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Sample records for hemosiderotic fibrohistiocytic lipomatous

  1. Lipomatous infiltration of the canine salivary gland.

    PubMed

    Brown, P J; Lucke, V M; Sozmen, M; Whitbread, T J; Wyatt, J M

    1997-06-01

    Benign connective tumours of the canine salivary glands are rare. This report describes lipomatous infiltration of parotid or submandibular salivary glands in seven dogs in which the glands were enlarged as a result of infiltration by fat cells; they appeared to have been successfully treated by local excision. The precise cause of the lipomatous infiltration in the dogs is unclear but different causes of similar lesions in humans are discussed.

  2. Pathologic and prognostic characteristics of splenomegaly in dogs due to fibrohistiocytic nodules: 98 cases.

    PubMed

    Spangler, W L; Kass, P H

    1998-11-01

    Ninety-eight canine splenectomy specimens consisting of combined nodular lymphoid and fibrohistiocytic cell proliferation were evaluated for seven light microscopic characteristics. Electron microscopic features in eight primary and two metastatic nodules (liver) were also evaluated. Nodular fibrohistiocytic proliferation in the canine spleen is characterized by a mixed population of histiocytoid and/or spindle cells in varying proportions intermixed with hematopoietic elements, plasma cells, and/or lymphocytes. These nodules seem to form a continuum between splenic lymphoid nodular hyperplasia and malignant splenic stromal neoplasms (malignant fibrous histiocytoma). Immunohistochemical methods used on 32/98 specimens showed uniform and strong positive staining among fibrohisiocytic cells for vimentin and desmin; S100 protein was similarly stained in general abundance. Individual cells strongly stained with smooth muscle actin were sparse but widely distributed. Proliferating cell nuclear antigen was not useful in the subjective differentiation of nodules taken from dogs that died of spleen-related causes and those surviving 12 months following splenectomy. A spectrum of cell types were observed by electron microscopy within each nodule. Fibroblasts, macrophages, intermediate fibrohistiocytic types, and several forms of splenic reticular cells were present. There were no consistent alterations in hematology or serum chemistry profiles of these dogs to provide useful diagnostic/prognostic information. Among the 93/98 dogs with complete (12 month) follow-up information, 48% (45/93) were alive and 52% (48/93) were dead. Dogs that died or were euthanatized during the follow-up period had a median survival of 5 and 5.5 months, respectively (range 0-15 months). Forty-four percent (21/48) died from causes linked to their splenic disease, and 35% (17/48) died from competing causes. The cause of death in 21% (10/48) was unknown. Lymphoid:fibrohistiocytic proportion and

  3. A Rare Case of Lipomatous Hypertrophy of the Interventricular Septum

    PubMed Central

    Matsiras, Sotirios; Vassilikos, Vassilios

    2016-01-01

    Asymmetrical left ventricular hypertrophy secondary to interventricular septum hypertrophy is usually considered a typical phenotype of hypertrophic cardiomyopathy. In rare cases other conditions such as tumors or lipomatous hypertrophy of the interventricular septum may have a similar presentation. We present a case of a male patient who presented for routine cardiology work up and was diagnosed of having ventricular septal hypertrophy secondary to localized lipomatous hypertrophy. PMID:27358712

  4. A Tete – a – tete of benign, borderline and malignant fibrohistiocytic tumor

    PubMed Central

    M.V, Rashmi.; P, Pavithra; G.M, Shivakumarappa

    2016-01-01

    Dermatofibrosarcoma protruberans is a relatively uncommon slow growing, locally aggressive fibrous tumor of the skin. It has a prospensity of progressing to fibrosarcomatous change in 5% of the cases. We present a case of a 56 yr old male with presented to the outpatient department of surgery, Sri Siddhartha Medical College, Tumkur with a chest swelling in 2013. FNAC was inconclusive and the mass was excised. On histopathology, areas of benign fibrohistiocytic tumor, dermatofibrosarcoma protruberans and fibrosarcomatous dermatofibrosarcoma were identified in the same tumor. Immunohistochemistry confirmed the diagnosis of DFSP with fibrosarcomatous change. Although, transformed DFSP is more aggressive, the prognosis is influenced by the extent of excision and with wide excision, there may be little increased risk for recurrence and metastasis over that of conventional DFSP.

  5. Lipomatous Change in Uveal Melanoma: Histopathological, Immunohistochemical and Cytogenetic Analysis

    PubMed Central

    Yavuzyigitoglu, Serdar; Kilic, Emine; Vaarwater, Jolanda; de Klein, Annelies; Paridaens, Dion; Verdijk, Robert M.

    2016-01-01

    Purpose The aim of this study was to describe a case of lipomatous change in uveal melanoma. Procedures The patient presented with a 2-year history of blurry vision. A full examination of the right eye revealed a dome-shaped pigmented subretinal mass in the choroid with a thickness of 9 mm and a diameter of 15 mm. The eye was enucleated and prepared for histopathologic, genetic and molecular investigation. Results Histopathology revealed a small circumscribed area consisting of mature adipocytic appearing cells with abundant clear cytoplasm and small peripheral flattened nuclei within a spindle-cell melanoma of the uvea. The cytoplasm of the adipocytic cells stained negative for periodic acid-Schiff and Alcian blue and positive for Melan-A, HMB-45 and tyrosinase, confirming melanocytic lineage. Fluorescence in situ hybridization analysis confirmed trisomy of chromosome 6p22 and disomy of chromosome 3p13 in the nuclei of both the tumor spindle type B cells and in the nuclei of lipomatous tumor cells. Conclusions Lipomatous change can be added to the many histopathologic faces of uveal melanoma. To our knowledge, this is the first report of lipomatous change in uveal melanoma performed with cytogenetic investigations. PMID:27239451

  6. An unusual lipomatous brain mass in a Golden Retriever dog.

    PubMed

    Scott, Steven J; Elliot, Kirsty; Philibert, Helene; Summers, Brian A; Godson, Dale; Singh, Baljit; Simko, Elemir

    2015-11-01

    A 9-year-old Golden Retriever dog was presented to the Veterinary Medical Center with a 3-week history of grand mal seizures and was subsequently euthanized. At autopsy, a discrete, firm, expansile mass was found in the right pyriform lobe, which compressed the ipsilateral hippocampus, thalamus, and cerebral cortex. Histologically, the mass was composed of well-differentiated adipose tissue supported by fibrous and mucinous stroma. Adipocytes exhibited strong immunoreactivity for vimentin and were negative for pancytokeratin (AE1/AE3), glial fibrillary acidic protein, neuron-specific enolase, and synaptophysin. These findings are most compatible with an intracranial lipomatous hamartoma, which is an extraparenchymal lesion that has been identified in several species. The current report describes an intracerebral lipomatous hamartoma in a veterinary species. PMID:26450836

  7. Pleomorphic rhabdomyosarcoma showing smooth-muscle and fibrohistiocytic differentiation: a single case report.

    PubMed

    Eyden, Brian

    2010-02-01

    Rhabdomyosarcoma has traditionally been subclassified into alveolar, embryonal, and pleomorphic variants. Less commonly, spindle-cell, neuroendocrine, sclerosing, and lipid-rich or clear-cell subtypes are seen. The author recently encountered a myogenic sarcoma, with all the common markers of rhabdomyosarcoma, but expressing the unusual features of alpha-smooth-muscle actin and abundant rough endoplasmic reticulum (rER). This myogenic sarcoma, therefore, exhibited four lines of differentiation, and is documented here. The patient was a 65-year-old man with an inguinal soft tissue mass. Following surgical excision, the patient was given radiotherapy and was well without disease after 6 years. The tumor was positive for vimentin, desmin, alpha-smooth-muscle actin, alpha-sarcomeric actin, myogenin, MyoD1, and CD68. Cytoplasm was dominated by abundant rER intermingled with lipid droplets and lysosomes. Cell surfaces exhibited microvillous processes and focal adhesions, but no lamina. Subplasmalemmal smooth-muscle-type myofilaments with focal densities and rare sarcomeric filaments were seen. The low level of expression of some markers was interpreted as consistent with a poorly differentiated tumor. Given the four lines of differentiation--striated muscle, smooth muscle, fibroblastic, and histiocytic--a name reflecting its phenotype would be pleomorphic rhabdomyosarcoma showing smooth-muscle and fibrohistiocytic differentiation. PMID:20070153

