDGF) receptor alpha (PDGFR-α) and its ligands are co-expressed in many types of cancer, including sarcomas. They are involved in stimulating growth and regulating stromal-derived fibroblasts and angiogenesis. PDGFR-α and its ligand may play an important role in tumorigenesis and be a potential target in the treatment of sarcomas. Olaratumab is a fully human IgG1-type anti-PDGFR-α monoclonal antibody with a high affinity and a low 50% inhibitory concentration (IC50). Areas covered: The authors review the role of olaratumab in the treatment of STS by focusing on the recent, randomized Phase II JDGD trial that challenged patients with unresectable or metastatic STS with doxorubicin in the presence or absence of olaratumab. This trial showed a great improvement in overall survival (OS), with an increase in survival from 14.7 months to 26.5 months for patients in the experimental arm and showed acceptable toxicity. Expert opinion: Results seem promising. However, it must be qualified, as the study includes several uncertainties. These uncertainties should be addressed by the ongoing Phase 3 JGDJ confirmatory trial, for which the final efficacy analysis is expected by 2019.

  10. [Primary sarcomas of the breast].

    PubMed

    Chirife, Ana M; Bello, Lorena; Celeste, Francisco; Giménez, Liliana; Gorostidy, Susana

    2006-01-01

    Primary sarcomas of the breast are extremely rare with less than 1% of all malignant tumours of the breast reported in literature. At our Institution 1315 malignant tumours of the breast were diagnosed between 1999-2004; nine of them corresponded to primary sarcomas: angiosarcoma (3), leiomyosarcoma (1), low-grade fibromyxoid sarcoma (1), dematofibrosarcoma protuberans (1), liposarcoma (1), osteosarcoma (1), malignant peripheral nerve sheath tumour (1). Histopathological specimens stained with routine techniques and immunoperoxidase were reviewed; proliferation index and p53 over-expression were also determined. Patients' clinical reports were also reviewed to determine prognosis (favorable and unfavorable). The incidence observed (0.7%) is similar to those already published by others authors. Proliferation index was correlated with type of evolution, being an unfavourable prognosis factor when it was equal or major to 30%. Most of the tumours (67%) showed p53 (mayor or equal to 20% of nuclear staining) over-expression but this did not show a direct relationship with the evolution of each neoplasm.

  11. Pathology of soft tissue sarcomas.

    PubMed

    Thway, K

    2009-11-01

    Sarcomas are a rare, complex group of childhood and adult neoplasms with differentiation towards mesenchymal tissue, which may arise almost anywhere in the body. Although pathologically diverse, they frequently exhibit similar clinical presentations and radiological features. Correct histopathological diagnosis is therefore crucial, but there is overlap between histological patterns of malignant tumours, between benign and malignant lesions, and with non-mesenchymal tumours. Immunohistochemistry and molecular genetic techniques, the latter to detect tumour-specific alterations, add significantly to histological interpretation, but several groups of tumours still lack reliable immunohistochemical markers or reproducible genetic changes. The classification of sarcomas is incomplete and continues to evolve, and although the biology of many remains relatively poorly understood, our increasing insight into molecular events occurring in these tumours is certain to aid future diagnosis and therapy. This paper aims to give a broad overview of several of the main soft tissue sarcomas from a clinicopathological perspective, discussing laboratory diagnosis and the use and limitations of ancillary investigations, including recent developments in molecular diagnosis.

  12. 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

  13. 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.

  14. 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

  15. Gastrointestinal Kaposi sarcoma with appendiceal involvement.

    PubMed

    Egwuonwu, Steve; Gatto-Weis, Cara; Miranda, Roberto; Casas, Luis De Las

    2011-04-01

    Kaposi sarcoma is a vascular tumor manifesting as nodular lesions on skin, mucous membranes, or internal organs. This is a case of a 42-year-old human immunodeficiency virus- (HIV) positive bisexual male, not on highly active antiretroviral therapy (HAART) since diagnosis four years ago. He presented with a three-day history of abdominal pains, fever, vomiting, and a one-week history of melena stools. Endoscopy revealed Kaposi sarcoma in the stomach and duodenum. Postendoscopy, he developed acute abdomen. Exploratory laparotomy revealed extensive Kaposi sarcoma of the gastrointestinal tract with appendiceal involvement. The patient underwent appendectomy and had an uneventful recovery. A review of the literature discusses appendiceal Kaposi sarcoma with appendicitis, a rare but critical manifestation of gastrointestinal Kaposi sarcoma.

  16. Ewing's Sarcoma Localized in the Mandible: A Case Report.

    PubMed

    Akbayram, S; Başaranoglu, M; Kaya, A; Açıkgöz, M; Üstyol, L; Taşkın, G A; Dogan, M

    2015-09-01

    Ewing's sarcoma is one of the most aggressive primary bone tumours. Ewing's sarcoma arising from the bones of the head and neck region is extremely rare; only 4-9% of all Ewing's sarcoma originate in this region. We report a case of Ewing's sarcoma localized in the mandible because of its unusual presentation.

  17. Socioeconomic factors and the risk for sarcoma.

    PubMed

    Hampras, Shalaka S; Moysich, Kirsten B; Marimuthu, Sathiya P; Ravi, Vinod; Jayaprakash, Vijayvel

    2014-11-01

    Sarcomas are a heterogeneous group of rare malignancies arising from mesenchymal tissue. Although several occupational exposures have been evaluated in association with sarcoma, little is known about the role of socioeconomic indicators such as education. Socioeconomic status has been found to be associated with risk of development of several types of cancers, primarily lung, gastric, and cervical cancers. We conducted a hospital-based case-control study to evaluate the association of socioeconomic level with the risk for sarcoma. A total of 371 incident cases of sarcoma were matched in terms of age, sex, and year of enrollment in the study with 742 cancer-free controls. Education and income levels were evaluated as the indicators of socioeconomic status. Higher education (college level) was associated with a significantly lower risk for sarcoma [odds ratio (OR)=0.48, 95% confidence interval (CI)=0.29-0.80], even after adjusting for important confounders. After stratifying by sex, significantly lower risk for sarcoma was observed among men who had college level education compared with men with a level of education of eighth grade or lower (OR=0.38, 95% CI=0.19-0.74). A significant association between education and the risk for sarcoma remained after stratifying by income (OR=0.49, 95% CI=0.28-0.86, among the low income group). When analyzed as a composite exposure, individuals with high education and high income status had significantly lower risk for sarcoma compared with those with low income and low education status (OR=0.41, 95% CI=0.23-0.71). Thus, socioeconomic factors may play a significant role in determining the risk for sarcoma and should be explored further to elucidate the underlying factors that may explain these sociodemographic inequalities related to sarcoma.

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

    ClinicalTrials.gov

    2017-04-11

    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

  19. Systemic Analysis of Gene Expression Profiles Identifies ErbB3 as a Potential Drug Target in Pediatric Alveolar Rhabdomyosarcoma

    PubMed Central

    Nordberg, Janne; Mpindi, John Patrick; Iljin, Kristiina; Pulliainen, Arto Tapio; Kallajoki, Markku; Kallioniemi, Olli; Elenius, Klaus; Elenius, Varpu

    2012-01-01

    Pediatric sarcomas, including rhabdomyosarcomas, Ewing’s sarcoma, and osteosarcoma, are aggressive tumors with poor survival rates. To overcome problems associated with nonselectivity of the current therapeutic approaches, targeted therapeutics have been developed. Currently, an increasing number of such drugs are used for treating malignancies of adult patients but little is known about their effects in pediatric patients. We analyzed expression of 24 clinically approved target genes in a wide variety of pediatric normal and malignant tissues using a novel high-throughput systems biology approach. Analysis of the Genesapiens database of human transcriptomes demonstrated statistically significant up-regulation of VEGFC and EPHA2 in Ewing’s sarcoma, and ERBB3 in alveolar rhabdomyosarcomas. In silico data for ERBB3 was validated by demonstrating ErbB3 protein expression in pediatric rhabdomyosarcoma in vitro and in vivo. ERBB3 overexpression promoted whereas ERBB3-targeted siRNA suppressed rhabdomyosarcoma cell gowth, indicating a functional role for ErbB3 signaling in rhabdomyosarcoma. These data suggest that drugs targeting ErbB3, EphA2 or VEGF-C could be further tested as therapeutic targets for pediatric sarcomas. PMID:23227212

  20. Sarcomas other than Kaposi sarcoma occurring in immunodeficiency: interpretations from a systematic literature review.

    PubMed

    Bhatia, Kishor; Shiels, Meredith S; Berg, Alexandra; Engels, Eric A

    2012-09-01

    In immunodeficiency, an increased sarcoma risk is confirmed for Kaposi's sarcoma. Whether rates of other sarcoma subtypes are elevated in the setting of immunodeficiency is not known. We therefore reviewed published case reports on HIV and AIDS patients and organ transplant recipients with sarcomas. For comparison, we assessed sarcomas in the U.S. general population using Surveillance Epidemiology End Results (SEER) data. A total of 176 non-Kaposi sarcoma were identified, 75 in people with HIV and AIDS and 101 in transplant recipients. Leiomyosarcomas (n = 101) were the most frequently reported sarcomas, followed by angiosarcomas (n = 23) and fibrohistiocytic tumors (n = 17). Leiomyosarcomas were reported with two age peaks, in children and young adults. Epstein-Barr virus (EBV) was detected in the tumor cells in 85 and 88% of leiomyosarcomas in HIV-infected people and transplant recipients, respectively. Angiosarcomas and fibrohistiocytic tumors were most frequently reported in men. Among kidney transplant recipients, 20% of sarcomas arose at the site of an arteriovenous fistula. In comparison, leiomyoscarcomas, angiosarcomas, and fibrohistiocytic tumors comprised 16.9, 3.8, and 18.7% of sarcomas in the U.S. general population. Leiomyosarcoma and angiosarcoma may occur disproportionately in immunodeficiency. Leiomyosarcomas appear causatively linked to EBV, whereas angiosarcomas might be correlated with an arteriovenous fistula. Additional studies are necessary to understand the contribution of immunodeficiency to the cause of these sarcomas.

  1. [Pulmonary alveolar microlithiasis. Study of pulmonary circulation].

    PubMed

    Orea Tejeda, A; Atencio, C; Sandoval, J; Lupi Herrera, E

    1982-01-01

    Pulmonary alveolar microlithiasis is a rare disease of unknown etiology which consists of alveolar deposit of calcium microspheres. We report the procedures for the diagnosis of this disease, as well as the hemodynamic features of the pulmonary circulation. Pulmonary arterial hypertension (PAH), and cor pulmonale were documented. The active and passive factors involved in PAH are analyzed. We conclude that alveolar hypoxia and estructural vascular changes play a major role in the genesis of PAH.

  2. [Disseminated interdigitating dendritic cell sarcoma].

    PubMed

    Santarelli, Ignacio M; Veltri, Mariano; Manzella, Diego J; Avagnina, María Alejandra; Pereyra, Pablo M; Chavín, Hernán C

    2017-01-01

    A 70 year-old woman was admitted to our hospital with a 3-month history of abdominal pain, weight loss and night sweats. On physical examination, she presented with a 5 cm diameter abdominal mass extended from epigastrium to the left flank, and at least three bilateral supraclavicular adenopathies. A disseminated interdigitating dendritic cell sarcoma was diagnosed through a biopsy of the abdominal mass. After that, a CHOP regime (cyclophosphamide, doxorubicin, vincristine and prednisone) was iniciated. She died after completion of the first cycle of treatment, six months after diagnosis.

  3. 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

  4. A new probable stem lineage crustacean with three-dimensionally preserved soft parts from the Herefordshire (Silurian) Lagerstätte, UK

    PubMed Central

    Siveter, Derek J; Sutton, Mark D; Briggs, Derek E.G; Siveter, David J

    2007-01-01

    A new arthropod with three-dimensionally preserved soft parts, Tanazios dokeron, is described from the Wenlock Series (Silurian) of Herefordshire, England, UK. Serial grinding, digital photographic and computer rendering techniques yielded ‘virtual fossils’ in the round for study. The body tagmata of T. dokeron comprise a head shield and a long trunk. The head shield bears six pairs of horn-like spines and the head bears five pairs of appendages. The antennule, antenna and mandible are all uniramous, and the mandible includes a gnathobasic coxa. Appendages four and five are biramous and similar to those of the trunk: each comprises a limb base with an endite, an enditic membrane, and two epipodites, plus an endopod and exopod. The hypostome bears a large cone-like projection centrally, and there may be a short labrum. The trunk has some 64 segments and at least 60 appendage pairs. A very small telson has the anus sited ventrally in its posterior part and also bears a caudal furca. Comparative morphological and cladistic analyses of T. dokeron indicate a crustacean affinity, with a probable position in the eucrustacean stem group. As such the epipodites in T. dokeron are the first recorded in a eucrustacean stem taxon. The new species is interpreted as a benthic or nektobenthic scavenger. PMID:17609185

  5. Digestive and appendicular soft-parts, with behavioural implications, in a large Ordovician trilobite from the Fezouata Lagerstätte, Morocco

    PubMed Central

    Gutiérrez-Marco, Juan C.; García-Bellido, Diego C.; Rábano, Isabel; Sá, Artur A.

    2017-01-01

    Trilobites were one of the most successful groups of marine arthropods during the Palaeozoic era, yet their soft-part anatomy is only known from a few exceptionally-preserved specimens found in a handful of localities from the Cambrian to the Devonian. This is because, even if the sclerotized appendages were not destroyed during early taphonomic stages, they are often overprinted by the three-dimensional, mineralised exoskeleton. Inferences about the ventral anatomy and behavioural activities of trilobites can also be derived from the ichnological record, which suggests that most Cruziana and Rusophycus trace fossils were possibly produced by the actions of trilobites. Three specimens of the asaphid trilobite Megistaspis (Ekeraspis) hammondi, have been discovered in the Lower Ordovician Fezouata Konservat-Lagerstätte of southern Morocco, preserving appendages and digestive tract. The digestive structures include a crop with digestive caeca, while the appendages display exopodal setae and slight heteropody (cephalic endopods larger and more spinose than thoracic and pygidial ones). The combination of these digestive structures and the heteropody has never been described together among trilobites, and the latter could assist in the understanding of the production of certain comb-like traces of the Cruziana rugosa group, which are extraordinarily abundant on the shallow marine shelves around Gondwana. PMID:28071705

  6. Digestive and appendicular soft-parts, with behavioural implications, in a large Ordovician trilobite from the Fezouata Lagerstätte, Morocco.

    PubMed

    Gutiérrez-Marco, Juan C; García-Bellido, Diego C; Rábano, Isabel; Sá, Artur A

    2017-01-10

    Trilobites were one of the most successful groups of marine arthropods during the Palaeozoic era, yet their soft-part anatomy is only known from a few exceptionally-preserved specimens found in a handful of localities from the Cambrian to the Devonian. This is because, even if the sclerotized appendages were not destroyed during early taphonomic stages, they are often overprinted by the three-dimensional, mineralised exoskeleton. Inferences about the ventral anatomy and behavioural activities of trilobites can also be derived from the ichnological record, which suggests that most Cruziana and Rusophycus trace fossils were possibly produced by the actions of trilobites. Three specimens of the asaphid trilobite Megistaspis (Ekeraspis) hammondi, have been discovered in the Lower Ordovician Fezouata Konservat-Lagerstätte of southern Morocco, preserving appendages and digestive tract. The digestive structures include a crop with digestive caeca, while the appendages display exopodal setae and slight heteropody (cephalic endopods larger and more spinose than thoracic and pygidial ones). The combination of these digestive structures and the heteropody has never been described together among trilobites, and the latter could assist in the understanding of the production of certain comb-like traces of the Cruziana rugosa group, which are extraordinarily abundant on the shallow marine shelves around Gondwana.

  7. Primary osteogenic sarcoma of the breast

    PubMed Central

    Ogundiran, Temidayo O; Ademola, Samuel A; Oluwatosin, Odunayo M; Akang, Effiong E; Adebamowo, Clement A

    2006-01-01

    Background Primary extra-osseous osteogenic sarcomas have been reported in many tissues of the body but their occurrence in the breast is extremely rare. It can arise as a result of osseous metaplasia in a pre-existing benign or malignant neoplasm of the breast or as non-phylloides sarcoma from the soft tissue of a previously normal breast. Case presentation A 40 year-old Nigerian woman was clinically diagnosed to have carcinoma of the left breast. The histology report of core-needle biopsy of the mass showed a malignant neoplasm comprising islands of chondroblastic and osteoblastic stromal cells. This report changed the diagnosis from carcinoma to osteogenic sarcoma of the breast. She had a left modified radical mastectomy, however there was significant post surgery skin deficit. A latissimus dorsi musculocutaneous flap was used to cover the anterior chest wall defect. Sections from the mastectomy specimen confirmed the diagnosis of osteogenic sarcoma. She died six months after mastectomy. Conclusion A diagnosis of osteogenic sarcoma of the breast was made based on histology report and after excluding an osteogenic sarcoma arising from underlying ribs and sternum. This is the second documented case of primary osteogenic sarcoma of the breast coming from Nigeria PMID:17156481

  8. [Demographic Analysis of Patients with Osteosarcoma, Chonddrosarcoma, Ewing's Sarcoma from one Sarcoma Center in Switzerland].

    PubMed

    Hodel, Sandro; Seeli, Franziska; Fuchs, Bruno

    2015-06-17

    Retrospective analysis of presentation, diagnosis and outcome of patients with osteosarcoma, chondrosarcoma and Ewing's sarcoma was performed for a single Sarcoma Center in Zurich at the University Hospital Balgrist. 201 patients were included. Overall survival at five and ten years were 74 ± 6%, 69 ± 7% for osteosarcoma (n = 85, since 2000), 85 ± 7%, 80 ± 9% for Ewing's sarcoma (n = 43, since 1990) and 86 ± 5%, 78 ± 9% for chondrosarcoma (n = 73, since 2000). The here presented overall survival rates from a single Sarcoma Center in Switzerland appear to be equivalent to other large international monocenter studies. The presentation and epidemiology of these patients are in accordance with large multicenter epidemiological studies. A nationwide sarcoma database (SwissSARCOS; www.sarcoma.ch) seems indispensable for more detailed analysis and quality management in such rare diseases.

  9. Radiation therapy for retroperitoneal sarcoma.

    PubMed

    Tuan, Jeffrey; Vitolo, Viviana; Vischioni, Barbara; Iannalfi, Alberto; Fiore, Maria Rosaria; Fossati, Piero; Orecchia, Roberto

    2014-10-01

    Retroperitoneal sarcomas (RPS) are rare tumours with an annual reported incidence of 2.7 per million persons. In spite of improvements in both diagnostic imaging and therapeutic strategies, patients afflicted by RPS still have poor prognoses. There are currently many different therapeutic strategies for these rare tumours and combining several different multi-modality strategies have not proved to have superior long-term clinical results. This review analyses the available published data and discusses multi-modality management of this rare entity. In particular, the role of radiation therapy, treatment-related side effects and the use of modern radiation treatment techniques will be discussed. A comprehensive literature search was conducted using PubMed in January 2011. Relevant international articles published from January 1980 to January 2011 were assessed. The keywords for search purposes were: retroperitoneum, sarcoma, radiotherapy, and radiation therapy. The search was limited to articles published in English. All articles were read in full by the authors and selected for inclusion based on relevance to this article. The addition of radiation therapy (RT) to wide surgical excision for RPS has improved local control rates when compared with surgery alone. Preoperative RT is preferred over postoperative RT. New types and delivery techniques in radiation therapy could further improve patient outcomes. Emerging therapies that employ charged particles (such as protons and carbon ions) are expected to be superior in sparing of normal tissues and efficacy over conventional photon therapy radiation, due to their physical and radiobiological properties.

  10. [AIDS-associated Kaposi's sarcoma: 22 cases].

    PubMed

    Dhrif, Asma Sioud; Kilani, Badreddine; Ammari, Lamia; Kanoun, Fakher; Tiouri, H; Ben Chaaben, Taoufik

    2007-06-01

    Kaposi's sarcoma is the most common acquired immune deficiency syndrome (AIDS)-associated malignancy. Our aim was to analyse the epidemiological, clinical, therapeutic findings in AIDS patients with Kaposi's sarcoma. This was a retrospective chart review of AIDS patients with Kaposi's sarcoma diagnosed between 1991 and 2005. Epidemiological data, the stage of human immunodeficiency virus's (HIV) infection, clinical characteristics of Kaposi's sarcoma, treatment rendered and outcome were collected. The search of HHV8 was not done. Twenty two patients were included. They were 17 men and 5 females (sex-ratio=3.4/ 1) with a mean age of 33.6 years at the diagnosis of HIV infection. The Kaposi's sarcoma appeared after a period varying between 0 and 10 years. The Kaposi's sarcoma uncovered the infection in 5 cases. There were 6 homosexual men. The mean rate of CD4 was 216 21/mm3 at the diagnosis of Kaposi's sarcoma. All patients had skin lesions. Mucocutaneous lesions were isolated in 12 cases and associated with visceral involvement in 10 cases; lung (10 cases), gastrointestinal tract (5 cases), lymphadenopathy (5 cases), liver (4 cases), spleen (2 cases). Antiretroviral therapy was prescribed for 13 patients. Six patients received chemotherapy and 3 others radiotherapy. Outcome was favourable in 4 cases with a partial improvement of the skin lesions in 3 cases and a complete regression in 1 case. Twelve patients died. AIDS associated Kaposi's sarcoma is a severe condition because of visceral localisations and the field of immunodeficiency. It requires a precocious diagnosis and collaboration. The identification of HHV8 in the aetiopathogenic mechanism of Kaposi's sarcoma can lead to the development new therapeutic approaches.

  11. 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

  12. Ewing's sarcoma of scapula: a rare entity.

    PubMed

    Shahid, Mohammad; Varshney, Manoranjan; Maheshwari, Veena; Mubeen, Aysha; Siddiqui, Mohammed Azfar; Julfiqar, Julfiqar; Gaur, Kavita

    2011-03-10

    Ewing's sarcoma is the second most common malignant bone tumour of childhood and adolescence. It may affect any bone, but it is frequent in the femur, ilium and the tibia. Here the authors are reporting a case of Ewing's sarcoma involving scapula in a 14-year-old boy presenting with pain and swelling around the shoulder. Swelling was removed which on histopathological examination showed solid sheets closely packed, poorly differentiated small cells that have a high nuclear-cytoplasmic ratio, fine chromatin and small nucleoli. A tentative diagnosis of Ewing's sarcoma was made which was confirmed by immunohistochemistry.

  13. Cell Cycle Deregulation in Ewing's Sarcoma Pathogenesis

    PubMed Central

    Kowalewski, Ashley A.; Randall, R. Lor; Lessnick, Stephen L.

    2011-01-01

    Ewing's sarcoma is a highly aggressive pediatric tumor of bone that usually contains the characteristic chromosomal translocation t(11;22)(q24;q12). This translocation encodes the oncogenic fusion protein EWS/FLI, which acts as an aberrant transcription factor to deregulate target genes necessary for oncogenesis. One key feature of oncogenic transformation is dysregulation of cell cycle control. It is therefore likely that EWS/FLI and other cooperating mutations in Ewing's sarcoma modulate the cell cycle to facilitate tumorigenesis. This paper will summarize current published data associated with deregulation of the cell cycle in Ewing's sarcoma and highlight important questions that remain to be answered. PMID:21052502

  14. Ewing's Sarcoma of the Adrenal Gland.

    PubMed

    Pal, Dilip Kumar; Chandra, Vipin; Ranjan, Kumar Rajiv; Chakrabortty, Debasis; Banerjee, Manju

    2016-01-01

    Ewing's sarcoma (ES) or primitive neuro-ectodermal tumor (PNET) typically occurs in long or flat bones, the chest wall, extra-skeletal soft tissue, and rarely in solid organs. Incidence of adrenal Ewing's sarcoma is very rare. Here we report a case of Ewing's sarcoma of the right adrenal gland in an 8-year-old girl who presented with an abdominal mass. The huge tumor was managed by preoperative neo-adjuvant chemotherapy followed by surgical resection. She died due to metastasis after five months of surgery.

  15. [Synovial sarcoma of the infratemporal fossa].

    PubMed

    Tamarit Conejeros, José Manuel; Estrems Navas, Paloma; Estellés Ferriol, Enrique; Dalmau Galofre, José

    2010-01-01

    Synovial sarcoma is the fourth most common type of sarcoma. It is usually found in the knee or ankle joints, and is exceptional in the head and neck. Most cases are diagnosed in men between 20 and 40 years of age. Diagnosis is often casual due to the infrequent nature of this tumour and its non-specific clinical and radiological characteristics. Confirmation is therefore based on immunohistochemistry and electron microscopy techniques. We report a case of biphasic sinovial sarcoma located in the infratemporal fossa treated at our hospital and we make a review of the literature. Copyright © 2009 Elsevier España, S.L. All rights reserved.

  16. Kaposi's sarcoma with HHV8 infection and ANCA-associated vasculitis in a hemodialysis patient.

    PubMed

    Fatma, Lilia Ben; Rais, Lamia; Mebazza, Amel; Azzouz, Haifa; Beji, Somaya; Krid, Madiha; Smaoui, Wided; Maiz, Hedi Ben; Zouaghi, Karim; Zitouna, Moncef; Osmane, Amel Ben; Moussa, Fatma Ben

    2013-11-01

    The association between Kaposi's sarcoma (KS) and human herpes virus eight (HHV-8) infection is rarely reported in hemodialysis (HD) patients. We report here the rare association of KS, HHV-8 and hepatitis C virus (HCV) infection as well as syphilis in a HD patient. We report the case of a 72-year-old woman who presented with microscopic polyangiitis with alveolar hemorrhage and pauci-immune necrosing and crescentic glomerulonephritis as well as renal failure requiring HD. Biological tests showed positive HCV and syphilis tests. The patient was treated by HD and intravenous pulse, followed by oral corticosteroids and six cyclophosphamide monthly pulses with remission of the alveolar hemorrhage, but without renal functional recovery as the patient remained HD dependent. Five months after the first treatment administration, she developed extensive purpuric lesions on her lower limbs, abdomen face and neck. A skin biopsy showed KS. The HHV-8 test was positive, with positive polymerase chain reaction-HHV8 in the serum and skin. After immunosuppression withdrawal, the KS skin lesions regressed rapidly without relapse after 12 months of follow-up, but alveolar hemorrhage relapsed after 16 months of follow-up. Our case showed that the immunosuppressed state related to multiple factors such as aging, vasculitis, HHV-8, HCV, syphilis, immunosuppressive therapy and HD may all have contributed to the development of KS in our patient.

  17. "Second" Primary Cardiac Sarcoma in a Patient With Ewing Sarcoma. Always Expect The Unexpected.

    PubMed

    Di Franco, Antonino; Gaudino, Mario; Weinsaft, Jonathan W; Pun, Shawn C; Narula, Navneet; Khan, Saadat A; Malik, Zahra M; Ohmes, Lucas B; Skubas, Nickolaos J; Girardi, Leonard N

    2017-02-01

    A high suspicion for relapsed metastatic disease must arise when an intracardiac mass is detected in a patient with a recent history of Ewing sarcoma. Nevertheless, the scenario may eventually turn out to be much more complex than expected, and the possibility that the intracardiac tumor may instead be a "second" primary sarcoma, although extremely rare, should also be considered. We describe the first case of concomitant diagnosis of Ewing sarcoma and low-grade myxoid spindle cell sarcoma in the same young patient. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  18. The Systemic Inflammation of Alveolar Hypoxia Is Initiated by Alveolar Macrophage–Borne Mediator(s)

    PubMed Central

    Chao, Jie; Wood, John G.; Blanco, Victor Gustavo; Gonzalez, Norberto C.

    2009-01-01

    Alveolar hypoxia produces widespread systemic inflammation in rats. The inflammation appears to be triggered by activation of mast cells by a mediator released from alveolar macrophages, not by the reduced systemic partial pressure of oxygen (PO2). If this is correct, the following should apply: (1) neither mast cells nor tissue macrophages should be directly activated by hypoxia; and (2) mast cells should be activated when in contact with hypoxic alveolar macrophages, but not with hypoxic tissue macrophages. We sought here to determine whether hypoxia activates isolated alveolar macrophages, peritoneal macrophages, and peritoneal mast cells, and to study the response of the microcirculation to supernatants of these cultures. Rat mesenteric microcirculation intravital microscopy was combined with primary cultures of alveolar macrophages, peritoneal macrophages, and peritoneal mast cells. Supernatant of hypoxic alveolar macrophages, but not of hypoxic peritoneal macrophages, produced inflammation in mesentery. Hypoxia induced a respiratory burst in alveolar, but not peritoneal macrophages. Cultured peritoneal mast cells did not degranulate with hypoxia. Immersion of mast cells in supernatant of hypoxic alveolar macrophages, but not in supernatant of hypoxic peritoneal macrophages, induced mast cell degranulation. Hypoxia induced release of monocyte chemoattractant protein-1, a mast cell secretagogue, from alveolar, but not peritoneal macrophages or mast cells. We conclude that a mediator released by hypoxic alveolar macrophages activates mast cells and triggers systemic inflammation. Reduced systemic PO2 and activation of tissue macrophages do not play a role in this phenomenon. The inflammation could contribute to systemic effects of diseases featuring alveolar hypoxia. PMID:19244200

  19. 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

  20. METASTATIC ANGIOSARCOMA PRESENTING AS DIFFUSE ALVEOLAR HEMORRHAGE

    PubMed Central

    Rai, SP; Barthwal, MS; Bhattacharya, P; Bhargava, S; Pethe, M

    2008-01-01

    Angiosarcoma is a rare malignant neoplasm of the vascular or lymphatic endothelium. Diffuse alveolar hemorrhage is a rare presenting manifestation of angiosarcoma. We describe a case of pulmonary metastasis of angiosarcoma who presented with diffuse alveolar hemorrhage as initial manifestation. PMID:20396655

  1. 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 ...

  2. 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.

  3. Pulmonary Artery Intimal Sarcoma: A Case Report.

    PubMed

    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.

  4. Pulmonary artery sarcoma mimicking a pulmonary embolism.

    PubMed

    Sandhu, A; Yates, T J; Kuriakose, P

    2008-01-01

    Sarcomas involving the lung are a rare occurrence, often a result of metastatic disease from primary malignancies involving the skin, liver, breast or heart. Primary pulmonary artery sarcomas are rarer still, with limited cases reported world-wide and consequently data regarding treatment modalities are sparse and largely experimental. These tumors are often mistaken for a pulmonary embolism and seemingly supported by radiological findings. Patients will often present without symptom resolution despite therapeutic anticoagulation. The following case illustrates how a soft tissue sarcoma of the pulmonary artery can mimic a pulmonary embolism, thus, resulting in both a diagnostic and therapeutic dilemma. A positron emission tomography scan was an invaluable tool in this case, showing increased radiotracer uptake and placing neoplasm at the top of the differential diagnosis. This ultimately led to a biopsy that was vimentin positive, cytokeratin negative and CD117 negative, thus consistent with soft tissue sarcoma.

  5. 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

  6. [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. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  7. Radiation Therapy for Soft Tissue Sarcomas

    MedlinePlus

    ... therapy to the brain for metastatic sarcoma include hair loss, headaches, and problems thinking. If given before surgery, ... Care Professionals Programs & Services Breast Cancer Support TLC Hair Loss & Mastectomy Products Hope Lodge® Lodging Rides To Treatment ...

  8. Neurogenic sarcomas of the neck in neurofibromatosis.

    PubMed

    Martin, G; Kleinsasser, O

    1981-01-01

    Based on two observations and a review of the literature, the pathological and clinical findings in sarcomas of the neck in patients with neurofibromatosis are described. Histologically these neurogenic tumours show a manifold picture; in addition to spindle-cell sarcomas pleomorphic structures are to be found, which can be similar to rhabdomyo-, lipo-, chondro-, angio-, or osteogenic sarcomas so that a histological diagnosis of a neurogenic sarcoma cannot always be made without clinical details. Up to the present surgical treatment is preferred; the value of cytostatic therapy and irradiation is controversial. The results of treating these tumours are unsatisfactory. Of 29 cases reported in the literature, only two could be found in which the patient survived without a recurrence for more than five years.

  9. Survival and Margin Status in Head and Neck Radiation-Induced Sarcomas and De Novo Sarcomas.

    PubMed

    Rosko, Andrew J; Birkeland, Andrew C; Chinn, Steven B; Shuman, Andrew G; Prince, Mark E; Patel, Rajiv M; McHugh, Jonathan B; Spector, Matthew E

    2017-04-01

    Objective To describe histologic subtypes and oncologic outcomes among patients with radiation-induced and de novo sarcomas of the head and neck. Study Design Retrospective case series with chart review. Setting Tertiary academic center. Subject and Methods In total, 166 adult patients with sarcoma of the head and neck treated from January 1, 1985, to January 1, 2010, were included. Tumors were characterized as radiation induced (15.1%) vs de novo sarcomas (84.9%). Clinical and tumor characteristics were compared. The primary outcomes were overall survival (OS) and disease-specific survival (DSS). Results Radiation-induced sarcomas were more likely to be high grade ( P = .006) and advanced stage ( P = .03). Chondrosarcoma was more common in de novo tumors ( P = .02) while leiomyosarcoma ( P = .01), sarcoma not otherwise specified ( P = .02), and undifferentiated pleomorphic sarcoma ( P < .001) were more common in radiation-induced sarcomas. Radiation-induced sarcomas were associated with statistically significantly worse DSS ( P = .019) and OS ( P = .005) compared with de novo sarcomas, but when only high-grade soft tissue sarcomas were analyzed, neither DSS ( P = .48) nor OS ( P = .29) differed. Margin status was a significant predictor of survival as both R0 and R1 resections correlated with statistically better DSS and OS compared with R2 ( P < .001) resections and patients treated with radiation therapy/chemoradiation therapy alone ( P = .005). Conclusion Radiation-induced sarcomas of the head and neck correlate with worse survival compared with de novo tumors; however, when controlling for tumor grade and resection status, there is no statistically significant difference in observed outcomes.

  10. Soft tissue sarcoma metastatic to pleura.

    PubMed

    Yildirim, Hüseyin; Metintaş, Muzaffer; Ak, Güntülü; Dündar, Emine; Erginel, Sinan

    2008-01-01

    Almost all cancers can cause distant pleural metastases. However, pleural metastases of soft tissue sarcoma that constitute less than 1% of adult solid malignancy are extremely rare. It is very difficult to distinguish them form sarcomatous malignant mesothelioma on histopathological features. We report a 57 year-old man who presented to us with left chest pain and progressive dyspnea and was diagnosed to have a pleural metastases of soft tissue sarcoma by thoracoscopic biopsy.

  11. 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.

  12. Outcomes of surgery for chest wall sarcomas.

    PubMed

    Putnam, Joe B

    2010-11-01

    Chest wall resection requires wide local excision, negative margins, and adequate reconstruction. Outcomes are generally good to excellent with wide local excision and negative margins. Mortality is nearly 0% to 1% with mild morbidity. Multispecialty surgical teams may be required for more complex situations. Early diagnosis of chest wall sarcomas, confirmation by an experienced sarcoma pathologist, and multidisciplinary discussion before treatment initiation, are all required for optimal and successful therapy.

  13. Primary granulocytic sarcoma of the ovary.

    PubMed

    Sreejith, G; Gangadharan, V P; Elizabath, K A; Preetha, S; Chithrathara, K

    2000-06-01

    Granulocytic sarcomas are rare extramedullary tumors of malignant myeloid precursor cells. Exceedingly rare in childhood, it commonly involves skin, lymph nodes, bone, and the spine. Ovarian involvement is rare. It can arise de novo, precede the development of acute nonlymphocytic leukemia, or be the sole manifestation of relapse. We describe a 26-year-old woman with granulocytic sarcoma of the ovary without any hematologic disorder.

  14. Surgical management of extensive pulmonary artery sarcoma.

    PubMed

    Shehatha, Jaffar; Saxena, Pankaj; Clarke, Belinda; Dunning, John; Konstantinov, Igor E

    2009-04-01

    Primary pulmonary artery sarcoma is a rare tumor that can be misdiagnosed as acute or chronic pulmonary thromboembolic disease. This article reports a patient with a preoperative diagnosis of pulmonary embolism who was found to have an extensive pulmonary artery tumor. Surgical resection of the primary pulmonary artery sarcoma and reconstruction of the central pulmonary arteries, followed by adjuvant chemoradiotherapy, provided significant improvement in his clinical symptoms.

  15. Status postpneumonectomy for pulmonary artery sarcoma.

    PubMed

    Siordia, Juan A; Garlish, Amanda; Truong, Huong

    2015-07-02

    Primary pulmonary artery sarcoma is a rare disease that has a poor survival prognosis due to misdiagnosis with pulmonary thromboembolism or metastatic embolisation, detailed image findings and complicated surgical procedures. Surgical procedures established for treatment include pneumonectomy and pulmonary endarterectomy. Survival after surgery still remains at 1 year survival of 50%. The following case report demonstrates a patient's status postpneumonectomy for pulmonary artery sarcoma that presented with no complications after 1.5-year follow-up.

  16. Undifferentiated Pleomorphic Sarcoma in Mandible

    PubMed Central

    Kim, Chul-Hwan; Jang, Jong-Won; Kim, Moon-Young; Kim, Yong-Hwan; Kim, Hang-Gul; Kim, Joo-Hwan

    2014-01-01

    Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, occurs commonly in the soft tissues in adult, but is rare in the maxillofacial region. It consists of undifferentiated mesenchymal tumor cells resembling histiocytes and fibroblasts. The purpose of this article is to report a case of UPS in the mandible. A 44-year-old patient presented with a painful growing mass in the mandible of two months’ duration. Computed tomography and positron emission tomography-computed tomography revealed an ill-defined heterogenous, hypermetabolic mass about 4 cm in size in the left mandible invading adjacent soft tissues. A left mandiblulectomy and reconstruction with a fibular free flap were performed. Immunohistochemical study gave a diagnosis of UPS. The patient was referred for adjuvant chemotherapy after surgical removal of the tumor. PMID:27489851

  17. Undifferentiated Pleomorphic Sarcoma in Mandible.

    PubMed

    Kim, Chul-Hwan; Jang, Jong-Won; Kim, Moon-Young; Kim, Yong-Hwan; Kim, Hang-Gul; Kim, Joo-Hwan

    2014-11-01

    Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, occurs commonly in the soft tissues in adult, but is rare in the maxillofacial region. It consists of undifferentiated mesenchymal tumor cells resembling histiocytes and fibroblasts. The purpose of this article is to report a case of UPS in the mandible. A 44-year-old patient presented with a painful growing mass in the mandible of two months' duration. Computed tomography and positron emission tomography-computed tomography revealed an ill-defined heterogenous, hypermetabolic mass about 4 cm in size in the left mandible invading adjacent soft tissues. A left mandiblulectomy and reconstruction with a fibular free flap were performed. Immunohistochemical study gave a diagnosis of UPS. The patient was referred for adjuvant chemotherapy after surgical removal of the tumor.

  18. Thromboembolism in pulmonary artery sarcoma.

    PubMed

    Farsad, Mohsen; Pernter, Patrizia; Triani, Antonio; Osele, Luzian; Wiedermann, Christian J

    2009-04-01

    Pulmonary artery sarcoma, although rare, must be considered in the differential diagnosis of pulmonary thromboembolism. Clinically and radiologically, it may imitate pulmonary embolism, making diagnosis difficult and delaying treatment. Patients often have no symptom resolution despite therapeutic anticoagulation. Visualization of filling defects within a pulmonary artery on contrast-enhanced CT cannot reliably differentiate between pulmonary thromboembolism and malignant lesions like leiomyosarcoma. FDG PET-CT offers the potential for identification of malignant lesions. The authors report a case with pulmonary artery thromboembolism due to thrombi formed on a pulmonary artery leiomyosarcoma. Integrated FDG PET-CT showed no FDG-uptake along the major part of the filling defect within the right main pulmonary artery suggesting blood clot and increased uptake along the posterior wall of the right main pulmonary artery and the left lower lobar artery suggesting malignancy.