  8. Lipomatous pleomorphic adenoma in the hard palate: Report of a rare case with cyto-histo correlation and review.

    PubMed

    Musayev, Jamal; Onal, Binnur; Hasanov, Adalat; Farzaliyev, Ismayil

    2014-01-01

    Pleomorphic adenoma is the most common benign tumor of the salivary glands. They are usually composed of epithelial/myoepithelial cells and chondromyxoid stroma. Extensive lipomatous differentiation is very rare. We report a case of lipomatous pleomorphic adenoma (LPA) that presented with a mass in the hard palate of a 32-year-old woman. The fine-needle aspiration cytology material was reported as benign cytology consistent with adenoma with major adipocytic component. Histopathological examination of the excision material displayed that more than 90% of the tumor was adipocytic in texture, containing scant epithelial and myoepithelial cells and chondromyxoid stromal fragments. Preoperative cytodiagnosis of lipomatous pleomorphic adenoma on FNA is based on cytomorphology intimately associated pleomorphic adenomatous and lipomatous tissue elements. LPA should be on the mental list of the (cyto)pathologist in differential diagnosis of lipomatous tumors or non-tumorous lipomatosis or carcinoma invasion in the adipose tissue of the minor salivary gland of the hard palate.

  9. Atypical lipomatous tumor of the lip with pleomorphic lipoma-like myxoid area, clinically simulating mucocele.

    PubMed

    Hattori, Hideo

    2002-10-01

    Lipomatous tumor is least common in the oral cavity. We present a case of atypical lipomatous tumor arising in the lower lip of an 81-year-old male. Clinical diagnosis was mucocele. The tumor measured 12 mm in diameter with a round shape. It was solid, firm, myxomatous and translucent with a small yellowish focus in the central portion. Histologically, the tumor was a circumscribed and well-encapsulated mass with prominent myxoid matrix. The central minor portion showed a well-differentiated liposarcoma. In the myxoid area, lipoblasts and large atypical cells with dense chromatin including floret-like giant cells were seen in abundance. Atypical lipomatous tumor may be indistinguishable from pleomorphic lipoma and its distinction from the latter is based on location as well as relative abundance of floret-type giant cells and lipoblasts. According to its abundance in lipoblasts over floret-like cells, pleomorphic lipoma-like area in our case could also be a part of atypical lipomatous tumor. It may be possible to interpret myxoid change in our case as to represent a superficial counterpart of myxoid malignant fibrous histiocytoma-like change reported in the retroperitoneal lesion. PMID:12269996

  10. Atypical lipomatous tumor of the lip with pleomorphic lipoma-like myxoid area, clinically simulating mucocele.

    PubMed

    Hattori, Hideo

    2002-10-01

    Lipomatous tumor is least common in the oral cavity. We present a case of atypical lipomatous tumor arising in the lower lip of an 81-year-old male. Clinical diagnosis was mucocele. The tumor measured 12 mm in diameter with a round shape. It was solid, firm, myxomatous and translucent with a small yellowish focus in the central portion. Histologically, the tumor was a circumscribed and well-encapsulated mass with prominent myxoid matrix. The central minor portion showed a well-differentiated liposarcoma. In the myxoid area, lipoblasts and large atypical cells with dense chromatin including floret-like giant cells were seen in abundance. Atypical lipomatous tumor may be indistinguishable from pleomorphic lipoma and its distinction from the latter is based on location as well as relative abundance of floret-type giant cells and lipoblasts. According to its abundance in lipoblasts over floret-like cells, pleomorphic lipoma-like area in our case could also be a part of atypical lipomatous tumor. It may be possible to interpret myxoid change in our case as to represent a superficial counterpart of myxoid malignant fibrous histiocytoma-like change reported in the retroperitoneal lesion.

  11. Spindle cell lipoma masquerading as lipomatous pleomorphic adenoma: A diagnostic dilemma on fine needle aspiration cytology.

    PubMed

    Agarwal, S; Nangia, A; Jyotsna, P Lalita; Pujani, M

    2013-01-01

    Spindle cell lipoma is a relatively uncommon benign adipocytic tumor that usually presents in subcutaneous fat of adult men. These are a rare form of lipoma, accounting for 1.5% of all lipomatous tumors, with a low rate of local recurrence and no risk of malignant behavior/dedifferentiation. Although few studies addressing the histological findings of spindle cell lipoma have been described, only a few descriptions of fine needle aspiration cytology (FNAC) findings have been documented in literature. We present a case of a 55-year-old male with a nodular swelling over left cheek (in the parotid region), which due to its location as well as prominent myxoid background prompted us to include the lipomatous salivary gland lesions in differential diagnosis. Our objective is to document and delineate the characteristic cytological features of spindle cell lipoma, which may permit a confident diagnosis on FNAC smears.

  12. Spindle Cell Lipoma Occurring in the Buccal Mucosa: An Unusual Location of This Benign Lipomatous Neoplasm

    PubMed Central

    Milhan, Noala Vicensoto Moreira; Cavalcante, Ana Sueli Rodrigues; Marques, Yonara Maria Freire Soares; Carvalho, Yasmin Rodarte; Anbinder, Ana Lia

    2015-01-01

    Spindle cell lipoma is a benign lipomatous neoplasm, which rarely occurs in the oral cavity. The aims of this paper are to report a case of spindle cell lipoma located in buccal mucosa and discuss the main clinical, histological, and immunohistochemical findings of this entity. Thus, we report a 4-year history of an asymptomatic smooth surface nodule in an elderly Caucasian man with clinical hypothesis of fibroma. The histopathological examination showed spindle cells, mature adipose tissue, and many mast cells in a stroma of connective tissue presenting ropey collagen fibers bundles. After immunohistochemical analysis, the final diagnosis was spindle cell lipoma. PMID:26491592

  13. A case report of spindle cell myoepithelioma with extensive lipomatous metaplasia and thick collagen bundles in the submandibular gland.

    PubMed

    Kwon, Mi Jung; Kim, Hye Jeong; Park, Bumjung; Cho, Seong Jin; Shin, Hyung Sik; Park, Hye-Rim; Min, Soo Kee; Seo, Jinwon; Min, Kyueng-Whan; Nam, Eun Sook

    2016-09-01

    Spindle cell myoepithelioma with extensive lipomatous metaplasia and thick collagen bundles has not yet been described, and there are no published reports on its cytological appearance in fine-needle aspiration (FNA). A 49-year-old man presented with a painless mass in the right submandibular area that had been gradually enlarging for a period of 5 years. The cytologic smears showed fascicles of cohesive spindle cells as well as individual bland cells with bipolar naked nuclei in a fibrillary background. Brightly eosinophilic bundles were intermingled with spindle cells and fat-like vacuoles. The FNA results were suggestive of neurogenic tumor. Patient underwent submandibular gland resection. Grossly, the cut surface showed a well-encapsulated, yellowish-white, soft, elastic mass, measuring 2.8 × 1.9 × 1.5 cm. The tumor consisted of uniform bland spindle cells arranged in short fascicles admixed with adipocyte-like cells and transversing thick collagen bundles, which demonstrated immunoreactivity for myoepithelial markers and ultrastructural features characteristic of myoepithelial cells, suggesting the presence of lipomatous metaplasia. The FNA cytology of spindle cell myoepithelioma with extensive lipometaplasia mimicked that of neurogenic tumor or lipomatous mesenchymal tumor. This case represents the first description of submandibular gland myoepithelioma with lipometaplasia, which is characterized by the coexistence of spindle cells, collagen bundles, and fat-like vacuoles in a fibrillary background. Diagn. Cytopathol. 2016;44:764-769. © 2016 Wiley Periodicals, Inc. PMID:27307392

  14. A case report of spindle cell myoepithelioma with extensive lipomatous metaplasia and thick collagen bundles in the submandibular gland.

    PubMed

    Kwon, Mi Jung; Kim, Hye Jeong; Park, Bumjung; Cho, Seong Jin; Shin, Hyung Sik; Park, Hye-Rim; Min, Soo Kee; Seo, Jinwon; Min, Kyueng-Whan; Nam, Eun Sook

    2016-09-01

    Spindle cell myoepithelioma with extensive lipomatous metaplasia and thick collagen bundles has not yet been described, and there are no published reports on its cytological appearance in fine-needle aspiration (FNA). A 49-year-old man presented with a painless mass in the right submandibular area that had been gradually enlarging for a period of 5 years. The cytologic smears showed fascicles of cohesive spindle cells as well as individual bland cells with bipolar naked nuclei in a fibrillary background. Brightly eosinophilic bundles were intermingled with spindle cells and fat-like vacuoles. The FNA results were suggestive of neurogenic tumor. Patient underwent submandibular gland resection. Grossly, the cut surface showed a well-encapsulated, yellowish-white, soft, elastic mass, measuring 2.8 × 1.9 × 1.5 cm. The tumor consisted of uniform bland spindle cells arranged in short fascicles admixed with adipocyte-like cells and transversing thick collagen bundles, which demonstrated immunoreactivity for myoepithelial markers and ultrastructural features characteristic of myoepithelial cells, suggesting the presence of lipomatous metaplasia. The FNA cytology of spindle cell myoepithelioma with extensive lipometaplasia mimicked that of neurogenic tumor or lipomatous mesenchymal tumor. This case represents the first description of submandibular gland myoepithelioma with lipometaplasia, which is characterized by the coexistence of spindle cells, collagen bundles, and fat-like vacuoles in a fibrillary background. Diagn. Cytopathol. 2016;44:764-769. © 2016 Wiley Periodicals, Inc.

  15. Lipomatous ependymoma: report of a rare differentiation pattern with a comprehensive review of literature.

    PubMed

    Gaur, Kavita; Batra, Vineeta V; Gupta, Rakesh; Sharma, M C; Narang, Poonam; Pandey, P N

    2016-07-01

    We report the case of a 13-year-old girl presenting with left-sided hemiparesis, altered sensorium and episodic headache with bouts of projectile vomiting. Imaging revealed a large heterodense intraventricular mass lesion displaying focal calcification and hyperintensity on T1- and T2- weighted fluid attenuated inversion recovery (FLAIR) magnetic resonance images suggesting the presence of intratumoral fat. Histologically, the tumour showed sheets of glial cells, focal perithelial rosettes and individual cells showing fat vacuoles. The morphological impression was of an ependymoma with lipomatous differentiation. Glial fibrillary acid protein (GFAP) immunohistochemistry revealed positivity in the cytoplasmic processes of the tumour cells as well as in the cytoplasmic rim of the cells having an adipocytic appearance. S100 and vimentin were also immunoreactive. Ultrastructural studies confirmed the ependymal differentiation of the tumour and the presence of an osmiophilic fat component confirming the diagnosis. After 1 year of follow-up, the patient presented with similar complaints and MRI evidence of recurrence of the tumour. A comprehensive literature review revealed that half of the reported cases of this pattern recurred suggesting a possibly tenacious clinical course. PMID:26942599

  16. Correlation between clinicopathological features and karyotype in lipomatous tumors. A report of 178 cases from the Chromosomes and Morphology (CHAMP) Collaborative Study Group.

    PubMed Central

    Fletcher, C. D.; Akerman, M.; Dal Cin, P.; de Wever, I.; Mandahl, N.; Mertens, F.; Mitelman, F.; Rosai, J.; Rydholm, A.; Sciot, R.; Tallini, G.; van den Berghe, H.; van de Ven, W.; Vanni, R.; Willen, H.

    1996-01-01

    Soft tissue tumors commonly show cytogenetic abnormalities, some of which are tumor specific. Lipomatous tumors represent the largest category of soft tissue neoplasms, and numerous karyotypic aberrations have been identified. However, clear-cut correlation between morphology and karyotype has not been undertaken on a systematic basis in a double-blind setting. The morphological features and histological diagnosis of 178 lipomatous neoplasms were reviewed independently without knowledge of the clinical data. The consensus diagnoses were then correlated with the clinical findings and compared with the tumors' karyotypes, using G-banded preparations from short-term cultures. The data were collated by a multicenter collaborative group of pathologists, geneticists, and surgeons. Clonal chromosomal abnormalities were identified in 149 cases studied (84%) and, to a large extent, the karyotype correlated with the morphological diagnosis. Specifically, 26 (96%) of 27 myxoid liposarcomas and its poorly differentiated variants showed a t(12;16); 29 (78%) of 37 atypical lipomatous tumors (including 5 dedifferentiated cases) showed ring chromosomes; 74 (80%) of 93 subcutaneous and intramuscular lipomas had karyotypic aberrations affecting mainly 12q, 6p, and 13q; 7 of 8 spindle cell and pleomorphic lipomas had aberrations of 16q; 3 lipoblastomas showed 8q rearrangements; and 2 hibernomas showed 11q abnormalities. We conclude that cytogenetic abnormalities are common in lipomatous tumors, correlate reliably with morphological sub-type in many cases, and can be of diagnostic value in histologically borderline or difficult cases. PMID:8579124

  17. Midline craniofacial malformations with a lipomatous cephalocele are associated with insufficient closure of the neural tube in the tuft mouse

    PubMed Central

    Fong, Keith S. K.; Adachi, Dana A. T.; Chang, Shaun B.; Lozanoff, Scott

    2015-01-01

    Genetic variations affecting neural tube closure along the head result in malformations to the face and brain, posing a significant impact on health care costs and the quality of life. We have established a mouse line from a mutation that arose spontaneously in our wildtype colony that we called tuft. Tuft mice have heritable midline craniofacial defects featuring an anterior lipomatous cephalocele. Whole mount skeletal stains indicated that affected newborns had a broader interfrontal suture where the cephalocele emerged between the frontal bones. Mice with a cephalocele positioned near the rostrum also presented craniofacial malformations such as ocular hypertelorism and midfacial cleft of the nose. Gross and histological examination revealed that the lipomatous cephalocele originated as a fluid filled cyst no earlier than E14.5 while embryos with a midfacial cleft was evident during craniofacial development at E11.5. Histological sections of embryos with a midfacial cleft revealed the cephalic neuroectoderm remained proximal or fused to the frontonasal ectoderm about the closure site of the anterior neuropore, indicating a defect to neural tube closure. We found the neural folds along the rostrum of E9-10.5 embryos curled inward and failed to close as well as embryos with exencephaly and anencephaly at later stages. Whole mount in situ hybridization of anterior markers Fgf8 and Shh indicated closure of the rostral site was compromised in severe cases. We present a model demonstrating how anterior cranial cephaloceles are generated following a defect to neural tube closure and relevance to subsequent craniofacial morphogenesis in the tuft mouse. PMID:24931720

  18. Detection of MDM2/CDK4 amplification in lipomatous soft tissue tumors from formalin-fixed, paraffin-embedded tissue: comparison of multiplex ligation-dependent probe amplification (MLPA) and fluorescence in situ hybridization (FISH).

    PubMed

    Creytens, David; van Gorp, Joost; Ferdinande, Liesbeth; Speel, Ernst-Jan; Libbrecht, Louis

    2015-02-01

    In this study, the detection of MDM2 and CDK4 amplification was evaluated in lipomatous soft tissue tumors using multiplex ligation-dependent probe amplification (MLPA), a PCR-based technique, in comparison with fluorescence in situ hybridization (FISH). These 2 techniques were evaluated in a series of 77 formalin-fixed, paraffin-embedded lipomatous tumors (27 benign adipose tumors, 28 atypical lipomatous tumors/well-differentiated liposarcomas, 18 dedifferentiated liposarcomas, and 4 pleomorphic liposarcomas). Using MLPA, with a cut-off ratio of >2, 36/71 samples (22 atypical lipomatous tumors/well-differentiated liposarcomas, and 14 dedifferentiated liposarcomas) showed MDM2 and CDK4 amplification. Using FISH as gold standard, MLPA showed a sensitivity of 90% (36/40) and a specificity of 100% (31/31) in detecting amplification of MDM2 and CDK4 in lipomatous soft tissue tumors. In case of high-level amplification (MDM2-CDK4/CEP12 ratio >5), concordance was 100%. Four cases of atypical lipomatous tumor/well-differentiated liposarcoma (4/26, 15%) with a low MDM2 and CDK4 amplification level (MDM2-CDK4/CEP12 ratio ranging between 2 and 2.5) detected by FISH showed no amplification by MLPA, although gain of MDM2 and CDK4 (ratios ranging between 1.6 and 1.9) was seen with MLPA. No amplification was detected in benign lipomatous tumors and pleomorphic liposarcomas. Furthermore, there was a very high concordance between the ratios obtained by FISH and MLPA. In conclusion, MLPA proves to be an appropriate and straightforward technique for screening MDM2/CDK4 amplification in lipomatous tumors, especially when a correct cut-off value and reference samples are chosen, and could be considered a good alternative to FISH to determine MDM2 and CDK4 amplification in liposarcomas. Moreover, because MLPA, as a multiplex technique, allows simultaneous detection of multiple chromosomal changes of interest, it could be in the future a very reliable and fast molecular analysis on

  19. Lipomatous Atrial Septal Hypertrophy: A Review of Its Anatomy, Pathophysiology, Multimodality Imaging, and Relevance to Percutaneous Interventions.