  19. New Therapeutic Targets in Soft Tissue Sarcoma

    PubMed Central

    Demicco, Elizabeth G; Maki, Robert G; Lev, Dina C.; Lazar, Alexander J

    2012-01-01

    Soft tissue sarcomas are an uncommon and diverse group of more than 50 mesenchymal malignancies. The pathogenesis of many of these is poorly understood, but others have begun to reveal the secrets of their inner workings. With considerable effort over recent years, soft tissue sarcomas have increasingly been classified on the basis of underlying molecular alterations. In turn, this has allowed the development and application of targeted agents in several specific, molecularly defined, sarcoma subtypes. This review will focus the rationale for targeted therapy in sarcoma, with emphasis on the relevance of specific molecular factors and pathways in both translocation-associated sarcomas and in genetically complex tumors. In addition, we will address some of the early successes in sarcoma targeted therapy as well as a few challenges and disappointments in this field. Finally we will discuss several possible opportunities represented by poorly understood, but potentially promising new therapeutic targets, as well as several novel biologic agents currently in preclinical and early phase I/II trials. This will provide the reader with context for understanding the current state this field and a sense of where it may be headed in the coming years. PMID:22498582

  20. Synovial sarcoma: defining features and diagnostic evolution.

    PubMed

    Thway, Khin; Fisher, Cyril

    2014-12-01

    Synovial sarcoma (SS) is a malignant mesenchymal neoplasm with variable epithelial differentiation, with a propensity to occur in young adults and which can arise at almost any site. It is generally viewed and treated as a high-grade sarcoma. As one of the first sarcomas to be defined by the presence of a specific chromosomal translocation leading to the production of the SS18-SSX fusion oncogene, it is perhaps the archetypal "translocation-associated sarcoma," and its translocation remains unique to this tumor type. Synovial sarcoma has a variety of morphologic patterns, but its chief forms are the classic biphasic pattern, of glandular or solid epithelial structures with monomorphic spindle cells and the monophasic pattern, of fascicles of spindle cells with only immunohistochemical or ultrastructural evidence of epithelial differentiation. However, there is significant morphologic heterogeneity and overlap with a variety of other neoplasms, which can cause diagnostic challenge, particularly as the immunoprofile is varied, SS18-SSX is not detected in 100% of SSs, and they may occur at unusual sites. Correct diagnosis is clinically important, due to the relative chemosensitivity of SS in relation to other sarcomas, for prognostication and because of the potential for treatment with specific targeted therapies in the near future. We review SS, with emphasis on the diagnostic spectrum, recent immunohistochemical and genetic findings, and the differential diagnosis. Copyright © 2014 Elsevier Inc. All rights reserved.

  1. 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

  2. Assessment of molecular genetic detection of chromosome translocations in the differential diagnosis of pediatric sarcomas.

    PubMed

    Dockhorn-Dworniczak, B; Schäfer, K L; Blasius, S; Christiansen, H; Koscielniak, E; Ritter, J; Winkelmann, W; Jürgens, H; Böcker, W

    1997-01-01

    Recent studies have shown that many types of soft-tissue sarcomas are characterized by specific chromosomal translocations, which are likely to be of etiologic significance. In order to evaluate their diagnostic impact, a panel of 129 sarcomas comprising 78 Ewing's tumors (ET), 19 rhabdomyosarcomas (RMS), 20 neuroblastomas (NB), 9 synovialsarcomas, 2 esthesioneuroblastomas, and 1 desmoplastic small-round-cell tumor (DSRCT) were analysed for the occurrence of the major recurrent translocations, such as t(11;22)(q24;q12), t(21;22)(q22;q12), t(11;22)(p13;q12), t(2;13)(q35;q14), t(1;13)(p36;q14), and t(X;18)(p11;q11). Nitrogen-frozen tissue material was analysed by means of Reverse Transcription followed by PCR (Polymerase-Chain Reaction) and nested PCR (RT-PCR). Specificity of the PCR products obtained was confirmed by non-isotopic Southern-Blot analysis with gene-specific probes and/or automated direct sequence analysis. 75 ETs have been shown to carry either a t(11;22) or t(21;22) translocation by identification of chimeric EWS-FLI-1 or EWS-ERG gene-fusion transcripts respectively. 3 ETs were lacking EWS/FLI-1 or EWS-ERG fusion products. 2 of these tumors were shown on review to have unusual morphological features for ETs. 8/19 RMS were initially diagnosed as alveolar RMS. These tumours were shown to carry either a t(2;13) translocation exhibiting chimeric PAX3-FKHR fusion transcripts or a t(1;13) translocation with PAX7-FKHR chimeric gene products. One RMS of the embryonal group also carried a t(1;13) translocation. Reevaluation demonstrated a partly alveolar morphology. In 8/9 synovial sarcomas a t(X;18) translocation was identified. Expression of a EWS-WTI gene-fusion product associated with a t(11;22) translocation was found in the DSRCT. None of these rearrangements were detected in the NBs and 2 esthesioneuroblastomas. Our results support the concept that the major recurrent translocations are histogenetically specific for a subset of sarcomas. Thus, the

  3. Predictors of alveolar air leaks.

    PubMed

    Loran, David B; Woodside, Kenneth J; Cerfolio, Robert J; Zwischenberger, Joseph B

    2002-08-01

    Persistent air leaks are caused by the failure of the postoperative lung to achieve a configuration that is physiologically amenable to healing. The raw pulmonary surface caused by the dissection of the fissure often is separated from the pleura, and the air leak fails to close. Additionally, higher air flow thorough an alveolar-pleural fistula seems to keep the fistula open. Other factors that interfere with wound healing, such as steroid use, diabetes, or malnutrition, can result in persistence of the leak. A thoracic surgeon can minimize the incidence of air leak through meticulous surgical technique and can identify patients in whom the balance of risks (Table 1) and benefits warrant operative intervention based on an understanding of the underlying pathophysiology.

  4. Vertical Alveolar Ridge Augmentation by Distraction Osteogenesis

    PubMed Central

    Kumar, N. Nanda; Ravindran, C.

    2015-01-01

    Introduction Compromised alveolar ridge in vertical and horizontal dimension is a common finding in patients visiting practitioners for dental prosthesis. Various treatment modalities are available for correction of deficient ridges among which alveolar distraction osteogenesis is one. Aim To study the efficacy of alveolar distraction osteogenesis in augmentation of alveolar ridges deficient in vertical dimension. Materials and Methods Ten patients aged 16 to 46 years with deficient alveolar ridge underwent ridge augmentation in 11 alveolar segments using the distraction osteogenesis method. For each patient a custom made distraction device was fabricated. The device was indigenously manufactured with SS-316 (ISO 3506). Results The vertical bone gain reached more than 10mm without the use of bone transplantation. Certain complications like incorrect vector of distraction, paresthesia, pain and loss of transport segment were encountered during the course of the study. Conclusion Alveolar vertical distraction osteogenesis is a reliable and predictable technique for both hard and soft tissue genesis. Implant placement is feasible with primary stability in neogenerated bone at the level of the distracted areas. PMID:26816991

  5. Vertical Alveolar Ridge Augmentation by Distraction Osteogenesis.

    PubMed

    Mohanty, Rajat; Kumar, N Nanda; Ravindran, C

    2015-12-01

    Compromised alveolar ridge in vertical and horizontal dimension is a common finding in patients visiting practitioners for dental prosthesis. Various treatment modalities are available for correction of deficient ridges among which alveolar distraction osteogenesis is one. To study the efficacy of alveolar distraction osteogenesis in augmentation of alveolar ridges deficient in vertical dimension. Ten patients aged 16 to 46 years with deficient alveolar ridge underwent ridge augmentation in 11 alveolar segments using the distraction osteogenesis method. For each patient a custom made distraction device was fabricated. The device was indigenously manufactured with SS-316 (ISO 3506). The vertical bone gain reached more than 10mm without the use of bone transplantation. Certain complications like incorrect vector of distraction, paresthesia, pain and loss of transport segment were encountered during the course of the study. Alveolar vertical distraction osteogenesis is a reliable and predictable technique for both hard and soft tissue genesis. Implant placement is feasible with primary stability in neogenerated bone at the level of the distracted areas.

  6. Inferior alveolar nerve block: Alternative technique

    PubMed Central

    Thangavelu, K.; Kannan, R.; Kumar, N. Senthil

    2012-01-01

    Background: Inferior alveolar nerve block (IANB) is a technique of dental anesthesia, used to produce anesthesia of the mandibular teeth, gingivae of the mandible and lower lip. The conventional IANB is the most commonly used the nerve block technique for achieving local anesthesia for mandibular surgical procedures. In certain cases, however, this nerve block fails, even when performed by the most experienced clinician. Therefore, it would be advantageous to find an alternative simple technique. Aim and Objective: The objective of this study is to find an alternative inferior alveolar nerve block that has a higher success rate than other routine techniques. To this purpose, a simple painless inferior alveolar nerve block was designed to anesthetize the inferior alveolar nerve. Materials and Methods: This study was conducted in Oral surgery department of Vinayaka Mission's dental college Salem from May 2009 to May 2011. Five hundred patients between the age of 20 years and 65 years who required extraction of teeth in mandible were included in the study. Out of 500 patients 270 were males and 230 were females. The effectiveness of the IANB was evaluated by using a sharp dental explorer in the regions innervated by the inferior alveolar, lingual, and buccal nerves after 3, 5, and 7 min, respectively. Conclusion: This study concludes that inferior alveolar nerve block is an appropriate alternative nerve block to anesthetize inferior alveolar nerve due to its several advantages. PMID:25885503

  7. Intratumoral oxygen gradients mediate sarcoma cell invasion

    PubMed Central

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

    2016-01-01

    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 mm3) 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

  8. 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.

  9. Expression of PSMA in tumor neovasculature of high grade sarcomas including synovial sarcoma, rhabdomyosarcoma, undifferentiated sarcoma and MPNST

    PubMed Central

    Heitkötter, Birthe; Trautmann, Marcel; Grünewald, Inga; Bögemann, Martin; Rahbar, Kambiz; Gevensleben, Heidrun; Wardelmann, Eva; Hartmann, Wolfgang; Steinestel, Konrad; Huss, Sebastian

    2017-01-01

    Aims PSMA (prostate specific membrane antigen) is physiologically expressed in normal prostate tissue. It is overexpressed in prostate cancer cells and has been suggested as a target for antibody-based radioligand therapy. As PSMA expression so far has not been systematically analyzed in soft tissue tumors, the current study aims at investigating a large cohort of different subtypes. Methods and Results Immunohistochemistry was used to detect PSMA expression in 779 samples of soft tissue tumors and Ewing sarcoma as a primary bone malignancy. CD34 coexpression was employed to study PSMA expression in the neovasculature. PSMA expression was found in the tumor-associated neovasculature of 151/779 soft tissue/bone tumors (19.38%) and was more frequent in malignant tumors compared to tumors with intermediate or benign biological potential (p=0.078). Strong neovascular PSMA expression was predominantly observed in subsets of different sarcomas including 3/20 rhabdomyosarcomas (15%), 4/21 malignant peripheral nerve sheath tumors (19.05%), 6/16 synovial sarcomas (35.29%) and 6/33 undifferentiated pleomorphic sarcomas (18.18%). Conclusion We conclude that PSMA is expressed in the neovasculature of a subset of soft tissue tumors to a variable extent. Our observation of strong PSMA expression predominantly occurring in sarcomas might provide a rationale to evaluate PSMA-targeted radioligand therapy in these entities. PMID:28002805

  10. Lung alveolar epithelium and interstitial lung disease.

    PubMed

    Corvol, Harriet; Flamein, Florence; Epaud, Ralph; Clement, Annick; Guillot, Loic

    2009-01-01

    Interstitial lung diseases (ILDs) comprise a group of lung disorders characterized by various levels of inflammation and fibrosis. The current understanding of the mechanisms underlying the development and progression of ILD strongly suggests a central role of the alveolar epithelium. Following injury, alveolar epithelial cells (AECs) may actively participate in the restoration of a normal alveolar architecture through a coordinated process of re-epithelialization, or in the development of fibrosis through a process known as epithelial-mesenchymal transition (EMT). Complex networks orchestrate EMT leading to changes in cell architecture and behaviour, loss of epithelial characteristics and gain of mesenchymal properties. In the lung, AECs themselves may serve as a source of fibroblasts and myofibroblasts by acquiring a mesenchymal phenotype. This review covers recent knowledge on the role of alveolar epithelium in the pathogenesis of ILD. The mechanisms underlying disease progression are discussed, with a main focus on the apoptotic pathway, the endoplasmic reticulum stress response and the developmental pathway.

  11. Toward therapeutic pulmonary alveolar regeneration in humans.

    PubMed

    Massaro, Donald; Massaro, Gloria Decarlo

    2006-11-01

    In humans, age results in loss of pulmonary alveoli; menopause accelerates loss of diffusing capacity, an index of alveolar surface area; and disease (e.g., chronic obstructive pulmonary disease) results in loss of alveoli. Thus, an important goal for investigators is to generate knowledge that allows induction of pulmonary alveolar regeneration in humans. Our enthusiasm for this goal and our assessment of its feasibility are based on work in several laboratories over the last decade that has disproved the notion that pulmonary alveoli are incapable of regeneration, and on the growing evidence that signals that regulate programs of alveolar turnover (loss and regeneration) are conserved from rodents to humans. We review animal models of alveolar loss and regeneration and their conservation during evolution, and hence their relevance to humans.

  12. Alveolarization Continues during Childhood and Adolescence

    PubMed Central

    Owers-Bradley, John; Beardsmore, Caroline S.; Mada, Marius; Ball, Iain; Garipov, Ruslan; Panesar, Kuldeep S.; Kuehni, Claudia E.; Spycher, Ben D.; Williams, Sian E.; Silverman, Michael

    2012-01-01

    Rationale: The current hypothesis that human pulmonary alveolarization is complete by 3 years is contradicted by new evidence of alveolarization throughout adolescence in mammals. Objectives: We reexamined the current hypothesis using helium-3 (3He) magnetic resonance (MR) to assess alveolar size noninvasively between 7 and 21 years, during which lung volume nearly quadruples. If new alveolarization does not occur, alveolar size should increase to the same extent. Methods: Lung volumes were measured by spirometry and plethysmography in 109 healthy subjects aged 7–21 years. Using 3HeMR we determined two independent measures of peripheral airspace dimensions: apparent diffusion coefficient (ADC) of 3He at FRC (n = 109), and average diffusion distance of helium (Xrms¯) by q-space analysis (n = 46). We compared the change in these parameters with lung growth against a model of lung expansion with no new alveolarization. Measurements and Main Results: ADC increased by 0.19% for every 1% increment in FRC (95% confidence interval [CI], 0.13–0.25), whereas the expected change in the absence of neoalveolarization is 0.41% (95% CI, 0.31–0.52). Similarly, increase of (Xrms¯) with FRC was significantly less than the predicted increase in the absence of neoalveolarization. The number of alveoli is estimated to increase 1.94-fold (95% CI, 1.64–2.30) across the age range studied. Conclusions: Our observations are best explained by postulating that the lungs grow partly by neoalveolarization throughout childhood and adolescence. This has important implications: developing lungs have the potential to recover from early life insults and respond to emerging alveolar therapies. Conversely, drugs, diseases, or environmental exposures could adversely affect alveolarization throughout childhood. PMID:22071328

  13. Monophasic synovial sarcoma of the pharynx: a case report

    PubMed Central

    Betal, Dibendu; Babu, Ramesh; Mehmet, Veysi

    2009-01-01

    Synovial sarcomas are a rare form of soft tissue sarcomas. We present a case of a 62 year-old male presenting with a left thyroid lump initially though to be a thyroid adenoma but subsequently diagnosed as a monophasic synovial sarcoma of the pharynx. We discuss the diagnosis and treatment of this case. PMID:19335917

  14. Monophasic synovial sarcoma of the pharynx: a case report.

    PubMed

    Betal, Dibendu; Babu, Ramesh; Mehmet, Veysi

    2009-03-31

    Synovial sarcomas are a rare form of soft tissue sarcomas. We present a case of a 62 year-old male presenting with a left thyroid lump initially though to be a thyroid adenoma but subsequently diagnosed as a monophasic synovial sarcoma of the pharynx. We discuss the diagnosis and treatment of this case.

  15. Primary Synovial Sarcoma of Lung: A Rare Tumor.

    PubMed

    Raj, Prince; Kumar, Parveen; Sarin, Yogesh Kumar

    2016-01-01

    Synovial sarcoma of lung is a rare tumor with few case reports in literature. Though named synovial sarcoma due to its resemblance to synovium on light microscopy, it arises from mesenchymal tissue. Here, we present a case of synovial sarcoma of lung in a 7-year old boy, with main emphasis on difficulty faced in the management.

  16. Nivolumab With or Without Ipilimumab in Treating Patients With Metastatic Sarcoma That Cannot Be Removed by Surgery

    ClinicalTrials.gov

    2017-10-12

    Dedifferentiated Liposarcoma; Gastrointestinal Stromal Tumor; Metastatic Liposarcoma; Metastatic Undifferentiated Pleomorphic Sarcoma; Pleomorphic Liposarcoma; Stage III Bone Sarcoma AJCC v7; Stage III Soft Tissue Sarcoma AJCC v7; Stage IV Bone Sarcoma AJCC v7; Stage IV Soft Tissue Sarcoma AJCC v7; Stage IVA Bone Sarcoma AJCC v7; Stage IVB Bone Sarcoma AJCC v7; Unresectable Liposarcoma

  17. Microsatellite instability in sarcoma: fact or fiction?

    PubMed

    Monument, Michael J; Lessnick, Stephen L; Schiffman, Joshua D; Randall, Rl Tx

    2012-01-01

    Microsatellite instability (MSI) is a unique molecular abnormality, indicative of a deficient DNA mismatch repair (MMR) system. Described and characterized in the colorectal cancer literature, the MSI-positive phenotype is predictive of disease susceptibility, pathogenesis, and prognosis. The clinical relevance of MSI in colorectal cancer has inspired similar inquisition within the sarcoma literature, although unfortunately, with very heterogeneous results. Evolving detection techniques, ill-defined sarcoma-specific microsatellite loci and small study numbers have hampered succinct conclusions. The literature does suggest that MSI in sarcoma is observed at a frequency similar to that of sporadic colorectal cancers, although there is little evidence to suggest that MSI-positive tumors share distinct biological attributes. Emerging evidence in Ewing sarcoma has demonstrated an intriguing mechanistic role of microsatellite DNA in the activation of key EWS/FLI-target genes. These findings provide an alternative perspective to the biological implications of microsatellite instability in sarcoma and warrant further investigation using sophisticated detection techniques, sensitive microsatellite loci, and appropriately powered study designs.

  18. Postirradiation sarcoma of the head and neck.

    PubMed

    Mark, R J; Bailet, J W; Poen, J; Tran, L M; Calcaterra, T C; Abemayor, E; Fu, Y S; Parker, R G

    1993-08-01

    With improvement in survival after cancer treatment, it is becoming increasingly important to study treatment-related morbidity and mortality. Sarcoma can develop in the irradiated field after radiation therapy. The authors performed a study to estimate the risk, and compared the risk of sarcoma after radiation therapy with that of other treatment modalities used against cancer. Between 1955 and 1988, 229 patients with sarcoma of the head and neck were seen at the University of California, Los Angeles (UCLA), Medical Center. Of these, 13 (6%) had a previous history of radiation therapy to the head and neck. Radiation doses were known in 10 of 13 patients and ranged from 30 to 124.4 Gy. The latency time from radiation therapy to the development of postirradiation sarcoma (PIS) ranged from 3 months to 50 years, with a median of 12 years. More than 2000 patients have received radiation therapy to the head and neck for various conditions at the UCLA Medical Center since 1955. The authors conclude that most head and neck sarcomas are not radiation related and that the risk of PIS after head and neck irradiation for other diseases is low. From a review of the literature comparing mortality risks of chemotherapy, general surgery, and anesthesia, the risk of PIS appears no worse. Given the large number of patients who can be cured or receive palliation with radiation therapy, concern about PIS should not be a major factor influencing treatment decisions in patients with cancer.

  19. Multidisciplinary management of soft tissue sarcomas.

    PubMed

    Luis, Ángel Montero; Aguilar, Damián Pérez; Martín, José Antonio López

    2010-08-01

    Musculoskeletal sarcomas are a heterogeneous group of malignant neoplasms derived from connective tissue. Sarcomas represent about 1% of cancer in adults. The annual incidence in adults in Europe is around 14,000 new cases of soft tissue sarcomas (STS) and 4,800 new cases of bone sarcomas. Musculoskeletal tumours arise anywhere in the body, although lower extremities are the most common site of appearance, followed by upper extremities, trunk, retroperitoneum and head and neck area. Adequate management of STS is a stimulating challenge for oncologists. The aim of treatment should be focused on four main aspects: improving survival, avoiding local recurrence, maximising organ function and, finally, minimising morbidity. Surgery, radiotherapy and, sometimes though increasingly, chemotherapy are the pillars on which rests the modern treatment of sarcomas. The optimal management of musculoskeletal tumour requires a multidisciplinary integration of these different approaches in treatment planning right from the initial diagnoses. Referring patients to qualified centres should be desirable to achieve the maximum probability of control and even cure for STS.

  20. Olaratumab for advanced soft tissue sarcoma.

    PubMed

    Tobias, Alexander; O'brien, Michael P; Agulnik, Mark

    2017-07-01

    Olaratumab is a humanized IgG1 monoclonal antibody that blocks the platelet-derived growth factor receptor alpha (PDGFRα). Its antagonistic behavior inhibits the receptor's tyrosine kinase activity, thereby, turning off the downstream signaling cascades responsible for soft tissue sarcoma tumorigenesis. In October 2016, olaratumab received Food and Drug Administration (FDA) approval for its use in combination with doxorubicin for treatment of advanced soft tissue sarcoma. Areas covered: This drug profile takes a comprehensive look at the clinical studies leading to FDA approval of olaratumab as well as its safety and efficacy as a front-line treatment option for sarcoma patients. The literature search was primarily conducted using PubMed. Expert commentary: The combination of olaratumab plus doxorubicin has provided a new front-line therapeutic option for soft tissue sarcoma patients. An open-label phase Ib and randomized phase II trial in patients with advanced soft tissue sarcoma demonstrated that the addition of olaratumab to doxorubicin prolonged progression-free survival by 2.5 months and overall survival by 11.8 months when compared to doxorubicin alone. Of importance, this clinically meaningful increase in overall survival did not come at the expense of a significantly greater number of toxicities. A phase III confirmatory trial (ClinicalTrials.gov Identifier NCT02451943) will be completed in 2020.

  1. Canine intrathoracic sarcoma with ultrastructural characteristics of human synovial sarcoma - case report.

    PubMed

    Lovell, Ser; Burchell, R K; Roady, P J; Fredrickson, R L; Gal, A

    2017-08-16

    Canine joint sarcomas, designated synovial sarcomas, are uncommon malignant mesenchymal neoplasms that occur in the large joints of the extremities of middle-aged, large-breed dogs. We report the diagnosis of an intrathoracic sarcoma with ultrastructural characteristics reminiscent of human synovial sarcoma in a dog. A 7-year-old female spayed Tibetan terrier crossbred dog was presented for acute severe labored breathing and diagnosed with an intrathoracic neoplastic mass. The neoplasm resulted in the accumulation of substantial amounts of viscous pleural fluid that led to dyspnea. The neoplastic mass consisted of interweaving bundles of large pleomorphic mesenchymal cells, supported by an alcian blue positive myxomatous matrix. The neoplastic cells were immunohistochemically negative for cytokeratin and CD18. Transmission electron microscopy indicated that the neoplastic cells had desmosome junctions, short microvilli-like structures and ample amounts of rough endoplasmic reticulum resembling type B-like synoviocytes and synovial sarcoma as reported in people. Despite complete surgical excision of the neoplastic mass, clinical signs recurred after a month and led to the euthanasia of the dog. Currently, there are no immunohistochemical markers specific for synovial sarcoma. Canine neoplasms with transmission electron microscopy characteristics resembling type B-like synoviocytes should be considered similar to the human sarcomas that carry the specific translocations between chromosomes X and 18.

  2. The Molecular Basis of Pulmonary Alveolar Proteinosis

    PubMed Central

    Carey, Brenna; Trapnell, Bruce C.

    2010-01-01

    Pulmonary alveolar proteinosis (PAP) comprises a heterogenous group of diseases characterized by abnormal surfactant accumulation resulting in respiratory insufficiency, and defects in alveolar macrophage- and neutrophil-mediated host defense. Basic, clinical and translational research over the past two decades have raised PAP from obscurity, identifying the molecular pathogenesis in over 90% of cases as a spectrum of diseases involving the disruption of GM-CSF signaling. Autoimmune PAP represents the vast majority of cases and is caused by neutralizing GM-CSF autoantibodies. Genetic mutations that disrupt GM-CSF receptor signaling comprise a rare form of hereditary PAP. In both autoimmune and hereditary PAP, loss of GM-CSF signaling blocks the terminal differentiation of alveolar macrophages in the lungs impairing the ability of alveolar macrophages to catabolize surfactant and to perform many host defense functions. Secondary PAP occurs in a variety of clinical diseases that presumedly cause the syndrome by reducing the numbers or functions of alveolar macrophages, thereby impairing alveolar macrophage-mediated pulmonary surfactant clearance. A similar phenotype occurs in mice deficient in the production of GM-CSF or GM-CSF receptors. PAP and related research has uncovered a critical and emerging role for GM-CSF in the regulation of pulmonary surfactant homeostasis, lung host defense, and systemic immunity. PMID:20338813

  3. Lung epithelial branching program antagonizes alveolar differentiation.

    PubMed

    Chang, Daniel R; Martinez Alanis, Denise; Miller, Rachel K; Ji, Hong; Akiyama, Haruhiko; McCrea, Pierre D; Chen, Jichao

    2013-11-05

    Mammalian organs, including the lung and kidney, often adopt a branched structure to achieve high efficiency and capacity of their physiological functions. Formation of a functional lung requires two developmental processes: branching morphogenesis, which builds a tree-like tubular network, and alveolar differentiation, which generates specialized epithelial cells for gas exchange. Much progress has been made to understand each of the two processes individually; however, it is not clear whether the two processes are coordinated and how they are deployed at the correct time and location. Here we show that an epithelial branching morphogenesis program antagonizes alveolar differentiation in the mouse lung. We find a negative correlation between branching morphogenesis and alveolar differentiation temporally, spatially, and evolutionarily. Gain-of-function experiments show that hyperactive small GTPase Kras expands the branching program and also suppresses molecular and cellular differentiation of alveolar cells. Loss-of-function experiments show that SRY-box containing gene 9 (Sox9) functions downstream of Fibroblast growth factor (Fgf)/Kras to promote branching and also suppresses premature initiation of alveolar differentiation. We thus propose that lung epithelial progenitors continuously balance between branching morphogenesis and alveolar differentiation, and such a balance is mediated by dual-function regulators, including Kras and Sox9. The resulting temporal delay of differentiation by the branching program may provide new insights to lung immaturity in preterm neonates and the increase in organ complexity during evolution.

  4. Kaposi sarcoma following postmastectomy lymphedema.

    PubMed

    Montero Pérez, Iria; Rodríguez-Pazos, Laura; Álvarez-Pérez, Adriana; Ferreirós, M Mercedes Pereiro; Aliste, Carlos; Suarez-Peñaranda, Jose Manuel; Toribio, Jaime

    2015-11-01

    Classical Kaposi sarcoma (KS) usually appears on lower extremities accompanied or preceded by local lymphedema. However, the development in areas of chronic lymphedema of the arms following mastectomy, mimicking a Stewart-Treves syndrome, has rarely been described. We report an 81-year-old woman who developed multiple, erythematous to purple tumors, located on areas of post mastectomy lymphedema. Histopathological examination evidenced several dermal nodules formed by spindle-shaped cells that delimitated slit-like vascular spaces with some red cell extravasation. Immunohistochemically, the human herpesvirus type 8 (HHV-8) latent nuclear antigen-1 was detected in the nuclei of most tumoral cells confirming the diagnosis of KS. Lymphedema could promote the development of certain tumors by altering immunocompetence. Although angiosarcoma (AS) is the most frequent neoplasia arising in the setting of chronic lymphedema, other tumors such as benign lymphangiomatous papules (BLAP) or KS can also develop in lymphedematous limbs. It is important to establish the difference between AS and KS because their prognosis and treatment are very different. Identification by immunohistochemistry of HHV-8 is useful for the distinction between KS and AS or BLAP.

  5. Early age renal synovial sarcoma.

    PubMed

    Romero-Rojas, Alfredo Ernesto; Díaz-Pérez, Julio Alexander; Messa-Botero, Oscar Alberto; Neira-Mejia, Fabían Enrique

    2010-01-01

    We report a primary renal Synovial Sarcoma (SS) case and analyze its features. A 15 year old male presented with left abdominal mass and weight loss. CT scan images showed a 13 cm mass located in the lower pole of the left kidney. Renal biopsy recognized an undifferentiated neoplasm, the immunohistochemistry suggesting the probability of neuroectodermic primitive tumor versus SS. Chemotherapy and radical nephrectomy were carried out. Pathological study showed a big multilobulated necrotic tumor 22 x 13 x 12.5 cm. Histopathological study demonstrated a neoplasm composed by immature cells. Currently, patient has survived 1,8 years. A structured bibliographical search was performed in the Medline, Imbiomed and Scielo databases. The final immunohistochemistry studies gave the diagnosis of poorly differentiated renal SS small cell variety. The renal SS is extremely infrequent, with less than 40 cases reported, of which this case reports the earlier age. These tumors, when located in the kidney, represent a great diagnostic challenge that requires adequate clinical, radiological, surgical, and pathological correlation for appropriate diagnosis and treatment.

  6. Cluster analysis of immunohistochemical profiles in synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma.

    PubMed

    Olsen, Stephen H; Thomas, Dafydd G; Lucas, David R

    2006-05-01

    As a result of overlapping morphologic and immunohistochemical features, it can be difficult to distinguish synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma/primitive neuroectodermal tumor in core biopsies. To analyze and compare immunohistochemical profiles, we stained tissue microarrays of 23 synovial sarcomas, 23 malignant peripheral nerve sheath tumors, and 27 Ewing sarcomas with 22 antibodies potentially useful in the differential diagnosis, and analyzed the data with cluster analysis. Stain intensity was scored as none, weak, or strong. For CD99, tumors with membranous accentuation were independently categorized. Cluster analysis sorted five groups, with like tumors clustering together. Synovial sarcoma clustered into two groups: one cytokeratin and EMA positive (n = 11), the other mostly cytokeratin negative, EMA positive, bcl-2 positive and mostly CD56 positive (n = 9). Malignant peripheral nerve sheath tumor clustered into two groups: one S100 positive, with nestin and NGFR positivity in most (n = 10), the other mostly S100 negative, and variably but mostly weakly positive for nestin and NGFR (n = 11). Ewing sarcomas clustered into a single group driven by membranous CD99 staining. Thirteen cases failed to cluster (outliers), while three Ewing sarcomas clustered into groups of other tumor types. Paired antibodies for each tumor type determined by visual assessment of cluster analysis data and statistical calculations of specificity, sensitivity, and predictive values showed that EMA/CK7 for synovial sarcoma, nestin/S100 for malignant peripheral nerve sheath tumor, and membranous CD99/Fli-1 for Ewing sarcoma yielded high specificity and positive predictive values. Cluster analysis also highlighted aberrant staining reactions and diagnostic pitfalls in these tumors. Hierarchical cluster analysis is an effective method for analyzing high-volume immunohistochemical data.

  7. Pregnancy and genital sarcoma: a systematic review of the literature.

    PubMed

    Matsuo, Koji; Eno, Michele L; Im, Dwight D; Rosenshein, Neil B

    2009-08-01

    We conducted a literature review to determine the clinical characteristics of genital sarcoma during pregnancy. The systematic literature search was conducted using the search engines PubMed and MEDLINE with keywords "sarcoma" and "pregnancy" and was limited to female genital organs such as ovary, uterus, cervix, vagina, vulva, and retroperitoneal sarcoma. Kaposi's sarcoma, metastatic sarcoma, history of sarcoma, bone sarcoma located in pelvis, and fetal sarcoma were excluded in this study. There were 40 cases of genital sarcoma during pregnancy between 1955 and 2007. The majority of the cases were uterine sarcoma (37.5%), followed by retroperitoneal sarcoma (27.5%), vulvar sarcoma (22.5%), and vaginal sarcoma (12.5%). Mean age of the patient was 27.8 +/- 7.0. The distribution in the onset of symptoms had two peaks: first trimester (27.5%) and third trimester (50.0%). Growing mass (42.5%), abdominal pain (30.0%), and vaginal bleeding (22.5%) were the three most common symptoms. Incidental diagnosis was made in 22.5% and included during cesarean section (12.5%) and routine pelvic exam (7.5%). The cases initially not suspicious for malignancy were 42.5%. Thirty-three (82.5%) cases had live-born infants with term delivery in 55.2%. Mean birth weight was 2843 +/- 791 g, and male infants were more common (66.7%). Intrauterine growth retardation was seen in 12.5% of cases. Preterm labor was a common complication. Median survival period was 2.5 years (95% confidence, 1.9 to 3.1). The 2-, 3-, and 5-year cumulative survival rates were 60%, 38%, and 17%, respectively. Genital sarcomas in pregnancy are rare. There is a delay in diagnosis due to low index of suspicion. A majority had live births, and the 5-year survival is similar to that of advanced-stage sarcoma in nonpregnant women.

  8. Sarcoma-like tumor originating from oligodendroglioma.

    PubMed

    Shoji, Takuhiro; Saito, Ryuta; Kanamori, Masayuki; Sonoda, Yukihiko; Watanabe, Mika; Tominaga, Teiji

    2016-10-01

    We present a case of sarcoma occurring at a site of resected oligodendroglioma without preceding radiotherapy or chemotherapy. Oligosarcoma occurring at sites of resected oligodendroglioma or anaplastic oligodendroglioma with sarcomatous components are rare. Although meningioma or sarcoma-like lesions are sometimes reported after glioma-targeted radiotherapy, those without preceding radiotherapy are quite rare. Moreover, cases of sarcoma without oligodendroglial components occurring at a site of resected oligodendroglioma have never been reported. In this case, fluorescent in situ hybridization analysis revealed 1p/19q co-deletion in both the first tumor and second tumors. Additionally, immunohistochemistry revealed mutated isocitrate dehydrogenase 1 in both tumors. Taken together, these findings suggest a monoclonal tumor origin. Consequently, this case may indicate a new mechanism of development of sarcomatous lesions occurring at the site of a resected glioma.

  9. Intraventricular Undifferentiated Pleomorphic Sarcoma: A Case Report.

    PubMed

    Sieg, Emily P; Stepanyan, Hayk; Payne, Russell; Frauenhoffer, Elizabeth; Specht, Charles S; Langan, Sara; Rizk, Elias

    2016-11-14

    Undifferentiated pleomorphic sarcoma is a histologic diagnosis based on cell morphology. These tumors are found throughout the body. They are rarely found in the central nervous system and almost never occur as a primary intraventricular tumor. We present the unusual case of a 68-year-old woman with an intraventricular undifferentiated pleomorphic sarcoma. We go on to discuss the clinical presentation, radiographic characteristics, and management paradigm for these rare lesions. Our patient presented with acute confusion, inability to balance a checkbook, and gait imbalance. CT and MRI demonstrated a 4 x 3.6 x 3.6 cm enhancing lesion in the left lateral ventricle abutting the foramen of Monro. Pathology revealed an undifferentiated pleomorphic sarcoma.

  10. Intraventricular Undifferentiated Pleomorphic Sarcoma: A Case Report

    PubMed Central

    Sieg, Emily P; Stepanyan, Hayk; Payne, Russell; Frauenhoffer, Elizabeth; Specht, Charles S; Rizk, Elias

    2016-01-01

    Undifferentiated pleomorphic sarcoma is a histologic diagnosis based on cell morphology. These tumors are found throughout the body. They are rarely found in the central nervous system and almost never occur as a primary intraventricular tumor. We present the unusual case of a 68-year-old woman with an intraventricular undifferentiated pleomorphic sarcoma. We go on to discuss the clinical presentation, radiographic characteristics, and management paradigm for these rare lesions. Our patient presented with acute confusion, inability to balance a checkbook, and gait imbalance. CT and MRI demonstrated a 4 x 3.6 x 3.6 cm enhancing lesion in the left lateral ventricle abutting the foramen of Monro. Pathology revealed an undifferentiated pleomorphic sarcoma. PMID:28003940

  11. Pulmonary artery sarcoma mimicking pulmonary thromboembolism.

    PubMed

    Celik, Gökhan; Ciledağ, Aydin; Yüksel, Cabir; Yenigün, Bülent Mustafa; Kutlay, Hakan; Yazicıoğlu, Levent; Perçinel, Sibel; Kaya, Akin

    2011-01-01

    A 30 years old male patient was referred to our hospital with a diagnosis of pulmonary thromboembolism due to thorax-computerized tomography (CT) angiography, revealing a thrombus totally occluding left main pulmonary artery. The lesion was evaluated as tumoural mass. Positron emission tomography (PET)-CT revealed pathologic uptake at pulmonary artery mass. Due to localization of tumour, left pneumonectomy was performed. The pathological diagnosis revealed to be pulmonary artery sarcoma. The patient was presented because pulmonary artery sarcomas are very rare tumors and can mimick pulmonary thromboembolism. The true prevalence is underestimated as many pulmonary artery sarcomas are misdiagnosed as pulmonary thromboembolism. PET-CT may help to make a differential diagnosis.

  12. Sarcoma of vulva, vagina and ovary.

    PubMed

    Magné, Nicolas; Pacaut, Cécile; Auberdiac, Pierre; Jacquin, Jean-Philippe; Chargari, Cyrus; Chauleur, Céline; Haie Meder, Christine; Merrouche, Yacine

    2011-12-01

    Less than 5% of vulvar, vaginal and ovarian malignant diseases are sarcomas. Adequate knowledge of these particular malignant diseases is essential for accurate diagnosis and for choice of surgical treatment, adjuvant therapy and efficient medical treatment in relapse. A crucial aspect in the management of women with these diseases is a multidisciplinary approach. Globally, presenting signs and symptoms of these sarcomas are non-specific of histological type but linked to initial location. In view of this, management should be undertaken by clinicians experienced in these particular malignancies. Long-term side-effects, particularly in children with sarcoma, adversely affect quality of life. New treatment strategies require special attention. Copyright © 2011 Elsevier Ltd. All rights reserved.

  13. 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

  14. Biphasic synovial sarcoma of the abdominal wall.

    PubMed

    Vera, Jesús; García, María-Dolores; Marigil, Miguel; Abascal, Manuel; Lopez, Jose-Ignacio; Ligorred, Luis

    2006-09-01

    Synovial sarcoma arising in the abdominal wall is a rare tumor. We report a case of a 38-year-old man who complained of abdominal pain. Physical examination revealed a firm mobile mass, 25 cm in diameter, in the left lower abdominal wall. The tumor was first thought to be a sarcoma arising from the omentum or mesentery. During surgery, a large tumor was found attached to the inner surface of the abdominal wall and compressing the gastrointestinal tract. On microscopic examination the tumor corresponded to a biphasic synovial sarcoma immunoreactive for cytokeratins (AE1/AE3, 7 and 19), epithelial membrane antigen and carcinoembryonic antigen in the epithelial tumor cells, for E-cadherin especially in their glandular structure, vimentin, CD99, and CD56 in the spindle cell component and for bcl-2 protein. The tumor recurred at the same site, and clinical course progressed to death 3 months after the initial diagnosis.