    PubMed

    Laura, Diana M; Donnino, Robert; Kim, Eugene E; Benenstein, Ricardo; Freedberg, Robin S; Saric, Muhamed

    2016-08-01

    Lipomatous atrial septal hypertrophy (LASH) is a histologically benign cardiac lesion characterized by excessive fat deposition in the region of the interatrial septum that spares the fossa ovalis. The etiology of LASH remains unclear, though it may be associated with advanced age and obesity. Because of the sparing of the fossa ovalis, LASH has a pathognomonic dumbbell shape. LASH may be mistaken for various tumors of the interatrial septum. Histologically, LASH is composed of both mature and brown (fetal) adipose tissue, but the role of brown adipose tissue remains unclear. In interventional procedures requiring access to the left atrium, LASH may interfere with transseptal puncture, as traversing the thickened area can reduce the maneuverability of catheters and devices. This may cause the needle to enter the epicardial space, causing dangerous pericardial effusions. LASH was once considered a contraindication to percutaneous device closure of atrial septal defects because of an associated increased risk for incorrect device deployment. However, careful attention to preprocedural imaging and procedural intracardiac echocardiography enable interventional cardiologists to perform procedures in patients with LASH without serious complications. In this review article, the authors describe anatomic and functional aspects of LASH, with emphasis on their roles in percutaneous interventions.

  20. Persistent high grade flurodeoxyglucose uptake in lipomatous hypertrophy of the interatrial septum on dual time point imaging and with ambient warming.

    PubMed

    Kamaleshwaran, K K; Sudhakar, N; Shibu, D K; Kurup, E R R; Shinto, Ajit

    2014-01-01

    Lipomatous hypertrophy of the interatrial septum (LHIS) is a relatively uncommon disorder of the heart characterized by benign fatty infiltration of the interatrial septum that usually spares the fossa ovalis. LHIS showing flurodeoxyglucose uptake has been reported, and is presumed to be due to activated brown adipose tissue (BAT). We here report a case of a patient who had isolated mediastinal uptake in interatrial septum, mimicking metastasis. Rescanning with external warming to deactivate BAT and a delayed time point image was done, which showed persistent and progressively increasing metabolic uptake respectively, suggesting that LHIS uptake might be unrelated to activated BAT or inflammation.

  1. Persistent High Grade Flurodeoxyglucose Uptake in Lipomatous Hypertrophy of the Interatrial Septum on Dual Time Point Imaging and with Ambient Warming

    PubMed Central

    Kamaleshwaran, K. K.; Sudhakar, N.; Shibu, D. K.; Kurup, E. R. R.; Shinto, Ajit

    2014-01-01

    Lipomatous hypertrophy of the interatrial septum (LHIS) is a relatively uncommon disorder of the heart characterized by benign fatty infiltration of the interatrial septum that usually spares the fossa ovalis. LHIS showing flurodeoxyglucose uptake has been reported, and is presumed to be due to activated brown adipose tissue (BAT). We here report a case of a patient who had isolated mediastinal uptake in interatrial septum, mimicking metastasis. Rescanning with external warming to deactivate BAT and a delayed time point image was done, which showed persistent and progressively increasing metabolic uptake respectively, suggesting that LHIS uptake might be unrelated to activated BAT or inflammation. PMID:25191115

  2. Malignant fat-forming solitary fibrous tumor (so-called "lipomatous hemangiopericytoma"): clinicopathologic analysis of 14 cases.

    PubMed

    Lee, Jen-Chieh; Fletcher, Christopher D M

    2011-08-01

    Fat-forming solitary fibrous tumor is a rare variant of solitary fibrous tumor (SFT). Generally regarded as benign, very few fat-forming SFTs with malignant histologic features have been reported. Here, we report 14 histologically malignant fat-forming SFTs to better characterize this subset. Seven patients were female and 7 were male, with ages ranging 20 to 93 years (median, 57 y). Five tumors were located in the lower limb, 3 in the trunk, 3 in abdominopelvic locations, 2 in the head and neck region, and 1 in the upper limb. The tumor size ranged from 3.4 to 20 cm (median, 8.6 cm). Histologically, all exhibited at least focal hypercellularity; 12 tumors had mitoses >4/10 high-power fields (range, 2 to 37; median, 8), 12 showed at least moderate atypia, and 8 showed necrosis. It should be noted that 7 tumors contained only mature adipose tissue, whereas 5 contained multivacuolated lipoblasts and 2 had areas resembling atypical lipomatous tumor (ALT). Immunohistochemically, CD34 and CD99 were positive in most cases (11 of 14 and 8 of 10, respectively); MDM2 and CDK4 were both negative in all 4 cases tested (including both tumors with ALT-like areas). Follow-up data from 10 cases (median duration, 47.5 mo; range, 5 to 76) showed 2 patients with multiple metastases (both to lung and bones, and 1 each to breast and to soft tissue), both of whom died of disease. In conclusion, fat-forming SFTs exhibiting malignant histologic features have potential for aggressive behavior. The presence of lipoblasts and/or ALT-like areas, although described in some "benign" examples of fat-forming SFT, seems much more common in the malignant subset and may prompt a careful search for morphologic evidence of malignancy in any case of fat-forming SFT.

  3. Atypical lipomatous tumor/"well-differentiated liposarcoma" of the skin clinically presenting as a skin tag: clinicopathologic, immunohistochemical, and molecular analysis of 2 cases.

    PubMed

    Paredes, Bruno E; Mentzel, Thomas

    2011-08-01

    Liposarcomas are extremely rare in the skin. When they involve the skin, it is usually by upward spread from a subcutaneous or deeper seated liposarcoma. Very rarely, liposarcoma metastasize to the skin or arise as a primary dermal lesion. We describe 2 cases of atypical lipomatous tumor "well-differentiated liposarcoma" located in dermis. Both presented clinically as a skin tag. The neoplasms arose in a 56-year-old female and a 69-year-old male patient. Both lesions were treated by excision and reexcision. In addition to classical morphology of atypical lipomatous tumor with evidence of lipoblasts and atypical adipocytes, immunohistochemistry with nuclear murine double-minute type 2 protein and cyclin-dependent kinase-4 expression as well as fluorescence in situ hybridization analysis showing an amplification of murine double-minute type 2 protein and cyclin-dependent kinase-4 were helpful to establish the diagnosis. None of the cases recurred after surgical treatment. These 2 cases show the importance of not to misdiagnose lesions which clinically may appear to be benign.

  4. Atypical lipomatous tumor/"well-differentiated liposarcoma" of the skin clinically presenting as a skin tag: clinicopathologic, immunohistochemical, and molecular analysis of 2 cases.

    PubMed

    Paredes, Bruno E; Mentzel, Thomas

    2011-08-01

    Liposarcomas are extremely rare in the skin. When they involve the skin, it is usually by upward spread from a subcutaneous or deeper seated liposarcoma. Very rarely, liposarcoma metastasize to the skin or arise as a primary dermal lesion. We describe 2 cases of atypical lipomatous tumor "well-differentiated liposarcoma" located in dermis. Both presented clinically as a skin tag. The neoplasms arose in a 56-year-old female and a 69-year-old male patient. Both lesions were treated by excision and reexcision. In addition to classical morphology of atypical lipomatous tumor with evidence of lipoblasts and atypical adipocytes, immunohistochemistry with nuclear murine double-minute type 2 protein and cyclin-dependent kinase-4 expression as well as fluorescence in situ hybridization analysis showing an amplification of murine double-minute type 2 protein and cyclin-dependent kinase-4 were helpful to establish the diagnosis. None of the cases recurred after surgical treatment. These 2 cases show the importance of not to misdiagnose lesions which clinically may appear to be benign. PMID:21358383

  5. Lipomatous hemangiopericytoma: a fat-containing variant of solitary fibrous tumor? Clinicopathologic, immunohistochemical, and ultrastructural analysis of a series in favor of a unifying concept.

    PubMed

    Guillou, L; Gebhard, S; Coindre, J M

    2000-09-01

    The dinicopathologic, immunohistochemical and ultrastructural features of 13 lipomatous hemangiopericytomas are presented. There were 6 male and 7 female patients whose ages at diagnosis ranged from 27 to 75 years (median 48) all presenting with a mass of variable duration. The tumor sizes ranged from 1.7 cm to 19 cm (median 5.5 cm). The locations included the orbit (1), neck (1), mediastinum (1), epicardium (1), retroperitoneum (3), right iliac fossa (1), and upper (1) and lower (4) extremity. Histologically, the lesions were composed of a varying admixture of spindle-shaped to round cells, variably collagenous stroma, adipose tissue, and branched, often thick-walled, hemangiopericytoma-like vessels. For 11 tumors, the mitotic activity ranged from 1 to 3 mitoses per 10 high-power fields (HPF). One tumor which contained hypercellular areas showed 13 mitoses per 10 HPF, and another hypercellular lesion showed up to 43 mitoses per 10 HPF, abnormal mitoses, and necrosis. Immunohistochemically, tumor cells were invariably positive for vimentin and CD99, and mostly for CD34 but negative for desmin, keratin, CD31, CD117 (c-kit), and inhibin. About half of the tumors showed reactivity for bcl-2. Occasionally, focal reactivity was also observed for smooth muscle actin, muscle-specific actin, S100 protein, and epithelial membrane antigen. Ultrastructural examination of seven cases showed features in keeping with fibroblastic, myofibroblastic, or pericytic differentiation. Treatment consisted of simple tumorectomy in 10 cases and wide excision in 3. Follow-up information on 10 patients (range: 6 to 77 months; median: 18 months) showed no recurrence. Lipomatous hemangiopericytoma which share the clinical, pathologic, immunohistochemical, and ultrastructural features of solitary fibrous tumor (SFT) is likely to represent, in most cases, a fat-containing variant of SFT.

  6. Evaluation of MDM2 and CDK4 amplification by real-time PCR on paraffin wax-embedded material: a potential tool for the diagnosis of atypical lipomatous tumours/well-differentiated liposarcomas.

    PubMed

    Hostein, I; Pelmus, M; Aurias, A; Pedeutour, F; Mathoulin-Pélissier, S; Coindre, J M

    2004-01-01

    Atypical lipomatous tumours/well-differentiated liposarcomas and dedifferentiated liposarcomas are characterized by 12q13-15 region amplification. In contrast, this molecular event has not been reported in benign lipomas. Within the 12q13-15 chromosomal region, the MDM2, SAS, HMGA2, and CDK4 genes are the most frequent targets of amplification. A series of lipomas (36 cases) and liposarcomas (48 cases) was analysed for MDM2 and CDK4 gene amplification by real-time PCR. MDM2 and CDK4 gene amplification was detected in 2.8% and 5.6% of lipomas and 98.2% and 82.4% of liposarcomas, respectively. Moreover, co-amplification of the two genes as well as a higher-level amplification was observed more frequently in dedifferentiated liposarcomas than in atypical lipomatous tumours/well-differentiated liposarcomas. Real-time PCR proved to be a fast and reliable method to characterize lipomas and liposarcomas by quantification of MDM2 and CDK4 gene amplification. It is applicable to paraffin wax-embedded tissues and could be useful when histological diagnosis is difficult.

  7. Fat-forming solitary fibrous tumour (lipomatous haemangiopericytoma) of the spine: case report and literature review.

    PubMed

    Aftab, Syed; Casey, Adrian; Tirabosco, Roberto; Kabir, Syed Rezaul; Saifuddin, Asif

    2010-10-01

    Solitary fibrous tumour (SFT) is a rare soft tissue tumour of uncertain histogenesis and unpredictable biological behaviour, which was first described in the pleura and subsequently in many extra-pleural locations. Fat-forming SFT is a sub-type of SFT and only a handful of cases have been reported in the literature. We present the clinical, radiological and histological features of a case of intraspinal fat-forming SFT, along with a literature review. This is the first known report of a fat-forming SFT in the spine.

  8. Significance of LRP and PPAR-γ Expression in Lipomatous Soft Tissue Tumors

    PubMed Central

    Tajima, Takashi; Morii, Takeshi; Kikuchi, Fumihito; Matsumine, Akihiko; Murata, Hiroaki; Nobuto, Hiroo; Mochizuki, Kazuo

    2010-01-01

    Background: Molecular mechanism of differentiation in lipogenic tumor is still unknown in detail. Low-density lipoprotein receptor-related protein (LRP) and peroxisome proliferator-activated receptor gamma (PPAR-γ), representative regulatory molecules of lipogenic differentiation, have been reported today as multi-functional molecules and to modulate tumorigenesis in various kind of cancer. To date, diagnostic and therapeutic significance of the expression of these molecules in lipogenic tumors are not defined. Methods: The immunohistochemical expression status of LRP and PPAR-γ in various grades of 54 lipogenic tumors was analyzed. Correlation between the expression levels and the differentiation of the tumors was confirmed. For statistical analyses, the Kruskal-Wallis test, the Steel-Dwass test and the Mann–Whitney U test were used. Results: LRP and PPAR-γ expression was detected in 50 (92.6%) and 44 (81.5%) cases, respectively. The expression level in LRP was significantly higher in cases with well differentiated liposarcoma, pleomorphic liposarcoma and dedifferentiated liposarcoma than in lipoma. Compared with lipoma or well differentiated liposarcoma, significant elevation in expression level of PPAR-γ was confirmed in myxoid liposarcoma, pleomorphic liposarcoma, dedifferentiated liposarcoma and the differentiated area of dedifferentiated liposarcoma. Conclusion: The up-regulation of LRP and PPAR-γ in higher grade cases, i.e. less differentiated tumors than in low grade cases was shown, suggesting the candidate role of these molecules as tumor progression modulators rather than regulatory molecules of differentiation in lipogenic tumors. PMID:20224740

  9. Bannayan-Riley-Ruvalcaba syndrome with deforming lipomatous hamartomas in infant - Case report*

    PubMed Central

    Gontijo, Gabriela Maria Abreu; Pinto, Clóvis Antonio Lopes; Rogatto, Silvia Regina; da Cunha, Isabela Werneck; Aguiar Junior, Samuel; Alves, Célia Antônia Xavier de Moraes

    2013-01-01

    Bannayan-Riley-Ruvalcaba Syndrome is a rare condition caused by mutations in the PTEN gene. It displays association of multiple lipomas, macrocephaly, hemangiomas, hamartomatous intestinal polyposis, developmental delay and speckled pigmented maculae on the male genitalia. We report the case of a nine-month-old boy who had fast growing and progressive tumors for three months, macrocephaly and lentigines on the penis. Imaging tests showed extensive lipomatosis with invasion of paraspinal muscles, enlargement of the spinal canal and spinal cord compression; after surgical excision of the mass, the pathology was consistent with lipoma. Adipocyte culture karyotype demonstrated PTEN mutation. We present this case for its rarity and exuberance. PMID:24474112

  10. [Report of a spindle cell myoepithelialioma of the minor salivary glands with extensive lipomatous component].

    PubMed

    Kern, M A; Kasper, H U; Drebber, U; Guntinas-Lichius, O; Veelken, F; Ortmann, M

    2005-06-01

    Myoepitheliomas of the salivary glands are rare benign tumors composed of spindle-shaped myoepithelial cells, but may show plasmacytoid, epitheloid and clear cell-types that principally exhibit myoepithelial but not ductal differentiation. These tumors are mainly located in the major salivary glands and have sometimes abundant, acellular, mucoid or hyaline stroma but lack chondroid and myxochondroid foci, probably representing the one end of the spectrum of pleomorphic adenoma. Lipomatosis in the form of isolated small islands or scattered single lipocytes, is quite uncommon, and a large amount of adipose tissue in a pleomorphic adenoma and myoepithelioma is a rarity and only described in major salivary glands. We present the case report of a 38-year old man with a myoepithelioma of the minor palatinal salivary glands with extensive lipomatosis as an example of this rare phenomena.