  15. Sarcoma derived from cultured mesenchymal stem cells.

    PubMed

    Tolar, Jakub; Nauta, Alma J; Osborn, Mark J; Panoskaltsis Mortari, Angela; McElmurry, Ron T; Bell, Scott; Xia, Lily; Zhou, Ning; Riddle, Megan; Schroeder, Tania M; Westendorf, Jennifer J; McIvor, R Scott; Hogendoorn, Pancras C W; Szuhai, Karoly; Oseth, Leann; Hirsch, Betsy; Yant, Stephen R; Kay, Mark A; Peister, Alexandra; Prockop, Darwin J; Fibbe, Willem E; Blazar, Bruce R

    2007-02-01

    To study the biodistribution of MSCs, we labeled adult murine C57BL/6 MSCs with firefly luciferase and DsRed2 fluorescent protein using nonviral Sleeping Beauty transposons and coinfused labeled MSCs with bone marrow into irradiated allogeneic recipients. Using in vivo whole-body imaging, luciferase signals were shown to be increased between weeks 3 and 12. Unexpectedly, some mice with the highest luciferase signals died and all surviving mice developed foci of sarcoma in their lungs. Two mice also developed sarcomas in their extremities. Common cytogenetic abnormalities were identified in tumor cells isolated from different animals. Original MSC cultures not labeled with transposons, as well as independently isolated cultured MSCs, were found to be cytogenetically abnormal. Moreover, primary MSCs derived from the bone marrow of both BALB/c and C57BL/6 mice showed cytogenetic aberrations after several passages in vitro, showing that transformation was not a strain-specific nor rare event. Clonal evolution was observed in vivo, suggesting that the critical transformation event(s) occurred before infusion. Mapping of the transposition insertion sites did not identify an obvious transposon-related genetic abnormality, and p53 was not overexpressed. Infusion of MSC-derived sarcoma cells resulted in malignant lesions in secondary recipients. This new sarcoma cell line, S1, is unique in having a cytogenetic profile similar to human sarcoma and contains bioluminescent and fluorescent genes, making it useful for investigations of cellular biodistribution and tumor response to therapy in vivo. More importantly, our study indicates that sarcoma can evolve from MSC cultures.

  16. FDG PET/CT findings in rare sarcomas.

    PubMed

    Ergül, N; Aydın, M

    2013-01-01

    The role of FDG PET/CT in management of soft tissue and bone sarcomas has been described in many studies up-to-date. However, contribution of PET/CT to diagnosis and treatment in some types of sarcomas that are seen with low incidence has not been identified properly yet. Clear cell sarcoma, synovial sarcoma of chest and myxoid lyposarcoma are rare types of sarcomas. We aimed to describe the FDG uptake patterns of these rare tumors and find out the role of FDG PET/CT in management of disease. Copyright © 2012 Elsevier España, S.L. and SEMNIM. All rights reserved.

  17. Kaposi's sarcoma following immune suppressive therapy for Wegener's granulomatosis.

    PubMed

    Deschênes, Isabelle; Dion, Louise; Beauchesne, Claude; de Brum-Fernandes, Artur

    2003-03-01

    The association between Kaposi's sarcoma and infection with human herpesvirus 8 is now well recognized. Immunologic impairment is associated with 2 forms of Kaposi's sarcoma, epidemic [associated with human immunodeficiency virus (HIV) infection] and iatrogenic (associated with immunosuppressive treatment); both forms have become more common during the last decade. We describe an HIV negative 54-year-old man who developed Kaposi's sarcoma 2 months after the beginning of immuno-suppressive therapy for Wegener's granulomatosis (WG). With tapering of medication, complete remission of Kaposi's sarcoma was achieved in one year. To our knowledge, this is the second reported case of iatrogenic Kaposi's sarcoma in a patient with WG.

  18. [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.

  19. Biomarkers of Osteosarcoma, Chondrosarcoma, and Ewing Sarcoma

    PubMed Central

    Evola, Francesco R.; Costarella, Luciano; Pavone, Vito; Caff, Giuseppe; Cannavò, Luca; Sessa, Andrea; Avondo, Sergio; Sessa, Giuseppe

    2017-01-01

    Osteosarcoma is the most frequent malignant bone neoplasm, followed by chondrosarcoma and Ewing sarcoma. The diagnosis of bone neoplasms is generally made through histological evaluation of a biopsy. Clinical and radiological features are also important in aiding diagnosis and to complete the staging of bone cancer. In addition to these, there are several non-specific serological or specific molecular markers for bone neoplasms. In bone tumors, molecular markers increase the accuracy of the diagnosis and assist in subtyping bone tumors. Here, we review these markers and discuss their role in the diagnosis and prognosis of the three most frequent malignant bone neoplasms, namely osteosarcoma, chondrosarcoma, and Ewing sarcoma. PMID:28439237

  20. Ewing Sarcoma: An Eponym Window to History

    PubMed Central

    Cripe, Timothy P.

    2011-01-01

    Ewing sarcoma was named after James R. Ewing, an eminent American pathologist at Cornell who described the first cases in 1921. Although he is best remembered for this singular achievement, Ewing's contributions to the study of cancer were far more profound and influential. He essentially launched oncology as a discipline with the publication of his seminal textbook and founded the major American cancer societies that exist today. His vision of comprehensive cancer centers still drives our research infrastructure. Since his initial report, these organizations have helped us achieve numerous milestones in understanding and treating patients with Ewing sarcoma. PMID:21151695

  1. Amputation for histiocytic sarcoma in a cat.

    PubMed

    Teshima, Takahiro; Hata, Takashi; Nezu, Yoko; Michishita, Masaki; Matsumoto, Hirotaka; Mizutani, Hisashi; Takahashi, Kimimasa; Koyama, Hidekazu

    2012-02-01

    A 9-year-old spayed female domestic shorthair cat presented with a skin lesion of the left tarsus. The lesion was biopsied and, based on the microscopic appearance and immunohistochemical characteristics, histiocytic sarcoma was diagnosed. Amputation was performed with improved demeanor seen postoperatively. However, between 44 and 60 days following the surgery, relapse of skin lesions appeared in multiple locations, including at the previous amputation site, and euthanasia was elected. This is the first report of a histiocytic sarcoma treated with amputation in a cat.

  2. Molecular piracy of Kaposi's sarcoma associated herpesvirus.

    PubMed

    Choi, J; Means, R E; Damania, B; Jung, J U

    2001-01-01

    Kaposi's Sarcoma associated Herpesvirus (KSHV) is the most recently discovered human tumor virus and is associated with the pathogenesis of Kaposi's sarcoma, primary effusion lymphoma, and Multicentric Casttleman's disease. KSHV contains numerous open reading frames with striking homology to cellular genes. These viral gene products play a variety of roles in KSHV-associated pathogenesis by disrupting cellular signal transduction pathways, which include interferon-mediated anti-viral responses, cytokine-regulated cell growth, apoptosis, and cell cycle control. In this review, we will attempt to cover our understanding of how viral proteins deregulate cellular signaling pathways, which ultimately contribute to the conversion of normal cells to cancerous cells.

  3. [Cleft lip, alveolar and palate sequelae. Proposal of new alveolar score by the Alveolar Cleft Score (ACS) classification].

    PubMed

    Molé, C; Simon, E

    2015-06-01

    The management of cleft lip, alveolar and palate sequelae remains problematic today. To optimize it, we tried to establish a new clinical index for diagnostic and prognostic purposes. Seven tissue indicators, that we consider to be important in the management of alveolar sequelae, are listed by assigning them individual scores. The final score, obtained by adding together the individual scores, can take a low, high or maximum value. We propose a new classification (ACS: Alveolar Cleft Score) that guides the therapeutic team to a prognosis approach, in terms of the recommended surgical and prosthetic reconstruction, the type of medical care required, and the preventive and supportive therapy to establish. Current studies are often only based on a standard radiological evaluation of the alveolar bone height at the cleft site. However, the gingival, the osseous and the cellular areas bordering the alveolar cleft sequelae induce many clinical parameters, which should be reflected in the morphological diagnosis, to better direct the surgical indications and the future prosthetic requirements, and to best maintain successful long term aesthetic and functional results. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  4. Targeting protein kinases to reverse multidrug resistance in sarcoma.

    PubMed

    Chen, Hua; Shen, Jacson; Choy, Edwin; Hornicek, Francis J; Duan, Zhenfeng

    2016-02-01

    Sarcomas are a group of cancers that arise from transformed cells of mesenchymal origin. They can be classified into over 50 subtypes, accounting for approximately 1% of adult and 15% of pediatric cancers. Wide surgical resection, radiotherapy, and chemotherapy are the most common treatments for the majority of sarcomas. Among these therapies, chemotherapy can palliate symptoms and prolong life for some sarcoma patients. However, sarcoma cells can have intrinsic or acquired resistance after treatment with chemotherapeutics drugs, leading to the development of multidrug resistance (MDR). MDR attenuates the efficacy of anticancer drugs and results in treatment failure for sarcomas. Therefore, overcoming MDR is an unmet need for sarcoma therapy. Certain protein kinases demonstrate aberrant expression and/or activity in sarcoma cells, which have been found to be involved in the regulation of sarcoma cell progression, such as cell cycle, apoptosis, and survival. Inhibiting these protein kinases may not only decrease the proliferation and growth of sarcoma cells, but also reverse their resistance to chemotherapeutic drugs to subsequently reduce the doses of anticancer drugs and decrease drug side-effects. The discovery of novel strategies targeting protein kinases opens a door to a new area of sarcoma research and provides insight into the mechanisms of MDR in chemotherapy. This review will focus on the recent studies in targeting protein kinase to reverse chemotherapeutic drug resistance in sarcoma. Copyright © 2015 Elsevier Ltd. All rights reserved.

  5. Targeting Protein Kinases to Reverse Multidrug Resistance in Sarcoma

    PubMed Central

    Chen, Hua; Shen, Jacson; Choy, Edwin; Hornicek, Francis J.; Duan, Zhenfeng

    2015-01-01

    Sarcomas are a group of cancers that arise from transformed cells of mesenchymal origin. They can be classified into over 50 subtypes, accounting for approximately 1% of adult and 15% of pediatric cancers. Wide surgical resection, radiotherapy, and chemotherapy are the most common treatments for the majority of sarcomas. Among these therapies, chemotherapy can palliate symptoms and prolong life for some sarcoma patients. However, sarcoma cells can have intrinsic or acquired resistance after treatment with chemotherapeutics drugs, leading to the development of multidrug resistance (MDR). MDR attenuates the efficacy of anticancer drugs and results in treatment failure for sarcomas. Therefore, overcoming MDR is an unmet need for sarcoma therapy. Certain protein kinases demonstrate aberrant expression and/or activity in sarcoma cells, which have been found to be involved in the regulation of sarcoma cell progression, such as cell cycle, apoptosis, and survival. Inhibiting these protein kinases may not only decrease the proliferation and growth of sarcoma cells, but also reverse their resistance to chemotherapeutic drugs to subsequently reduce the doses of anticancer drugs and decrease drug side-effects. The discovery of novel strategies targeting protein kinases opens a door to a new area of sarcoma research and provides insight into the mechanisms of MDR in chemotherapy. This review will focus on the recent studies in targeting protein kinase to reverse chemotherapeutic drug resistance in sarcoma. PMID:26827688

  6. ESF-EMBO Symposium "Molecular Biology and Innovative Therapies in Sarcomas of Childhood and Adolescence" Sept 29-Oct 4, Polonia Castle Pultusk, Poland.

    PubMed

    Schäfer, Beat W; Koscielniak, Ewa; Kovar, Heinrich; Fulda, Simone

    2013-01-01

    Rhabdomyosarcoma (RMS) and Ewing sarcoma (ES) are among the most common pediatric sarcomas (Arndt et al., 2012). Despite sarcomas representing a highly heterogeneous group of tumors, ES and alveolar RMS (ARMS) typically share one common genetic characteristic, namely a specific chromosomal translocation (Helman and Meltzer, 2003; Lessnick and Ladanyi, 2012). These translocations generate fusion proteins, which are composed of two transcription factors (TF). Typically, one TF is a developmentally regulated factor that is essential for proper specification of a given lineage and provides the DNA-binding domain, while the partner TF contributes a transactivation domain that drives aberrant expression of target genes. Based on these common genetic characteristics, the first ESF-EMBO research conference entitled "Molecular Biology and Innovative Therapies in Sarcomas of Childhood and Adolescence" with special focus on RMS and ES was held at the Polonia Castle in Pultusk, Poland. The conference gathered 70 participants from more than 15 countries and several continents representing most research groups that are active in this field.

  7. ESF-EMBO Symposium “Molecular Biology and Innovative Therapies in Sarcomas of Childhood and Adolescence” Sept 29–Oct 4, Polonia Castle Pultusk, Poland

    PubMed Central

    Schäfer, Beat W.; Koscielniak, Ewa; Kovar, Heinrich; Fulda, Simone

    2013-01-01

    Rhabdomyosarcoma (RMS) and Ewing sarcoma (ES) are among the most common pediatric sarcomas (Arndt et al., 2012). Despite sarcomas representing a highly heterogeneous group of tumors, ES and alveolar RMS (ARMS) typically share one common genetic characteristic, namely a specific chromosomal translocation (Helman and Meltzer, 2003; Lessnick and Ladanyi, 2012). These translocations generate fusion proteins, which are composed of two transcription factors (TF). Typically, one TF is a developmentally regulated factor that is essential for proper specification of a given lineage and provides the DNA-binding domain, while the partner TF contributes a transactivation domain that drives aberrant expression of target genes. Based on these common genetic characteristics, the first ESF-EMBO research conference entitled “Molecular Biology and Innovative Therapies in Sarcomas of Childhood and Adolescence” with special focus on RMS and ES was held at the Polonia Castle in Pultusk, Poland. The conference gathered 70 participants from more than 15 countries and several continents representing most research groups that are active in this field. PMID:23761860

  8. AIDS-related Kaposi sarcoma: findings on thallium-201 scintigraphy

    SciTech Connect

    Lee, V.W.; Rosen, M.P.; Baum, A.; Cohen, S.E.; Cooley, T.P.; Liebman, H.A.

    1988-12-01

    No simple, noninvasive method is available for evaluating extracutaneous Kaposi sarcoma in AIDS patients or for following the tumor's response to treatment. We report our preliminary experience with thallium-201 scintigraphy in nine AIDS patients with proved Kaposi sarcoma. Eight of the nine had abnormal uptake of the radionuclide in skin, lymph nodes, oral cavity, vagina, and lungs. Only four of the nine had cutaneous Kaposi sarcoma at the time of scanning. All cutaneous and mucosal lesions were thallium avid. Two of the six patients with thallium-avid nodes underwent nodal biopsy. Both biopsies confirmed the diagnosis of Kaposi sarcoma. Cutaneous Kaposi sarcoma developed later in one of these patients, showing the efficacy of thallium scintigraphy for the early detection of extracutaneous lesions. These preliminary results show thallium avidity in Kaposi sarcoma involving the skin and various extracutaneous sites (lymph nodes, lung, mucosa, and vagina). Thallium scintigraphy is a potentially useful procedure for detecting extracutaneous Kaposi sarcoma in AIDS patients.

  9. A New Device for Alveolar Bone Transportation

    PubMed Central

    Vega, Omar; Pérez, Daniel; Páramo, Viviana; Falcón, Jocelyn

    2011-01-01

    We present a retrospective review of a new technique for the transportation of alveolar bone using a Hyrax device modified by the principal author (O.A.V.). There were seven patients (five males and two females), including five patients with cleft palate and lip diagnosis, one patient with a high-speed gunshot wound, and one patient with facial trauma sequel due to mandibular fracture. They were all treated with an alveolar bone transportation technique (ABT) through the use of the modified Hyrax device (VEGAX). Before surgery, distraction osteogenesis of the bifocal type was performed on four patients, and the trifocal type was performed on the other three patients. However, in one case, direct dental anchorage was not used, only orthodontic appliances. In all the cases, new bone formation and gingival tissue around the defect were obtained, posterior to the alveolar distraction process; no complications were observed in any patient. In one case, two teeth involved in the disk of the ABT were extracted, due to a previous condition of periodontal disease. The alveolar bone transport with the VEGAX device is an accessible technique for almost every patient with alveolar defects due to diverse causes. In all the presented cases, predictability and success were demonstrated. PMID:22655120

  10. Keratin subsets in spindle cell sarcomas. Keratins are widespread but synovial sarcoma contains a distinctive keratin polypeptide pattern and desmoplakins.

    PubMed Central

    Miettinen, M.

    1991-01-01

    The presence of individual keratin polypeptides and desmoplakins was immunohistochemically studied in 25 spindle cell sarcomas of different types using acetone-fixed frozen sections. Results revealed that keratins 8 and 18 were present in a high number of tumors: 9 of 9 synovial sarcomas, 5 of 7 leiomyosarcomas, 5 of 5 malignant schwannomas, and 1 of 4 undifferentiated spindle cell sarcomas. In addition to keratins 8 and 18, the glandular component of synovial sarcoma showed prominent reactivity with antibodies to keratins 7 and 19. Also the glandular epithelial cells in synovial sarcoma showed desmoplakin immunoreactivity preferentially in a luminal distribution, but desmoplakin was absent in other spindle cell sarcomas. Furthermore keratin 13 was seen focally in 4 of 9 synovial sarcomas. In contrast, keratins 7, 13, and 19 were practically absent in leiomyosarcomas, malignant schwannomas, and undifferentiated spindle cell sarcomas. The widespread presence of keratins 8 and 18 in various spindle cell sarcomas may reflect aberrant keratin expression in mesenchymal cells, previously described in cultured transformed fibroblasts. The presence of keratins 7 and 19 and desmoplakin is highly associated with morphologically observable epithelial differentiation restricted to synovial sarcoma among spindle cell sarcomas. Images Figure 1 Figure 2 Figure 3 PMID:1704194

  11. Radiographic appearance of Ewing sarcoma of the hands and feet: report from the Intergroup Ewing Sarcoma Study

    SciTech Connect

    Reinus, W.R.; Gilula, L.A.; Shirley, S.K.; Askin, F.B.; Siegal, G.P.

    1985-02-01

    Review of current data from the Intergroup Ewing Sarcoma Study (IESS) shows that Ewing sarcoma is rare in bones of the hands and feet. The 12 patients from the IESS protocols with hand or foot Ewing sarcoma are comparable to those already reported in the literature. With the exception of lesions in the calcaneus, the prognosis for disease-free survival is excellent. The radiographic features of hand and foot Ewing sarcoma are generally those of classic Ewing sarcoma: permeation, soft-tissue mass, and often, associated sclerotic reaction. However, with the exception of sclerosis, features suggesting bone reaction and slow tumor growth in these patients were distinctly uncommon compared with Ewing sarcoma in general. Apparently location of the lesion is important, since in the reported cases in the literature and in this series, lesions of the calcaneus fared poorly. The importance of this set of patients therefore relates to awareness and early recognition of an unusual appearance and location of Ewing sarcoma.

  12. 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

  13. Advances in sarcoma diagnostics and treatment

    PubMed Central

    Dancsok, Amanda R; Asleh-Aburaya, Karama; Nielsen, Torsten O

    2017-01-01

    The heterogeneity of sarcomas with regard to molecular genesis, histology, clinical characteristics, and response to treatment makes management of these rare yet diverse neoplasms particularly challenging. This review encompasses recent developments in sarcoma diagnostics and treatment, including cytotoxic, targeted, epigenetic, and immune therapy agents. In the past year, groups internationally explored the impact of adding mandatory molecular testing to histological diagnosis, reporting some changes in diagnosis and/or management; however, the impact on outcomes could not be adequately assessed. Transcriptome sequencing techniques have brought forward new diagnostic tools for identifying fusions and/or characterizing unclassified entities. Next-generation sequencing and advanced molecular techniques were also applied to identify potential targets for directed and epigenetic therapy, where preclinical studies reported results for agents active within the receptor tyrosine kinase, mTOR, Notch, Wnt, Hedgehog, Hsp90, and MDM2 signaling networks. At the level of clinical practice, modest developments were seen for some sarcoma subtypes in conventional chemotherapy and in therapies targeting the pathways activated by various receptor tyrosine kinases. In the burgeoning field of immune therapy, sarcoma work is in its infancy; however, elaborate protocols for immune stimulation are being explored, and checkpoint blockade agents advance from preclinical models to clinical studies. PMID:27732970

  14. Transplacental cancerization by Rous sarcoma virus.

    PubMed

    Nastac, E; Stoian, M; Hozoc, M; Athanasiu, P

    1979-01-01

    Experimental data obtained in the mouse are presented, pointing to the possibility of transplacental transmission of the genetic information contained in the genome of Rous sarcoma virus(RSV), by either intact RSV virions or nucleic acids extracted from infectant suspensions of tumors induced by the Carr(Ziber) on Bryan RSV strains.

  15. Preoperative diagnosis of a pulmonary artery sarcoma.

    PubMed Central

    Velebit, V.; Christenson, J. T.; Simonet, F.; Maurice, J.; Schmuziger, M.; Hauser, H.; Didier, D.

    1995-01-01

    A pulmonary artery sarcoma was diagnosed preoperatively by magnetic resonance imaging enhanced with gadolinium and confirmed by percutaneous computed tomographic guided needle biopsy. Accurate preoperative diagnosis allowed planned curative surgery with removal of the right ventricular outflow tract and reconstructive surgery using a cryopreserved homograft. Images PMID:8539663

  16. Feline postvaccinal sarcoma: 20 years later.

    PubMed

    Wilcock, Brian; Wilcock, Anne; Bottoms, Katherine

    2012-04-01

    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.

  17. Pulmonary artery sarcoma mimicking pulmonary embolism.

    PubMed

    El-Sayed Ahmed, Magdy M; Aftab, Muhammad; Al-Najjar, Raed M; de la Cruz, Kim I; Benjamin, Robert S; Hallman, Charles H

    2014-10-01

    Primary sarcomas that arise from major blood vessels are exceedingly rare, and some of the published cases have been autopsy reports. Most patients are adults. We report a case of pulmonary artery sarcoma in a 77-year-old man who presented with acute onset of dyspnea. Magnetic resonance imaging of the chest revealed a large mass within the pulmonary trunk and its main branches. Because massive pulmonary embolism was suspected, both anticoagulant and thrombolytic therapies were initiated. The patient responded poorly to these therapies, which then necessitated resection of both the mass and the pulmonary valve. A bioprosthetic porcine valve replaced the native valve, and we reconstructed the right ventricular outflow tract with a Dacron patch. Histopathologic examination revealed a high-grade sarcoma with focal myogenic and chondrogenic differentiation. The patient tolerated the procedure well and was discharged from the hospital on postoperative day 7. He was subsequently treated with chemotherapy and radiation and continued to show no evidence of disease. The diagnosis of pulmonary artery sarcoma should be suspected in patients who present with manifestations of pulmonary embolism, especially when there is no evidence of deep venous thrombosis and poor response to anticoagulant therapy. Multimodal therapy can provide prolonged survival.

  18. Pulmonary Artery Sarcoma Mimicking Pulmonary Embolism

    PubMed Central

    Aftab, Muhammad; Al-Najjar, Raed M.; de la Cruz, Kim I.; Benjamin, Robert S.; Hallman, Charles H.

    2014-01-01

    Primary sarcomas that arise from major blood vessels are exceedingly rare, and some of the published cases have been autopsy reports. Most patients are adults. We report a case of pulmonary artery sarcoma in a 77-year-old man who presented with acute onset of dyspnea. Magnetic resonance imaging of the chest revealed a large mass within the pulmonary trunk and its main branches. Because massive pulmonary embolism was suspected, both anticoagulant and thrombolytic therapies were initiated. The patient responded poorly to these therapies, which then necessitated resection of both the mass and the pulmonary valve. A bioprosthetic porcine valve replaced the native valve, and we reconstructed the right ventricular outflow tract with a Dacron patch. Histopathologic examination revealed a high-grade sarcoma with focal myogenic and chondrogenic differentiation. The patient tolerated the procedure well and was discharged from the hospital on postoperative day 7. He was subsequently treated with chemotherapy and radiation and continued to show no evidence of disease. The diagnosis of pulmonary artery sarcoma should be suspected in patients who present with manifestations of pulmonary embolism, especially when there is no evidence of deep venous thrombosis and poor response to anticoagulant therapy. Multimodal therapy can provide prolonged survival. PMID:25425986

  19. Osteogenic sarcoma presenting with lung metastasis.

    PubMed

    Staddon, Arthur P; Lackman, Richard; Robinson, Keith; Shrager, Joseph B; Warhol, Michael

    2002-01-01

    A patient with osteogenic sarcoma presenting with lung metastases is discussed with attention to appropriate diagnosis, staging, and treatment. Multimodality treatment options using chemotherapy, orthopedic surgery and thoracic surgery are presented. Physical medicine and rehabilitation evaluation and treatment are included. Current research options are discussed.

  20. Lateralization Technique and Inferior Alveolar Nerve Transposition

    PubMed Central

    Sanches, Marco Antonio; Ramalho, Gabriel Cardoso; Manzi, Marcello Roberto

    2016-01-01

    Bone resorption of the posterior mandible can result in diminished bone edge and, therefore, the installation of implants in these regions becomes a challenge, especially in the presence of the mandibular canal and its contents, the inferior alveolar nerve. Several treatment alternatives are suggested: the use of short implants, guided bone regeneration, appositional bone grafting, distraction osteogenesis, inclined implants tangential to the mandibular canal, and the lateralization of the inferior alveolar nerve. The aim was to elucidate the success rate of implants in the lateralization technique and in inferior alveolar nerve transposition and to determine the most effective sensory test. We conclude that the success rate is linked to the possibility of installing implants with long bicortical anchor which favors primary stability and biomechanics. PMID:27433360

  1. Matrix Metalloproteinase 1: Role in Sarcoma Biology

    PubMed Central

    Jawad, Muhammad Umar; Garamszegi, Nandor; Garamszegi, Susanna P.; Correa-Medina, Mayrin; Diez, Juan A.; Wen, Rong; Scully, Sean P.

    2010-01-01

    In carcinomas stromal cells participate in cancer progression by producing proteases such as MMPs. The expression MMP1 is a prognostic factor in human chondrosarcoma, however the role in tumor progression is unknown. Laser capture microdissection and In Situ hybridization were used to determine cellular origin of MMP1 in human sarcomas. A xenogenic model of tumor progression was then used and mice were divided in two groups: each harboring either the control or a stably MMP1 silenced cell line. Animals were sacrificed; the neovascularization, primary tumor volumes, and metastatic burden were assessed. LCM and RNA-ISH analysis revealed MMP1 expression was predominantly localized to the tumor cells in all samples of sarcoma (p = 0.05). The percentage lung metastatic volume at 5 weeks (p = 0.08) and number of spontaneous deaths secondary to systemic tumor burden were lower in MMP1 silenced cell bearing mice. Interestingly, this group also demonstrated a larger primary tumor size (p<0.04) and increased angiogenesis (p<0.01). These findings were found to be consistent when experiment was repeated using a second independent MMP1 silencing sequence. Prior clinical trials employing MMP1 inhibitors failed because of a poor understanding of the role of MMPs in tumor progression. The current findings indicating tumor cell production of MMP1 by sarcoma cells is novel and highlights the fundamental differences in MMP biology between carcinomas and sarcomas. The results also emphasize the complex roles of MMP in tumor progression of sarcomas. Not only does metastasis seem to be affected by MMP1 silencing, but also local tumor growth and angiogenesis are affected inversely. PMID:21170377

  2. Remodeling of alveolar septa after murine pneumonectomy

    PubMed Central

    Ysasi, Alexandra B.; Wagner, Willi L.; Bennett, Robert D.; Ackermann, Maximilian; Valenzuela, Cristian D.; Belle, Janeil; Tsuda, Akira; Konerding, Moritz A.

    2015-01-01

    In most mammals, removing one lung (pneumonectomy) results in the compensatory growth of the remaining lung. In mice, stereological observations have demonstrated an increase in the number of mature alveoli; however, anatomic evidence of the early phases of alveolar growth has remained elusive. To identify changes in the lung microstructure associated with neoalveolarization, we used tissue histology, electron microscopy, and synchrotron imaging to examine the configuration of the alveolar duct after murine pneumonectomy. Systematic histological examination of the cardiac lobe demonstrated no change in the relative frequency of dihedral angle components (Ends, Bends, and Junctions) (P > 0.05), but a significant decrease in the length of a subset of septal ends (“E”). Septal retraction, observed in 20–30% of the alveolar ducts, was maximal on day 3 after pneumonectomy (P < 0.01) and returned to baseline levels within 3 wk. Consistent with septal retraction, the postpneumonectomy alveolar duct diameter ratio (Dout:Din) was significantly lower 3 days after pneumonectomy compared to all controls except for the detergent-treated lung (P < 0.001). To identify clumped capillaries predicted by septal retraction, vascular casting, analyzed by both scanning electron microscopy and synchrotron imaging, demonstrated matted capillaries that were most prominent 3 days after pneumonectomy. Numerical simulations suggested that septal retraction could reflect increased surface tension within the alveolar duct, resulting in a new equilibrium at a higher total energy and lower surface area. The spatial and temporal association of these microstructural changes with postpneumonectomy lung growth suggests that these changes represent an early phase of alveolar duct remodeling. PMID:26078396

  3. Increased alveolar plasminogen activator in early asbestosis

    SciTech Connect

    Cantin, A.; Allard, C.; Begin, R.

    1989-03-01

    Alveolar macrophage-derived plasminogen activator (PA) activity is decreased in some chronic interstitial lung diseases such as idiopathic pulmonary fibrosis and sarcoidosis but increased in experimental models of acute alveolitis. Although asbestos fibers can stimulate alveolar macrophages (AM) to release PA in vitro, the effect of chronic asbestos exposure of the lower respiratory tract on lung PA activity remains unknown. The present study was designed to evaluate PA activity of alveolar macrophages and bronchoalveolar lavage (BAL) fluid in asbestos-exposed sheep and asbestos workers. Forty-three sheep were exposed to either 100 mg UICC chrysotile B asbestos in 100 ml phosphate-buffered saline (PBS) or to 100 ml PBS by tracheal infusion every 2 wk for 18 months. At Month 18, chest roentgenograms were analyzed and alveolar macrophage and extracellular fluid PA activity were measured in samples obtained by BAL. Alveolar macrophage PA activity was increased in the asbestos-exposed sheep compared to control sheep (87.2 +/- 17.3 versus 41.1 +/- 7.2 U/10(5) AM-24 h, p less than 0.05) as was the BAL fluid PA activity (674.9 +/- 168.4 versus 81.3 +/- 19.7 U/mg alb-24 h, p less than 0.01). Among the asbestos-exposed sheep, 10 had normal chest roentgenograms (Group SA) and 15 had irregular interstitial opacities (Group SB). Strikingly, whereas Group SA did not differ from the control group in BAL cellularity or PA activity, Group SB had marked increases in alveolar macrophages (p less than 0.005), AM PA activity (p less than 0.02), and BAL PA activity (p less than 0.001) compared to the control group.

  4. Integrity of the alveolar-capillary barrier and alveolar surfactant system in smokers.

    PubMed Central

    Schmekel, B; Bos, J A; Khan, A R; Wohlfart, B; Lachmann, B; Wollmer, P

    1992-01-01

    BACKGROUND: The permeability of the alveolar-capillary barrier to technetium-99m labelled diethylenetriamine pentaacetate (99mTc DTPA) is known to be greatly increased in smokers, but the underlying mechanism is poorly understood. Abnormal permeability of the alveolar epithelium as well as impaired surfactant function has been suggested. The purpose of this study was to examine transudation of urea and albumin into the alveoli and alveolar surfactant function in smokers and non-smokers and to relate these variables to the rate of alveolar-capillary transfer of 99mTc DTPA. METHODS: Standardised bronchoalveolar lavage was performed and the yield of urea and albumin measured in the lavage fluid. The integrity of the alveolar surfactant system was assessed by measurement of the surface activity and of the yield of phospholipids in alveolar lavage fluid. RESULTS: The mean decay constant for the pulmonary clearance of 99mTc DTPA was 0.028/min in the smokers and 0.009/min in the non-smokers. The recovery of albumin and urea in alveolar lavage fluid was very similar in the two groups. The surface activity of alveolar lavage fluid was lower in smokers than in non-smokers (minimum surface tension 37.9 versus 28.6 mN/m) and the yield of phospholipids was reduced (2.08 versus 3.86 mg). The rate constant for the pulmonary clearance of 99mTc DTPA correlated with the yield of phospholipids at bronchoalveolar lavage. CONCLUSIONS: The study shows that increased alveolar-capillary transfer of 99mTc DTPA in smokers is not accompanied by increased transudation of small or large molecules into the alveoli. The findings support the hypothesis that increased clearance of 99mTc DTPA in smokers is related to surfactant dysfunction. PMID:1412116

  5. Bone graft healing in alveolar osteoplasty in patients with unilateral lip, alveolar process, and palate clefts.

    PubMed

    Rychlik, Dariusz; Wójcicki, Piotr

    2012-01-01

    Secondary osteoplasty by means of autogenic spongy bone grafting is the most common procedure used in the reconstruction of the continuity of the maxillary alveolar process. The aim of the study was to analyze retrospectively the effect of certain factors on the course of the bone graft healing process in patients with unilateral complete clefts of the lip, alveolar process, and palate. The investigations involved 62 children aged 8 to 14 years (mean age, 11 years) with unilateral complete cleft of the lip, alveolar process, and palate operated on at the Clinic of Plastic Surgery in Polanica Zdrój from November 2007 to April 2009. All the procedures consisted in the reconstruction of the maxillary alveolar process by means of autogenic spongy bone grafting from the iliac bone. The analysis was performed on the basis of computed tomography scans presenting maxillary alveolar processes in the horizontal cross-sectional planes performed on the second or third postoperative day and after 6 months. They were used as the basis for the measurement of the volume and density (condensation) of the bone graft, the surface of its adhesion to the maxillary alveolar bone, and the volume and density of the healed bone. The following correlation coefficients were determined: between the adhesion surface of the bone to the alveolar bone and the volume of the healed bone, between the adhesion surface of the bone to the alveolar bone and the density of the healed bone, and between the density of the graft and the volume of the healed bone. Increasing the surface of the graft adhesion to the bone ridges of the alveolar cleft contributes to increased volume of the healed bone and slows down the increase in its density (on 6-month follow-up). Crushing of the bone graft increases its resorption and reduces volume of the healed bone.

  6. 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.

  7. 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

  8. ALVEOLAR BREATH SAMPLING AND ANALYSIS IN HUMAN EXPOSURE ASSESSMENT STUDIES

    EPA Science Inventory

    Alveolar breath sampling and analysis can be extremely useful in exposure assessment studies involving volatile organic compounds (VOCs). Over recent years scientists from the EPA's National Exposure Research Laboratory have developed and refined an alveolar breath collection ...

  9. ALVEOLAR BREATH SAMPLING AND ANALYSIS IN HUMAN EXPOSURE ASSESSMENT STUDIES

    EPA Science Inventory

    Alveolar breath sampling and analysis can be extremely useful in exposure assessment studies involving volatile organic compounds (VOCs). Over recent years scientists from the EPA's National Exposure Research Laboratory have developed and refined an alveolar breath collection ...

  10. Pulmonary alveolar microlithiasis: review of Turkish reports.

    PubMed Central

    Ucan, E S; Keyf, A I; Aydilek, R; Yalcin, Z; Sebit, S; Kudu, M; Ok, U

    1993-01-01

    Pulmonary alveolar microlithiasis is a rare disorder, only 173 cases having been reported worldwide. Fifty two cases from Turkey are reported, 49 of which have previously been described only in Turkish publications. The mean age of the patients was 27 (SD 12) years, 34 were male, and 10 were symptomless. In 40 of the 52 cases diagnosis was confirmed histopathologically. Nineteen cases were diagnosed in siblings. This high rate suggests that pulmonary alveolar microlithiasis is a familial disease, which, though rare, is for unknown reasons most common in Turkey. Images PMID:8493634

  11. Idiopathic inflammatory myopathy with diffuse alveolar damage.

    PubMed

    Lee, C-S; Chen, T-L; Tzen, C-Y; Lin, F-J; Peng, M-J; Wu, C-L; Chen, P-J

    2002-09-01

    Interstitial lung disease (ILD) in patients with myositis is defined by the presence of interstitial changes on radiographic examination. The reported prevalence of ILD varies from 0% to nearly 50%. However, only rarely has the pathological pattern of diffuse alveolar damage (DAD) associated with idiopathic inflammatory myopathy (IIM) been reported. We report five patients with IIM (one with dermatomyositis, one with polymyositis, and three with amyopathic dermatomyositis) and respiratory failure. Four underwent open lung biopsy with pathological proof of diffuse alveolar damage (DAD). Despite intensive immunosuppressive therapy, all of them died. In addition to the case reports, we discuss DAD in patients with IIM.

  12. Lung Transplant Recipient with Pulmonary Alveolar Proteinosis

    PubMed Central

    Hahn, M. Frances; Abdelrazek, Hesham; Patel, Vipul J.; Walia, Rajat

    2016-01-01

    Pulmonary alveolar proteinosis (PAP) is a progressive lung disease characterized by accumulated surfactant-like lipoproteinaceous material in the alveoli and distal bronchioles. This accumulation is the result of impaired clearance by alveolar macrophages. PAP has been described in 11 solid organ transplant recipients, 9 of whom were treated with mammalian target of rapamycin inhibitors. We report a case of a lung transplant recipient treated with prednisone, mycophenolate mofetil (MMF), and tacrolimus who ultimately developed PAP, which worsened when MMF was replaced with everolimus. PMID:27213073

  13. Alternative lengthening of telomeres does exist in various canine sarcomas.

    PubMed

    Kreilmeier, Theresa; Sampl, Sandra; Deloria, Abigail J; Walter, Ingrid; Reifinger, Martin; Hauck, Marlene; Borst, Luke B; Holzmann, Klaus; Kleiter, Miriam

    2017-03-01

    Alternative lengthening of telomeres (ALT) is a telomere maintenance mechanism (TMM) found in some human tumors such as sarcomas. Canine tumors are not characterized for ALT and the study aim was to identify if the ALT phenotype exists in canine sarcomas. Sixty-four canine sarcoma samples (20 snap-frozen, 44 FFPE) as well as six canine sarcoma cell lines were screened for ALT by C-circle assay. ALT was further evaluated by measuring telomere length via qPCR and telomere restriction-fragments including pulsed-field electrophoresis. ALT-associated proteins were validated by immunohistochemistry. Further, telomerase activity (TA) and gene expression were analyzed by TRAP and qPCR. DNA from 20 human neuroblastomas and 8 sarcoma cell lines served as comparative controls. ALT was detected in 9.4% (6/64) canine sarcomas including aggressive subtypes as hemangiosarcoma, osteosarcoma, and histiocytic sarcoma. C-circle levels were comparable with human ALT-positive controls. All ALT tumors demonstrated loss of ATRX expression and 5/6 showed strong p53 expression. TA was detected in 93% (14/15) snap-frozen samples including a sarcoma with ALT activity. This tumor showed long heterogeneous telomeres, and a high level of colocalization of DAXX with telomeres. One sarcoma was ALT and TA negative. All canine and human sarcoma cell lines were ALT negative. In this study, we demonstrated that canine sarcomas use ALT. As in humans, ALT was identified in aggressive sarcomas subtypes and coexisted with TA in one tumor. Overall, canine sarcomas seem to share many similarities with their human counterparts and appear an attractive model for comparative telomere research. © 2016 Wiley Periodicals, Inc.