  11. Malignant fat-forming solitary fibrous tumor (lipomatous hemangiopericytoma) in the neck: Imaging and histopathological findings of a case.

    PubMed

    de Carvalho, Alice Duarte; Abrahão-Machado, Lucas Faria; Viana, Cristiano Ribeiro; de Castro Capuzzo, Renato; Mamere, Augusto Elias

    2013-03-01

    Fat-forming solitary fibrous tumor (SFT) is a rare variant of solitary fibrous tumor, a mesenchymal fibroblastic neoplasia with a particular branching hypervascular pattern. This tumor is usually classified as benign and only very few fat-forming SFTs with malignant histologic features have been reported. We report a histologically malignant fat-forming solitary fibrous tumor in a 61-year-old man, located in his neck. Ultrasonography examination was first performed showing a heterogeneous lesion, predominantly hyperechoic, with sound beam attenuation, containing two hypoechoic solid nodules. Magnetic resonance imaging and computed tomography examinations demonstrated a heterogeneous and predominantly adipose mass, containing post contrast enhancing solid nodules and thin septations. Treatment consisted of total removal of the lesion. Histologically, the tumor showed hypercellularity, numerous mitoses and cytological atypia, fulfilling the criteria for malignancy. The patient had no metastasis. This rare tumor may be confused with other fat-containing lesions on imaging examinations, mainly liposarcoma.

  12. Oral soft-tissue angiolipoma: report of two cases of rare oral lipomatous lesion with emphasis on morphological and immunohistochemical features.

    PubMed

    Silva-Junior, Geraldo O; Picciani, Bruna L; Costa, Raphael C; Barbosa, Saulo M; Silvares, Marcelo G; Souza, Ramiro B; Cantisano, Marília H; Pires, Fabio R

    2013-03-01

    Oral angiolipomas are exceedingly rare and little is known about their morphological and etiological features. Here, we report two cases of oral angiolipoma and discuss their clinicopathological and immunohistochemical features, focusing on endothelial markers. Both lesions presented mature adipocytes interspersed by small blood vessels containing fibrin thrombi. Immunohistochemical analysis showed numerous mast cells and expression of CD34, vascular endothelial growth factor, intercellular adhesion molecule-1, interferon-γ and interleukin 6 in most endothelial and stromal cells. Mast cell-endothelial cell interaction may be responsible for the reactive or neoplastic origin of the vascular proliferation of these entities.

  13. Multiple Eruptive Dermatofibromas Masquerading as Cutaneous Lymphoma

    PubMed Central

    Monteiro, Rowan; Aithal, Vijay; Tirumalae, Rajalakshmi

    2016-01-01

    A 34-year-old male came with complaints of multiple firms to hard nontender, nonitchy nodules, measuring 2 cm × 2 cm to 1 cm × 1.5 cm, present predominantly over the lower limbs. This condition started 5 years back as a single nodule over the thigh but gradually increased in number and size with time to involve both lower limbs. A differential diagnosis of cutaneous lymphoma and dermatofibroma was considered. He underwent a biopsy and immunohistochemistry for the same which were consistent with hemosiderotic histiocytoma and positive for vimentin. The patient was advised surgical excision for the same. The occurrence of multiple dermatofibromas although rare has been reported in a few case reports; however, the occurrence of multiple dermatofibromas of the hemosiderotic variant has not been documented yet. PMID:27688463

  14. Multiple Eruptive Dermatofibromas Masquerading as Cutaneous Lymphoma.

    PubMed

    Monteiro, Rowan; Aithal, Vijay; Tirumalae, Rajalakshmi

    2016-01-01

    A 34-year-old male came with complaints of multiple firms to hard nontender, nonitchy nodules, measuring 2 cm × 2 cm to 1 cm × 1.5 cm, present predominantly over the lower limbs. This condition started 5 years back as a single nodule over the thigh but gradually increased in number and size with time to involve both lower limbs. A differential diagnosis of cutaneous lymphoma and dermatofibroma was considered. He underwent a biopsy and immunohistochemistry for the same which were consistent with hemosiderotic histiocytoma and positive for vimentin. The patient was advised surgical excision for the same. The occurrence of multiple dermatofibromas although rare has been reported in a few case reports; however, the occurrence of multiple dermatofibromas of the hemosiderotic variant has not been documented yet. PMID:27688463

  15. Multiple Eruptive Dermatofibromas Masquerading as Cutaneous Lymphoma

    PubMed Central

    Monteiro, Rowan; Aithal, Vijay; Tirumalae, Rajalakshmi

    2016-01-01

    A 34-year-old male came with complaints of multiple firms to hard nontender, nonitchy nodules, measuring 2 cm × 2 cm to 1 cm × 1.5 cm, present predominantly over the lower limbs. This condition started 5 years back as a single nodule over the thigh but gradually increased in number and size with time to involve both lower limbs. A differential diagnosis of cutaneous lymphoma and dermatofibroma was considered. He underwent a biopsy and immunohistochemistry for the same which were consistent with hemosiderotic histiocytoma and positive for vimentin. The patient was advised surgical excision for the same. The occurrence of multiple dermatofibromas although rare has been reported in a few case reports; however, the occurrence of multiple dermatofibromas of the hemosiderotic variant has not been documented yet.

  16. Vertebral choristoma in lipomyelomeningocele: a case report.

    PubMed

    Joshua, S P; Singh, Pankaj Kumar; Mahapatra, A K

    2012-01-01

    Lipomeningocele is a type of occult spinal dysraphism characterized by a subcutaneous lipomatous mass that protrudes through a midline bony defect. We report a rare presentation of this condition--a vertebral choristoma wherein the lipomatous mass displaced the normally formed posterior elements--lamina and spinous process of L4 vertebra dorsally to an abnormal location in the absence of a bony defect.

  17. Lipomatosis: a diverse form of hemifacial hyperplasia

    PubMed Central

    Umarji, Hemant R.; Arora, Aman; Ramaswami, Easwaran

    2012-01-01

    A case of hemifacial hyperplasia that presented with muscular, skeletal, and dental hyperplasia along with lipomatous infiltration was described. Advanced imaging was useful in identifying the lipomatous infiltration present in the lesion, which raises the possibility of lipomatosis having a diverse presentation in hemifacial hyperplasia. As there was a scarcity of related literature in the field of dentomaxillofacial radiology, this report would make us familiar with its computed tomographic and magnetic resonance image findings. PMID:23071970

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

    ClinicalTrials.gov

    2016-11-04

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

  19. Soft tissue tumors of the head and neck.

    PubMed

    Katenkamp, D

    1987-01-01

    From the tumor register of the Institute of Pathology of Jena all soft tissue tumors of the head and neck collected between 1959 and 1984 were retrieved and reclassified. 562 out of 646 tumors (87%) were benign. Three quarter of these growths could be diagnosed as nerve sheath tumors (schwannomas and neurofibromas), hemangiomas, fibrohistiocytic tumors and lipomas. 84 tumors were malignant (13%). As the most frequent subtypes we found fibrohistiocytic sarcomas (malignant fibrous histiocytomas and atypical fibroxanthomas), muscularly differentiated sarcomas (rhabdo- and leiomyosarcomas) and unclassified sarcomas. The age and sex distribution as well as the localization and histologic peculiarities were analysed and compared with findings reported in the literature. The significance of knowing such data for diagnostic and differential diagnostic considerations is stressed and exemplified. PMID:3592924

  20. A Challenging Giant Dermatofibrosarcoma Protuberans on the Face.

    PubMed

    Pérez, Gimena Castro; Arias, Cintia; Luna, Paula; Sorín, Irene; Mazzuoccolo, Luis Daniel

    2016-01-01

    Dermatofibrosarcoma protuberans (DFSP) is a malignant fibrohistiocytic tumor that appears exclusively on the skin. It is a low-grade malignant soft tissue tumor of subcutaneous tissues that has a propensity for local recurrence but seldom metastasizes. It may rarely occur on the head and neck accounting for less than one percent of total head and neck malignancies. We present a man with a giant DFSP on the face. Oncological, functional, and aesthetic aspects are set forth. PMID:27437151

  1. Fine-needle aspiration of pigmented villonodular synovitis of the temporomandibular joint masquerading as a primary parotid gland lesion.