  14. 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

  15. Cabozantinib-s-malate in Treating Patients With Relapsed Osteosarcoma or Ewing Sarcoma

    ClinicalTrials.gov

    2017-09-04

    Metastatic Ewing Sarcoma; Metastatic Osteosarcoma; Recurrent Ewing Sarcoma; Recurrent Osteosarcoma; Stage III Osteosarcoma AJCC v7; Stage IV Osteosarcoma AJCC v7; Stage IVA Osteosarcoma AJCC v7; Stage IVB Osteosarcoma AJCC v7; Unresectable Ewing Sarcoma; Unresectable Osteosarcoma

  16. [An unusual primary vascular tumor: intimal sarcoma of the pulmonary artery].

    PubMed

    Brochériou, I; Quillard, A; Gatecel, C; Wassef, M

    2000-01-01

    Primary sarcomas of great vessels are rare and involve the aorta, pulmonary artery and inferior vena cava. The pathologic classification of these tumors can be made on the location of the sarcoma in relation to the vessel wall, luminal or mural. Luminal sarcomas are usually intimal sarcoma and mural sarcoma are most frequently leiomyosarcoma. The myofibroblastic or endothelial differentiation of these tumors is still debated. We report a case of intimal sarcoma of the pulmonary artery.

  17. The Roles of Sox Family Genes in Sarcoma.

    PubMed

    Li, Jingyuan; Shen, Jacson; Wang, Kunzheng; Hornicek, Francis; Duan, Zhenfeng

    2016-01-01

    Sox (SRY-related HMG-box) family genes are important regulators of cell development, homeostasis, and regeneration. Deregulation of certain members of the Sox gene family has been implicated in a number of human malignancies, including in sarcoma. Accumulating evidence suggests that Sox genes play crucial roles in sarcoma cell pathogenesis, growth, and proliferation. Here, we review the biological relevance of Sox2 and Sox9 genes in osteosarcoma, chondrosarcoma and chordoma; Sox2, Sox6, and Sox17 genes in Ewing's sarcoma; Sox2, Sox9, and Sox10 genes in synovial sarcoma; Sox2 gene in fibrosarcoma; and Sox21 gene in liposarcoma. These findings potentiate the targeting of Sox genes for novel therapeutic interventions in sarcoma and may also hold valuable clinical potential to improve the care of patients with sarcoma.

  18. Clinical trial enrollment of adolescents and young adults with sarcoma.

    PubMed

    Davis, Lara E; Janeway, Katherine A; Weiss, Aaron R; Chen, Yen-Lin E; Scharschmidt, Thomas J; Krailo, Mark; Glade Bender, Julia L; Kopp, Lisa M; Patel, Shreyaskumar R; Schwartz, Gary K; Horvath, L Elise; Hawkins, Douglas S; Chuk, Meredith K; Reinke, Denise K; Gorlick, Richard G; Randall, R Lor

    2017-09-15

    More than half of all sarcomas occur in adolescents and young adults (AYAs) aged 15 to 39 years. After the publication of the AYA series in the April 1, 2016 issue of Cancer, several leaders in the field of sarcoma across disciplines gathered to discuss the status of sarcoma clinical research in AYAs. They determined that a focused effort to include the underrepresented and understudied AYA population in current and future sarcoma clinical trials is overdue. Trial enrichment for AYA-aged sarcoma patients will produce more meaningful results that better represent the disease's biology, epidemiology, and treatment environment. To address the current deficit, this commentary outlines changes believed to be necessary to expediently achieve an increase in the enrollment of AYAs in sarcoma clinical trials. Cancer 2017;123:3434-40. © 2017 American Cancer Society. © 2017 American Cancer Society.

  19. Alveolar proteinosis associated with aluminium dust inhalation.

    PubMed

    Chew, R; Nigam, S; Sivakumaran, P

    2016-08-01

    Secondary alveolar proteinosis is a rare lung disease which may be triggered by a variety of inhaled particles. The diagnosis is made by detection of anti-granulocyte-macrophage colony-stimulating factor antibodies in bronchoalveolar lavage fluid, which appears milky white and contains lamellar bodies. Aluminium has been suggested as a possible cause, but there is little evidence in the literature to support this assertion. We report the case of a 46-year-old former boilermaker and boat builder who developed secondary alveolar proteinosis following sustained heavy aluminium exposure. The presence of aluminium was confirmed both by histological examination and metallurgical analysis of a mediastinal lymph node. Despite cessation of exposure to aluminium and treatment with whole-lung lavage which normally results in improvements in both symptoms and lung function, the outcome was poor and novel therapies are now being used for this patient. It may be that the natural history in aluminium-related alveolar proteinosis is different, with the metal playing a mediating role in the disease process. Our case further supports the link between aluminium and secondary alveolar proteinosis and highlights the need for measures to prevent excessive aluminium inhalation in relevant industries. © The Author 2016. Published by Oxford University Press on behalf of the Society of Occupational Medicine. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  20. Pulmonary sarcoidosis with an alveolar radiographic pattern.

    PubMed Central

    Battesti, J P; Saumon, G; Valeyre, D; Amouroux, J; Pechnick, B; Sandron, D; Georges, R

    1982-01-01

    Thirty-three cases of sarcoidosis (4.4% of 746 patients) showed an alveolar radiological pattern. A study of pulmonary function was carried out in 25 patients and compared with that of 46 patients with the interstitial radiological type of sarcoidosis. Twenty-two cases have been followed up from one to six years after the initial examination. The radiographic lesions were most often bilateral and included nodules greater than 15 mm with ill-defined margins or diffuse, infiltrative, non-retractile opacities with fluffy margins. Bilateral mediastinal lymph nodes were present in 27 patients. In 20 patients an associated reticulation was found on radiography. In four patients an open lung biopsy was done. The granulomatous nodules were identical to those found in other forms of sarcoidosis, although they were more confluent in the affected areas. Clinical and functional findings did not differ from those in the more common forms of sarcoidosis. Alveolar sarcoidosis has a sudden course. The alveolar radiological patterns always disappeared, with or without steroid treatment, while reticular patterns persisted in four patients. Rapid radiological changes were observed. Some functional abnormalities persisted in cases that were followed. It is concluded that alveolar sarcoidosis is a distinct acute form of sarcoidosis. Images PMID:7135279

  1. Tirofiban-Induced Diffuse Alveolar Hemorrhage

    PubMed Central

    Guo, Jincheng; Xu, Min; Xi, Yutao

    2012-01-01

    Platelets play an important role in the development of acute coronary syndromes. Evidence indicates that platelet-inhibiting drugs, such as glycoprotein IIb/IIIa inhibitors, can be beneficial when they are administered at the time of primary percutaneous coronary intervention for acute ST-segment-elevation myocardial infarction. However, an associated increase in the risk of bleeding is well documented. Diffuse alveolar hemorrhage is a rare but life-threatening and underdiagnosed complication of therapy with glycoprotein IIb/IIIa inhibitors. Diffuse alveolar hemorrhage can easily be mistaken for acute pulmonary edema, a condition commonly seen in patients with acute coronary syndrome. Physicians need to be aware of this diagnostic dilemma, because early treatment increases the chance that the patients will survive. Herein, we report the fatal outcome of diffuse alveolar hemorrhage in a 73-year-old man who presented with acute ST-segment-elevation myocardial infarction and was treated with tirofiban in conjunction with primary percutaneous coronary intervention. In addition, we review the medical literature pertaining to the sequelae of glycoprotein IIb/IIIa inhibitor therapy in the presence of diffuse alveolar hemorrhage. PMID:22412240

  2. Teaching Alveolar Ventilation with Simple, Inexpensive Models

    ERIC Educational Resources Information Center

    DiCarlo, Stephen E.

    2008-01-01

    When teaching and learning about alveolar ventilation with our class of 300 first-year medical students, we use four simple, inexpensive "models." The models, which encourage research-oriented learning and help our students to understand complex ideas, are distributed to the students before class. The students anticipate something new every day,…

  3. Teaching Alveolar Ventilation with Simple, Inexpensive Models

    ERIC Educational Resources Information Center

    DiCarlo, Stephen E.

    2008-01-01

    When teaching and learning about alveolar ventilation with our class of 300 first-year medical students, we use four simple, inexpensive "models." The models, which encourage research-oriented learning and help our students to understand complex ideas, are distributed to the students before class. The students anticipate something new every day,…

  4. Alveolar mechanics using realistic acinar models

    NASA Astrophysics Data System (ADS)

    Kumar, Haribalan; Lin, Ching-Long; Tawhai, Merryn H.; Hoffman, Eric A.

    2009-11-01

    Accurate modeling of the mechanics in terminal airspaces of the lung is desirable for study of particle transport and pathology. The flow in the acinar region is traditionally studied by employing prescribed boundary conditions to represent rhythmic breathing and volumetric expansion. Conventional models utilize simplified spherical or polygonal units to represent the alveolar duct and sac. Accurate prediction of flow and transport characteristics may require geometries reconstructed from CT-based images and serve to understand the importance of physiologically realistic representation of the acinus. In this effort, we present a stabilized finite element framework, supplemented with appropriate boundary conditions at the alveolar mouth and septal borders for simulation of the alveolar mechanics and the resulting airflow. Results of material advection based on Lagrangian tracking are presented to complete the study of transport and compare the results with simplified acinar models. The current formulation provides improved understanding and realization of a dynamic framework for parenchymal mechanics with incorporation of alveolar pressure and traction stresses.

  5. [Classic and aggressive Kaposi sarcoma with bone involvement].

    PubMed

    Sbiyaa, Mouhcine; El Alaoui, Adil; El Bardai, Mohammed; Mezzani, Amine; Lahrach, Kamal; Marzouki, Amine; Boutayeb, Fawzi

    2016-01-01

    Classic Kaposi sarcoma is a multifocal rare tumor originating from vascular endothelial cells with progressive evolution and little malignant predisposition. Although Kaposi sarcoma with extensive visceral involvement is sometimes observed among HIV-positive patients, tumor dissemination to visceral lymph nodes in classic SK remains very rare. We report a rare case of aggressive classic Kaposi sarcoma of the hand with a rapid and destructive development.

  6. Radiation-induced prostatic sarcoma: A case report

    SciTech Connect

    Scully, J.M.; Uno, J.M.; McIntyre, M.; Mosely, S. )

    1990-09-01

    A 64-year-old man had a prostatic sarcoma 8 years after transurethral prostatectomy and radical bilateral pelvic lymph node dissection with insertion of 125-iodine implants for stage B1N carcinoma of the prostate. Therapy for the sarcoma consisted of isolated pelvic perfusion and then pelvic exenteration with creation of an ileal conduit and colostomy. The pathology report showed well encapsulated grade 2 spindle cell sarcoma of the prostate. Multiple small metallic particles were embedded in the tumor specimen.

  7. Primary extraskeletal Ewing's sarcoma/primitive neuroectodermal tumour of breast.

    PubMed

    Ikhwan, S M; Kenneth, V K T; Seoparjoo, A; Zin, A A M

    2013-06-21

    Primary primitive neuroectodermal tumour (PNET) and extraskeletal Ewing's sarcoma belongs to the Ewing's family of tumours. Primary tumours arising from breast are very rare. There are only a few case reports published on primary extraskeletal Ewing's sarcoma and PNET arising from breast. We present an extremely rare case of an inoperable primary Ewing's sarcoma arising from left breast with contralateral breast, lymphatic and lung metastasis.

  8. Recurrent pulmonary intimal sarcoma involving the right ventricular outflow tract.

    PubMed

    Shah, Dipesh K; Joyce, Lyle D; Grogan, Martha; Aubry, Marie Christine; Miller, John A; Ding, Wei; Haddock, Michael G

    2011-03-01

    Intimal sarcoma of the pulmonary artery is commonly misdiagnosed as chronic pulmonary embolism. Rarely, it can involve the right ventricular outflow tract and the pulmonary valve. We report a patient who was treated surgically for an intimal sarcoma of the pulmonary artery involving the right ventricular outflow tract and the pulmonary valve. The sarcoma recurred in about 8 weeks. It responded favorably to chemoradiation therapy and shows some signs of regression.

  9. Overcoming Therapeutic Resistance of Bone Sarcomas: Overview of the Molecular Mechanisms and Therapeutic Targets for Bone Sarcoma Stem Cells

    PubMed Central

    Ozaki, Toshifumi

    2016-01-01

    Bone sarcomas are heterogeneous malignant tumors that exhibit clinical, histological, and molecular heterogeneity. Recent progress in their multimodal treatment has gradually improved patient prognosis; however, drug resistance and distant metastasis remain unresolved clinical problems. Recent investigations have suggested the existence of cancer stem-like cells (CSCs) in bone sarcomas, which represent a subpopulation of tumor cells with high tumor-forming ability. The hallmarks of CSCs include tumor- and metastasis-forming potential and drug resistance, which are responsible for poor prognoses of bone sarcoma patients. Therefore, elucidation of the molecular mechanisms of CSCs and identification of therapeutic targets could contribute to novel treatment strategies for bone sarcomas and improve patient prognosis. This paper provides an overview of the accumulating knowledge on bone sarcoma stem cells and preclinical analyses to overcome their lethal phenotypes, in addition to a discussion of their potential for novel therapeutics for bone sarcomas. PMID:28115942

  10. 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

  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. Primary pulmonary synovial sarcoma: a rare primary pulmonary tumor.

    PubMed

    Falkenstern-Ge, Roger Fei; Kimmich, Martin; Grabner, Andreas; Horn, Heike; Friedel, Godehard; Ott, German; Kohlhäufl, Martin

    2014-02-01

    Pulmonary sarcomas overall are very uncommon and comprise only 0.5 % of all primary lung malignancies. The diagnosis is established only after sarcoma-like primary lung malignancies and a metastatic extrathoracic sarcoma have been excluded. Synovial sarcoma accounts for ~8 % of soft-tissue sarcomas. Synovial sarcoma arising from the pleura has rarely been reported. We report a case of a 58-year-old woman who complained of right-sided chest pain and shortness of breath. Chest CT scan revealed a large heterogeneous mass, occupying most of the right hemithorax. Histologic diagnosis was supplemented by interphase cytogenetic (FISH) analysis. Computed tomography guided Tru-cut biopsy was suspicious for a sarcomatous or fibrous malignancy. However, intraoperative frozen-section diagnostics confirmed the diagnosis of a sarcoma. Immunohistochemistry showed that tumor cells expressed epithelial membrane antigen, CD99 and BCL2. Based on immunohistochemistry, the diagnosis of synovial sarcoma was suspected and was confirmed by FISH analysis. The patient was treated with right upper bilobectomy. Due to R1-resection status, postsurgical systemic chemotherapy was administered. Primary pulmonary synovial sarcoma is a rare primary lung tumor. Due to extensive size of the tumor with pleural and mediastinal invasion only a R1-resection status could be achieved by thoracic surgery.

  13. Dermoscopy of Kaposi's sarcoma: areas exhibiting the multicoloured 'rainbow pattern'.

    PubMed

    Hu, S C-S; Ke, C-L K; Lee, C-H; Wu, C-S; Chen, G-S; Cheng, S-T

    2009-10-01

    Kaposi's sarcoma is a vascular tumour characterized by a proliferation of spindle cells and endothelial cells to form closely arranged slit-like vascular spaces. Currently, the definitive diagnosis of Kaposi's sarcoma relies on histology. The dermoscopic features of Kaposi's sarcoma are not clearly defined in the scientific literature. We seek to evaluate the dermoscopic features of Kaposi's sarcoma and compare them with other vascular tumours. One hundred forty-one lesions from seven patients with histologically proven Kaposi's sarcoma were evaluated using polarized light dermoscopy for the presence of various dermoscopic features. Twenty patients with other vascular tumours were also examined. Dermoscopic examination revealed bluish-reddish coloration (84% of lesions), multicoloured areas showing various colours of the rainbow spectrum (36%), scaly surface (29%), and small brown globules (15%). The 'rainbow pattern' was found in six out of seven patients with Kaposi's sarcoma and was not observed in other vascular tumours. In addition, there was an absence of dermoscopic features specific for other vascular and non-vascular skin tumours, such as well-defined lacunae or structured vascular pattern, in most of the Kaposi's sarcoma lesions. The most frequent dermoscopic patterns in Kaposi's sarcoma were found to be bluish-reddish coloration, the 'rainbow pattern', and scaly surface. The rainbow pattern is a dermoscopic feature which has not been previously described. We propose that dermoscopy, as an adjunct to clinical examination, may enhance accuracy in the preoperative diagnosis of Kaposi's sarcoma.

  14. [Primary pharynx synovial sarcoma: a case report].

    PubMed

    Qingying, Cui; Youmei, Zhang; Shuai, Fu; Changbin, Zhang; Ming, Li

    2017-04-01

    A case of primary pharynx synovial sarcoma was reported in this paper. A 15-year-old male patient experienced painless pharyngeal swelling that gradually proliferated for 1 month. Special examination showed an 8 cm × 4 cm × 3 cm tumor located in the left pharynx and the supratonsillar crypt. Imaging tests revealed an irregular mass on the left side of the oropharynx and an unclear boundary. Immunohistochemical examination yielded the following results: epithelial membrane antigen (+), cytokeratin (CK)19 (+), CD7(+), vimentin (+), CK10(-), E-cadherin (+), B-cell lymphoma-2 (-), CD2 (-), CD10 (-), CD138 (+), CD99 (+), leukocyte common antigen (+), and Ki-67 (20%+). This condition was pathologically diagnosed as primary pharynx synovial sarcoma.

  15. Animal models of soft-tissue sarcoma

    PubMed Central

    Dodd, Rebecca D.; Mito, Jeffery K.; Kirsch, David G.

    2010-01-01

    Soft-tissue sarcomas (STSs) are rare mesenchymal tumors that arise from muscle, fat and connective tissue. Currently, over 75 subtypes of STS are recognized. The rarity and heterogeneity of patient samples complicate clinical investigations into sarcoma biology. Model organisms might provide traction to our understanding and treatment of the disease. Over the past 10 years, many successful animal models of STS have been developed, primarily genetically engineered mice and zebrafish. These models are useful for studying the relevant oncogenes, signaling pathways and other cell changes involved in generating STSs. Recently, these model systems have become preclinical platforms in which to evaluate new drugs and treatment regimens. Thus, animal models are useful surrogates for understanding STS disease susceptibility and pathogenesis as well as for testing potential therapeutic strategies. PMID:20713645

  16. Polypoid uterine lesions mimicking endometrial stromal sarcoma.

    PubMed Central

    McCluggage, W G; Alderdice, J M; Walsh, M Y

    1999-01-01

    Two polypoid submucosal uterine lesions were examined histologically and immunohistochemically with monoclonal antibodies to desmin and alpha smooth muscle actin. One case comprised a leiomyoma and the other a polypoid form of adenomyosis. Both polyps had prolapsed through the external cervical os. The lesions had an ulcerated surface with focal areas of marked increased cellularity and pronounced vascularity throughout, such that they mimicked a low grade endometrial stromal sarcoma infiltrating the myometrium. The cellular areas showed diffuse positivity for desmin and alpha smooth muscle actin, confirming them to be of smooth muscle origin. The changes of marked hypercellularity and pronounced vascularity within polypoid submucosal uterine lesions have not been emphasised in published reports up to now. Pathologists should be aware of these morphological features in order to avoid misdiagnosis of such cases as endometrial stromal sarcomas. The changes described here are likely to be secondary to trauma associated with a polypoid lesion prolapsing through the external cervical os. Images PMID:10605413

  17. Kaposi's visceral sarcoma in liver transplant recipients.

    PubMed

    García-Sesma, A; Jiménez, C; Loinaz, C; Meneu, J C; Colina, F; Marqués, E; Gómez, R; Abradelo, M; Garcia, J I; Moreno González, E

    2003-08-01

    We report three cases of Kaposi's sarcoma after orthotopic liver transplantation performed for cirrhosis related to hepatitis C virus (one case), ethanol (one case), or both (one case). All patients displayed disease within the first year after liver transplantation, and only in one case was the diagnosis obtained before the patient died. All three patients were on tacrolimus-steroid therapy, and in one case mycophenolate mofetil was added to treat acute persistent rejection.

  18. Ewing's Sarcoma of the Cranial Vault

    PubMed Central

    Narayanan, Geetha; Sreelesh, K. P.; Somanathan, Thara; Soman, Lali V.

    2016-01-01

    Primary Ewing's sarcoma (EWS) arising from cranial bones is rare and accounts for only 1%–4% of all EWS. We report the case of a 15-year-old girl with EWS of the frontoparietal region of the skull. She underwent excision following which she received combination chemotherapy with vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide, and VP16 and local radiation of 45 Gy. She is alive in complete remission at 40 months. PMID:28163522

  19. 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.

  20. Carbon Ion Radiotherapy for Unresectable Retroperitoneal Sarcomas

    SciTech Connect

    Serizawa, Itsuko; Kagei, Kenji; Kamada, Tadashi; Imai, Reiko; Sugahara, Shinji; Okada, Tohru; Tsuji, Hiroshi; Ito, Hisao; Tsujii, Hirohiko

    2009-11-15

    Purpose: To evaluate the applicability of carbon ion radiotherapy (CIRT) for unresectable retroperitoneal sarcomas with regard to normal tissue morbidity and local tumor control. Methods and Materials: From May 1997 to February 2006, 24 patients (17 male and 7 female) with unresectable retroperitoneal sarcoma received CIRT. Age ranged from 16 to 77 years (median, 48.6 years). Of the patients, 16 had primary disease and 8 recurrent disease. Histologic diagnoses were as follows: malignant fibrous histiocytoma in 6, liposarcoma in 3, malignant peripheral nerve sheath tumor in 3, Ewing/primitive neuroectodermal tumor (PNET) in 2, and miscellaneous in 10 patients. The histologic grades were as follows: Grade 3 in 15, Grade 2-3 in 2, Grade 2 in 3, and unknown in 4. Clinical target volumes ranged between 57 cm{sup 3} and 1,194 cm{sup 3} (median 525 cm{sup 3}). The delivered carbon ion dose ranged from 52.8 to 73.6 GyE in 16 fixed fractions over 4 weeks. Results: The median follow-up was 36 months (range, 6-143 months). The overall survival rates at 2 and 5 years were 75% and 50%, respectively. The local control rates at 2 and 5 years were 77% and 69%. No complications of the gastrointestinal tract were encountered. No other toxicity greater than Grade 2 was observed. Conclusions: Use of CIRT is suggested to be effective and safe for retroperitoneal sarcomas. The results obtained with CIRT were a good overall survival rate and local control, notwithstanding the fact that most patients were not eligible for surgical resection and had high-grade sarcomas.

  1. De Novo Osteogenic Sarcoma of Mastoid Bone

    PubMed Central

    Işikdogan, Abdurrahman; Erekul, Selim; Pamir, Ali

    2002-01-01

    The most common primary malignant tumor of the bone is osteosarcoma. Primary involvement of the craniofacial bones in osteosarcoma is relatively rare. The mandible and the maxillae are the most commonly affected bones of the head. Here, we report a rare case of de novo high-grade osteogenic sarcoma of the mastoid region of the temporal bone and discuss the diagnostic and therapeutic properties. PMID:18521333

  2. 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.

  3. 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

  4. In vivo radiolocalization of antiosteogenic sarcoma monoclonal antibodies in osteogenic sarcoma xenografts

    SciTech Connect

    Nakamura, T.; Sakahara, H.; Hosoi, S.; Yamamuro, T.; Higashi, S.; Mikawa, H.; Endo, K.; Toyama, S.

    1984-05-01

    Monoclonal antibodies Ost6 and Ost7 (mouse Immunoglobulin G1) to human osteogenic sarcoma were isolated from ascitic fluid and labeled with radioiodine. After injection into athymic nu/nu mice with s.c. xenografts of human osteogenic sarcoma, the uptake of radioactivity in tumors, visceral organs, and blood was determined. Five days after injection, Ost6 and Ost7 showed preferential accumulation in tumors (tumor:blood ratio, 4.3). Furthermore, with testicular and bladder tumors, both unreactive with Ost7, there was no localization of radiolabeled Ost7 in xenograft growths. When Ost7 was labeled with /sup 131/I, its accumulation into human osteogenic sarcoma could be clearly visualized by whole-body gamma-scintigraphy without computer-assisted data processing.

  5. Alveolar Bone Fracture: Pathognomonic Sign for Clinical Diagnosis

    PubMed Central

    Gutmacher, Zvi; Peled, Eli; Norman, Doron; Lin, Shaul

    2017-01-01

    Aim: Dental injuries, especially luxation and avulsion, are common. Dental trauma can cause alveolar bone fracture that can lead to tooth loss and malocclusion. Single tooth alveolar bone fractures are difficult to identify unless it protrudes through the overlying mucosa and can be visualized. Pain, malocclusion, and tooth mobility provide signs of suspected alveolar bone fractures. Integrity of the proximate alveolar bone should be examined for fractures where avulsion, luxation, or other tooth trauma is detected. Any suggestion of alveolar fractures should be further investigated with an appropriate radiograph. Summary: This case report shows a pathognomonic sign that detects and diagnosis single tooth alveolar bone fractures, i.e., a localized hematoma crossing the attached gingiva from the free gingival margin to the vestibular mucosa. This should serve as a warning for localized alveolar bone fracture. A visualized hematoma and gentle, careful palpation may help detect covered fractures when the overlying mucosa is not perforated. PMID:28400864

  6. Lymphangioma-like Kaposi sarcoma: case report.

    PubMed

    Posada García, Celia; García-Cruz, Aranzazu; García-Doval, Ignacio; De La Torre, Carlos; Cruces, Manuel José

    2009-09-15

    Kaposi sarcoma (KS) is a multifocal vascular disease with uncertain histogenesis. It is characterized by clinical and histologic polymorphism. The "lymphangioma-like" variant is very uncommon, accounting for less than 5% of all cases. We report the case of a 76-year-old woman, HIV negative, with a 4-year history of classic Kaposi sarcoma treated with cryotherapy who developed new bullous lesions on her lower extremities. Biopsy revealed histologic findings of lymphangioma-like KS (LLKS), together with areas of classic KS; HHV-8 staining was positive. Diagnosis of LLKS was made and the patient was proposed for radiotherapy. The lymphangioma-like Kaposi sarcoma is a rare morphologic expression of KS characterized by dilated and bizarrely shaped vascular channels lined by flattened endothelium permeating the dermis. "Bulla-like" lesions have been considered as a clinical hallmark of this variant. Its histologic appearance suggests a lymphatic origin of KS and it may resemble other vascular tumors. Findings of areas of typical KS and positive staining for HHV-8 may help to make a definitive diagnosis.

  7. 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

  8. Tumor Lysis Syndrome in a Retroperitoneal Sarcoma.

    PubMed

    Zakharia, Yousef; Mansour, Joshua; Vasireddi, Srinivasa; Zakharia, Kais; Fatakhov, Eduard; Koch, Christopher; Hrinczenko, Borys

    2014-01-01

    In the present case, a 49-year-old white female presented to the clinic with a 2-month history of nausea, vomiting, and right upper quadrant pain. On examination a 3-cm mass on the right anterior scalene muscle was noted. A computed tomography scan was performed revealing a 8.7 × 7.7 × 6.1 cm retroperitoneal mass with possible invasion of the inferior vena cava and right renal and left common iliac veins. An excisional biopsy was performed with pathology compatible with spindle cell sarcoma. The patient was then sent for follow-up at the sarcoma clinic as an outpatient. However, before chemotherapy was to be started the patient would be admitted to the hospital with progressively worse nausea and vomiting. At that time the patient's lab work showed lactic acidosis, acute renal failure, hyperuricemia, hyperphosphatemia, and hypocalcemia, which met the Cairo-Bishop criteria for tumor lysis syndrome (TLS). The patient was admitted to the intensive care unit and kidney dialysis initiated. The patient would become progressively obtunded at which time the family opted for hospice care. The patient eventually succumbed peacefully 3 days after her last admission. In this case report, we briefly review the literature on TLS in solid tumors, and we present a rare case of spontaneous TLS in a retroperitoneal sarcoma.

  9. 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.

  10. Osteogenic sarcoma. Malignant fibrous histiocytoma subtype.

    PubMed

    Ballance, W A; Mendelsohn, G; Carter, J R; Abdul-Karim, F W; Jacobs, G; Makley, J T

    1988-08-15

    A distinctly different entity from the now well-delineated malignant fibrous histiocytoma (MFH) of bone is the MFH histopathologic subtype of osteogenic sarcoma. Although uncommon, recently the authors have encountered six cases of this neoplasm, in each of which the soft tissue component was devoid of bone elements and was microscopically indistinguishable from MFH of bone or soft tissue. Neoplastic osteoid and woven bone were present in the osseous component of each tumor, however. Radiologically, the lesions generally were osteoblastic but focally osteolytic with features typical of osteogenic sarcoma. Pain was the most common presenting symptom. There was no age or sex predilection. Immunocytochemical staining showed strong positivity with alpha-1-antichymotrypsin within malignant bizarre giant cells and occasional neoplastic osteoblasts in five cases. The biological behavior followed a very aggressive course. Four of the six patients developed pulmonary metastases 6 to 12 months after initial surgery; one patient presented initially with pulmonary metastases. Adequate tumor sampling as well as optimal correlation with clinical and radiographic information are required to distinguish the MFH subtype of osteogenic sarcoma from MFH of bone, both being high-grade neoplasms, however.

  11. The role of Kaposi sarcoma-associated herpesvirus in the pathogenesis of Kaposi sarcoma.

    PubMed

    Gramolelli, Silvia; Schulz, Thomas F

    2015-01-01

    Kaposi sarcoma (KS) is an unusual vascular tumour caused by an oncogenic-herpesvirus, Kaposi sarcoma-associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV 8). KS lesions are characterized by an abundant inflammatory infiltrate, the presence of KSHV-infected endothelial cells that show signs of aberrant differentiation, as well as faulty angiogenesis/ vascularization. Here we discuss the molecular mechanisms that lead to the development of these histological features of KS, with an emphasis on the viral proteins that are responsible for their development.

  12. Knockout Mice Reveal a Major Role for Alveolar Epithelial Type I Cells in Alveolar Fluid Clearance.

    PubMed

    Flodby, Per; Kim, Yong Ho; Beard, LaMonta L; Gao, Danping; Ji, Yanbin; Kage, Hidenori; Liebler, Janice M; Minoo, Parviz; Kim, Kwang-Jin; Borok, Zea; Crandall, Edward D

    2016-09-01

    Active ion transport by basolateral Na-K-ATPase (Na pump) creates an Na(+) gradient that drives fluid absorption across lung alveolar epithelium. The α1 and β1 subunits are the most highly expressed Na pump subunits in alveolar epithelial cells (AEC). The specific contribution of the β1 subunit and the relative contributions of alveolar epithelial type II (AT2) versus type I (AT1) cells to alveolar fluid clearance (AFC) were investigated using two cell type-specific mouse knockout lines in which the β1 subunit was knocked out in either AT1 cells or both AT1 and AT2 cells. AFC was markedly decreased in both knockout lines, revealing, we believe for the first time, that AT1 cells play a major role in AFC and providing insights into AEC-specific roles in alveolar homeostasis. AEC monolayers derived from knockout mice demonstrated decreased short-circuit current and active Na(+) absorption, consistent with in vivo observations. Neither hyperoxia nor ventilator-induced lung injury increased wet-to-dry lung weight ratios in knockout lungs relative to control lungs. Knockout mice showed increases in Na pump β3 subunit expression and β2-adrenergic receptor expression. These results demonstrate a crucial role for the Na pump β1 subunit in alveolar ion and fluid transport and indicate that both AT1 and AT2 cells make major contributions to these processes and to AFC. Furthermore, they support the feasibility of a general approach to altering alveolar epithelial function in a cell-specific manner that allows direct insights into AT1 versus AT2 cell-specific roles in the lung.

  13. Endoscopic sensing of alveolar pH

    PubMed Central

    Choudhury, D.; Tanner, M. G.; McAughtrie, S.; Yu, F.; Mills, B.; Choudhary, T. R.; Seth, S.; Craven, T. H.; Stone, J. M.; Mati, I. K.; Campbell, C. J.; Bradley, M.; Williams, C. K. I.; Dhaliwal, K.; Birks, T. A.; Thomson, R. R.

    2016-01-01

    Previously unobtainable measurements of alveolar pH were obtained using an endoscope-deployable optrode. The pH sensing was achieved using functionalized gold nanoshell sensors and surface enhanced Raman spectroscopy (SERS). The optrode consisted of an asymmetric dual-core optical fiber designed for spatially separating the optical pump delivery and signal collection, in order to circumvent the unwanted Raman signal generated within the fiber. Using this approach, we demonstrate a ~100-fold increase in SERS signal-to-fiber background ratio, and demonstrate multiple site pH sensing with a measurement accuracy of ± 0.07 pH units in the respiratory acini of an ex vivo ovine lung model. We also demonstrate that alveolar pH changes in response to ventilation. PMID:28101415

  14. Endoscopic sensing of alveolar pH.

    PubMed

    Choudhury, D; Tanner, M G; McAughtrie, S; Yu, F; Mills, B; Choudhary, T R; Seth, S; Craven, T H; Stone, J M; Mati, I K; Campbell, C J; Bradley, M; Williams, C K I; Dhaliwal, K; Birks, T A; Thomson, R R

    2017-01-01

    Previously unobtainable measurements of alveolar pH were obtained using an endoscope-deployable optrode. The pH sensing was achieved using functionalized gold nanoshell sensors and surface enhanced Raman spectroscopy (SERS). The optrode consisted of an asymmetric dual-core optical fiber designed for spatially separating the optical pump delivery and signal collection, in order to circumvent the unwanted Raman signal generated within the fiber. Using this approach, we demonstrate a ~100-fold increase in SERS signal-to-fiber background ratio, and demonstrate multiple site pH sensing with a measurement accuracy of ± 0.07 pH units in the respiratory acini of an ex vivo ovine lung model. We also demonstrate that alveolar pH changes in response to ventilation.

  15. Alveolar distraction osteogenesis for implant site development.

    PubMed

    Batal, Hussam S; Cottrell, David A

    2004-02-01

    Alveolar distraction osteogenesis can be a valuable tool for implant site development. Simultaneous regeneration of hard and soft tissue and an overall decrease in treatment time compared with other methods of site preparation can be an advantage. The authors advocate the concept of "prosthetically driven alveolar distraction." Surgical planning should begin with visualization of the final restoration to determine the volume and position of the soft and hard tissue deficiency. Surgical guides will help the surgeon determine the vector of distraction. Adherence to surgical principles to avoid damage to adjacent vital structures and maintain vascular supply to the transport segment is necessary for success. Bone grafting may be necessary before or after distraction to increase the surgical success of the procedure. Close follow-up is needed to verify the appropriate distraction vector and volume. Patient management and acceptance should be considered in distractor design and placement.

  16. Diffuse Alveolar Hemorrhage in Autoimmune Diseases.

    PubMed

    Martínez-Martínez, Marco Ulises; Oostdam, David Alejandro Herrera-van; Abud-Mendoza, Carlos

    2017-05-01

    The present paper establishes a narrative and analytical review of diffuse alveolar hemorrhage (DAH) in ANCA-associated vasculitis, systemic lupus erythematosus, and antiphospholipid syndrome. Recent studies found a frequent association between DAH and infections and systemic lupus erythematosus and its associated factors. Biological therapies like rituximab have demonstrated benefit mainly in patients with ANCA-associated vasculitis. Main clinical manifestations of diffuse alveolar hemorrhage in these three diseases include dyspnea, pulmonary infiltrates, cough, and hypoxemia. The presence of hemorrhagic bronchoalveolar lavage, hemosiderin containing macrophages, or an increase of carbon monoxide diffusing capacity have been described in some series as helpful findings for the diagnosis. Hemoptysis has been seen mainly in systemic lupus erythematosus. The cornerstone of therapy includes glucocorticoids and cyclophosphamide, and recent findings in ANCA-associated vasculitis suggest the similar benefit of rituximab. Future evaluations and systematic reviews will help to define the real benefit for therapies that appeared to be controversial at the moment.

  17. Treatment of Adult Primary Alveolar Proteinosis.

    PubMed

    Rodríguez Portal, José Antonio

    2015-07-01

    Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of surfactant-like lipoproteinaceous material in the distal air spaces and terminal bronchi, which may lead to impaired gas exchange. This accumulation of surfactant is due to decreased clearance by the alveolar macrophages. Its primary, most common form, is currently considered an autoimmune disease. Better knowledge of the causes of PAP have led to the emergence of alternatives to whole lung lavage, although this is still considered the treatment of choice. Most studies are case series, often with limited patient numbers, so the level of evidence is low. Since the severity of presentation and clinical course are variable, not all patients will require treatment. Due to the low level of evidence, some objective criteria based on expert opinion have been arbitrarily proposed in an attempt to define in which patients it is best to initiate treatment.

  18. Rare lung diseases II: Pulmonary alveolar proteinosis

    PubMed Central

    Juvet, Stephen C; Hwang, David; Waddell, Thomas K; Downey, Gregory P

    2008-01-01

    The present article is the second in a series on rare lung diseases. It focuses on pulmonary alveolar proteinosis (PAP), a disorder in which lipoproteinaceous material accumulates in the alveolar space. PAP was first described in 1958, and for many years the nature of the material accumulating in the lungs was unknown. Major insights into PAP have been made in the past decade, and these have led to the notion that PAP is an autoimmume disorder in which autoantibodies interfere with signalling through the granulocyte-macrophage colony-stimulating factor receptor, leading to macrophage and neutrophil dysfunction. This has spurred new therapeutic approaches to this disorder. The discussion of PAP will begin with a case report, then will highlight the classification of PAP and review recent insights into the pathogenesis of PAP. The approach to therapy and the prognosis of PAP will also be discussed. PMID:18551202

  19. Dephasing and diffusion on the alveolar surface

    NASA Astrophysics Data System (ADS)

    Buschle, L. R.; Kurz, F. T.; Kampf, T.; Wagner, W. L.; Duerr, J.; Stiller, W.; Konietzke, P.; Wünnemann, F.; Mall, M. A.; Wielpütz, M. O.; Schlemmer, H. P.; Ziener, C. H.

    2017-02-01

    We propose a surface model of spin dephasing in lung tissue that includes both susceptibility and diffusion effects to provide a closed-form solution of the Bloch-Torrey equation on the alveolar surface. The nonlocal susceptibility effects of the model are validated against numerical simulations of spin dephasing in a realistic lung tissue geometry acquired from synchotron-based μ CT data sets of mouse lung tissue, and against simulations in the well-known Wigner-Seitz model geometry. The free induction decay is obtained in dependence on microscopic tissue parameters and agrees very well with in vivo lung measurements at 1.5 Tesla to allow a quantification of the local mean alveolar radius. Our results are therefore potentially relevant for the clinical diagnosis and therapy of pulmonary diseases.

  20. [Alveolar haemorrhage following a cannabis water pipe].

    PubMed

    Moatemri, Z; Zaibi, H; Dabboussi, S; Mhamedi, S; Aichaouia, C; Khadhraoui, M; Cheikh, R

    2016-10-01

    Respiratory toxicity of cannabis is well-known today particularly with the new consumption patterns. We report the case of a 25-year-old man admitted for haemoptysis, with unfavourable outcome and acute respiratory failure. Various explorations concluded to acute respiratory distress syndrome secondary to diffuse alveolar haemorrhage. Etiological assessment was initially negative. Outcome was favourable during hospitalization, authorizing the discharge of our patient. Two days later, alveolar haemorrhage recur, with positive toxicological tests for cannabis and the patient admits smoking cannabis by plastic "bang". We illustrate, through this case, the severity of respiratory complications caused by new methods of using cannabis, particularly with plastic 'bang', hence the need to insist of the importance of supported withdrawal and to inform young people how these techniques are serious.ssss.

  1. 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.

  2. Mouse mesenchymal stem cells expressing PAX-FKHR form alveolar rhabdomyosarcomas by cooperating with secondary mutations.

    PubMed

    Ren, Yue-Xin; Finckenstein, Friedrich Graf; Abdueva, Diana A; Shahbazian, Violette; Chung, Brile; Weinberg, Kenneth I; Triche, Timothy J; Shimada, Hiroyuki; Anderson, Michael J

    2008-08-15

    Alveolar rhabdomyosarcomas (ARMS) are highly malignant soft-tissue sarcomas that arise in children, adolescents, and young adults. Although formation and expression of the PAX-FKHR fusion genes is thought to be the initiating event in this cancer, the role of PAX-FKHR in the neoplastic process remains largely unknown in a progenitor cell that is undefined. We hypothesize that PAX-FKHR determine the ARMS progenitor to the skeletal muscle lineage, which when coupled to the inactivation and/or activation of critical cell signaling pathways leads to the formation of ARMS. Because a number of studies have proposed that mesenchymal stem cells (MSC) are the progenitor for several of the sarcomas, we tested this hypothesis in MSCs. We show that PAX-FKHR induce skeletal myogenesis in MSCs by transactivating MyoD and myogenin. Despite exhibiting enhanced growth in vitro, the PAX-FKHR-expressing populations do not form colonies in soft agar or tumors in mice. Expression of dominant-negative p53, or the SV40 early region, elicits tumor formation in some of the PAX-FKHR-expressing populations. Additional activation of the Ras signaling pathway leads to highly malignant tumor formation for all of the populations. The PAX-FKHR-expressing tumors were shown to have histologic, immunohistochemical, and gene expression profiles similar to human ARMS. Our results show the critical role played by PAX-FKHR in determining the molecular, myogenic, and histologic phenotype of ARMS. More importantly, we identify MSCs as a progenitor that can give rise to ARMS.

  3. Adult Soft Tissue Sarcoma Treatment (PDQ®)—Patient Version

    Cancer.gov

    Soft tissue sarcomas can form almost anywhere in the body, but are most common in the head, neck, arms, legs, truck, and abdomen. Find out about risk and genetic factors, symptoms, tests to diagnose, prognosis, staging, and treatment for soft tissue sarcoma.

  4. Oncolytic Maraba Virus MG1 as a Treatment for Sarcoma.

    PubMed

    Le Boeuf, Fabrice; Selman, Mohammed; Son, Hwan Hee; Bergeron, Anabel; Chen, Andrew; Tsang, Jovian; Butterwick, Derek; Arulanandam, Rozanne; Forbes, Nicole E; Tzelepis, Fanny; Bell, John C; Werier, Joel; Abdelbary, Hesham; Diallo, Jean-Simon

    2017-09-15

    The poor prognosis of patients with advanced bone and soft-tissue sarcoma has not changed in the past several decades, highlighting the necessity for new therapeutic approaches. Immunotherapies, including oncolytic viral (OV) therapy, have shown great promise in a number of clinical trials for a variety of tumor types. However, the effective application of OV in treating sarcoma still remains to be demonstrated. Although few pre-clinical studies using distinct OVs have been performed and demonstrated therapeutic benefit in sarcoma models, a side-by-side comparison of clinically relevant OV platforms has not been performed. Four clinically relevant OV platforms (Reovirus, Vaccinia virus, Herpes-simplex virus and Rhabdovirus) were screened for their ability to infect and kill human and canine sarcoma cell lines in vitro, and human sarcoma specimens ex vivo. In vivo treatment efficacy was tested in a murine model. The rhabdovirus MG1 demonstrated the highest potency in vitro. Ex vivo, MG1 productively infected more than 80% of human sarcoma tissues tested, and treatment in vivo led to a significant increase in long-lasting cures in sarcoma-bearing mice. Importantly, MG1 treatment induced the generation of memory immune response that provided protection against a subsequent tumor challenge. This study opens the door for the use of MG1-based oncolytic immunotherapy strategies as treatment for sarcoma or as a component of a combined therapy. © 2017 UICC.

  5. Therapeutic applications of histone deacetylase inhibitors in sarcoma.

    PubMed

    Tang, Fan; Choy, Edwin; Tu, Chongqi; Hornicek, Francis; Duan, Zhenfeng

    2017-09-01

    Sarcomas are a rare group of malignant tumors originating from mesenchymal stem cells. Surgery, radiation and chemotherapy are currently the only standard treatments for sarcoma. However, their response rates to chemotherapy are quite low. Toxic side effects and multi-drug chemoresistance make treatment even more challenging. Therefore, better drugs to treat sarcomas are needed. Histone deacetylase inhibitors (HDAC inhibitors, HDACi, HDIs) are epigenetic modifying agents that can inhibit sarcoma growth in vitro and in vivo through a variety of pathways, including inducing tumor cell apoptosis, causing cell cycle arrest, impairing tumor invasion and preventing metastasis. Importantly, preclinical studies have revealed that HDIs can not only sensitize sarcomas to chemotherapy and radiotherapy, but also increase treatment responses when combined with other chemotherapeutic drugs. Several phase I and II clinical trials have been conducted to assess the efficacy of HDIs either as monotherapy or in combination with standard chemotherapeutic agents or targeted therapeutic drugs for sarcomas. Combination regimen for sarcomas appear to be more promising than monotherapy when using HDIs. This review summarizes our current understanding and therapeutic applications of HDIs in sarcomas. Copyright © 2017 Elsevier Ltd. All rights reserved.

  6. Is There a Predisposition Gene for Ewing's Sarcoma?

    PubMed

    Randall, R L; Lessnick, S L; Jones, K B; Gouw, L G; Cummings, J E; Cannon-Albright, L; Schiffman, J D

    2010-01-01

    Ewing's sarcoma is a highly malignant tumor of children and young adults. The molecular mechanisms that underlie Ewing's Sarcoma development are beginning to be understood. For example, most cases of this disease harbor somatic chromosomal translocations that fuse the EWSR1 gene on chromosome 22 with members of the ETS family. While some cooperative genetic events have been identified, such as mutations in TP53 or deletions of the CDKN2A locus, these appear to be absent in the vast majority of cases. It is therefore uncertain whether EWS/ETS translocations are the only consistently present alteration in this tumor, or whether there are other recurrent abnormalities yet to be discovered. One method to discover such mutations is to identify familial cases of Ewing's sarcoma and to then map the susceptibility locus using traditional genetic mapping techniques. Although cases of sibling pairs with Ewing's sarcoma exist, familial cases of Ewing's sarcoma have not been reported. While Ewing's sarcoma has been reported as a 2nd malignancy after retinoblastoma, significant associations of Ewing's sarcoma with classic tumor susceptibility syndromes have not been identified. We will review the current evidence, or lack thereof, regarding the potential of a heritable condition predisposing to Ewing's sarcoma.

  7. 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

  8. Intimal sarcoma of the pulmonary artery--diagnostic challenge.

    PubMed

    Fukuda, Wakako; Morohashi, Satoko; Fukuda, Ikuo

    2011-08-01

    Pulmonary artery intimal sarcoma is a rare tumour and the diagnosis is often delayed. We report the case of a woman with a primary pulmonary artery intimal sarcoma who presented with massive pulmonary embolism. The definitive diagnosis was elucidated after the patient's death by autopsy specimen. We discuss the diagnosis and lessons learned from this case.

  9. 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.

  10. Osteoplasty of the alveolar cleft defect.

    PubMed

    Rychlik, Dariusz; Wójcicki, Piotr; Koźlik, Maciej

    2012-01-01

    Cleft of lip, alveolar process and palate is the most common congenital defect affecting the face. It occurs at the time of early embryogenesis as a result of disturbed differentiation of the primordial cell layer and is associated with genetic and environmental factors. The most severe type of the defect is complete cleft of the lip, alveolar process and palate, unilateral or bilateral, which is accompanied by impaired breathing, sucking, swallowing, chewing, hearing and speaking. The treatment consists in the surgical reconnection (reconstruction) of the cleft anatomical structures and their formation to gain proper appearance, occlusal conditions and speech. The part of the surgical treatment is reconstruction of alveolar bone by means of autogenic spongy bone grafting (osteoplasty). The surgery performed at the stage of mixed dentition following an orthodontic treatment is a recognized standard management modality. Its effects provide stabilization of the dental arches fixed in the orthodontic treatment, possibility of growth of permanent teeth adjoining the cleft as well as separation of the nasal and oral cavities. The grafted bone becomes a platform for the collapsed base of the ala nasi and facilitates restoration of teeth loss. In the graft healing process the volume of the regenerated bone tissue is lower than the graft volume. Methods to augment the healed bone volume are being searched for, as this factor decides substantially on successful outcome of the surgery.

  11. Variations of Surveillance Practice for Patients with Bone Sarcoma: A Survey of Australian Sarcoma Clinicians

    PubMed Central

    Thompson, Kate; Bae, Susie; Desai, Jayesh; Strong, Robyn; Caruso, Denise; Howell, Deborah; Herschtal, Alan; Sullivan, Michael; Orme, Lisa

    2017-01-01

    Introduction. After treatment, bone sarcoma patients carry a high chance of relapse and late effects from multimodal therapy. We hypothesize that significant variation in surveillance practice exists between pediatric medical oncology (PO) and nonpediatric medical oncology (NP) sarcoma disciplines. Methods. Australian sarcoma clinicians were approached to do a web based survey that assessed radiologic surveillance (RS) strategies, late toxicity assessment, and posttreatment psychosocial interventions. Results. In total, 51 clinicians responded. No differences were identified in local disease RS. In metastatic disease response assessment, 100% of POs (23/23) and 93% of NPs (24/26) conducted CT chest. However, this was more likely to occur for NPs in the context of a CT chest/abdomen/pelvis (NP: 10/26; PO: 1/23; p = 0.006). POs were more likely to use CXR for RS (p = 0.006). POs showed more prescriptive intensity in assessment of heart function (p = 0.001), hearing (p < 0.001), and fertility (p = 0.02). POs were more likely to deliver written information for health maintenance/treatment summary (p = 0.04). The majority of respondents described enquiring about psychosocial aspects of health (n = 33/37, 89%), but a routine formal psychosocial screen was only used by 23% (n = 6/26). Conclusion. There is high variability in bone sarcoma surveillance between PO and NP clinicians. Efforts to harmonize approaches would allow early and late effects recognition/intervention and facilitate improved patient care/transition and research. PMID:28348507

  12. Characteristics of 64 sarcoma patients referred to a sarcoma center after unplanned excision.

    PubMed

    Dyrop, Heidi Buvarp; Safwat, Akmal; Vedsted, Peter; Maretty-Kongstad, Katja; Hansen, Bjarne Hauge; Jørgensen, Peter Holmberg; Baad-Hansen, Thomas; Keller, Johnny

    2016-02-01

    Unplanned excision of sarcoma before referral to specialist centers can affect prognosis and surgical outcome. The diagnostic pathway of these patients is uncertain and needs to be reviewed. We aimed to describe patient and tumor characteristics, initial symptoms, initial and final diagnosis, and explore reasons for unplanned excision in this patient group. From a previous study on 258 sarcoma patients, we identified 64 patients referred after surgery. Medical records were reviewed. The majority were soft tissue sarcomas, most often with thoracic location. Leiomyosarcoma was the most frequent final diagnosis, lipoma, and fibroma/dermatofibroma the most frequent initial diagnoses. Fifty percent were superficial small tumors, and 60.9% had not received diagnostic imaging before surgery. Fifty percent were referred from public surgical departments, and 1/3 from private specialists. Twenty-three patients had initial presence of alarm symptoms registered before surgery, the remaining 2/3 fell outside referral criteria or alarm symptoms were not discovered. Patients referred after unplanned excision often have small superficial tumors and the majority fall outside of defined referral criteria. Referral criteria are not a guarantee for detection of all sarcomas and surgeons should always be aware of the possibility of malignancy when removing a tumor. © 2016 Wiley Periodicals, Inc.

  13. Variations of Surveillance Practice for Patients with Bone Sarcoma: A Survey of Australian Sarcoma Clinicians.

    PubMed

    Lewin, Jeremy; Thompson, Kate; Bae, Susie; Desai, Jayesh; Strong, Robyn; Caruso, Denise; Howell, Deborah; Herschtal, Alan; Sullivan, Michael; Orme, Lisa

    2017-01-01

    Introduction. After treatment, bone sarcoma patients carry a high chance of relapse and late effects from multimodal therapy. We hypothesize that significant variation in surveillance practice exists between pediatric medical oncology (PO) and nonpediatric medical oncology (NP) sarcoma disciplines. Methods. Australian sarcoma clinicians were approached to do a web based survey that assessed radiologic surveillance (RS) strategies, late toxicity assessment, and posttreatment psychosocial interventions. Results. In total, 51 clinicians responded. No differences were identified in local disease RS. In metastatic disease response assessment, 100% of POs (23/23) and 93% of NPs (24/26) conducted CT chest. However, this was more likely to occur for NPs in the context of a CT chest/abdomen/pelvis (NP: 10/26; PO: 1/23; p = 0.006). POs were more likely to use CXR for RS (p = 0.006). POs showed more prescriptive intensity in assessment of heart function (p = 0.001), hearing (p < 0.001), and fertility (p = 0.02). POs were more likely to deliver written information for health maintenance/treatment summary (p = 0.04). The majority of respondents described enquiring about psychosocial aspects of health (n = 33/37, 89%), but a routine formal psychosocial screen was only used by 23% (n = 6/26). Conclusion. There is high variability in bone sarcoma surveillance between PO and NP clinicians. Efforts to harmonize approaches would allow early and late effects recognition/intervention and facilitate improved patient care/transition and research.

  14. Two Cases of Ectopic Hamartomatous Thymoma Masquerading as Sarcoma.

    PubMed

    Kondo, Takahito; Sato, Yukiko; Tanaka, Hiroko; Sasaki, Toru; Kawabata, Kazuyoshi; Mitani, Hiroki; Yonekawa, Hiroyuki; Fukushima, Hirofumi; Shimbashi, Wataru

    2017-01-01

    Ectopic hamartomatous thymoma (EHT) is an extremely rare benign tumor. EHTs are difficult to differentiate from sarcomas, especially synovial sarcomas. We encountered two cases of EHT that were referred from other hospitals because sarcoma was suspected. In these cases, fusion gene detection via polymerase chain reaction or fluorescence in situ hybridization was useful for differentiating EHT from synovial sarcoma. EHT requires accurate diagnosis before surgery to avoid excessive treatment. Both tumor location and the presence of fat inside the tumor are important imaging findings for EHT, and confirmation of spindle cells, epithelial cells, and mature adipose cells in the tumor is an important pathological finding. It is important to exclude synovial sarcoma from the differential diagnosis via fusion gene analysis.

  15. The roles and implications of exosomes in sarcoma

    PubMed Central

    Min, Li; Shen, Jacson; Tu, Chongqi; Hornicek, Francis; Duan, Zhenfeng

    2016-01-01

    Better diagnostic biomarkers and therapeutic options are still necessary for patients with sarcomas due to the current limitations of diagnosis and treatment. Exosomes are small extracellular membrane vesicles that are released by various cells and are found in most body fluids. Tumor-derived exosomes have been proven to mediate tumorigenesis, intercellular communication, microenvironment modulation, and metastasis in different cancers, including in sarcomas. Recently, exosomes have been considered as potential biomarkers for sarcoma diagnosis, prognosis, and possible targets for sarcoma therapy. Moreover, due to their specific cell-tropism and bioavailability, exosomes can also be engineered as vehicles for drug delivery. In this review, we discuss recent advances in the roles of tumor-derived exosomes in sarcoma and their potential clinical applications. PMID:27342745

  16. The roles and implications of exosomes in sarcoma.

    PubMed

    Min, Li; Shen, Jacson; Tu, Chongqi; Hornicek, Francis; Duan, Zhenfeng

    2016-09-01

    Better diagnostic biomarkers and therapeutic options are still necessary for patients with sarcomas due to the current limitations of diagnosis and treatment. Exosomes are small extracellular membrane vesicles that are released by various cells and are found in most body fluids. Tumor-derived exosomes have been proven to mediate tumorigenesis, intercellular communication, microenvironment modulation, and metastasis in different cancers, including in sarcomas. Recently, exosomes have been considered as potential biomarkers for sarcoma diagnosis and prognosis, and as possible targets for sarcoma therapy. Moreover, due to their specific cell tropism and bioavailability, exosomes can also be engineered as vehicles for drug delivery. In this review, we discuss recent advances in the roles of tumor-derived exosomes in sarcoma and their potential clinical applications.

  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. Two Cases of Ectopic Hamartomatous Thymoma Masquerading as Sarcoma

    PubMed Central

    Sato, Yukiko; Tanaka, Hiroko; Sasaki, Toru; Kawabata, Kazuyoshi; Mitani, Hiroki; Yonekawa, Hiroyuki; Fukushima, Hirofumi; Shimbashi, Wataru

    2017-01-01

    Ectopic hamartomatous thymoma (EHT) is an extremely rare benign tumor. EHTs are difficult to differentiate from sarcomas, especially synovial sarcomas. We encountered two cases of EHT that were referred from other hospitals because sarcoma was suspected. In these cases, fusion gene detection via polymerase chain reaction or fluorescence in situ hybridization was useful for differentiating EHT from synovial sarcoma. EHT requires accurate diagnosis before surgery to avoid excessive treatment. Both tumor location and the presence of fat inside the tumor are important imaging findings for EHT, and confirmation of spindle cells, epithelial cells, and mature adipose cells in the tumor is an important pathological finding. It is important to exclude synovial sarcoma from the differential diagnosis via fusion gene analysis. PMID:28168073

  19. Role of genetic and molecular profiling in sarcomas.

    PubMed

    Norberg, Scott M; Movva, Sujana

    2015-05-01

    The treatment of sarcomas has been challenging due to their heterogeneity, rarity in the general population, relative insensitivity to chemotherapeutics, and lack of effective targeted agents. One of the first major breakthroughs in the treatment of sarcomas was the use of imatinib to treat gastrointestinal stromal tumors (GISTs). Since then, advanced molecular techniques and genetic profiling have revolutionized the approach to sarcoma classification, diagnosis, prognosis and, most importantly, treatment. As the sarcoma genetic database continues to expand, the basis for how we classify, diagnose, and treat these challenging malignancies will be redefined. The overall goal of these types of techniques has been to determine a molecular blueprint for each sarcoma subtype and discover actionable alterations that lend themselves to targeted therapies. Other important information derived from these large genomic databases includes biomarkers, prognostic indicators, and information regarding tumorigenesis. Eventually, advanced molecular techniques will provide a personalized-medicine approach that tailors each treatment regimen to the patient's own tumor genome.

  20. Intimal sarcoma of the superficial femoral artery with osteosarcomatous differentiation.

    PubMed

    Ebaugh, James L; Yuan, Minsheng; Hu, Jeffery; Chen, Ahchean; Raffetto, Joseph D

    2011-05-01

    Sarcomas of the large vessels usually present centrally in the aorta, pulmonary artery, and inferior vena cava. Peripheral arterial sarcomas are exceptionally rare. They have been reported in the iliac and common or profunda femoral arteries, and are frequently undifferentiated. In this study, we describe a differentiated intimal sarcoma of the superficial femoral artery with abundant osteosarcoma within the specimen. Before knowing the diagnosis, treatment was for a presumed pseudoaneurysm using excision and bypass. Postoperatively, the patient received palliative radiation therapy. The tumor's location and histopathology are unique. A differentiated intimal sarcoma has never been reported in the superficial femoral artery, and it represents the second peripheral arterial intimal sarcoma reported with osteosarcomatous differentiation.

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

    ClinicalTrials.gov

    2017-02-08

    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

  2. Current State of Pediatric Sarcoma Biology and Opportunities for Future Discovery: A Report from the Sarcoma Translational Research Workshop

    PubMed Central

    Hingorani, Pooja; Janeway, Katherine; Crompton, Brian D.; Kadoch, Cigall; Mackall, Crystal L.; Khan, Javed; Shern, Jack F.; Schiffman, Joshua; Mirabello, Lisa; Savage, Sharon A.; Ladanyi, Marc; Meltzer, Paul; Bult, Carol J.; Adamson, Peter C.; Lupo, Philip J.; Mody, Rajen; DuBois, Steven G.; Parsons, D. Williams; Khanna, Chand; Lau, Ching; Hawkins, Douglas S.; Randall, R. Lor; Smith, Malcolm; Sorensen, Poul H.; Plon, Sharon E.; Skapek, Stephen X.; Lessnick, Stephen; Gorlick, Richard; Reed, Damon R.

    2017-01-01

    Sarcomas are a rare subgroup of pediatric cancers comprised of a variety of bone and soft-tissue tumors. While significant advances have been made in improving outcomes of patients with localized pediatric sarcomas since the addition of systemic chemotherapy to local control many decades ago, outcomes for patients with metastatic and relapsed sarcoma remain poor with few novel therapeutics identified to date. With the advent of new technologies to study cancer genomes, transcriptomes and epigenomes, our understanding of sarcoma biology has improved tremendously in a relatively short period of time. However, much remains to be accomplished in this arena especially with regard to translating all of this new knowledge to the bedside. To this end, a meeting was convened in Philadelphia, PA on April 18, 2015 sponsored by the QuadW foundation, Children’s Oncology Group and CureSearch for Children’s Cancer that brought together sarcoma clinicians and scientists from North America to review the current state of pediatric sarcoma biology and ongoing/planned genomics based clinical trials in an effort to identify and bridge knowledge gaps that continue to exist at the current time. At the conclusion of the workshop, three key objectives that would significantly further our understanding of sarcoma were identified and a proposal was put forward to develop an all-encompassing pediatric sarcoma biology protocol that would address these specific needs. This review summarizes the proceedings of the workshop. PMID:27132463

  3. Current state of pediatric sarcoma biology and opportunities for future discovery: A report from the sarcoma translational research workshop.

    PubMed

    Hingorani, Pooja; Janeway, Katherine; Crompton, Brian D; Kadoch, Cigall; Mackall, Crystal L; Khan, Javed; Shern, Jack F; Schiffman, Joshua; Mirabello, Lisa; Savage, Sharon A; Ladanyi, Marc; Meltzer, Paul; Bult, Carol J; Adamson, Peter C; Lupo, Philip J; Mody, Rajen; DuBois, Steven G; Parsons, D Williams; Khanna, Chand; Lau, Ching; Hawkins, Douglas S; Randall, R Lor; Smith, Malcolm; Sorensen, Poul H; Plon, Sharon E; Skapek, Stephen X; Lessnick, Stephen; Gorlick, Richard; Reed, Damon R

    2016-05-01

    Sarcomas are a rare subgroup of pediatric cancers comprised of a variety of bone and soft-tissue tumors. While significant advances have been made in improving outcomes of patients with localized pediatric sarcomas since the addition of systemic chemotherapy to local control many decades ago, outcomes for patients with metastatic and relapsed sarcoma remain poor with few novel therapeutics identified to date. With the advent of new technologies to study cancer genomes, transcriptomes and epigenomes, our understanding of sarcoma biology has improved tremendously in a relatively short period of time. However, much remains to be accomplished in this arena especially with regard to translating all of this new knowledge to the bedside. To this end, a meeting was convened in Philadelphia, PA, on April 18, 2015 sponsored by the QuadW foundation, Children's Oncology Group and CureSearch for Children's Cancer that brought together sarcoma clinicians and scientists from North America to review the current state of pediatric sarcoma biology and ongoing/planned genomics based clinical trials in an effort to identify and bridge knowledge gaps that continue to exist at present. At the conclusion of the workshop, three key objectives that would significantly further our understanding of sarcoma were identified and a proposal was put forward to develop an all-encompassing pediatric sarcoma biology protocol that would address these specific needs. This review summarizes the proceedings of the workshop.

  4. Imaging investigations before referral to a sarcoma center delay the final diagnosis of musculoskeletal sarcoma.

    PubMed

    Dyrop, Heidi Buvarp; Vedsted, Peter; Rædkjær, Mathias; Safwat, Akmal; Keller, Johnny

    2017-04-01

    Background and purpose - The use of point-of-care or local investigations before referral to specialist sarcoma centers as part of a fast-track diagnostic pathway varies, and may affect the time to diagnosis. We wanted to investigate differences in time intervals and proportion of malignancy in patients who were referred after initial diagnostic investigations were performed locally and in patients who were referred without these investigations. Patients and methods - We included 545 consecutive patients who were referred to Aarhus Sarcoma Center for suspected musculoskeletal sarcoma. Data on time intervals and investigations performed were collected from questionnaires and patient records. Patients who were referred from outside Aarhus uptake area after initial MRI/CT or histology performed locally were compared with patients who were referred from Aarhus uptake area without these investigations. Results - The median total interval from first symptom to diagnosis was 166 days for outside patients referred with MRI/CT or histology, which was 91 (95% CI: 76-106) days longer than for local patients who were referred without MRI/CT or histology. Comparing the same groups, the median diagnostic interval was 41 (95% CI: 30-51) days longer for outside patients including both primary care and hospital intervals. Both the proportion of malignancies (38% vs. 14%) and the proportion of sarcomas (24% vs. 7%) were higher in the outside group referred with MRI/CT or histology than in the local group without MRI/CT or histology. Interpretation - Pre-referral investigations at a local hospital increased the diagnostic interval by at least 1 month for 50% of the patients, and the proportion of malignancy was more than doubled-to almost 40%. If investigations are to be performed before referral to a sarcoma center, they should be part of the fast-track pathway in order to ensure timely diagnosis.

  5. Pazopanib in advanced vascular sarcomas: an EORTC Soft Tissue and Bone Sarcoma Group (STBSG) retrospective analysis.

    PubMed

    Kollár, A; Jones, R L; Stacchiotti, S; Gelderblom, H; Guida, M; Grignani, G; Steeghs, N; Safwat, A; Katz, D; Duffaud, F; Sleijfer, S; van der Graaf, W T; Touati, N; Litière, S; Marreaud, S; Gronchi, A; Kasper, B

    2017-01-01

    Pazopanib is a multitargeted tyrosine kinase inhibitor approved for the treatment of patients with selective subtypes of advanced soft tissue sarcoma (STS) who have previously received standard chemotherapy including anthracyclines. Data on the efficacy in vascular sarcomas are limited. The main objective of this study was to investigate the activity of pazopanib in vascular sarcomas. A retrospective study of patients with advanced vascular sarcomas, including angiosarcoma (AS), epithelioid hemangioendothelioma (HE) and intimal sarcoma (IS) treated with pazopanib in real life practice at EORTC centers as well as patients treated within the EORTC phase II and III clinical trials (62043/62072) was performed. Patient and tumor characteristics were collected. Response was assessed according to RECIST 1.1. and survival analysis was performed. Fifty-two patients were identified, 40 (76.9%), 10 (19.2%) and two (3.8%) with AS, HE and IS, respectively. The response rate was eight (20%), two (20%) and two (100%) in the AS, HE and IS subtypes, respectively. There was no significant difference in response rate between cutaneous and non-cutaneous AS and similarly between radiation-associated and non-radiation-associated AS. Median progression-free survival (PFS) and median overall survival (OS; from commencing pazopanib) were three months (95% CI 2.1-4.4) and 9.9 months (95% CI 6.5-11.3) in AS, respectively. The activity of pazopanib in AS is comparable to its reported activity in other STS subtypes. In this study, the activity of pazopanib was similar in cutaneous/non-cutaneous and in radiation/non-radiation-associated AS. In addition, pazopanib showed promising activity in HE and IS, worthy of further evaluation.

  6. Imaging investigations before referral to a sarcoma center delay the final diagnosis of musculoskeletal sarcoma

    PubMed Central

    Dyrop, Heidi Buvarp; Vedsted, Peter; Rædkjær, Mathias; Safwat, Akmal; Keller, Johnny

    2017-01-01

    Background and purpose The use of point-of-care or local investigations before referral to specialist sarcoma centers as part of a fast-track diagnostic pathway varies, and may affect the time to diagnosis. We wanted to investigate differences in time intervals and proportion of malignancy in patients who were referred after initial diagnostic investigations were performed locally and in patients who were referred without these investigations. Patients and methods We included 545 consecutive patients who were referred to Aarhus Sarcoma Center for suspected musculoskeletal sarcoma. Data on time intervals and investigations performed were collected from questionnaires and patient records. Patients who were referred from outside Aarhus uptake area after initial MRI/CT or histology performed locally were compared with patients who were referred from Aarhus uptake area without these investigations. Results The median total interval from first symptom to diagnosis was 166 days for outside patients referred with MRI/CT or histology, which was 91 (95% CI: 76–106) days longer than for local patients who were referred without MRI/CT or histology. Comparing the same groups, the median diagnostic interval was 41 (95% CI: 30–51) days longer for outside patients including both primary care and hospital intervals. Both the proportion of malignancies (38% vs. 14%) and the proportion of sarcomas (24% vs. 7%) were higher in the outside group referred with MRI/CT or histology than in the local group without MRI/CT or histology. Interpretation Pre-referral investigations at a local hospital increased the diagnostic interval by at least 1 month for 50% of the patients, and the proportion of malignancy was more than doubled—to almost 40%. If investigations are to be performed before referral to a sarcoma center, they should be part of the fast-track pathway in order to ensure timely diagnosis. PMID:28077058

  7. 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.

  8. [Alveolar hemorrhage associated with intestinal inflammatory disease and Hashimoto thyroiditis].

    PubMed

    Rabec, C; Barcat, J; Rey, D

    2003-06-01

    Diffuse alveolar hemorrhage (DAH) is characterized by diffuse bleeding into alveolar spaces. Three histopathological patterns may be seen: 1) pulmonary capillaritis due to immunological aggression to the membrane, 2) diffuse alveolar damage within the context of acute respiratory distress syndrome, and 3) and "bland" DAH without alveolar or capillary damage. In the first two groups, pulmonary damage usually occurs within the context of a systemic disease. In the last, injury is usually found only in the lung, an entity called pulmonary hemosiderosis. We present a case of DAH with neither capillaritis nor diffuse alveolar damage in association with inflammatory bowel disease and Hashimoto thyroiditis. The case is interesting both because the association has not yet been described in the literature and because the presence of alveolar bleeding without evident tissue damage within the context of known autoimmune diseases may extend the field to include a new pathophysiological mechanism of pulmonary hemorrhage.

  9. Alveolar air volatile organic compound extractor for clinical breath sampling.

    PubMed

    de Silva, Geethanga; Beyette, Fred R

    2014-01-01

    Alveolar air Volatile Organic Compound (VOC) extractor is a handheld breath-sampling device for clinical breath analysis. The device consists two main components: (1) An alveolar air separator, (2) A VOC extractor. The alveolar air separator splits exhaled air based on total exhaled air volume directing alveolar air towards the VOC extractor and dead space air to into an exhaust channel. The VOC extractor collects the VOCs from alveolar air into a modified Sold Phase Micro Extraction (SPME) filament. Feasibility of using the SPME filament to collect a quantifiable breath sample directly from exhaled breath is experimentally validated. Exhaled breath acetone is quantified using alveolar air VOC extractor and a GC/MS system.

  10. Mast cells in the human alveolar wall: an electronmicroscopic study.

    PubMed Central

    Fox, B; Bull, T B; Guz, A

    1981-01-01

    Mast cells were identified by electronmicroscopy in the alveolar wall of the lung in 20 subjects (10 normal, 10 abnormal). A quantitative and qualitative study was made of the mast cells. In the normal lung there was an average concentration of 350 mast cells/mm2 of alveolar wall and in the abnormal 523/mm2. Mast cells occupied approximately 1.6-2.1% of the area of the alveolar wall. There was marked variation in the structure of the mast cell granules but no differences between those in the normal and abnormal lungs. There was evidence that constant degranulation of mast cells may be occurring in the lung. The role that alveolar mast cells may play in the vasoconstrictor response to alveolar hypoxia is discussed. It is suggested that the tachypnoea present in asthma may partly be due to release of mediators from sensitised mast cells within the alveolar wall. Images PMID:7328180

  11. Recent advances in alveolar biology: some new looks at the alveolar interface.

    PubMed

    Possmayer, Fred; Hall, Stephen B; Haller, Thomas; Petersen, Nils O; Zuo, Yi Y; Bernardino de la Serna, Jorge; Postle, Anthony D; Veldhuizen, Ruud A W; Orgeig, Sandra

    2010-08-31

    This article examines the manner in which some new methodologies and novel concepts have contributed to our understanding of how pulmonary surfactant reduces alveolar surface tension. Investigations utilizing small angle X-ray diffraction, inverted interface fluorescence microscopy, time of flight-secondary ion mass spectroscopy, atomic force microscopy, two-photon fluorescence microscopy and electrospray mass spectroscopy are highlighted and a new model of ventilation-induced acute lung injury described. This contribution attempts to emphasize how these new approaches have resulted in a fuller appreciation of events presumably occurring at the alveolar interface. Copyright (c) 2010 Elsevier B.V. All rights reserved.

  12. Inhibition of MDM2 by RG7388 confers hypersensitivity to X-radiation in xenograft models of childhood sarcoma.

    PubMed

    Phelps, Doris; Bondra, Kathryn; Seum, Star; Chronowski, Christopher; Leasure, Justin; Kurmasheva, Raushan T; Middleton, Steven; Wang, Dian; Mo, Xiaokui; Houghton, Peter J

    2015-08-01

    Curative therapy for childhood sarcoma presents challenges when complete resection is not possible. Ionizing radiation (XRT) is used as a standard modality at diagnosis or recurrence for childhood sarcoma; however, local recurrence is still problematic. Most childhood sarcomas are TP53 wild type at diagnosis, although approximately 5-10% have MDM2 amplification or overexpression. The MDM2 inhibitor, RG7388, was examined alone or in combination with XRT (20Gy given in 2 Gy daily fractions) to immune-deficient mice bearing Rh18 (embryonal) or a total of 30 Gy in 2 Gy fractions to mice bearing Rh30 (alveolar) rhabdomyosarcoma xenografts. RG7388 was administered by oral gavage using two schedules (daily ×5; schedule 1 or once weekly; schedule 2). TP53-responsive gene products (p21, PUMA, DDB2, and MIC1) as well as markers of apoptosis were analyzed. RG7388 showed no significant single agent antitumor activity. Twenty Grays XRT induced complete regressions (CR) of Rh18 with 100 percent tumor regrowth by week 7, but no tumor regrowth at 20 weeks when combined with RG7388. RG7388 enhanced time to recurrence combined with XRT in Rh30 xenografts compared to 30 Gy XRT alone. RG7388 did not enhance XRT-induced local skin toxicity. Combination treatments induced TP53 responsive genes more rapidly and to a greater magnitude than single agent treatments. RG7388 enhanced the activity of XRT in both rhabdomyosarcoma models without increasing local XRT-induced skin toxicity. Changes in TP53-responsive genes were consistent with the synergistic activity of RG7388 and XRT in the Rh18 model. © 2015 Wiley Periodicals, Inc.

  13. 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.

  14. Pulmonary artery intimal sarcoma: case report.

    PubMed

    Hou, Yulong; Shen, Zhenya; Gao, Wei; Ye, Wenxue

    2010-01-01

    A 72-year-old woman with pulmonary artery intimal sarcoma was successfully treated with surgery. With heightened clinical awareness and technological advancement, more and more cases were diagnosed definitely before operation. Computed tomography of the chest showed a mass in right ventricular extending to pulmonary trunk and the left pulmonary artery. The patient underwent complete surgical resection and repair of the pulmonary artery with no evidence of recurrence during the 12-month follow-up, suggesting that early identification and aggressive surgical intervention would improve survival.

  15. [SURGERY FOR SARCOMA OF THE PULMONARY ARTERY].

    PubMed

    Parshin, V D; Motus, I Ya; Belov, Yu V; Chernyavsky, A M; Neretin, A V; Rusinov, V V

    2015-01-01

    Sarcoma of the pulmonary artery is a rare tumor. At present the literature describes single cases. However the number of publications increases in recent time due to improved diagnostics. There are appeared papers, which provide a series of observations of surgical treatment for this kind of tumor exceeded more than 10 cases. It can be assumed that today the number of these cases in the literature contains several hundreds. Thus despite the rarity of this tumor there is a certain understanding of the clinical picture of this disease and treatment that we tried to do in this paper being studied the available literature and bringing four of our observation.

  16. Histological variants of cutaneous Kaposi sarcoma

    PubMed Central

    Grayson, Wayne; Pantanowitz, Liron

    2008-01-01

    This review provides a comprehensive overview of the broad clinicopathologic spectrum of cutaneous Kaposi sarcoma (KS) lesions. Variants discussed include: usual KS lesions associated with disease progression (i.e. patch, plaque and nodular stage); morphologic subtypes alluded to in the older literature such as anaplastic and telangiectatic KS, as well as several lymphedematous variants; and numerous recently described variants including hyperkeratotic, keloidal, micronodular, pyogenic granuloma-like, ecchymotic, and intravascular KS. Involuting lesions as a result of treatment related regression are also presented. PMID:18655700

  17. Granulocytic sarcoma: a rare cause of sciatica

    PubMed Central

    Glover, Thomas Edward

    2017-01-01

    We describe a case report of a man aged 56 years with a 4-month history of right-sided sciatica-type pain with subclinical disc prolapse evident on MRI. Worsening pain together with the appearance of a tender mass in his right buttock prompted further imaging, which demonstrated an infiltrative mass engulfing the lumbosacral plexus. This was later shown to be a granulocytic sarcoma on biopsy. Intervertebral disc herniation can be an incidental finding and is not always the cause of sciatica. PMID:28202486

  18. Intranasal Fentanyl Intoxication Leading to Diffuse Alveolar Hemorrhage.

    PubMed

    Ruzycki, Shannon; Yarema, Mark; Dunham, Michael; Sadrzadeh, Hossein; Tremblay, Alain

    2016-06-01

    Increasing rates of opioid abuse, particularly fentanyl, may lead to more presentations of unusual effects of opioid toxicity. Diffuse alveolar hemorrhage is a rare complication of fentanyl overdose. A 45-year-old male presented in hypoxic respiratory failure secondary to diffuse alveolar hemorrhage requiring intubation. Comprehensive drug screening detected fentanyl without exposure to cocaine. Further history upon the patient's recovery revealed exposure to snorted fentanyl powder immediately prior to presentation. Diffuse alveolar hemorrhage is a potential, though rare, presentation of opioid intoxication. Recognition of less common complications of opioid abuse such as diffuse alveolar hemorrhage is important in proper management of overdoses.

  19. Alveolar haemorrhage in a case of high altitude pulmonary oedema.

    PubMed

    Grissom, C K; Albertine, K H; Elstad, M R

    2000-02-01

    A case of high altitude pulmonary oedema (HAPE) in a climber who made a rapid ascent on Mt McKinley (Denali), Alaska is described. The bronchoalveolar lavage (BAL) fluid contained increased numbers of red blood cells and an abundance of haemosiderin laden macrophages consistent with alveolar haemorrhage. The timing of this finding indicates that alveolar haemorrhage began early during the ascent, well before the onset of symptoms. Although evidence of alveolar haemorrhage has been reported at necropsy in individuals dying of HAPE, previous reports have not shown the same abundance of haemosiderin laden macrophages in the BAL fluid. These findings suggest that alveolar haemorrhage is an early event in HAPE.

  20. Novel Roles for Staufen1 in Embryonal and Alveolar Rhabdomyosarcoma via c-myc-dependent and -independent events

    PubMed Central

    Crawford Parks, Tara E.; Marcellus, Kristen A.; Langill, Jonathan; Ravel-Chapuis, Aymeric; Michaud, Jean; Cowan, Kyle N.; Côté, Jocelyn; Jasmin, Bernard J.