    PubMed

    Yu, G H; Staerkel, G A; Kershisnik, M M; Varma, D G

    1997-01-01

    The fine-needle aspiration findings in a case of pigmented villonodular synovitis of the temporomandibular joint are presented. The characteristic cytomorphologic and clinical features of this uncommon, benign fibrohistiocytic lesion are discussed. In addition, due to the initial clinical impression of a primary parotid gland lesion, the differential diagnosis for the cytomorphologic features observed (histiocytoid cells admixed with osteoclast-like giant cells) are discussed within the context of a primary salivary gland mass.

  2. The Molecular Signature of HIV-1-Associated Lipomatosis Reveals Differential Involvement of Brown and Beige/Brite Adipocyte Cell Lineages.

    PubMed

    Cereijo, Rubén; Gallego-Escuredo, José Miguel; Moure, Ricardo; Villarroya, Joan; Domingo, Joan Carles; Fontdevila, Joan; Martínez, Esteban; Gutiérrez, Maria del Mar; Mateo, María Gracia; Giralt, Marta; Domingo, Pere; Villarroya, Francesc

    2015-01-01

    Highly active antiretroviral therapy has remarkably improved quality of life of HIV-1-infected patients. However, this treatment has been associated with the so-called lipodystrophic syndrome, which conveys a number of adverse metabolic effects and morphological alterations. Among them, lipoatrophy of subcutaneous fat in certain anatomical areas and hypertrophy of visceral depots are the most common. Less frequently, lipomatous enlargements of subcutaneous fat at distinct anatomic areas occur. Lipomatous adipose tissue in the dorso-cervical area ("buffalo hump") has been associated with a partial white-to-brown phenotype transition and with increased cell proliferation, but, to date, lipomatous enlargements arising in other parts of the body have not been characterized. In order to establish the main molecular events associated with the appearance of lipomatosis in HIV-1 patients, we analyzed biopsies of lipomatous tissue from "buffalo hump" and from other anatomical areas in patients, in comparison with healthy subcutaneous adipose tissue, using a marker gene expression approach. Both buffalo-hump and non-buffalo-hump lipomatous adipose tissues exhibited similar patterns of non-compromised adipogenesis, unaltered inflammation, non-fibrotic phenotype and proliferative activity. Shorter telomere length, prelamin A accumulation and SA-β-Gal induction, reminiscent of adipocyte senescence, were also common to both types of lipomatous tissues. Buffalo hump biopsies showed expression of marker genes of brown adipose tissue (e.g. UCP1) and, specifically, of "classical" brown adipocytes (e.g. ZIC1) but not of beige/brite adipocytes. No such brown fat-related gene expression occurred in lipomatous tissues at other anatomical sites. In conclusion, buffalo hump and other subcutaneous adipose tissue enlargements from HIV-1-infected patients share a similar lipomatous character. However, a distorted induction of white-to-"classical brown adipocyte" phenotype appears unique of

  3. A Benign Cardiac Growth but Not So Indolent

    PubMed Central

    Reddy, Sahadev T.; Biederman, Robert W. W.

    2016-01-01

    Cardiac lipomatous hypertrophy is a rare benign condition that usually involves the interatrial septum. Due to its benign nature it rarely requires intervention. Its presence outside the interatrial septum is reported infrequently. We present a case of lipomatous hypertrophy in the intraventricular septum that was complicated by a severe, symptomatic, and disabling dynamic left ventricular outflow tract obstruction. The symptoms significantly improved following the excision of the mass. In our case transthoracic echocardiogram was used to visualize the mass and measure the severity of the obstruction; Cardiac Magnetic Resonance Imaging was used to characterize the mass and histopathology confirmed the diagnosis. PMID:27293911

  4. The Molecular Signature of HIV-1-Associated Lipomatosis Reveals Differential Involvement of Brown and Beige/Brite Adipocyte Cell Lineages

    PubMed Central

    Cereijo, Rubén; Gallego-Escuredo, José Miguel; Moure, Ricardo; Villarroya, Joan; Domingo, Joan Carles; Fontdevila, Joan; Martínez, Esteban; Gutiérrez, Maria del Mar; Mateo, María Gracia; Giralt, Marta; Domingo, Pere; Villarroya, Francesc

    2015-01-01

    Highly active antiretroviral therapy has remarkably improved quality of life of HIV-1-infected patients. However, this treatment has been associated with the so-called lipodystrophic syndrome, which conveys a number of adverse metabolic effects and morphological alterations. Among them, lipoatrophy of subcutaneous fat in certain anatomical areas and hypertrophy of visceral depots are the most common. Less frequently, lipomatous enlargements of subcutaneous fat at distinct anatomic areas occur. Lipomatous adipose tissue in the dorso-cervical area (“buffalo hump”) has been associated with a partial white-to-brown phenotype transition and with increased cell proliferation, but, to date, lipomatous enlargements arising in other parts of the body have not been characterized. In order to establish the main molecular events associated with the appearance of lipomatosis in HIV-1 patients, we analyzed biopsies of lipomatous tissue from “buffalo hump” and from other anatomical areas in patients, in comparison with healthy subcutaneous adipose tissue, using a marker gene expression approach. Both buffalo-hump and non-buffalo-hump lipomatous adipose tissues exhibited similar patterns of non-compromised adipogenesis, unaltered inflammation, non-fibrotic phenotype and proliferative activity. Shorter telomere length, prelamin A accumulation and SA-β-Gal induction, reminiscent of adipocyte senescence, were also common to both types of lipomatous tissues. Buffalo hump biopsies showed expression of marker genes of brown adipose tissue (e.g. UCP1) and, specifically, of “classical” brown adipocytes (e.g. ZIC1) but not of beige/brite adipocytes. No such brown fat-related gene expression occurred in lipomatous tissues at other anatomical sites. In conclusion, buffalo hump and other subcutaneous adipose tissue enlargements from HIV-1-infected patients share a similar lipomatous character. However, a distorted induction of white-to-“classical brown adipocyte” phenotype

  5. A Benign Cardiac Growth but Not So Indolent.

    PubMed

    Wani, Adil S; Reddy, Sahadev T; Harinath, Lakshmi; Biederman, Robert W W

    2016-01-01

    Cardiac lipomatous hypertrophy is a rare benign condition that usually involves the interatrial septum. Due to its benign nature it rarely requires intervention. Its presence outside the interatrial septum is reported infrequently. We present a case of lipomatous hypertrophy in the intraventricular septum that was complicated by a severe, symptomatic, and disabling dynamic left ventricular outflow tract obstruction. The symptoms significantly improved following the excision of the mass. In our case transthoracic echocardiogram was used to visualize the mass and measure the severity of the obstruction; Cardiac Magnetic Resonance Imaging was used to characterize the mass and histopathology confirmed the diagnosis. PMID:27293911

  6. [Adipocytic tumors].

    PubMed

    Stock, Nathalie

    2015-01-01

    Adipocytic tumors are the most common mesenchymal neoplasms, liposarcoma accounting for approximately 20% of soft tissue sarcomas. The differential diagnosis between benign and malignant tumors is often problematic and represents a significant proportion of consultation cases. The goal of this article is to review liposarcoma subtypes, the main benign adipocytic neoplasms: lipoblastoma, hibernoma, spindle/pleomorphic cell lipoma, chondroid lipoma, as well as non adipocytic neoplasms with a lipomatous component such as lipomatous solitary fibrous tumor, emphasizing on practical differential diagnosis issues, and immunohistochemical and molecular tools allowing their resolution.