    2017-01-01

    Rhabdomyosarcoma is the most common soft tissue sarcoma in children and young adults. Rhabdomyosarcomas are skeletal muscle-like tumours that typically arise in muscle beds, and express key myogenic regulatory factors. However, their developmental program remains blocked in the proliferative phase with cells unable to exit the cell cycle to fuse into myotubes. Recently, we uncovered a key role for the RNA-binding protein Staufen1 during myogenic differentiation through the regulation of c-myc translation. Given the known implication of c-myc in rhabdomyosarcoma, we hypothesized in the current work that Staufen1 controls rhabdomyosarcoma tumorigenesis. Here, we report for the first time the novel role of Staufen1 in cancer, specifically in rhabdomyosarcoma. We demonstrate that Staufen1 is markedly upregulated in human rhabdomyosarcoma tumours and cell lines as compared to normal skeletal muscle. Moreover, we show that Staufen1 promotes the tumorigenesis of embryonal and alveolar rhabdomyosarcoma subtypes both in cell culture and in animal models. Finally, our data demonstrate that Staufen1 has differential roles in embryonal versus alveolar rhabdomyosarcoma through the control of proliferative and apoptotic pathways, respectively. Together, these results provide the first evidence for Staufen1’s direct implication in cancer biology. Accordingly, Staufen1 thus represents a novel target for the development of future therapeutic strategies for rhabdomyosarcoma. PMID:28211476

  1. 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.

  2. Ewing's sarcoma precursors are highly enriched in embryonic osteochondrogenic progenitors.

    PubMed

    Tanaka, Miwa; Yamazaki, Yukari; Kanno, Yohei; Igarashi, Katsuhide; Aisaki, Ken-ichi; Kanno, Jun; Nakamura, Takuro

    2014-07-01

    Ewing's sarcoma is a highly malignant bone tumor found in children and adolescents, and the origin of this malignancy is not well understood. Here, we introduced a Ewing's sarcoma-associated genetic fusion of the genes encoding the RNA-binding protein EWS and the transcription factor ETS (EWS-ETS) into a fraction of cells enriched for osteochondrogenic progenitors derived from the embryonic superficial zone (eSZ) of long bones collected from late gestational murine embryos. EWS-ETS fusions efficiently induced Ewing's sarcoma-like small round cell sarcoma formation by these cells. Analysis of the eSZ revealed a fraction of a precursor cells that express growth/differentiation factor 5 (Gdf5), the transcription factor Erg, and parathyroid hormone-like hormone (Pthlh), and selection of the Pthlh-positive fraction alone further enhanced EWS-ETS-dependent tumor induction. Genes downstream of the EWS-ETS fusion protein were quite transcriptionally active in eSZ cells, especially in regions in which the chromatin structure of the ETS-responsive locus was open. Inhibition of β-catenin, poly (ADP-ribose) polymerase 1 (PARP1), or enhancer of zeste homolog 2 (EZH2) suppressed cell growth in a murine model of Ewing's sarcoma, suggesting the utility of the current system as a preclinical model. These results indicate that eSZ cells are highly enriched in precursors to Ewing's sarcoma and provide clues to the histogenesis of Ewing's sarcoma in bone.

  3. Quantitative morphology in canine cutaneous soft tissue sarcomas.

    PubMed

    Simeonov, R; Ananiev, J; Gulubova, M

    2015-12-01

    Stained cytological specimens from 24 dogs with spontaneous soft tissue sarcomas [fibrosarcoma (n = 8), liposarcoma (n = 8) and haemangiopericytoma (n = 8)], and 24 dogs with reactive connective tissue lesions [granulation tissue (n = 12) and dermal fibrosis (n = 12)] were analysed by computer-assisted nuclear morphometry. The studied morphometric parameters were: mean nuclear area (MNA; µm(2)), mean nuclear perimeter (MNP; µm), mean nuclear diameter (MND mean; µm), minimum nuclear diameter (Dmin; µm) and maximum nuclear diameter (Dmax; µm). The study aimed to evaluate (1) possibility for quantitative differentiation of soft tissue sarcomas from reactive connective tissue lesions and (2) by using cytomorphometry, to differentiate the various histopathological soft tissue sarcomas subtypes in dogs. The mean values of all nuclear cytomorphometric parameters (except for Dmax) were statistically significantly higher in reactive connective tissue processes than in soft tissue sarcomas. At the same time, however, there were no considerable differences among the different sarcoma subtypes. The results demonstrated that the quantitative differentiation of reactive connective tissue processes from soft tissue sarcomas in dogs is possible, but the same was not true for the different canine soft tissue sarcoma subtypes. Further investigations on this topic are necessary for thorough explication of the role of quantitative morphology in the diagnostics of mesenchymal neoplasms and tumour-like fibrous lesions in dogs. © 2014 John Wiley & Sons Ltd.

  4. HIV-associated Kaposi sarcoma and related diseases.

    PubMed

    Gonçalves, Priscila H; Uldrick, Thomas S; Yarchoan, Robert

    2017-09-10

    : The search for the etiologic agent for Kaposi sarcoma led to the discovery of Kaposi sarcoma-associated herpesvirus (KSHV) in 1994. KSHV, also called human herpesvirus-8, has since been shown to be the etiologic agent for several other tumors and diseases, including primary effusion lymphoma (PEL), an extracavitary variant of PEL, KSHV-associated diffuse large B-cell lymphoma, a form of multicentric Castleman disease, and KSHV inflammatory cytokine syndrome. KSHV encodes several genes that interfere with innate and specific immunity, thwart apoptosis, enhance cell proliferation and cytokine production, and promote angiogenesis, and these play important roles in disease pathogenesis. HIV is an important cofactor in Kaposi sarcoma pathogenesis, and widespread use of antiretroviral therapy has reduced Kaposi sarcoma incidence. However, Kaposi sarcoma remains the second most frequent tumor arising in HIV-infected patients in the United States and is particularly common in sub-Saharan Africa. KSHV prevalence varies substantially in different populations. KSHV is secreted in saliva, and public health measures to reduce its spread may help reduce the incidence of KSHV-associated diseases. Although there have been advances in the treatment of Kaposi sarcoma, KSHV-multicentric Castleman disease, and PEL, improved therapies are needed, especially those that are appropriate for Kaposi sarcoma in resource-poor regions.

  5. Effective transduction of osteogenic sarcoma cells by a baculovirus vector.

    PubMed

    Song, Sun U; Shin, Seok-Hwan; Kim, Soon-Ki; Choi, Gwang-Seong; Kim, Woo-Chul; Lee, Moon-Hee; Kim, Sei-Joong; Kim, In-Ho; Choi, Mi-Sook; Hong, Young-Jin; Lee, Kwan-Hee

    2003-03-01

    Efficient gene delivery of a baculovirus-derived vector (BV-p53-lacZ) to a human osteogenic sarcoma cell line, Saos-2, was serendipitously found while evaluating the vector for gene delivery to human p53-null tumour cells in a previous study. Therefore, we investigated other human, rat and mouse osteogenic sarcoma and other types of tumour cell lines for transduction efficiency via baculovirus vectors containing a lacZ reporter gene under the control of either a cytomegalovirus or Rous sarcoma virus promoter. The expression of beta-galactosidase protein, assessed by X-Gal staining and beta-galactosidase ELISA, demonstrated an extremely high level of transduction efficiency in some osteogenic sarcoma cell lines, such as U-2OS, Saos-2 and Saos-LM2. These human osteogenic sarcoma cell lines showed levels of beta-galactosidase expression 5-40 times greater than HepG2 cells, which were previously thought to be the mammalian cells most susceptible to baculovirus-mediated gene delivery. The level of acetylated histone proteins in these tumour lines did not correlate well with the high level of reporter gene expression. These results strongly suggest that some osteogenic sarcoma cells are highly susceptible to baculovirus-mediated gene delivery and that a baculovirus-derived vector is an efficient gene delivery vehicle into human osteogenic sarcoma cells.

  6. 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.

  7. 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

  8. [Primary sarcoma of the mammary gland].

    PubMed

    Coronel-Brizio, Pedro Guillermo; Quistian Navarrete, Fernando; Guzmán Garcia, Raúl E; Zoloeta Dominguez, Pedro A; Venegas Espinosa, Berenice

    2012-12-01

    Breast cancer occupies the chief place in incidence after the cervix. This gland sarcomas are rare with less than 1% at this location. Its diagnosis is difficult and its evolution is aggressive. Primary breast osteogenic sarcomas are a subset of lower frequency so that their behavior, evolution, prognosis ytratamiento no much experience and often are diagnosed as benign tumors. We report the case of a woman of 59 years which initially arose from a tumor in the right breast was performed with tru-cut biopsy with inconclusive results, using the tumor 7 months after the presence of a multilobulated solid tumor 20 cm, tumor compatible with mammography Phylodes (BIRADS 2). It was decided to perform right total mastectomy and pectoral muscle resection reconstruction with latissimus dorsi more and histochemical review pathology diagnosed with malignant mesenchymal neoplasm, osteosarcoma osteoblast osteoclast type and complemented the treatment with radiotherapy and chemotherapy. The prognosis is poor because the five-year survival is less than 40%. In the fast-growing breast tumors and large mammographic calcifications with signs and antecedentesde irradiation, trauma and Phyllodes tumor must be aware of this disease and that early diagnosis improves survival. In the surgical treatment of axillary dissection is not indicated and adjuvant treatment with radiotherapy and chemotherapy.

  9. Targeted therapies for soft-tissue sarcomas.

    PubMed

    Tap, William D; Federman, Noah; Eilber, Fritz C

    2007-05-01

    The past several decades have revealed certain challenges that are inherent to soft-tissue sarcomas with regards to devising, testing and setting treatment paradigms for such a rare and heterogeneous malignancy. Despite this, significant advances have been made through the efforts of scientists, clinicians and patients alike. We are now entering a molecular era of cancer and current biotechnology is beginning to unravel the pathogenic enigma of these often devastating tumors. As our understanding of these malignancies improves, so does our list of potential treatment options. The impetus now lies with the medical/scientific community to direct translational research and subsequently the development and clinical testing of novel compounds in a fashion that best serves this unique patient population. To do so, we must continue to integrate the lessons of the past with the resources and promise of the future. This review will outline current areas of therapeutic interest in soft-tissue sarcomas with regard to agents that have reached clinical testing.

  10. 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.

  11. An estimation of mechanical stress on alveolar walls during repetitive alveolar reopening and closure.

    PubMed

    Chen, Zheng-Long; Song, Yuan-Lin; Hu, Zhao-Yan; Zhang, Su; Chen, Ya-Zhu

    2015-08-01

    Alveolar overdistension and mechanical stresses generated by repetitive opening and closing of small airways and alveoli have been widely recognized as two primary mechanistic factors that may contribute to the development of ventilator-induced lung injury. A long-duration exposure of alveolar epithelial cells to even small, shear stresses could lead to the changes in cytoskeleton and the production of inflammatory mediators. In this paper, we have made an attempt to estimate in situ the magnitudes of mechanical stresses exerted on the alveolar walls during repetitive alveolar reopening by using a tape-peeling model of McEwan and Taylor (35). To this end, we first speculate the possible ranges of capillary number (Ca) ≡ μU/γ (a dimensionless combination of surface tension γ, fluid viscosity μ, and alveolar opening velocity U) during in vivo alveolar opening. Subsequent calculations show that increasing respiratory rate or inflation rate serves to increase the values of mechanical stresses. For a normal lung, the predicted maximum shear stresses are <15 dyn/cm(2) at all respiratory rates, whereas for a lung with elevated surface tension or viscosity, the maximum shear stress will notably increase, even at a slow respiratory rate. Similarly, the increased pressure gradients in the case of elevated surface or viscosity may lead to a pressure drop >300 dyn/cm(2) across a cell, possibly inducing epithelial hydraulic cracks. In addition, we have conceived of a geometrical model of alveolar opening to make a prediction of the positive end-expiratory pressure (PEEP) required to splint open a collapsed alveolus, which as shown by our results, covers a wide range of pressures, from several centimeters to dozens of centimeters of water, strongly depending on the underlying pulmonary conditions. The establishment of adequate regional ventilation-to-perfusion ratios may prevent recruited alveoli from reabsorption atelectasis and accordingly, reduce the required levels of

  12. Decompression of inferior alveolar nerve: case report.

    PubMed

    Marques, Tiago Miguel Santos; Gomes, Joana Marques

    2011-01-01

    Paresthesia as a result of mechanical trauma is one of the most frequent sensory disturbances of the inferior alveolar nerve. This case report describes surgical treatment for paresthesia caused by a compressive phenomenon within the mandibular canal. The cause of the compression, a broken instrument left in the patient's mouth during previous endodontic therapy, was identified during routine radiography and computed tomography. Once the foreign object was removed by surgery, the paresthesia resolved quickly. This case highlights the potential for an iatrogenic mechanical cause of paresthesia.

  13. AIDS-related primary Kaposi sarcoma of the nasopharynx.

    PubMed

    Çelenk, Fatih; Yilmaz, Metin; Asal, Korhan; Ekinci, Özgür; Tokgöz, Nil

    2011-06-01

    Primary nasopharyngeal Kaposi sarcoma is extremely rare, as only 1 case has been previously reported in the literature. We report a new case, which occurred in a 37-year-old man with a known history of acquired immune deficiency syndrome (AIDS). The patient presented with complaints of recurrent epistaxis and postnasal hemorrhage. Endoscopic examination detected a bluish, smooth, firm, nonpulsatile mass in the nasopharyngeal wall. Histopathologic findings on biopsy were consistent with Kaposi sarcoma. The tumor was successfully treated with radiotherapy. Kaposi sarcoma should be considered in the differential diagnosis of any AIDS patient who presents with recurrent unilateral nasal bleeding.

  14. Pseudo-Kaposi sarcoma (acroangiodermatitis): occurring after bullous erysipelas.

    PubMed

    Kutlubay, Zekayi; Yardimci, Gürkan; Engin, Burhan; Demirkesen, Cuyan; Aydin, Övgü; Khatib, Rashid; Tuzun, Yalçın

    2015-05-18

    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.

  15. Biological Extremity Reconstruction after Sarcoma Resection: Past, Present, and Future

    PubMed Central

    Holzer, Lukas A.; Leithner, Andreas

    2013-01-01

    In sarcoma surgery besides a wide local resection, limb salvage became more and more important. Reconstruction of bone and soft tissue defects after sarcoma resection poses a major challenge for surgeons. Nowadays a broad range of reconstructive methods exist to deal with bony defects. Among these are prostheses, bone autografts, or bone allografts. Furthermore a variety of plastic reconstructive techniques exist that allow soft tissue reconstruction or coverage after sarcoma resection. Here we discuss the historical highlights, the present role, and possible future options for biological reconstruction. PMID:23840167

  16. Sarcoma epidemiology and etiology: potential environmental and genetic factors.

    PubMed

    Lahat, Guy; Lazar, Alexander; Lev, Dina

    2008-06-01

    Sarcomas are a heterogeneous group of tumors that may have many etiologies. The incidence of histologic subtypes differs significantly between children and adults. The increase in incidence may be due to improved registry systems, diagnostic tools, and pathologic definitions. Environmental causes may contribute to increased incidence. Genetic alternations may play a role in sarcoma development. As a result of rapidly evolving genomic and proteomic technologies, increased knowledge of the oncogenic mechanisms underlying sarcomagenesis is being generated. Understanding the mechanisms involved in sarcomagenesis is rudimentary. Insight into the molecular basis of sarcoma inception, proliferation, and dissemination hopefully will lead to more effective therapies.

  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. Pulmonary artery sarcoma masquerading as chronic pulmonary thromboembolism.

    PubMed

    Coskun, Ugur; Sinan, Umit Yasar; Calpar, Ilknur; Yildizeli, Bedrettin; Yanartas, Mehmet; Filinte, Deniz; Kucukoglu, Mehmet Serdar

    2014-10-01

    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.

  19. Intimal Sarcoma of the Pulmonary Artery Treated with Pazopanib.

    PubMed

    Funatsu, Yohei; Hirayama, Miwa; Shiraishi, Junichi; Asakura, Takanori; Wakaki, Misa; Yamada, Erina; Fujimoto, Kazuyuki; Satomi, Ryosuke; Inaki, Shunsuke; Murata, Yuya; Oyamada, Yoshitaka

    2016-01-01

    Intimal sarcoma is a rare disease with a poor prognosis. We herein report the case of a 71-year-old man with intimal sarcoma of the pulmonary artery treated with pazopanib. The tumor showed regression after 1 month of treatment. Hand-foot syndrome led to cessation of pazopanib, which triggered a disease flare. Pazopanib should be considered in patients with intimal sarcoma of the pulmonary artery that is unresectable or recurrent after surgery or cytotoxic chemotherapy. We must be careful about drug cessation, as it can lead to a disease flare.

  20. 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

  1. Low-grade myofibroblastic sarcoma of the parapharyngeal space.

    PubMed

    Takahama, A; Nascimento, A G; Brum, M C; Vargas, P A; Lopes, M A

    2006-10-01

    Low-grade myofibroblastic sarcoma was recently described as representing malignant mesenchymal tumours that show myofibroblastic differentiation; few cases have been reported. Here, a low-grade myofibroblastic sarcoma of the parapharyngeal space is described. A 42-year-old man presented with swelling on the right side of the temporal bone. Based on histological and immunohistochemical features, the diagnosis of low-grade myofibroblastic sarcoma was established. The tumour had invaded the orbit and the brain, and therefore surgical excision was not possible. There are thought to have been no cases affecting this region reported previously in the English-language literature.

  2. Vaccine-associated sarcomas in cats: a unique cancer model.

    PubMed

    McNiel, E A

    2001-01-01

    Epidemiologic evidence supports a relationship between vaccination of cats for rabies and feline leukemia virus with the development of soft tissue sarcomas at the site of administration. These tumors are locally invasive and histologically aggressive. As with high-grade soft tissue sarcoma in humans, combination treatment with radiation therapy and surgery provides for optimum tumor control. Feline vaccine-associated sarcoma has become a difficult issue for the veterinary profession for legal, ethical, and clinical reasons. Although most research efforts have focused on therapeutic intervention, this tumor has great potential to provide an informative model for carcinogenesis and genetic susceptibility applicable to cancer in all species, including humans.

  3. Deficiency of vitamin E in the alveolar fluid of cigarette smokers. Influence on alveolar macrophage cytotoxicity.

    PubMed

    Pacht, E R; Kaseki, H; Mohammed, J R; Cornwell, D G; Davis, W B

    1986-03-01

    Cigarette smoking produces oxidant-mediated changes in the lung important to the pathogenesis of emphysema. Since vitamin E can neutralize reactive oxygen species and prevent peroxidation of unsaturated lipids, it may constitute an important component of the lung's defense against oxidant injury. To better characterize the antioxidant protective role of vitamin E, young asymptomatic smokers and nonsmokers were evaluated by bronchoalveolar lavage before and immediately after a 3-wk course of oral vitamin E (2,400 IU/d). Smoker alveolar fluid at baseline was relatively deficient in vitamin E compared with nonsmoker fluid (3.1 +/- 0.7 ng/ml vs. 20.7 +/- 2.4 ng/ml, P less than 0.005). Although smoker alveolar fluid vitamin E levels increased to 9.3 +/- 2.3 ng/ml after supplementation, the levels remained significantly lower than nonsmoker baseline levels (P less than 0.01). This deficiency was explained, in part, by the increased oxidative metabolism of vitamin E to the quinone form in the lungs of smokers compared with nonsmokers. Although the significance of a lower concentration of alveolar fluid vitamin E is unclear, it may compromise the antioxidant protection afforded by the alveolar fluid as it coats the lung's epithelial surface. The protective role of vitamin E was assessed by cytotoxicity experiments, which demonstrated that the killing of normal rat lung parenchymal cells by smoker alveolar macrophages was inversely related to the vitamin E content of the parenchymal cells. These findings suggest that vitamin E may be an important lower respiratory tract antioxidant, and that the deficiency seen in young smokers may predispose them to an enhanced oxidant attack on their lung parenchymal cells.

  4. Proteomic Analysis of Gingival Tissue and Alveolar Bone during Alveolar Bone Healing*

    PubMed Central

    Yang, Hee-Young; Kwon, Joseph; Kook, Min-Suk; Kang, Seong Soo; Kim, Se Eun; Sohn, Sungoh; Jung, Seunggon; Kwon, Sang-Oh; Kim, Hyung-Seok; Lee, Jae Hyuk; Lee, Tae-Hoon

    2013-01-01

    Bone tissue regeneration is orchestrated by the surrounding supporting tissues and involves the build-up of osteogenic cells, which orchestrate remodeling/healing through the expression of numerous mediators and signaling molecules. Periodontal regeneration models have proven useful for studying the interaction and communication between alveolar bone and supporting soft tissue. We applied a quantitative proteomic approach to analyze and compare proteins with altered expression in gingival soft tissue and alveolar bone following tooth extraction. For target identification and validation, hard and soft tissue were extracted from mini-pigs at the indicated times after tooth extraction. From triplicate experiments, 56 proteins in soft tissue and 27 proteins in alveolar bone were found to be differentially expressed before and after tooth extraction. The expression of 21 of those proteins was altered in both soft tissue and bone. Comparison of the activated networks in soft tissue and alveolar bone highlighted their distinct responsibilities in bone and tissue healing. Moreover, we found that there is crosstalk between identified proteins in soft tissue and alveolar bone with respect to cellular assembly, organization, and communication. Among these proteins, we examined in detail the expression patterns and associated networks of ATP5B and fibronectin 1. ATP5B is involved in nucleic acid metabolism, small molecule biochemistry, and neurological disease, and fibronectin 1 is involved in cellular assembly, organization, and maintenance. Collectively, our findings indicate that bone regeneration is accompanied by a profound interaction among networks regulating cellular resources, and they provide novel insight into the molecular mechanisms involved in the healing of periodontal tissue after tooth extraction. PMID:23824910

  5. The proprotein convertase furin is required to maintain viability of alveolar rhabdomyosarcoma cells

    PubMed Central

    Jaaks, Patricia; Meier, Gianmarco; Alijaj, Nagjie; Brack, Eva; Bode, Peter; Koscielniak, Ewa; Wachtel, Marco; Schäfer, Beat W.; Bernasconi, Michele

    2016-01-01

    Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Success of current therapies is still limited and outcome is particularly poor for metastatic alveolar rhabdomyosarcoma (aRMS). We previously identified the proprotein convertase furin as potential target for specific drug delivery with RMS-homing peptides. Furin is a protease that converts inactive precursor proteins into bioactive proteins and peptides. In this study, we investigate the biological role of furin in aRMS progression in vitro and in vivo. Furin expression was confirmed in over 86% RMS biopsies in a tissue microarray (n=89). Inducible furin silencing in vitro led to significant impairment of cell viability and proliferation in all investigated aRMS cell lines, but not in MRC5 fibroblasts. Furthermore, the aRMS cell lines Rh3 and Rh4 revealed to be very sensitive to furin silencing, undergoing caspase-dependent cell death. Notably, furin silencing in vivo led to complete remission of established Rh4 tumors and to delayed growth in Rh30 tumors. Taken together, these findings identify furin as an important factor for aRMS progression and survival. Thus, we propose furin as a novel therapeutic target for treatment of aRMS. PMID:27572312

  6. Caveolin-1 is down-regulated in alveolar rhabdomyosarcomas and negatively regulates tumor growth

    PubMed Central

    Huertas-Martínez, Juan; Rello-Varona, Santiago; Herrero-Martín, David; Barrau, Ignasi; García-Monclús, Silvia; Sáinz-Jaspeado, Miguel; Lagares-Tena, Laura; Núñez-Álvarez, Yaiza; Mateo-Lozano, Silvia; Mora, Jaume; Roma, Josep; Toran, Nuria; Moran, Sebastian; López-Alemany, Roser; Gallego, Soledad; Esteller, Manel; Peinado, Miguel A.; Xavier García del, Muro; Tirado, Oscar M.

    2014-01-01

    Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood and adolescence. Despite advances in therapy, patients with histological variant of rhabdomyosarcoma known as alveolar rhabdomyosarcoma (ARMS) have a 5-year survival of less than 30%. Caveolin-1 (CAV1), encoding the structural component of cellular caveolae, is a suggested tumor suppressor gene involved in cell signaling. In the present study we report that compared to other forms of rhabdomyosarcoma (RMS) CAV1 expression is either undetectable or very low in ARMS cell lines and tumor samples. DNA methylation analysis of the promoter region and azacytidine-induced re-expression suggest the involvement of epigenetic mechanisms in the silencing of CAV1. Reintroduction of CAV1 in three of these cell lines impairs their clonogenic capacity and promotes features of muscular differentiation. In vitro, CAV1-expressing cells show high expression of Caveolin-3 (CAV3), a muscular differentiation marker. Blockade of MAPK signaling is also observed. In vivo, CAV1-expressing xenografts show growth delay, features of muscular differentiation and increased cell death. In summary, our results suggest that CAV1 could function as a potent tumor suppressor in ARMS tumors. Inhibition of CAV1 function therefore, could contribute to aberrant cell proliferation, leading to ARMS development. PMID:25313138

  7. Alveolar rhabdomyosarcoma–associated PAX3-FOXO1 promotes tumorigenesis via Hippo pathway suppression

    PubMed Central

    Crose, Lisa E.S.; Galindo, Kathleen A.; Kephart, Julie Grondin; Chen, Candy; Fitamant, Julien; Bardeesy, Nabeel; Bentley, Rex C.; Galindo, Rene L.; Ashley Chi, Jen-Tsan; Linardic, Corinne M.

    2013-01-01

    Alveolar rhabdomyosarcoma (aRMS) is an aggressive sarcoma of skeletal muscle characterized by expression of the paired box 3-forkhead box protein O1 (PAX3-FOXO1) fusion oncogene. Despite its discovery nearly two decades ago, the mechanisms by which PAX3-FOXO1 drives tumor development are not well characterized. Previously, we reported that PAX3-FOXO1 supports aRMS initiation by enabling bypass of cellular senescence checkpoints. We have now found that this bypass occurs in part through PAX3-FOXO1–mediated upregulation of RASSF4, a Ras-association domain family (RASSF) member. RASSF4 expression was upregulated in PAX3-FOXO1–positive aRMS cell lines and tumors. Enhanced RASSF4 expression promoted cell cycle progression, senescence evasion, and tumorigenesis through inhibition of the Hippo pathway tumor suppressor MST1. We also found that the downstream Hippo pathway target Yes-associated protein 1 (YAP), which is ordinarily restrained by Hippo signaling, was upregulated in RMS tumors. These data suggest that Hippo pathway dysfunction promotes RMS. This work provides evidence for Hippo pathway suppression in aRMS and demonstrates a progrowth role for RASSF4. Additionally, we identify a mechanism used by PAX3-FOXO1 to inhibit MST1 signaling and promote tumorigenesis in aRMS. PMID:24334454

  8. Radioimmunoimaging of osteogenic sarcoma xenografts in nude mice using monoclonal antibodies to osteogenic sarcoma

    SciTech Connect

    Sakahara, H.; Endo, K.; Nakashima, T.; Koizumi, M.; Ohta, H.; Kunimatsu, M.; Torizuka, K.; Nakamura, T.; Tanaka, H.; Kotoura, Y.

    1985-05-01

    The authors have developed several monoclonal antibodies against human osteogenic sarcoma, one of which; OST7 (IgGl) selectively localized in osteogenic sarcoma xenografts in nude mice. In the present study, F(ab')/sub 2/ fragment was compared with whole IgG and those labeled with In-111 as well as I-131 were used as a radiotracer for the scintigraphic imaging of tumors. IgC and F(ab')/sub 2/ were labeled with I-131 using chloramine-T method and injected into nude mice bearing human osteogenic sarcoma. Scintigrams at day 2 clearly delineated the site of tumors with almost no radioactivity in other organs with F(ab')/sub 2/, which yielded much better images than whole IgG. Tumor-to-blood ratio of 6.09-27.87 was obtained at day 2 using F(ab')/sub 2/, whereas it was 0.76-1.12 at day 2 and 2.05-3.27 at day 7 with IgG. I-131 labeled nonspecific F(ab')/sub 2/ or IgG resulted in no or very low tumor uptake with tumor-to-blood ratio of 0.94-1.18 at day 2 for F(ab')/sub 2/ and 0.67-0.76 at day 7 for IgG, respectively. In-111 labeled F(ab')/sub 2/ fragment of OST7, which was prepared using DTPA as a bifunctional chelate, also showed a high tumor accumulation with tumor-to-blood ratio of 11.67-17.54 at day 2, but higher background activity in the liver and kidney was observed than I-131 labeled one. These results indicate that F(ab')/sub 2/ fragment of OST7 labeled with either I-131 or In-111, has a great potential for the radioimmunoimaging of osteogenic sarcoma.

  9. [The in vivo penetration of erythromycin into alveolar macrophages].

    PubMed

    Carré, P; Piva, F; Aerts, C; Voisin, C; Wallaert, B

    1990-01-01

    In order to appreciate the in vivo penetration of erythromycin the alveolar spaces a broncho-alveolar lavage was carried out in 24 guinea pigs, 30 minutes, 1 hour 30 minutes and three hours after a single intraperitoneal injection of 50 mgms. of erythromycin. The erythromycin dose was assessed by a microbiological method in the alveolar macrophages and the supernatant of the broncho-alveolar lavage liquid. The intramacrophage concentrations of erythromycin were 3.9, 11.5 and 12 times higher than the serum concentrations at 30 minutes, 1 hour 30 and three hours respectively. The concentrations in the broncho-alveolar lavage liquid was always higher than the serum concentrations tacking account of the different dilutions estimated with relation to the glucose concentrations. At 30 minutes, 1 hour 30 minutes and three hours the alveolar macrophages contained 1.9; 7.6 and 6 times more erythromycin respectively than the lavage supernatant. From the first half hour of its administration the erythromycin was concentrated in the alveolar spaces, in particular within the macrophages. Already noted in vitro, this rapidity of erythromycin concentration in vivo in alveolar macrophages appears to be one of the reasons to explain its activity against micro-organisms developing within macrophages.

  10. Tongue-Palate Contact of Perceptually Acceptable Alveolar Stops

    ERIC Educational Resources Information Center

    Lee, Alice; Gibbon, Fiona E.; O'Donovan, Cliona

    2013-01-01

    Increased tongue-palate contact for perceptually acceptable alveolar stops has been observed in children with speech sound disorders (SSD). This is a retrospective study that further investigated this issue by using quantitative measures to compare the target alveolar stops /t/, /d/ and /n/ produced in words by nine children with SSD (20 tokens of…

  11. Diffuse alveolar haemorrhage associated with progressive systemic sclerosis.

    PubMed Central

    Griffin, M T; Robb, J D; Martin, J R

    1990-01-01

    A 41 year old man with an eight year history of progressive systemic sclerosis developed severe diffuse alveolar haemorrhage and died. The importance of diffuse alveolar haemorrhage as a rare but potentially serious complication of connective tissue disease should not be overlooked. Images PMID:2256025

  12. Diffuse alveolar haemorrhage associated with aerosol propellant use.

    PubMed

    Kelchen, Phillip; Jamous, Fady; Huntington, Mark K

    2013-08-16

    Diffuse alveolar haemorrhage (DAH) is a clinical syndrome resulting from injury to the alveolar microcirculation, most commonly associated with not only autoimmune disorders or connective tissue disease, but also a variety of infections, neoplasms and toxins. We report here a case of an otherwise healthy young man with DAH attributable to an inhalation injury resulting from use of aerosol spray paint.

  13. Diffuse alveolar haemorrhage associated with aerosol propellant use

    PubMed Central

    Kelchen, Phillip; Jamous, Fady; Huntington, Mark K

    2013-01-01

    Diffuse alveolar haemorrhage (DAH) is a clinical syndrome resulting from injury to the alveolar microcirculation, most commonly associated with not only autoimmune disorders or connective tissue disease, but also a variety of infections, neoplasms and toxins. We report here a case of an otherwise healthy young man with DAH attributable to an inhalation injury resulting from use of aerosol spray paint. PMID:23955981

  14. Alveolar Epithelial Dynamics in Post-pneumonectomy Lung Growth

    PubMed Central

    Chamoto, Kenji; Gibney, Barry C.; Ackermann, Maximilian; Lee, Grace S.; Konerding, Moritz A.; Tsuda, Akira; Mentzer, Steven J.

    2013-01-01

    The intimate anatomic and functional relationship between epithelial cells and endothelial cells within the alveolus suggests the likelihood of a coordinated response during post-pneumonectomy lung growth. To define the population dynamics and potential contribution of alveolar epithelial cells to alveolar angiogenesis, we studied alveolar Type II and Type I cells during the 21 days after pneumonectomy. Alveolar Type II cells were defined and isolated by flow cytometry using a CD45−, MHC class II+, phosphine+ phenotype. These phenotypically defined alveolar Type II cells demonstrated an increase in cell number after pneumonectomy; the increase in cell number preceded the increase in Type I (T1α+) cells. Using a parabiotic wild type/GFP pneumonectomy model, less than 3% of the Type II cells and 1% of the Type I cells were positive for GFP—a finding consistent with the absence of a blood-borne contribution to alveolar epithelial cells. The CD45−, MHC class II+, phosphine+ Type II cells demonstrated the active transcription of angiogenesis-related genes both before and after pneumonectomy. When the Type II cells on day 7 after pneumonectomy were compared to non-surgical controls, 10 genes demonstrated significantly increased expression (p<.05). In contrast to the normal adult Type II cells, there was notable expression of inflammation-associated genes (Ccl2, Cxcl2, Ifng) as well as genes associated with epithelial growth (Ereg, Lep). Together, the data suggest an active contribution of local alveolar Type II cells to alveolar growth. PMID:23408540

  15. Estrogen regulates pulmonary alveolar formation, loss, and regeneration in mice.

    PubMed

    Massaro, Donald; Massaro, Gloria Decarlo

    2004-12-01

    Lung tissue elastic recoil and the dimension and number of pulmonary gas-exchange units (alveoli) are major determinants of gas-exchange function. Loss of gas-exchange function accelerates after menopause in the healthy aged and is progressively lost in individuals with chronic obstructive pulmonary disease (COPD). The latter, a disease of midlife and later, though more common in men than in women, is a disease to which women smokers and never smokers may be more susceptible than men; it is characterized by diminished lung tissue elastic recoil and presently irremediable alveolar loss. Ovariectomy in sexually immature rats diminishes the formation of alveoli, and estrogen prevents the diminution. In the present work, we found that estrogen receptor-alpha and estrogen receptor-beta, the only recognized mammalian estrogen receptors, are required for the formation of a full complement of alveoli in female mice. However, only the absence of estrogen receptor-beta diminishes lung elastic tissue recoil. Furthermore, ovariectomy in adult mice results, within 3 wk, in loss of alveoli and of alveolar surface area without a change of lung volume. Estrogen replacement, after alveolar loss, induces alveolar regeneration, reversing the architectural effects of ovariectomy. These studies 1) reveal estrogen receptors regulate alveolar size and number in a nonredundant manner, 2) show estrogen is required for maintenance of already formed alveoli and induces alveolar regeneration after their loss in adult ovariectomized mice, and 3) offer the possibility estrogen can slow alveolar loss and induce alveolar regeneration in women with COPD.

  16. Correction of alveolar cleft with calcium-based bone substitutes.

    PubMed

    Lazarou, Spiros A; Contodimos, George B; Gkegkes, Ioannis D

    2011-05-01

    The criterion standard of alveolar cleft repair is iliac crest bone graft before secondary canine eruption. Tooth eruption has never been shown to occur in synthetic bone substitute, and there is no ideal autologous bone graft for primary repair. This prospective study evaluated alveolar cleft grafting with a calcium substitute before primary canine eruption. Ten consecutive patients with complete cleft lip, palate, and unilateral alveolar cleft with reasonably aligned arches were grafted beginning in January 2003 to March 2007. Mean age at surgery was 10.4 months. Follow-up ranged from 3 to 7 years. Radiologic evaluation of alveolar ridge was performed at the age of 4.All 10 patients were operated on by the same surgeon using the same technique, that is, conservative elevation of nasal, oral, and anterior alveolar mucosal flaps around the cleft, closure of nasal and oral flaps, placement of 1 to 3 mL of calcium substitute paste or crystals in the pocket, and closure of the anterior alveolar mucosa. All 10 patients healed without complication. Clinical evaluation revealed a well-healed arch with primary canine growth in the area of the previous cleft. Adequate normal bone formation and often a descending secondary canine were radiologically confirmed. Calcium substitutes offer significant advantages over other biomaterials as well as autologous bone grafts particularly in the primary alveolar cleft reconstruction. Our study has shown for the first time that teeth can erupt through this material, which turns into a normal functioning bone in the alveolar ridge.

  17. Tongue-Palate Contact of Perceptually Acceptable Alveolar Stops

    ERIC Educational Resources Information Center

    Lee, Alice; Gibbon, Fiona E.; O'Donovan, Cliona

    2013-01-01

    Increased tongue-palate contact for perceptually acceptable alveolar stops has been observed in children with speech sound disorders (SSD). This is a retrospective study that further investigated this issue by using quantitative measures to compare the target alveolar stops /t/, /d/ and /n/ produced in words by nine children with SSD (20 tokens of…

  18. Alveolar bone changes in autogenous tooth transplantation.

    PubMed

    Waikakul, Aurasa; Punwutikorn, Jirapun; Kasetsuwan, Julalux; Korsuwannawong, Suwanna

    2011-03-01

    To assess the alveolar bone formation after autogenous tooth transplantation by conventional radiographic method and digital subtraction radiography. This retrospective study was done in 54 of 136 patients who received the third molar tooth transplantation and attended the first week, as well as the 1-, 3-, 6-, 9-, and 12-month follow-up. Postoperative periapical radiographs were subsequently evaluated by direct visual interpretation and digital subtraction radiography. The data were analyzed by using McNemar test and 1-way repeated-measure analysis of variance as well as Bonferroni multiple comparison. Fifty-four cases of transplantation were studied. Most of them had normal wound healing. The direct radiographic interpretation and digital subtraction radiography found significant alveolar bone formation in the first-and the third-month follow-ups (P < .05). Lamina dura appeared in the third month and kept increasing until the sixth month. Postoperative radiographs revealed the distinctive bone formation up to the third month. The clinical and radiographic assessment found that the third molar transplants could bear a normal chewing load within 3 months. Copyright © 2011 Mosby, Inc. All rights reserved.

  19. Reversible transdifferentiation of alveolar epithelial cells.

    PubMed

    Danto, S I; Shannon, J M; Borok, Z; Zabski, S M; Crandall, E D

    1995-05-01

    Alveolar epithelial type II (AT2) cells have been thought to be the progenitors of terminally differentiated type I (AT1) cells in the adult animal in vivo. In this study, we used an AT1 cell-specific monoclonal antibody (mAb VIII B2) to investigate expression of the AT1 cell phenotype accompanying reversible changes in expression of the AT2 cell phenotype. AT2 cells were isolated and cultured either on attached collagen gels or on gels detached 1 or 4 days after plating and maintained thereafter as floating gels. Monolayers on both attached and floating gels were harvested on days 4 and 8 and analyzed by electron microscopy for changes in morphology and binding of mAb VIII B2. Results indicate that: (1) alveolar epithelial cells (AEC) on attached gels develop characteristics of the AT1 cell phenotype, (2) AEC on gels detached on day 1 maintain features of the AT2 cell phenotype (and do not react with mAb VIII B2), and (3) the expression of AT1 cell phenotypic traits seen by day 4 on attached gels is reversed after detachment. We conclude that commitment to the AT1 and AT2 cell lineages requires continuous regulatory input to maintain the differentiated states, and that transdifferentiation between AT2 and AT1 cells may be reversible.

  20. Diffuse Alveolar Hemorrhage in Acute Myeloid Leukemia.

    PubMed

    Nanjappa, Sowmya; Jeong, Daniel K; Muddaraju, Manjunath; Jeong, Katherine; Hill, Ebone D; Greene, John N

    2016-07-01

    Diffuse alveolar hemorrhage is a potentially fatal pulmonary disease syndrome that affects individuals with hematological and nonhematological malignancies. The range of inciting factors is wide for this syndrome and includes thrombocytopenia, underlying infection, coagulopathy, and the frequent use of anticoagulants, given the high incidence of venous thrombosis in this population. Dyspnea, fever, and cough are commonly presenting symptoms. However, clinical manifestations can be variable. Obvious bleeding (hemoptysis) is not always present and can pose a potential diagnostic challenge. Without prompt treatment, hypoxia that rapidly progresses to respiratory failure can occur. Diagnosis is primarily based on radiological and bronchoscopic findings. This syndrome is especially common in patients with hematological malignancies, given an even greater propensity for thrombocytopenia as a result of bone marrow suppression as well as the often prolonged immunosuppression in this patient population. The syndrome also has an increased incidence in individuals with hematological malignancies who have received a bone marrow transplant. We present a case series of 5 patients with acute myeloid leukemia presenting with diffuse alveolar hemorrhage at our institution. A comparison of clinical manifestations, radiographic findings, treatment course, and outcomes are described. A review of the literature and general overview of the diagnostic evaluation, differential diagnoses, pathophysiology, and treatment of this syndrome are discussed.

  1. Chest ultrasound findings in pulmonary alveolar microlithiasis.

    PubMed

    Rea, Gaetano; Sperandeo, Marco; Sorrentino, Nunzia; Stanziola, Anna Agnese; D'Amato, Maria; Bocchino, Marialuisa

    2015-10-01

    Pulmonary alveolar microlithiasis (PAM) is a rare disorder of unknown etiology affecting people at any age. It is characterized by multiple and microscopic calcium deposits diffusely localized within the alveoli. Thorax high-resolution computed tomography is considered the gold standard for PAM imaging. Herein we report for the first time the use of trans-thoracic ultrasound (TUS) examination in a young severely obese PAM female patient, diagnosed at the age of 10, and referred to our clinic for re-staging purposes at the age of 36. Unlike expected, no reverberation or additional artifacts were appreciated on TUS examination despite the severity of the interstitial/alveolar involvement seen on conventional CT imaging. To date, no ring-down or comet-tail artifacts were detected. The only TUS finding was an increased thickness and irregular profile, more evident in the dorsal lower lung regions, of the hyper-echoic pleural line. TUS has recently aroused increasing interest among clinicians and radiologists as a useful noninvasive diagnostic tool for studying pleuro-pulmonary diseases, including interstitial lung diseases (ILDs). The peculiarity of our case is represented by the discrepancy between TUS and CT findings. Further efforts to address the usefulness and US patterns in diffuse ILDs, with the inclusion of rare disorders, are needed.

  2. Secondary alveolar bone grafting: An outcome analysis

    PubMed Central

    Murthy, Ananth S; Lehman, James A

    2006-01-01

    OBJECTIVE To review the outcome of secondary alveolar bone grafting in unilateral and bilateral cleft lip and palate. DESIGN A surgeon’s experience, by retrospective chart review, of 70 consecutive patients at a tertiary care centre. OUTCOME MEASURE Periapical radiographs were taken at least six months after secondary alveolar bone grafting. The Enemark grading system was used to stratify graft-take. RESULTS In unilateral clefts, 33% were level 1, 36% were level 2, 20% were level 3 and 11% were level 4. In bilateral clefts, 29% were level 1, 50% were level 2, 14% were level 3 and 7% were level 4. There was no statistically significant difference between the level of take and the type of cleft. Complications encountered were infection (n=3), fistula (n=3), pain (n=4) and bone graft exposure that led to failure (n=2). Two patients required reoperation for bone grafting. CONCLUSIONS The iliac crest is a good donor site with excellent results and minimal morbidity. PMID:19554111

  3. Radiological aspects of diffuse alveolar haemorrhage.

    PubMed

    Cortese, G; Nicali, R; Placido, R; Gariazzo, G; Anrò, P

    2008-02-01

    This paper describes chest X-ray (CXR) and computed tomography (CT) findings of diffuse alveolar haemorrhage (DAH). We retrospectively reviewed 23 episodes of DAH in 20 patients, 17 of known aetiology and three of unknown aetiology. All cases were studied by CXR and 15 also by CT. Parenchymal consolidations and ground-glass opacities were evaluated after dividing each lung into three regions (upper, middle, lower) for a total of six zones. Consolidations or ground-glass opacities were identified on CXR in 16/20 patients, mainly in the middle fields (73%). In 4/20 patients, all with Wegener's granulomatosis, CXR was negative or demonstrated only nodular opacities; in two of these cases, CT revealed ground-glass opacities. A complete follow-up was available for ten patients: initially, they showed consolidation opacities in 36/60 zones, which persisted in 16/60 after 7 days and in 11/60 after 15 days. Conversely, ground-glass opacities increased after 7 days owing to the partial regression of consolidation opacities, and they markedly diminished after 15 days. DAH is radiologically characterised by a nonspecific alveolar-filling pattern. Diagnosis or suspicion of DAH needs to be supported by the evidence of haemoptysis and/or rapid-onset anaemia. CT is superior in detecting ground-glass opacities and is required in cases of suspected DAH with normal CXR findings.

  4. An unusual association of malignant gastrointestinal neuroectodermal tumor (clear cell sarcoma-like) and Ewing sarcoma.

    PubMed

    Insabato, Luigi; Guadagno, Elia; Natella, Valentina; Somma, Anna; Bihl, Michel; Pizzolorusso, Antonio; Mainenti, Pier Paolo; Apice, Gaetano; Tornillo, Luigi

    2015-09-01

    Very recently a new designation of "Malignant Neuroectodermal Gastrointestinal Tumor" has been proposed for an aggressive form of neuroectodermal tumor with features similar to that of Clear Cell Sarcoma of Soft Tissue, however without a melanocytic differentiation. Also known as "clear cell sarcoma-like tumors of the gastrointestinal tract", these tumors show some features strongly suggesting an origin from a gastrointestinal neuroectodermal precursor cell unable to differentiate along the melanocytic lineage. They occur mainly in young and middle-aged adults, and have a poor prognosis with a high rate of liver and lymphnode metastases. Histologically they are composed of epithelioid or oval-to spindle cells with a sheet-like or nested pattern of growth, strongly positive for neural markers (S-100, SOX10, and vimentin) and negative for the melanocytic ones. EWSR1 gene rearrangements including EWSR1-ATF1 or EWSR1-CREB1 GENE fusions are typically assessed in these tumors. Here we report a case of malignant neuroectodermal gastrointestinal tumor which immunophenotypically unusually expressed FLI-1, occurring in a 29-year-old man with a previous medical history of Ewing sarcoma. We finally suggest that this case might be a further evidence of a link between these two entities.

  5. Secondary alveolar bone grafting: our experience with olecranon bone graft.

    PubMed

    Nadal, Emmanuela; Sabás, Mariana; Dogliotti, Pedro; Espósito, Raquel

    2010-03-01

    Management of alveolar cleft has dramatically changed during the last century: secondary alveolar bone grafting is now an integral part of cleft palate and craniofacial center's protocols. The objectives of alveolar repair and bone grafting are as follows: providing a continuous and stable maxillary dental arch, closure of oronasal fistulae, adequate bone for tooth eruption or orthodontic movement, and nasal base support, improving facial aesthetic. Although cancellous iliac bone is the donor site selected more frequently, bone grafts harvested from different sites have been advocated to decrease donor site morbidity.The aim of this study was to propose and evaluate the use of olecranon as a donor site in 24 patients with secondary alveolar cleft. The graft is taken as a single piece to fit the alveolar cleft defect, and it includes periosteum and corticocancellous bone to improve early vascularization and greater volume maintenance.

  6. Relative effects of asbestos and wollastonite on alveolar macrophages.

    PubMed

    Pailes, W H; Judy, D J; Resnick, H; Castranova, V

    1984-01-01

    Rabbit alveolar macrophages were exposed in culture to chrysotile asbestos, wollastonite, or latex, and the effects on various biochemical and physiological parameters related to cellular viability and fibrogenicity were determined. Exposure of alveolar macrophages to asbestos, wollastonite, or latex for 3 d has no effect on oxygen consumption or cellular volume. However, treatment of alveolar macrophages with as little as 25 micrograms asbestos/ml for 1 d increases lysosomal enzyme release and decreases membrane integrity, i.e., decreases trypan blue exclusion and increases leakage of cytosolic enzymes. In contrast, exposure of alveolar macrophages to wollastonite or latex at 250 micrograms/ml does not induce lysosomal enzyme release or alter membrane integrity even after 3 d of exposure in culture. These data suggest that chrysotile asbestos damages rabbit alveolar macrophages, while wollastonite, a potential substitute for asbestos, is far less cytotoxic.

  7. A Comparative Study between Carcinoma and Sarcoma Using Raman Spectroscopy

    NASA Astrophysics Data System (ADS)

    Dehghani-Bidgoli, Z.; Baygi, M. H. Miran; Kabir, E.; Malekfar, R.

    2014-01-01

    The purpose of this study was to find discriminating Raman spectral features between two major types of cancer, i.e., carcinoma and sarcoma. To this end, Raman spectra from adenocarcinoma, liposarcoma and fibrosarcoma samples were compared. A Raman system was used for the tissue Raman spectroscopic measurements at 785-nm laser excitation. After pre-processings, the Raman spectra were investigated, in major bands associated with protein and lipids, in the adenocarcinoma, liposarcoma, and fibrosarcoma groups. Principal component analysis and nonnegative matrix factorization were performed for finding most significant features in discriminating the spectra of carcinoma from those of sarcoma samples. The findings of this study show that the lipid content in the sarcoma samples decreases compared with the carcinoma samples. The achieved accuracy in discriminating carcinoma from sarcoma by linear discriminant analysis is 93.75 % and 90.63 % using the first nine principal components and nonnegative matrix factorization analysis, respectively.

  8. [Sarcoma of the spleen with MDM2 expression].

    PubMed

    Hansen, T; Titze, U; Deeb, A; Eikötter, B; Schütz, M; Schildhaus, H U

    2016-07-01

    Primary sarcomas and sarcoma metastases are a rarity in the spleen. We report on the case of a 69-year-old male patient presenting with unclear abdominal symptoms and computed tomography (CT) revealed a tumor mass in the spleen. Histologically the tumor mass predominantly showed features of a spindle cell sarcoma with lymphoid infiltrates. The expression and amplification of MDM2 could be demonstrated by means of immunohistochemistry and fluorescence in situ hybridization (FISH). Furthermore, staging examinations did not reveal indications of any other primary tumors. These preliminary findings were suggestive of a dedifferentiated liposarcoma; however, in the further diagnostic work-up the tumor showed strong expression of CD21 and CD23 and was ultimately diagnosed as a follicular dendritic cell sarcoma (FDCS). The case emphasizes that MDM2 expression represents a possible pitfall in the diagnosis of spindle cell tumors. The differential diagnostic distinction between FDCS and a dedifferentiated liposarcoma is discussed.

  9. Is time of the essence? Delayed diagnosis of Ewing's sarcoma.

    PubMed

    Edwards, Madeline; Halton, Jacqueline; Ramphal, Raveena; Johnston, Donna

    2015-01-27

    Rarely in modern medicine are we able to observe the natural history of a patient with a sarcoma. This unusual case provides that opportunity. A CT scan was performed on the leg of a 15-year-old boy with a tender soft tissue mass on the lateral aspect of his left calf. Despite showing a lesion consistent with a sarcoma, neither the patient nor his family was informed. Almost a year and a half later, the patient returned and was diagnosed with Ewing's sarcoma. A staging work up showed no metastatic disease. After undergoing chemotherapy and a complete surgical resection of the tumour, the patient remains disease-free 10 years later, indicating that the biology of Ewing's sarcoma may be more important than time to diagnosis in determining outcome.

  10. Molecular diagnostics in soft tissue sarcomas and gastrointestinal stromal tumors.

    PubMed

    Smith, Stephen M; Coleman, Joshua; Bridge, Julia A; Iwenofu, O Hans

    2015-04-01

    Soft tissue sarcomas are rare malignant heterogenous tumors of mesenchymal origin with over fifty subtypes. The use of hematoxylin and eosin stained sections (and immunohistochemistry) in the morphologic assessment of these tumors has been the bane of clinical diagnosis until recently. The last decade has witnessed considerable progress in the understanding and application of molecular techniques in refining the current understanding of soft tissue sarcomas and gastrointestinal stromal tumors beyond the limits of traditional approaches. Indeed, the identification of reciprocal chromosomal translocations and fusion genes in some subsets of sarcomas with potential implications in the pathogenesis, diagnosis and treatment has been revolutionary. The era of molecular targeted therapy presents a platform that continues to drive biomarker discovery and personalized medicine in soft tissue sarcomas and gastrointestinal stromal tumors. In this review, we highlight how the different molecular techniques have enhanced the diagnosis of these tumors with prognostic and therapeutic implications.

  11. Aggressive multiple surgical interventions to pulmonary artery sarcoma.

    PubMed

    Tanaka, Akiko; Shirasaka, Tomonori; Okada, Kenji; Okita, Yutaka

    2015-02-01

    We describe our experience with a patient who had metastasized pulmonary artery sarcoma, but survived 7 years after diagnosis. A 61-year-old man was diagnosed with pulmonary artery intimal sarcoma after resection of metastatic tumours to the bilateral lungs. The primary lesion in the pulmonary artery trunk extending into the bilateral branches was treated by tumour endoarterectomy followed by chemotherapy. He underwent resections of lung metastases two more times before detection of recurrent obstructive pulmonary artery sarcoma 4 years after the tumour endoarterectomy. En bloc resection of the tumour including the pulmonary artery trunk, valve and interventricular septum was performed, and the right ventricular out flow tract was reconstructed with a stentless pulmonary valve and equine pericardium. He died of the disease soon after an operation for metastatic brain tumour 3 years later. Pulmonary artery sarcoma has a dismal prognosis, but aggressively repeated surgical interventions may lengthen survival.

  12. Poorly differentiated synovial sarcoma in the wrist - Case report*

    PubMed Central

    Maia, Daniela Cristina Caetano; Menezes, Carla Kellen da Silva; Bastos, Thales Costa; Ferreira, Luiz Carlos de Lima; Francesconi, Fabio

    2014-01-01

    Synovial sarcomas are rare malignant tumors affecting mainly young adults, presenting as a slow growth mass located in deep soft tissues of extremities, near the joints. In this report a 34-year-old male patient, presented an ulcerovegetative lesion on the right wrist which was completely excised. Histopathology and immunohistochemistry confirmed synovial sarcomas with poorly differentiated cells. This patient presented 11 months later with ipsilateral axillary lymph node metastasis, which emphasizes the unfavorable prognosis of this synovial sarcoma variant. The indolent growth pattern of this sarcoma justifies the well circumscribed initial stages, which progressively infiltrate adjacent structures with lung metastasis (80%) and lymph node involvement (20%) and thus corroborates the importance of early diagnosis and proper treatment. PMID:25184926

  13. Ewing's sarcoma of maxilla: A rare case report

    PubMed Central

    Jairamdas Nagpal, Deepak Kumar; Prabhu, Prashant Ramesh; Palaskar, Sangeeta Jayant; Patil, Swati

    2014-01-01

    Ewing's sarcoma is uncommon malignancy of childhood, frequently involving the mandible. The occurrence in maxilla is rare. It is histopathologically characterized by sheets of round cells positive for CD99. Although the prognosis is poor but early diagnosis and long term follow up can improve the survival. This article presents a rare case of Ewing's sarcoma of maxilla in a 15 year old male patient showing excessive fibro-osseous response which is not a frequent presentation. A retrospective analysis of cases of Ewings sarcoma of maxilla published in the English litreture is reviewed. In our case, diagnosis was confirmed by immunohistochemistry where sheets of round tumor cells were positive for CD 99. Ewings sarcoma of maxilla is a rare and aggressive tumor. Hence early diagnosis, combined therapy and long term follow up is suggested in such cases. PMID:25328307

  14. Epiphyseal ewing sarcoma: first reported case with molecular confirmation.

    PubMed

    Pérez-González, Yosmar; García-Esparza, Elena; Conde, Esther; Azorín, Daniel

    2013-04-01

    Ewing sarcoma is the second most common pediatric malignant bone neoplasm after osteosarcoma. Ewing sarcoma comprises "small, round, blue-cell" tumors thought to arise from neural crest cells. The authors report the case of a 14-year-old boy that presented with a nonpainful circumscribed lesion. The radiographs showed a lytic lesion at the tibial epiphysis with a large soft tissue mass, best depicted in the magnetic resonance imaging scan that suggested an aggressive lesion. A needle biopsy of the lesion was performed. The diagnosis of Ewing sarcoma was made based on microscopic, immunohistochemical, polymerase chain reaction, and fluorescence in situ hybridization. This is the third case report about a primary epiphyseal Ewing sarcoma and the fist one with molecular confirmation.

  15. 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

  16. Clinical exuberance of classic Kaposi's sarcoma and response to radiotherapy.

    PubMed

    Trujillo, Jeniffer Muñoz; Alves, Natália Ribeiro de Magalhães; Medeiros, Paula Mota; Azulay-Abulafia, Luna; Alves, Maria de Fátima Guimarães Scotelaro; Gripp, Alexandre Carlos

    2015-01-01

    Kaposi's sarcoma (KS) is a multicentric vascular neoplasm, with cutaneous and extracutaneous involvement. Different clinical and epidemiological variants have been identified. The classic form is manifested mainly in elderly men with indolent and long-term evolution, with lesions localized primarily in the lower extremities. We present two cases of classic Kaposi's sarcoma (CKS) in two female patients with extensive, exuberant skin involvement and rapid evolution, with good response to radiotherapy.

  17. Pulmonary artery sarcoma mimicking massive pulmonary embolus: a case report.

    PubMed

    Alsoufi, Bahaaldin; Slater, Matthew; Smith, Pamela P; Karamlou, Tara; Mansoor, Atiya; Ravichandran, Pasala

    2006-08-01

    Intimal sarcomas of the pulmonary artery are rare tumors that are often difficult to distinguish from pulmonary thromboembolic disease, complicating accurate diagnosis and timely therapy. We report the case of a gentleman with a primary pulmonary artery sarcoma who presented with a massive pulmonary embolism and complete right ventricular outflow tract obstruction. The patient's condition was successfully managed with urgent pulmonary artery thromboendarterectomy, pulmonary valve replacement, and tricuspid valve annuloplasty.

  18. Postirradiation sarcoma (malignant fibrous histiocytoma) following cervix cancer

    SciTech Connect

    Pinkston, J.A.; Sekine, I.

    1982-02-01

    A case of postirradiation sarcoma is described. The tumor, a malignant fibrous histiocytoma, occurred in the radiation field 11 years following postoperative external beam radiation therapy (7000 rad) for carcinoma of the cervix. Reports of postirradiation malignant fibrous histiocytoma are rare, and the occurrence of this neoplasm following treatment of cervix cancer has not previously been described. The literature concerning postirradiation bone and soft tissue sarcomas is briefly reviewed, with special attention to malignant fibrous histiocytomas.

  19. 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.

  20. Localized Ewing sarcoma of the tibia

    PubMed Central

    2013-01-01

    Ewing sarcoma (ES) is a high-grade malignant primary round cell tumour of bone in which there is commonly extension into extraosseous soft tissues at the time of diagnosis. This report details the clinical, radiological and pathological features of a case of ES of the tibia in which there was extensive osseous involvement but no infiltration beyond the periosteum into surrounding soft tissue. We also record the findings of one other ES case that exhibited similar behaviour. Both cases were male, involved the tibia and had the characteristic t (11;22) (q24;q12) translocation. No recurrence of tumour or metastasis has been seen in these two cases, both of which have had 6 years follow-up. Our findings indicate that there is heterogeneity in the behaviour of ES and show that localized ES is associated with a good prognosis. PMID:23379545

  1. Kaposi's sarcoma: DOX-SL approval recommended.

    PubMed

    1995-02-17

    The Food and Drug Administration (FDA) drug approval committee has recommended that DOX-SL be given accelerated approval for treatment of AIDS-related Kaposi's sarcoma (KS) in patients who have failed conventional treatment. This decision, coming after several votes against the drug's approval, brings up an ongoing problem with the current drug-approval system, i.e., the advisory committee for drug approvals has developed its own "corporate culture" for judging approval applications that can be disadvantageous to the smaller biotechnology company less familiar with the system. In order to prevent mistakes that may stop approval of a necessary drug, it may be necessary for the FDA to develop a body of knowledge, based on past experience with its committee system, to help committee members avoid certain perennial mistakes in drug approval.

  2. [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.

  3. Potential for immunotherapy in soft tissue sarcoma

    PubMed Central

    Tseng, William W; Somaiah, Neeta; Engleman, Edgar G

    2015-01-01

    Soft tissue sarcomas (STS) are rare, heterogeneous tumors of mesenchymal origin. Despite optimal treatment, a large proportion of patients will develop recurrent and metastatic disease. For these patients, current treatment options are quite limited. Significant progress has been made recently in the use of immunotherapy for the treatment of other solid tumors (e.g. prostate cancer, melanoma). There is a strong rationale for immunotherapy in STS, based on an understanding of disease biology. For example, STS frequently have chromosomal translocations which result in unique fusion proteins and specific subtypes have been shown to express cancer testis antigens. In this review, we discuss the current status of immunotherapy in STS, including data from human studies with cancer vaccines, adoptive cell therapy, and immune checkpoint blockade. Further research into STS immunology is needed to help design logical, subtype-specific immunotherapeutic strategies. PMID:25625925

  4. Intimal sarcoma of the descending aorta.

    PubMed

    Shirani, Shapour; Soleymanzadeh-Ardabili, Maryam; Arami, Mitra

    2007-04-01

    Primary intimal angiosarcoma of the aorta (i.e., mostly intraluminal sarcomas with evidence of endothelial differentiation) is extraordinarily rare. We report a case in which the diagnosis was accurately made using immunohistochemistry in an embolectomy specimen. The patient was a 78-year-old man with a two-month history of bilateral claudication. Doppler ultrasound proved an embolus in both popliteal arteries, which was removed. The highly atypical cells comprising these emboli were positive immunohistochemically for CD68, vimentin, and CD31. Magnetic resonance imaging also showed an irregular tumor (invasion to the left main bronchus). This case emphasizes the need for a wide panel of immunohistochemical studies in tumor emboli of unknown origin.

  5. Cytogenetics Findings in a Histiocytic Sarcoma Case

    PubMed Central

    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

  6. Primary Kaposi sarcoma of the subcutaneous tissue

    PubMed Central

    Pantanowitz, Liron; Mullen, John; Dezube, Bruce J

    2008-01-01

    Background Involvement of the subcutis by Kaposi sarcoma (KS) occurs primarily when cutaneous KS lesions evolve into deep penetrating nodular tumors. Primary KS of the subcutaneous tissue is an exceptional manifestation of this low-grade vascular neoplasm. Case presentation We present a unique case of acquired immune deficiency syndrome (AIDS)-associated KS manifesting primarily in the subcutaneous tissue of the anterior thigh in a 43-year-old male, which occurred without overlying visible skin changes or concomitant KS disease elsewhere. Radiological imaging and tissue biopsy confirmed the diagnosis of KS. Conclusion This is the first documented case of primary subcutaneous KS occurring in the setting of AIDS. The differential diagnosis of an isolated subcutaneous lesion in an human immunodeficiency virus (HIV)-infected individual is broad, and requires both imaging and a histopathological diagnosis to guide appropriate therapy. PMID:18764944

  7. Myxoinflammatory fibroblastic sarcoma of the face.

    PubMed

    Gómez Martín, Cristina; Ortega, María I; Aramburu, José A; Fernández-Cañamaque, José L

    2012-08-01

    Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare low-grade tumor of modified fibroblasts, with tendency to local recurrence. This unusual entity typically presents as a slow-growing painless mass in the distal extremities of middle-aged subjects. A 48-year-old woman presented to our clinic with a painless subcutaneous mass in the right temporal region. Excisional biopsy made the rare diagnosis of MIFS. Histologic examination showed the unique features that characterize this lesion: a myxoid component with a superimposed inflammatory infiltrate and the presence of distinctive, large, and bizarre Reed-Stemberg-like cells. A second wide tumor bed resection was performed, achieving clear margins. No adjuvant therapy was administered, and the patient is free of disease at 18 months postoperatively. To the best of our knowledge, this is the first reported case of MIFS presenting in the face. This adds another possibility for differential diagnoses of soft tissue tumors of the face.

  8. 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.

  9. Testicular Synovial Sarcoma: A Case Report

    PubMed Central

    Nesrine, Mejri; Sellami, Rym; Doghri, Raoudha; Rifi, Hela; Raies, Henda; Mezlini, Amel

    2012-01-01

    This paper reports a case of testicular synovial sarcoma with molecular genetic analysis. A 24-year-old male presented with painless scrotal mass. Ultrasonography showed a heterogeneous mass of 66 mm × 34 mm in size involving the inguinal region. Histological examination of a surgical biopsy showed a grade III monophasic growth pattern of spindle cell proliferation. Immunohistochemical analyses indicated positive staining for pancytokeratine and epithelial membrane antigen. Cytogenetic analysis showed the presence of CYT-SSX1 mutation, and CT scan showed non-specific pleural micro-nodules with a size of 7.5 mm. The patient had an extended left orchidectomy but was lost to follow-up for 1 year. A local recurrent scrotal mass of 32 mm × 25 mm, multiple inguinal lymph nodes, and increased pleural nodules, which were confirmed by histological examination, were treated with three cycles of adriamycine and ifosfamide chemotherapy, surgical resection, and radiotherapy with complete response. After 3 months, the patient developed local recurrence and pulmonary metastases that did not respond to second-line chemotherapy based on gemcitabine and paclitaxel. The patient had dyspnea at the time of this writing and chest pain, and is under third-line chemotherapy based on Deticene after 30 months of following up. This patient died on November 16, 2012 after a resperatory failure and malignant pelural effusion. Synovial sarcoma should be considered in the differential diagnosis of soft tissue tumor and it should be aggressively treated to improve prognosis. Although our patient has shown numerous factors of bad prognosis, he has had a relatively long survival time. PMID:23691490

  10. Testicular synovial sarcoma: a case report.

    PubMed

    Nesrine, Mejri; Sellami, Rym; Doghri, Raoudha; Rifi, Hela; Raies, Henda; Mezlini, Amel

    2012-12-01

    This paper reports a case of testicular synovial sarcoma with molecular genetic analysis. A 24-year-old male presented with painless scrotal mass. Ultrasonography showed a heterogeneous mass of 66 mm × 34 mm in size involving the inguinal region. Histological examination of a surgical biopsy showed a grade III monophasic growth pattern of spindle cell proliferation. Immunohistochemical analyses indicated positive staining for pancytokeratine and epithelial membrane antigen. Cytogenetic analysis showed the presence of CYT-SSX1 mutation, and CT scan showed non-specific pleural micro-nodules with a size of 7.5 mm. The patient had an extended left orchidectomy but was lost to follow-up for 1 year. A local recurrent scrotal mass of 32 mm × 25 mm, multiple inguinal lymph nodes, and increased pleural nodules, which were confirmed by histological examination, were treated with three cycles of adriamycine and ifosfamide chemotherapy, surgical resection, and radiotherapy with complete response. After 3 months, the patient developed local recurrence and pulmonary metastases that did not respond to second-line chemotherapy based on gemcitabine and paclitaxel. The patient had dyspnea at the time of this writing and chest pain, and is under third-line chemotherapy based on Deticene after 30 months of following up. This patient died on November 16, 2012 after a resperatory failure and malignant pelural effusion. Synovial sarcoma should be considered in the differential diagnosis of soft tissue tumor and it should be aggressively treated to improve prognosis. Although our patient has shown numerous factors of bad prognosis, he has had a relatively long survival time.

  11. 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

  12. 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.

  13. Granulocytic sarcoma of pediatric head and neck: an institutional experience.

    PubMed

    Roby, Brianne Barnett; Drehner, Dennis; Sidman, James D

    2013-08-01

    To demonstrate a case series of granulocytic sarcoma of the head and neck found in the pediatric population and review long-term outcomes. A pathology database at a tertiary hospital was searched for patients with biopsy specimens from the head and neck diagnosed as granulocytic sarcoma. There were 6 cases between 1992 and 2004 that met inclusion criteria. Subjects' age ranged from 22 months to 14 years old. In three cases, the patients were diagnosed with acute myeloid leukemia (AML) based on biopsy results; 2 patients were already diagnosed with AML when diagnosed with granulocytic sarcoma, and in 1 case, a relapse of AML was diagnosed. In all cases, patients began induction chemotherapy. Two patients died during induction chemotherapy from infection. The remaining 4 patients underwent bone marrow transplants. One patient had a relapse post-transplant and died. Only one patient was healthy two years post-transplant. The results of this series suggest granulocytic sarcoma must be on the differential when tumors present in the head and neck region in pediatric patients. In our series, 100% of the patients with granulocytic sarcoma had underlying AML. The long-term prognosis of patients with AML who developed granulocytic sarcoma is quite poor. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  14. Primary breast sarcoma: prevalence, clinical signs, and radiological features.

    PubMed

    Surov, Alexey; Holzhausen, Hans-Jürgen; Ruschke, Kathrin; Spielmann, Rolf Peter

    2011-07-01

    Primary breast sarcoma is very rare. Most reports regarding sarcoma of the breast are clinical observations or pathological series and provide either no or inconstant radiological information. Radiological publications consist predominantly of isolated case reports or small series. To determine the prevalence, clinical signs, and radiological features of primary breast sarcoma. This is a retrospective review of 21 patients with breast sarcoma. All patients were female and their median age was 66 years (range 27-86). In all patients the diagnosis was confirmed histopathologically. The prevalence of breast sarcoma was 0.1% of all identified cases with breast malignancies. Clinically, all patients presented with solitary painless breast lumps. There was no uni- or bilateral axillary lymphadenopathy. On mammography (n = 19), two mammographic patterns could be identified: breast masses (68%), and architectural distortion (32%). On ultrasound (n = 8), most lesions were homogeneously hypoechoic, lobular or oval in shape with microlobulated or indistinct margins. On magnetic resonance imaging (n = 3), marked inhomogeneous contrast enhancement was seen in all investigated cases. The imaging findings of primary breast sarcoma are not pathognomonic. However, they should be taken into consideration in the differential diagnosis of breast lesions.

  15. Treatment of early uterine sarcomas: disentangling adjuvant modalities

    PubMed Central

    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. PMID:19356236

  16. Somatic mutations in histiocytic sarcoma identified by next generation sequencing.

    PubMed

    Liu, Qingqing; Tomaszewicz, Keith; Hutchinson, Lloyd; Hornick, Jason L; Woda, Bruce; Yu, Hongbo

    2016-08-01

    Histiocytic sarcoma is a rare malignant neoplasm of presumed hematopoietic origin showing morphologic and immunophenotypic evidence of histiocytic differentiation. Somatic mutation importance in the pathogenesis or disease progression of histiocytic sarcoma was largely unknown. To identify somatic mutations in histiocytic sarcoma, we studied 5 histiocytic sarcomas [3 female and 2 male patients; mean age 54.8 (20-72), anatomic sites include lymph node, uterus, and pleura] and matched normal tissues from each patient as germ line controls. Somatic mutations in 50 "Hotspot" oncogenes and tumor suppressor genes were examined using next generation sequencing. Three (out of five) histiocytic sarcoma cases carried somatic mutations in BRAF. Among them, G464V [variant frequency (VF) of 43.6 %] and G466R (VF of 29.6 %) located at the P loop potentially interfere with the hydrophobic interaction between P and activating loops and ultimately activation of BRAF. Also detected was BRAF somatic mutation N581S (VF of 7.4 %), which was located at the catalytic loop of BRAF kinase domain: its role in modifying kinase activity was unclear. A similar mutational analysis was also performed on nine acute monocytic/monoblastic leukemia cases, which did not identify any BRAF somatic mutations. Our study detected several BRAF mutations in histiocytic sarcomas, which may be important in understanding the tumorigenesis of this rare neoplasm and providing mechanisms for potential therapeutical opportunities.

  17. [Interventional Bronchoscopy for the Treatment of Pulmonary Sarcoma].

    PubMed

    Wang, Hongwu; Zhang, Nan; Li, Dongmei; Zou, Hang; Zhang, Jieli; Zhou, Yunzhi; Bai, Xiuyun

    2016-09-20

    Pulmonary sarcoma is a rare malignant tumor in soft tissues. Resection is the preferred option to treat this tumor. The aim of this study is to explore the effect of interventional bronchoscopies in the treatment of pulmonary sarcoma if the patient is inoperable. Sixteen cases with pulmonary sarcoma were retrospectively reviewed in our hospital from November 2008 to July 2014. The mean age was (53.1±5.4) years old. Rigid bronchoscopy was applied for the first procedure with general anesthesia, and electronic bronchoscopy was used for the second procedure or slight patients. Sixteen cases, which include 10 sarcomatoid carcinoma, 2 fibrosarcoma, 2 sarcoma, 1 fibromucoid sarcoma, and 1 spindle cell synovial sarcoma, were collected in this study. Eleven cases (68.8%) were peripheral and mainly located in the right upper lobe and left lower lobe. Five cases (31.2%) were central. Of these cases, 82% (9/11) were mixed and primary tumors in pulmonary tumor. Meanwhile, 56% (9/16) were intraluminal and 69% (11/16) were metastatic in central airway. All of the four cases with whole atelectasis were completely relieved through postbronchoscopic interventions. Three of the seven cases with segment atelectasis were completely reopened; two of them were partially relieved; and the remaining two had no response. The obstructive degree, Karnofsky performance status (KPS), and shortness of breathless score improved significantly after the treatment. Interventional bronchoscopy could rapidly and efficiently remove endobronchial tumor, relieve airway obstruction, and improve clinical symptoms.

  18. 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.

  19. RUNX3 facilitates growth of Ewing sarcoma cells.

    PubMed

    Bledsoe, Krista L; McGee-Lawrence, Meghan E; Camilleri, Emily T; Wang, Xiaoke; Riester, Scott M; van Wijnen, Andre J; Oliveira, Andre M; Westendorf, Jennifer J

    2014-12-01

    Ewing sarcoma is an aggressive pediatric small round cell tumor that predominantly occurs in bone. Approximately 85% of Ewing sarcomas harbor the EWS/FLI fusion protein, which arises from a chromosomal translocation, t(11:22)(q24:q12). EWS/FLI interacts with numerous lineage-essential transcription factors to maintain mesenchymal progenitors in an undifferentiated state. We previously showed that EWS/FLI binds the osteogenic transcription factor RUNX2 and prevents osteoblast differentiation. In this study, we investigated the role of another Runt-domain protein, RUNX3, in Ewing sarcoma. RUNX3 participates in mesenchymal-derived bone formation and is a context dependent tumor suppressor and oncogene. RUNX3 was detected in all Ewing sarcoma cells examined, whereas RUNX2 was detected in only 73% of specimens. Like RUNX2, RUNX3 binds to EWS/FLI via its Runt domain. EWS/FLI prevented RUNX3 from activating the transcription of a RUNX-responsive reporter, p6OSE2. Stable suppression of RUNX3 expression in the Ewing sarcoma cell line A673 delayed colony growth in anchorage independent soft agar assays and reversed expression of EWS/FLI-responsive genes. These results demonstrate an important role for RUNX3 in Ewing sarcoma.

  20. Primary pulmonary sarcoma: a clinicopathologic study of 26 cases.

    PubMed

    Keel, S B; Bacha, E; Mark, E J; Nielsen, G P; Rosenberg, A E

    1999-12-01

    The clinical and pathologic features of 26 primary pulmonary sarcomas were analyzed. Fourteen patients were male and 12 were female; ranging in age from 18 to 75 years (mean, 48 yr). The tumors measured from 0.9 cm in greatest diameter to filling the entire hemithorax. Thirteen tumors were in the left lung and nine in the right lung; one was bilateral, two were in the pulmonary artery, and the location of one tumor was not available. The histologic diagnoses were malignant fibrous histiocytoma (7), synovial sarcoma (6), malignant peripheral-nerve sheath tumor (3), leiomyosarcoma (3), angiosarcoma (2), intimal sarcoma (2), fibrosarcoma (2), and one case of epithelioid hemangioendothelioma. Immunohistochemical and ultrastructural examination supported these diagnoses. Morphologically, the differential diagnosis often included sarcomatoid carcinoma or desmoplastic malignant mesothelioma Patients were treated with surgery, chemotherapy, radiation therapy, or a combination of these. Follow-up was available for 22 patients and ranged from 2 to 183 months (mean, 45 mo). Fourteen patients are free of disease, four died of disease, three are alive with disease, and one died of surgical complications. A variety of sarcomas, especially malignant fibrous histiocytoma and synovial sarcoma, arise within the pulmonary parenchyma. These tumors have the potential to behave aggressively but can be cured by resection, with or without adjuvant therapy. Immunohistochemistry and electron microscopy can be helpful in distinguishing primary pulmonary sarcoma from other tumors in the differential diagnosis.

  1. Kaposi sarcoma herpesvirus-associated cancers and related diseases.

    PubMed

    Goncalves, Priscila H; Ziegelbauer, Joseph; Uldrick, Thomas S; Yarchoan, Robert

    2017-01-01

    This review discusses the pathogenesis and recent advances in the management of Kaposi sarcoma herpesvirus (KSHV)-associated diseases. KSHV, a gammaherpesvirus, causes several tumors and related diseases, including Kaposi sarcoma, a form of multicentric Castleman disease (KSHV-MCD), and primary effusion lymphoma. These most often develop in patients infected with human immunodeficiency virus (HIV). KSHV inflammatory cytokine syndrome (KICS) is a newly described syndrome with high mortality that has inflammatory symptoms-like MCD but not the pathologic lymph node findings. KSHV-associated diseases are often associated with dysregulated human interleukin-6, and KSHV encodes a viral interleukin-6, both of which contribute to disease pathogenesis. Treatment of HIV is important in HIV-infected patients. Strategies to prevent KSHV infection may reduce the incidence of these tumors. Pomalidomide, an immunomodulatory agent, has activity in Kaposi sarcoma. Rituximab is active in KSHV-MCD but can cause Kaposi sarcoma exacerbation; rituximab plus liposomal doxorubicin is useful to treat KSHV-MCD patients with concurrent Kaposi sarcoma. KSHV is the etiological agents of all forms of Kaposi sarcoma and several other diseases. Strategies employing immunomodulatory agents, cytokine inhibition, and targeting of KSHV-infected cells are areas of active research.

  2. 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

  3. [Soft tissue sarcoma in children and adolescents: experiences of the cooperative Soft Tissue Sarcoma Group Studies (CWS-81 - 96)].

    PubMed

    Brecht, I B; Treuner, J

    2004-10-01

    The very heterogeneous group of paediatric soft tissue sarcomas account for approximately 7 % of all malignant childhood tumours. More than one half of all cases are rhabdomyosarcomas, some of the over 20 entities are very rare. The prognosis and biology of soft tissue sarcomas in children and adolescents vary greatly depending on histological subtype, the age of the patient, the primary site, the tumour size, tumour invasiveness and the extent of disease at diagnosis. Since 1981, 2918 children and adolescents with soft tissue sarcomas were treated prospectively according to the common treatment protocols of the Cooperative Soft Tissue Sarcoma Study Group (CWS-81 - 96). The known prognostic factors were used to develop a more and more detailed risk stratification. The multimodal treatment includes the use of surgery, chemotherapy and radiotherapy and should be planned by a multidisciplinary team. That way, an overall survival of nearly 70 % over all risk groups could be achieved.

  4. Genetics of rare mesenchymal tumors: implications for targeted treatment in DFSP, ASPS, CCS, GCTB and PEComa.

    PubMed

    Rutkowski, Piotr; Przybył, Joanna; Świtaj, Tomasz

    2014-08-01

    Soft tissue and bone sarcomas comprise a heterogeneous group of mesenchymal tumors that include roughly 130 distinct diagnostic entities. Many of them are exceptionally rare, with only few cases diagnosed worldwide each year. Development of novel targeted treatment in this group of tumors is of special importance since many sarcoma subtypes are resistant to conventional chemotherapy and the effective therapeutic options are limited. In this review we aim to discuss the molecular implications for targeted therapy in selected rare soft tissue and bone sarcoma subtypes, including dermatofibrosarcoma protuberans (DFSP), alveolar soft part sarcoma (ASPS), clear cell sarcoma (CCS), giant cell tumor of bone (GCTB) and perivascular epithelioid cell neoplasms (PEComas). This article is part of a Directed Issue entitled: Rare cancers. Copyright © 2014 Elsevier Ltd. All rights reserved.

  5. Diagnostic systems for assessing alveolar bone loss.

    PubMed

    Ivanusa, T; Babic, A; Petelin, M

    1997-01-01

    Radiological diagnostics serves as a basic monitoring technique for alveolar bone loss which is a severe consequence of periodontal disease. To evaluate efficacy of Conventional Visual Radiography (CVR), and to assess a complete clinical status, we had used two more diagnostic systems. These are Digital Subtraction Radiography (DSR) and Probing Pocket Depth (PPD). Experimental Periodontitis was studied in 20 beagle dogs based on the measurements taken in the beginning (baseline), and before (11th month) and after the medical treatment (12th month). Data analyses pointed out the same clinical trend, i.e. a significant bone loss prior to medical treatment and its recovery to the initial state. Differences in metrics and measurement errors could be identified as causes for discrepancies between the systems, but a relationship between the CVR and PPD is worth of further research, as these systems do not appear to be entirely compatible, but rather complementary to each other.

  6. [Clinical aspect of diffuse alveolar hemorrhage syndrome].

    PubMed

    Ishida, Kazuo; Seki, Reiko; Inoue, Takeo; Iwamoto, Tokuzen; Hoshino, Makoto; Nakagawa, Takemasa

    2003-12-01

    Thirteen cases of diffuse alveolar hemorrhage (DAH) were encountered in our Hospital between January 1996 and October 2001. Eight patients were men and five were women, their mean age being 59.5 +/- 19.2 years (range, 18-88 years). Three patients had systemic lupus erythematosus (SLE), three (23%) had polyarteritis nodosa (including microscopic PN), one (7.7%) had allergic granulomatous angitis, one (7.7%) had Goodpasture syndrome, one (7.7%) had MPO-ANCA-associated vasculitis, one (7.7%) had Behçet's disease, one (7.7%) had chronic heart failure caused by mitral stenosis, one (7.7%) had chronic renal failure (etiology unknown), and the last had no particular disorder. Nine episodes (69%) had occurred as complications of primary diseases, four (31%) as the first symptoms of underlying diseases. Prognosis was poor in the former cases but in the latter, the prognosis was relatively favorable.

  7. Vestibuloplasty after secondary alveolar bone grafting.

    PubMed

    Iino, M; Fukuda, M; Murakami, K; Horiuchi, T; Niitsu, K; Seto, K

    2001-11-01

    This paper introduces a surgical technique for vestibuloplasty after secondary alveolar bone grafting of patients with cleft lip and palate (CLP). This paper also reports on the patients who underwent this modified vestibuloplasty. The vestibuloplasty technique described in this paper consists of: (1) reduction of submucosal scar tissue of the upper lip, (2) V-Y plasty of the superficial mucosa, (3) placement of horizontal mattress sutures between nostril floor skin and freed marginal mucosa, (4) application of artificial skin to cover the exposed periosteal surface, and (5) use of a removable retention splint. This surgical procedure appears to be very useful for patients with CLP. The technique enables the surgeon to obtain an adequate sulcus depth around the graft area. In addition, this technique releases the mucosal scar contraction and improves the shape and mobility of the upper lip.

  8. 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.

  9. Oncological outcomes of patients with Ewing's sarcoma: is there a difference between skeletal and extra-skeletal Ewing's sarcoma?

    PubMed

    Pradhan, A; Grimer, R J; Spooner, D; Peake, D; Carter, S R; Tillman, R M; Abudu, A; Jeys, L

    2011-04-01

    The aim of this study was to identify whether there was any difference in patient, tumour, treatment or outcome characteristics between patients with skeletal or extra-skeletal Ewing's sarcoma. We identified 300 patients with new primary Ewing's sarcoma diagnosed between 1980 and 2005 from the centres' local database. There were 253 (84%) with skeletal and 47 (16%) with extra-skeletal Ewing's sarcomas. Although patients with skeletal Ewing's were younger (mean age 16.8 years) than those with extra-skeletal Ewing's sarcoma (mean age 27.5 years), there was little difference between the groups in terms of tumour stage or treatment. Nearly all the patients were treated with chemotherapy and most had surgery. There was no difference in the overall survival of patients with skeletal (64%) and extra-skeletal Ewing's sarcoma (61%) (p = 0.85), and this was also the case when both groups were split by whether they had metastases or not. This large series has shown that the oncological outcomes of Ewing's sarcoma are related to tumour characteristics and patient age, and not determined by whether they arise in bone or soft tissue.

  10. Improved Prognosis for Patients with Ewing Sarcoma in the Sacrum Compared with the Innominate Bones: The Scandinavian Sarcoma Group Experience.

    PubMed

    Hesla, Asle Charles; Tsagozis, Panagiotis; Jebsen, Nina; Zaikova, Olga; Bauer, Henrik; Brosjö, Otte

    2016-02-03

    Treatment of Ewing sarcoma of the pelvic bones remains one of the most difficult tasks in the treatment of bone sarcomas. Whether surgery or radiation therapy is the best local treatment is still a matter of debate. The aim of the present study was to compare sacral and nonsacral sites with regard to the treatment and outcome of pelvic Ewing sarcomas. Patients with Ewing sarcoma of the osseous pelvis diagnosed between 1986 and 2011 were identified through the Scandinavian Sarcoma Group registry. Data regarding tumor size, local treatment (surgery or radiation therapy), metastatic disease, surgical margins, local recurrence, and overall survival were analyzed. Of the 117 patients examined, eighty-eight had tumors in the innominate bones and twenty-nine, in the sacrum. Radiation therapy was the sole local treatment for 40% of the innominate bone tumors in contrast to 79% of the sacral tumors. The five-year disease-free survival rate in the latter group (66%) was greater than that in the group with tumors in the innominate bones (40%) (p = 0.02 adjusted for size). Disease-free survival among patients with Ewing sarcoma was improved when the tumor was localized in the sacrum compared with the innominate bones, where these tumors are generally larger. Local radiation therapy alone appears to result in good local tumor control and may be the treatment of choice for sacral tumors. Copyright © 2016 by The Journal of Bone and Joint Surgery, Incorporated.

  11. Arterial hypertension perpetuates alveolar bone loss.

    PubMed

    de Medeiros Vanderlei, Janine Montenegro Toscano Moura; Messora, Michel Reis; Fernandes, Patrícia Garani; Novaes, Arthur B; Palioto, Daniela Bazan; de Moraes Grisi, Marcio Fernando; Scombatti de Souza, Sergio Luis; Gerlach, Raquel Fernanda; Antoniali, Cristina; Taba, Mario

    2013-01-01

    Few studies have focused on the impact of hypertension on the progression of periodontitis (PD). The purpose of this study was to evaluate whether hypertension affects PD by enhancing bone loss even after the stimulus for PD induction is removed. Ligature-induced PD was created on the first mandibular molars of spontaneously hypertensive rats (SHR) and normotensive rats (Wistar Kyoto-WKY). The animals were assigned to non-ligated controls (C) and PD groups: WKY-C, WKY-PD, SHR-C, and SHR-PD. After 10 days, five animals of each group were killed and the ligatures of the other animals were removed. On the 21st day (11 days without PD induced), the remaining animals were killed. The jaws were defleshed and the amount of bone loss was measured. After 10 days, the PD groups showed more bone loss than its controls (P < .05); SHR-PD = 0.72 ± 0.05 mm, SHR-C = 0.39 ± 0.04 mm, WKY-PD = 0.75 ± 0.04 mm, and WKY-C = 0.56 ± 0.04 mm. The cumulative bone loss on day 21 (0.94 ± 0.13 mm) was significantly worse than on day 10 only in SHR-PD group (P < .05). The final bone loss differences between PD and C groups accounted for 102% (SHR) and 26% (WKY) increase in comparison with the initial control levels. Hypertension is associated with progressive alveolar bone loss even when the stimulus for PD induction is removed and it may be speculated that host condition perpetuates alveolar bone loss.

  12. [Clinicopathological diagnosis of diffuse alveolar hemorrhage].

    PubMed

    Fang, F; Li, Y M; Hu, S T; Wang, H T; Liu, D G; Wang, C

    2016-01-12

    To improve knowledge about the clinical and pathological features of diffuse alveolar hemorrhage (DAH). Six cases DAH with intact clinical and pathological data were retrospectively analyzed during the period from May 1999 to May 2015 in Beijing Hospital. There were altogether 2 males and 4 females, with age ranging from 32 to 68 years (mean 58.8 years). Specimens were obtained by autopsy (3 cases), open lung biopsy (2 cases) and renal biopsy (2 cases), including 1 case of open lung biopsy in 2003, renal biopsy in 2012. Clinically, the patients presented with cough, shortness of breath and dyspnea, including 5 cases of hemoptysis, 4 cases of fever, 3 cases of skin and mucosa bleeding, 2 cases of gross hematuria, 2 cases of microscopic hematuria, 3 cases of renal functional impairment. A total of 5 cases had different levels of elevated erythrocyte sedimentation rate and C-reactive protein, 6 cases had moderate anemia, hypoxemia, diffuse infiltrates with alveolar filling in chest CT. Serum antineutrophil cytoplasmic antibody was positive in 3 cases, anti-glomerular basement membrane antibody was present in 1 case. Pathological diagnosis: 2 cases of Granulomatosis with polyangiitis (GPA), 2 cases of Microscopic polyangiitis (MPA), 1 case of Goodpasture syndrome, 1 case of pulmonary veno-occlusive disease (PVOD). 3 cases died; 2 cases were discharged; 1 case received symptomatic treatment, follow-up after discharge. The mainly clinical characteristics of DAH are varied degree of dyspnea, anemia, hypoxemia, and extensive ground-glass opacification or consolidation in image, with or without haemoptysis; diffuse acute or chronic pulmonary hemorrhage in lung tissue is the main pathological feature.

  13. Immunogenicity of surfactant. I. Human alveolar surfactant.

    PubMed Central

    Strayer, D S; Hallman, M; Merritt, T A

    1991-01-01

    The immunogenicity of lung surfactant derived from amniotic fluid has been well established. We have set out to examine the antigenic similarity of human surfactant to non-human alveolar surfactants currently being used therapeutically in clinical trials with neonatal respiratory distress syndrome. To this end, we raised a series of eight monoclonal antibodies in rats directed to human surfactant (H1 to H8). All antibodies bound human surfactant as measured by ELISA. Four of these monoclonal antibodies bound surfactant components by Western blot analysis: all bound a 9-10-kD species. In addition, one antibody (H2) bound a protein of 16 kD, one (H8) a 6-kD protein, and one (H6) a 30-kD protein. When mixed with surfactant, three antibodies, H4, H7 and H8, profoundly altered surfactant activity in vitro in the pulsating bubble surfactometer. Three other antibodies, H1, H2, and H5 moderately inhibited surfactant's surface activity. We also examined the cross-reactivity of these monoclonal antibodies with bovine (CLSE) and porcine (Curosurf) surfactants. By Western blot analysis, only H6 bound these heterologous surfactants. Other antibodies did so by ELISA. However, functional assays indicated that antibodies H7, H8 and H4 all greatly inhibited CLSE surface activity in vitro. Five antibodies (H1-H4 and H8) inhibited Curosurf function. Thus, human surfactant species, especially low molecular weight species, are highly antigenic. Antibodies to alveolar surfactants may inhibit surfactant function in vitro. As indicated by Western blot and cross-inhibition data, human lower molecular weight surfactants share epitopes with proteins from therapeutically important porcine and bovine surfactants. The potential importance of these findings to treatment of neonatal respiratory distress syndrome with heterologous surfactants is discussed. PMID:1988229

  14. Bacillus anthracis lethal toxin reduces human alveolar epithelial barrier function.

    PubMed

    Langer, Marybeth; Duggan, Elizabeth Stewart; Booth, John Leland; Patel, Vineet Indrajit; Zander, Ryan A; Silasi-Mansat, Robert; Ramani, Vijay; Veres, Tibor Zoltan; Prenzler, Frauke; Sewald, Katherina; Williams, Daniel M; Coggeshall, Kenneth Mark; Awasthi, Shanjana; Lupu, Florea; Burian, Dennis; Ballard, Jimmy Dale; Braun, Armin; Metcalf, Jordan Patrick

    2012-12-01

    The lung is the site of entry for Bacillus anthracis in inhalation anthrax, the deadliest form of the disease. Bacillus anthracis produces virulence toxins required for disease. Alveolar macrophages were considered the primary target of the Bacillus anthracis virulence factor lethal toxin because lethal toxin inhibits mouse macrophages through cleavage of MEK signaling pathway components, but we have reported that human alveolar macrophages are not a target of lethal toxin. Our current results suggest that, unlike human alveolar macrophages, the cells lining the respiratory units of the lung, alveolar epithelial cells, are a target of lethal toxin in humans. Alveolar epithelial cells expressed lethal toxin receptor protein, bound the protective antigen component of lethal toxin, and were subject to lethal-toxin-induced cleavage of multiple MEKs. These findings suggest that human alveolar epithelial cells are a target of Bacillus anthracis lethal toxin. Further, no reduction in alveolar epithelial cell viability was observed, but lethal toxin caused actin rearrangement and impaired desmosome formation, consistent with impaired barrier function as well as reduced surfactant production. Therefore, by compromising epithelial barrier function, lethal toxin may play a role in the pathogenesis of inhalation anthrax by facilitating the dissemination of Bacillus anthracis from the lung in early disease and promoting edema in late stages of the illness.

  15. Bacillus anthracis Lethal Toxin Reduces Human Alveolar Epithelial Barrier Function

    PubMed Central

    Langer, Marybeth; Duggan, Elizabeth Stewart; Booth, John Leland; Patel, Vineet Indrajit; Zander, Ryan A.; Silasi-Mansat, Robert; Ramani, Vijay; Veres, Tibor Zoltan; Prenzler, Frauke; Sewald, Katherina; Williams, Daniel M.; Coggeshall, Kenneth Mark; Awasthi, Shanjana; Lupu, Florea; Burian, Dennis; Ballard, Jimmy Dale; Braun, Armin

    2012-01-01

    The lung is the site of entry for Bacillus anthracis in inhalation anthrax, the deadliest form of the disease. Bacillus anthracis produces virulence toxins required for disease. Alveolar macrophages were considered the primary target of the Bacillus anthracis virulence factor lethal toxin because lethal toxin inhibits mouse macrophages through cleavage of MEK signaling pathway components, but we have reported that human alveolar macrophages are not a target of lethal toxin. Our current results suggest that, unlike human alveolar macrophages, the cells lining the respiratory units of the lung, alveolar epithelial cells, are a target of lethal toxin in humans. Alveolar epithelial cells expressed lethal toxin receptor protein, bound the protective antigen component of lethal toxin, and were subject to lethal-toxin-induced cleavage of multiple MEKs. These findings suggest that human alveolar epithelial cells are a target of Bacillus anthracis lethal toxin. Further, no reduction in alveolar epithelial cell viability was observed, but lethal toxin caused actin rearrangement and impaired desmosome formation, consistent with impaired barrier function as well as reduced surfactant production. Therefore, by compromising epithelial barrier function, lethal toxin may play a role in the pathogenesis of inhalation anthrax by facilitating the dissemination of Bacillus anthracis from the lung in early disease and promoting edema in late stages of the illness. PMID:23027535

  16. Impairment of phagocytic functions of alveolar macrophages by hydrogen peroxide

    SciTech Connect

    Oosting, R.S.; van Bree, L.; van Iwaarden, J.F.; van Golde, L.M.; Verhoef, J. )

    1990-08-01

    Hydrogen peroxide (H2O2) inhibited phagocytosis and superoxide anion production by rat alveolar macrophages. The inhibition was irreversible and concentration and exposure time dependent. The potential relationship between H2O2-induced biochemical perturbations and impaired alveolar macrophage phagocytic functions was investigated. Alveolar macrophage viability and Fc receptor binding capacity were not affected by H2O2. There was probably no correlation between a H2O2-induced rise in cytosolic (Ca2+) ((Ca2+)i) and the impairment of phagocytosis by alveolar macrophages, as was suggested by the following findings. First, the H2O2-induced rise in (Ca2+)i could be inhibited by chelation of extracellular Ca2+, whereas the H2O2-induced impairment of phagocytosis could not. Second, the H2O2-induced rise in (Ca2+)i was reversible, whereas the impairment of phagocytosis was not. And finally, a rise in (Ca2+)i by incubation of alveolar macrophages with the calcium ionophore A23187 did not affect phagocytosis. Various experiments suggested that ATP depletion may play an important role in the H2O2 toxicity for alveolar macrophages. Comparable concentrations of H2O2 caused an irreversible decrease both in cellular ATP and in phagocytosis and superoxide production by alveolar macrophages. In addition, time course of ATP depletion and induction of impaired alveolar macrophage function were similar. In view of the fact that the strong oxidant H2O2 may react with a large variety of biological substances, possible other toxic lesions may not be excluded as underlying mechanism for H2O2-induced inhibition of phagocytic functions of alveolar macrophages.

  17. Zinc hydroxide stimulates superoxide production by rat alveolar macrophages.

    PubMed

    Ogino, K; Izumi, Y; Ishiyama, H; Murata, T; Kobayashi, H; Houbara, T

    1992-06-30

    The effect of zinc hydroxide on superoxide (O2-) production by rat alveolar macrophages was determined by chemiluminescence and by cytochrome c reduction. Zinc ions had no effect on the chemiluminescence of unstimulated alveolar macrophages. By contrast, zinc hydroxide (ZnOH2), a neutralized form of zinc ions, increased the chemiluminescence level and O2- release. Increased O2- release was inhibited by pertussis toxin, isoquinoline sulfonamide and pretreatment with EGTA. These findings indicate that zinc hydroxide formation from zinc compounds can stimulate the O2- production by alveolar macrophages by receptor-mediated and Ca(2+)-dependent process.

  18. In vitro study of gas effects on alveolar macrophages.

    PubMed

    Wallaert, B; Voisin, C

    1992-01-01

    To evaluate the biological effects of gas pollutants on alveolar macrophages several in vitro systems have been developed. We described here an original method of cell culture in aerobiosis, which permitted direct contact between the atmosphere and the target cells. We studied the long term (24 h) and short term (30 min) effects of NO2 on alveolar macrophages. Our results demonstrated that exposure of alveolar macrophages to gas pollutants may be responsible for either cell injury or cell activation associated with the release of various bioactive mediators (superoxide anion, neutrophil chemotactic activity). Cell culture in aerobiosis opens new ways for the research on the biological effects of gas pollutants.

  19. A Suspected Case of an Alveolar Haemorrhage Caused by Dasatinib

    PubMed Central

    Sakoda, Yoritake; Arimori, Yojiro; Ueno, Masakatsu; Matsumoto, Takafumi

    2017-01-01

    A 39-year-old man treated with dasatinib for chronic myelogenous leukaemia presented to our hospital with haemoptysis, coughing, and dyspnoea. Chest radiography and computed tomography revealed ground-glass opacities and a crazy-paving pattern. Bronchoalveolar lavage was not performed due to serious hypoxemia and bleeding. Significant bleeding from the peripheral bronchi led to a diagnosis of an alveolar haemorrhage. Dasatinib-induced alveolar haemorrhaging was suspected based on the clinical findings. His condition improved immediately after dasatinib withdrawal and initiation of steroid therapy. Reports of alveolar haemorrhaging induced by dasatinib are rare. As such, this is considered an important case. PMID:28090053

  20. [Two Cases of Stromal Sarcoma of the Breast].

    PubMed

    Takahashi, Hiroyuki; Inaba, Satoshi; Yabuki, Hidehiko

    2016-11-01

    Stromal sarcoma of the breast is a very rare disease accounting for 0.03%of primary breast malignant tumors. From 1995 to 2014, 384 patients with primary breast malignant tumors underwent resection at our institution, of which 2 cases(0.5%) were stromal sarcoma of the breast. Case 1: A woman aged in her 50's presented with a painful lump spanning the right breast identified 7-8 months previously by palpation. The patient first visited the outpatient department at our hospital when the lump gradually increased in size and bleeding from the breast was observed. The tumor measured 8 cm in its greatest dimension and it was elastic soft with a smooth surface, exhibited sphericity, and existed in combination with a partial skin ulcer. Biopsy indicated possibility of stromal sarcoma; therefore, simple mastectomy was performed and the patient was pathologically diagnosed with stromal sarcoma. Presently, the patient is alive and recurrence-free 3 years after the surgery. Case 2: A woman aged in her 80's presented with a left breast tumor identified 4-5 days previously via palpation. The patient visited a general practitioner who referred her to our hospital. The tumor measured 1.6 cm in its greatest dimension and it was elastic hard, irregular in shape, and exhibited dimpling. Biopsy indicated the possibility of malignancy; therefore, a partial resection was performed and the patient was pathologically diagnosed with stromal sarcoma. After 6 months, another lesion was detected and extirpation was performed. A recurrent lesion was detected after 7 months and extirpation was performed again. Presently, the patient is alive and recurrence-free 17 months after the third surgery. In stromal sarcoma of the breast, surgical resection with a negative margin is the only curative treatment. Tumor resection should be planned carefully and the possibility of sarcoma in breast tumors with atypical features as breast cancer should be considered.

  1. 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

  2. Efficacy of paclitaxel in the treatment of Kaposi sarcoma.

    PubMed

    Ercolak, V; Sahin, B; Gunaldi, M; Duman, B B; Afsar, C U

    2015-11-01

    Kaposi sarcoma is an angioproliferative disease. Kaposi sarcoma is clinicopathologically classified into four subgroups based on epidemiological data. For its systemic treatment, in addition to some chemotherapeutics, taxanes have also been used during the recent years for their anti-angiogenic properties. In this study, we aimed to compare paclitaxel and non-paclitaxel chemotherapeutic regimens in terms of efficacy and side effects. In our center, demographical, clinical and histopathological characteristics of a total of 13 patients diagnosed with Kaposi sarcoma who received therapy were retrospectively recorded based on their medical files Among these subjects, 7 have been treated with paclitaxel and 6 with non-paclitaxel therapies. Eleven patients were male. Twelve patients were found to have classical type of Kaposi Sarcoma. The recurrence was observed in 2 patients treated with paclitaxel and in 1 patient treated with non-paclitaxel therapy. No statistically significant difference was found between the therapeutic modality, the stage of the disease and the percentage of the recurrence. Neuropathy developed in 3 patients treated with paclitaxel, whereas there was no neuropathy in the other group. Although the recurrence-free survival was worse in the patients treated with paclitaxel, there was no statistically significant difference. Cytotoxic chemotherapy is effective in treating patients with Kaposi Sarcoma, although it is palliative. Taxanes have demonstrated effectiveness against AIDS-associated Kaposi Sarcoma. The experience suggests that paclitaxel is an effective alternative in the treatment of classical form Kaposi's sarcoma. There was no difference in efficacy between paclitaxel and non-paclitaxel therapies whereas difference in occurrence of neuropathy which is one of the side effects, showed borderline statistical significance.

  3. ECCO Essential Requirements for Quality Cancer Care: Soft Tissue Sarcoma in Adults and Bone Sarcoma. A critical review.

    PubMed

    Andritsch, Elisabeth; Beishon, Marc; Bielack, Stefan; Bonvalot, Sylvie; Casali, Paolo; Crul, Mirjam; Delgado Bolton, Roberto; Donati, Davide Maria; Douis, Hassan; Haas, Rick; Hogendoorn, Pancras; Kozhaeva, Olga; Lavender, Verna; Lovey, Jozsef; Negrouk, Anastassia; Pereira, Philippe; Roca, Pierre; de Lempdes, Godelieve Rochette; Saarto, Tiina; van Berck, Bert; Vassal, Gilles; Wartenberg, Markus; Yared, Wendy; Costa, Alberto; Naredi, Peter

    2017-02-01

    ECCO essential requirements for quality cancer care (ERQCC) are checklists and explanations of organisation and actions that are necessary to give high-quality care to patients who have a specific tumour type. They are written by European experts representing all disciplines involved in cancer care. ERQCC papers give oncology teams, patients, policymakers and managers an overview of the elements needed in any healthcare system to provide high quality of care throughout the patient journey. References are made to clinical guidelines and other resources where appropriate, and the focus is on care in Europe. Sarcoma: essential requirements for quality care • Sarcomas - which can be classified into soft tissue and bone sarcomas - are rare, but all rare cancers make up more than 20% of cancers in Europe, and there are substantial inequalities in access to high-quality care. Sarcomas, of which there are many subtypes, comprise a particularly complex and demanding challenge for healthcare systems and providers. This paper presents essential requirements for quality cancer care of soft tissue sarcomas in adults and bone sarcomas. • High-quality care must only be carried out in specialised sarcoma centres (including paediatric cancer centres) which have both a core multidisciplinary team and an extended team of allied professionals, and which are subject to quality and audit procedures. Access to such units is far from universal in all European countries. • It is essential that, to meet European aspirations for high-quality comprehensive cancer control, healthcare organisations implement the requirements in this paper, paying particular attention to multidisciplinarity and patient-centred pathways from diagnosis and follow-up, to treatment, to improve survival and quality of life for patients. Taken together, the information presented in this paper provides a comprehensive description of the essential requirements for establishing a high-quality service for soft

  4. Synovial Sarcoma of the Larynx: Report of a Case and Review of Literature

    PubMed Central

    Baby, Anto; Somanathan, Thara; Konoth, Sreedevi

    2017-01-01

    Sarcomas account for less than 1% of malignant neoplasms arising in the head and neck in adults. Laryngeal synovial sarcoma is an extremely rare form of laryngeal malignancy with less than 20 cases reported in the literature. We report the case of a 48-year-old man with synovial sarcoma of the larynx. He underwent excision of the tumor followed by radiation. He is alive in remission at 36 months. The literature on synovial sarcoma of the larynx is reviewed. PMID:28280643

  5. Current update on the diagnosis and management of head and neck soft tissue sarcomas.

    PubMed

    Tudor-Green, Ben; Gomez, Ricardo; Brennan, Peter A

    2017-10-01

    Head and neck soft tissue sarcomas are a group of rare heterogeneous tumours arising from embryonic mesoderm. They comprise <1% of all head and neck malignancies and 5-15% of all sarcomas with most head and neck sarcomas arising from soft tissues. Although rare, they are associated with both high recurrence and mortality rates. We review the current management of head and neck soft tissue sarcomas. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  6. Soft tissue sarcoma with metastasis to the stomach: A case report

    PubMed Central

    Dent, Lemuel Leon; Cardona, Cesar Yamil; Buchholz, Michael Clause; Peebles, Roosevelt; Scott, Julie Denise; Beech, Derrick Jerome; Ballard, Billy Ray

    2010-01-01

    Soft tissue sarcomas are unusual malignancies comprising 1% of cancer diagnoses in the United States. Undifferentiated pleomorphic sarcoma accounts for approximately 5% of sarcomas occurring in adults. The most common site of metastasis is the lung, with other sites being bone, the brain, and the liver. Metastasis to the gastrointestinal tract has rarely been documented. We present an unusual case of high-grade pleomorphic sarcoma with metastasis to the stomach, complicated by upper gastrointestinal bleeding. PMID:20976852

  7. *Ambient Particluate Matter Supresses Alveolar Macrophage Cytokine Response to Lipopolysaccharide

    EPA Science Inventory

    Reports link ambient particulate matter (PM) exposure with cardiopulmonary mortality and morbidity, including the exacerbation of inflammatory disease and increased hospitalization for lung infections. Alveolar macrophages (AM) play an important defense role against infections v...

  8. AMBIENT PARTICULATE MATTER DECREASED IN HUMAN ALVEOLAR MACHROPHAGE CYTOKINE RELEASE

    EPA Science Inventory

    Human exposure to ambient airborne particulate matter (PM) is associated with cardiopulmonary mortality and morbidity, including increased hospitalizations for lung infection. Normal lung immune responses to bacterial infection include alveolar macrophage cytokine production and...

  9. Interrelationships between the Human Alveolar Macrophage and Alpha-1-Antitrypsin

    PubMed Central

    Cohen, Allen B.

    1973-01-01

    Alveolar macrophages lavaged from human lungs contain protease activity at an optimum pH of 3.0 and possibly a lesser peak of activity at pH 5.5. Protease activity measured at pH 4.1 is inhibited by purified alpha-1-antitrypsin. Fluorescent antibody studies of human alveolar macrophages showed that alpha-1-antitrypsin is present in normal alveolar macrophages. In addition, macrophages from a patient with a homozygous deficiency of alpha-1-antitrypsin exhibited less fluorescence when incubated in autologous serum than the same macrophages incubated in normal serum. Macrophages from normal subjects showed maximal fluorescence when removed from the lung and additional incubation with serum did not increase fluorescence. These results implicate the human alveolar macrophage as a possible source of an enzyme that may cause emphysema in patients deficient in alpha-1-antitrypsin. They also show that alpha-1-antitrypsin has access to the alveolus in normal subjects. Images PMID:4201266

  10. *Ambient Particluate Matter Supresses Alveolar Macrophage Cytokine Response to Lipopolysaccharide

    EPA Science Inventory

    Reports link ambient particulate matter (PM) exposure with cardiopulmonary mortality and morbidity, including the exacerbation of inflammatory disease and increased hospitalization for lung infections. Alveolar macrophages (AM) play an important defense role against infections v...

  11. Alveolar distraction osteogenesis: revive and restore the native bone.

    PubMed

    Sant, Sumedha; Jagtap, Amit

    2009-12-01

    In prosthodontics, knife-edge bony alveolar ridges can cause a problem in their rehabilitation. The distraction osteogenesis process raises the medullary component of the alveolus, allowing the labial plate of the existing natural bone to be displaced. This process involves mobilization, transport, and fixation of a healthy segment of bone adjacent to the deficient site. It entails use of the gradual controlled displacement of surgically created fractures, which results in simultaneous expansion of soft tissue and bone volume. A mechanical device, the alveolar distraction device, is used for this purpose. This modality of treatment can be used in implant dentistry cases for rehabilitation of resorbed ridges. The objective of this overview is to explain this procedure wherein the alveolar housing, including the osseous and soft-tissue components, is enlarged in a single, simultaneous process, which makes creation of an appropriate alveolar morphology possible.

  12. Alveolar haemorrhage in a case of high altitude pulmonary oedema

    PubMed Central

    Grissom, C.; Albertine, K.; Elstad, M.

    2000-01-01

    A case of high altitude pulmonary oedema (HAPE) in a climber who made a rapid ascent on Mt McKinley (Denali), Alaska is described. The bronchoalveolar lavage (BAL) fluid contained increased numbers of red blood cells and an abundance of haemosiderin laden macrophages consistent with alveolar haemorrhage. The timing of this finding indicates that alveolar haemorrhage began early during the ascent, well before the onset of symptoms. Although evidence of alveolar haemorrhage has been reported at necropsy in individuals dying of HAPE, previous reports have not shown the same abundance of haemosiderin laden macrophages in the BAL fluid. These findings suggest that alveolar haemorrhage is an early event in HAPE.

 PMID:10639537

  13. Pulmonary alveolar microlithiasis in Afghan pika (Ochotona rufescens rufescens).

    PubMed

    Madarame, H; Kumagai, M; Suzuki, J; Watanabe, A; Konno, S

    1989-07-01

    Lung lesions of 60 Afghan pikas (Ochotona rufescens rufescens) were examined histologically; 38 cases of pulmonary alveolar microlithiasis were found. Neither sex was predominantly affected, and there was no predilection for age.

  14. Pulmonary involvement in Kaposi sarcoma: correlation between imaging and pathology.

    PubMed

    Gasparetto, Taisa Davaus; Marchiori, Edson; Lourenço, Sílvia; Zanetti, Gláucia; Vianna, Alberto Domingues; Santos, Alair A S M D; Nobre, Luiz Felipe

    2009-07-14

    Kaposi sarcoma is a low-grade mesenchymal tumor involving blood and lymphatic vessels. There are four variants of this disease, each presenting a different clinical manifestation: classic or sporadic, African or endemic, organ transplant-related or iatrogenic, and AIDS-related or epidemic. Kaposi sarcoma is the most common tumor among patients with HIV infection, occurring predominantly in homosexual or bisexual men. The pulmonary involvement in Kaposi sarcoma occurs commonly in critically immunosupressed patients who commonly have had preceding mucocutaneous or digestive involvement.The etiology of Kaposi sarcoma is not precisely established; genetic, hormonal, and immune factors, as well as infectious agents, have all been implicated. There is evidence from epidemiologic, serologic, and molecular studies that Kaposi sarcoma is associated with human herpes virus type 8 infection. The disease starts as a reactive polyclonal angioproliferative response towards this virus, in which polyclonal cells change to form oligoclonal cell populations that expand and undergo malignant transformation.The diagnosis of pulmonary involvement in Kaposi sarcoma usually can be made by a combination of clinical, radiographic, and laboratory findings, together with the results of bronchoscopy and transbronchial biopsy. Chest high-resolution computed tomography scans commonly reveal peribronchovascular and interlobular septal thickening, bilateral and symmetric ill-defined nodules in a peribronchovascular distribution, fissural nodularity, mediastinal adenopathies, and pleural effusions. Correlation between the high-resolution computed tomography findings and the pathology revealed by histopathological analysis demonstrate that the areas of central peribronchovascular infiltration represent tumor growth involving the bronchovascular bundles, with nodules corresponding to proliferations of neoplastic cells into the pulmonary parenchyma. The interlobular septal thickening may represent

  15. Like or Dislike? Impact of Facebook on Ewing Sarcoma Treatment.

    PubMed

    Ruckenstuhl, Paul; Schippinger, Michael; Liebmann, Paul; Leithner, Andreas; Bernhardt, Gerwin

    2016-08-25

    An increasing number of patients are raising their voices in online forums to exchange health-related information. Facebook is the leading social media platform with more than 1 billion international daily users recorded in the summer of 2015. Facebook has a dynamic audience and is utilized in a number of ways, discussing medical issues being one of them. Ewing sarcoma mainly affects teenagers and young adults. Additionally, many individuals within this age group are regular users of Facebook. However, little is known about the impact of this modern way of communication via Web-based platforms on patients with Ewing sarcoma and their social environment. The aim of this study was to analyze and compare Ewing sarcoma patients' and relatives' behavior on Facebook to draw conclusions regarding the impact of Facebook on Ewing sarcoma treatment. We examined a Facebook group named "Ewing Sarcoma Awareness" that is used to exchange information for both patients and relatives regarding Ewing sarcoma. A self-designed questionnaire was used to compare patients' and relatives' answers. Additionally, we analyzed all processes (posts, likes, threads, links) in the group for 6 consecutive months. A total of 65 members of the Facebook group (26 patients, 39 relatives) out of 2227 international group members participated in our study. More than 70% (46/65) of all participants reported that they use the group Ewing Sarcoma Awareness as a source of information about Ewing sarcoma. Of the participants, 89% (58/65) agreed on our scale from a little to a lot that being in contact with other affected people through the group makes it easier to handle the diagnosis. In this study, 20% (13/65) of all participants reported that the group affected their choice of treatment and 15% (10/65) of participants were influenced in the selection of their specialist. Regarding the recommendation of the Facebook group toward other people, significant differences (P=.003) were found comparing patients

  16. Like or Dislike? Impact of Facebook on Ewing Sarcoma Treatment

    PubMed Central

    Schippinger, Michael; Liebmann, Paul; Leithner, Andreas; Bernhardt, Gerwin

    2016-01-01

    Background An increasing number of patients are raising their voices in online forums to exchange health-related information. Facebook is the leading social media platform with more than 1 billion international daily users recorded in the summer of 2015. Facebook has a dynamic audience and is utilized in a number of ways, discussing medical issues being one of them. Ewing sarcoma mainly affects teenagers and young adults. Additionally, many individuals within this age group are regular users of Facebook. However, little is known about the impact of this modern way of communication via Web-based platforms on patients with Ewing sarcoma and their social environment. Objective The aim of this study was to analyze and compare Ewing sarcoma patients’ and relatives’ behavior on Facebook to draw conclusions regarding the impact of Facebook on Ewing sarcoma treatment. Methods We examined a Facebook group named “Ewing Sarcoma Awareness” that is used to exchange information for both patients and relatives regarding Ewing sarcoma. A self-designed questionnaire was used to compare patients’ and relatives’ answers. Additionally, we analyzed all processes (posts, likes, threads, links) in the group for 6 consecutive months. A total of 65 members of the Facebook group (26 patients, 39 relatives) out of 2227 international group members participated in our study. Results More than 70% (46/65) of all participants reported that they use the group Ewing Sarcoma Awareness as a source of information about Ewing sarcoma. Of the participants, 89% (58/65) agreed on our scale from a little to a lot that being in contact with other affected people through the group makes it easier to handle the diagnosis. In this study, 20% (13/65) of all participants reported that the group affected their choice of treatment and 15% (10/65) of participants were influenced in the selection of their specialist. Regarding the recommendation of the Facebook group toward other people, significant

  17. 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

  18. A case report of pancreatic metastasis from synovial sarcoma successfully treated by metastasectomy with adjuvant chemotherapy

    PubMed Central

    Makino, Yuki; Shigekawa, Minoru; Kegasawa, Tadashi; Suda, Takahiro; Yoshioka, Teppei; Iwahashi, Kiyoshi; Ikezawa, Kenji; Sakamori, Ryotaro; Yakushijin, Takayuki; Kajihara, Jun; Tomimaru, Yoshito; Eguchi, Hidetoshi; Imura, Yoshinori; Outani, Hidetatsu; Naka, Norifumi; Honma, Keiichiro; Morii, Eiichi; Tatsumi, Tomohide; Hiramatsu, Naoki; Takehara, Tetsuo

    2016-01-01

    Abstract Introduction: Synovial sarcoma is a malignant soft tissue sarcoma which arises near joints. The most frequent metastasis sites of synovial sarcoma are the lungs, lymph nodes, and bone. Pancreatic metastasis is quite rare; only 3 cases have been reported worldwide to date. We herein present the 4th case of pancreatic metastasis from synovial sarcoma. Methods and Results: A 32-year-old man underwent extended excision of synovial sarcoma in the left pelvis and femur in 2009. In 2013, follow-up contrast-enhanced computed tomography revealed a 35-mm heterogeneously enhanced mass in the pancreas body. Endoscopic ultrasound-guided fine needle aspiration of the mass revealed a diffuse proliferation of atypical spindle cells in a fascicular arrangement. Because the histology was quite similar to the resected specimen of synovial sarcoma in 2009, the mass was suspected to be a metastasis from synovial sarcoma. Laparoscopic distal pancreatectomy with adjuvant adriamycin/ifosfamide chemotherapy was subsequently performed. Synovial sarcoma-specific SS18-SSX1 (synovial sarcoma translocation, chromosome 18-synovial sarcoma X1) or SS18-SSX2 chimera mRNA was detected in the resected specimen, confirming the diagnosis of metastasis from synovial sarcoma. The patient did well for 30 months without recurrence. Conclusion: This case suggests that pancreatic metastasis from synovial sarcoma can be successfully treated by metastasectomy with adjuvant chemotherapy. PMID:27684804

  19. 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

  20. 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.