  7. The echographic characteristics of fatty tissues and tumors.

    PubMed

    Behan, M; Kazam, E

    1978-10-01

    Fatty tissues and tumors have been described as characteristically cyst-like on ultrasound examination, with relatively few internal echoes. The authors offer several examples of lipomatous masses and tissues which are markedly echogenic. In vitro studies suggest that this is an inherent property of body fat and is not due to coexistent fibrous tissue or tumor vascularity. The echogenicity of fat has important implications for diagnostic ultrasound: (a) the presence of fat in tissues and masses may be demonstrated by ultrasound, and (b) because the high-level internal echoes blend with the echo pattern of bowel gas and retroperitoneal fat, lipomatous abdominal masses may easily be overlooked.

  8. Intramuscular myxoma of the deltoid muscle: report of a case

    PubMed Central

    Costamagna, Daniela; Erra, Stefania; Durando, Riccardo

    2009-01-01

    Intramuscular myxoma is a rare, benign lesion of mesenchymal origin, affecting the skeletal muscles. We report the case of a 75-year-old woman presenting with a mass of the right deltoid region. On the MRI examination it was interpreted as a lipomatous lesion. She underwent marginal excision. The pathological examination revealed the diagnosis of intramuscular myxoma. PMID:21686685

  9. Atypical spindle cell lipoma: a clinicopathologic, immunohistochemical, and molecular study emphasizing its relationship to classical spindle cell lipoma.

    PubMed

    Creytens, David; van Gorp, Joost; Savola, Suvi; Ferdinande, Liesbeth; Mentzel, Thomas; Libbrecht, Louis

    2014-07-01

    We studied a series of spindle cell lipomas arising in atypical sites and showing unusual morphologic features (which we called atypical spindle cell lipoma) to assess if these lesions have the same chromosomal alterations as classical spindle cell lipoma but different from those found in atypical lipomatous tumor/well-differentiated liposarcoma. We investigated alterations of different genes in the 13q14 region and the amplification status of the MDM2 and CDK4 genes at 12q14-15 by multiplex ligation-dependent probe amplification (MLPA) and fluorescence in situ hybridization (FISH) analysis. In the atypical spindle cell lipomas, MLPA revealed deletions in the two nearest flanking genes of RB1 (ITM2B and RCBTB2) and in multiple important exons of RB1. In contrast, in classical spindle cell lipomas, a less complex loss of RB1 exons was found but no deletion of ITM2B and RCBTB2. Moreover, MLPA identified a deletion of the DLEU1 gene, a finding which has not been reported earlier. We propose an immunohistochemical panel for lipomatous tumors which comprises of MDM2, CDK4, p16, Rb, which we have found useful in discriminating between atypical or classical spindle cell lipomas and other adipocytic neoplasms, especially atypical lipomatous tumor/well-differentiated liposarcoma. Our findings strengthen the link between atypical spindle cell lipoma and classical spindle cell lipoma, and differentiate them from atypical lipomatous tumor/well-differentiated liposarcoma.

  10. Rectal angiolipoma: a case report and review of literature.

    PubMed

    Kacar, Sabite; Kuran, Sedef; Temucin, Tulay; Odemis, Bulent; Karadeniz, Nilufer; Sasmaz, Nurgul

    2007-03-01

    Angiolipoma is a rare vascular variant of the benign lipomatous tumors and is generally seen in subcutaneous tissues. We report a 70-year-old female with abdominal distension not related to rectal small polypoid mass with peduncule described as angiolipoma by histologically, and review the literature.

  11. [Spinal epidural angiolipoma: a case report].

    PubMed

    Dufrenot, Leïla; Pelé, Eric; Cursolle, Jean-Christophe; Coindre, Jean-Michel; Lepreux, Sébastien

    2010-02-01

    Spinal epidural angiolipoma is a rare tumor revealed by a slowly progressive paraplegia. We reported a case of a 44-year-old female and point out the peculiar pattern of this lesion characterized by the prominence of the vascular component over the lipomatous component. Recognition of this entity is important because this is a benign and curable cause of paraplegia.

  12. Atypical spindle cell lipoma: a clinicopathologic, immunohistochemical, and molecular study emphasizing its relationship to classical spindle cell lipoma.

    PubMed

    Creytens, David; van Gorp, Joost; Savola, Suvi; Ferdinande, Liesbeth; Mentzel, Thomas; Libbrecht, Louis

    2014-07-01

    We studied a series of spindle cell lipomas arising in atypical sites and showing unusual morphologic features (which we called atypical spindle cell lipoma) to assess if these lesions have the same chromosomal alterations as classical spindle cell lipoma but different from those found in atypical lipomatous tumor/well-differentiated liposarcoma. We investigated alterations of different genes in the 13q14 region and the amplification status of the MDM2 and CDK4 genes at 12q14-15 by multiplex ligation-dependent probe amplification (MLPA) and fluorescence in situ hybridization (FISH) analysis. In the atypical spindle cell lipomas, MLPA revealed deletions in the two nearest flanking genes of RB1 (ITM2B and RCBTB2) and in multiple important exons of RB1. In contrast, in classical spindle cell lipomas, a less complex loss of RB1 exons was found but no deletion of ITM2B and RCBTB2. Moreover, MLPA identified a deletion of the DLEU1 gene, a finding which has not been reported earlier. We propose an immunohistochemical panel for lipomatous tumors which comprises of MDM2, CDK4, p16, Rb, which we have found useful in discriminating between atypical or classical spindle cell lipomas and other adipocytic neoplasms, especially atypical lipomatous tumor/well-differentiated liposarcoma. Our findings strengthen the link between atypical spindle cell lipoma and classical spindle cell lipoma, and differentiate them from atypical lipomatous tumor/well-differentiated liposarcoma. PMID:24659226

  13. Malignant fibrous histiocytomas of salivary glands.

    PubMed

    Benjamin, E; Wells, S; Fox, H; Reeve, N L; Knox, F

    1982-09-01

    The light microscopic, immunohistological and ultrastructural findings in two cases of malignant fibrous histiocytoma arising in salivary glands are presented and the features of seven previously reported cases are reviewed. This neoplasm is extremely rare in this site and may pose problems in diagnosis. It has to be distinguished from other spindled cell tumours, in particular from epithelial tumours of predominantly spindled cell pattern; immunohistological markers for histiocytic cells may be of value. The histogenesis of this neoplasm is controversial but our electron microscopic findings support an origin from mesenchymal cells which differentiate along a broad fibrohistiocytic spectrum.

  14. [An unusual case of massive funicular lipoma].

    PubMed

    Masciovecchio, Stefano; Saldutto, Pietro; Del Rosso, Alessandro; Galatioto, Giuseppe Paradiso; Vicentini, Carlo

    2014-01-01

    Intrascrotal lipomas are benign diseases that originate from adipose tissue inside the scrotal sac or constituting the scrotal wall itself. Paratesticular spermatic cord lipomas are a rare type of intrascrotal lipomas. A 78 years old patient in good health comes to our attention for massive tense-elastic swelling of left hemiscrotum appeared about 3 years ago and slowly increasing. Ultrasound evaluation of the intrascrotal mass showed inhomogeneous images mainly hyperechoic, not infiltrating the ipsilateral testis and scrotal wall. The ultrasonography found a lipomatous lesion with benign characteristics and a surgical excision has been performed. The lipomatous mass removed presented a size equal to 25 cm x 14 cm x 11 cm and a weight of about 490 gr. The microscopic examination of the tissue showed only the presence of mature adipocytes in the absence of cellular atypia, of other cellular types and of vascular abnormalities.

  15. Angiolipoma of the Orbit: A Rare Tumor in an Unusual Location.

    PubMed

    Carruth, Bryant P; Meyer, Dale R

    2015-01-01

    Despite their relatively frequent occurrence on the trunk and extremities, lipomas rarely present in the orbit. Rarer still are variants of lipoma such as fibrolipoma, myxoid lipoma, and angiolipoma. The authors report a 66-year-old woman who presented with a large, slowly growing tumor of the forehead and orbit. The case presentation conforms to the tenets of the Declaration of Helsinki and is HIPAA compliant. Clinical and radiographic evidence suggested a lipomatous type of tumor, and excisional biopsy revealed adipose proliferation with numerous small vessels and fibrin thrombi consistent with angiolipoma. The tumor was completely excised without ophthalmic sequelae or recurrence in 6 months of follow up. To the authors' knowledge, this tumor represents only the second reported case of this type of highly vascularized lipomatous lesion within the orbit. This case is a novel entity in the differential diagnosis of orbital tumors and demonstrates the value of total excision in such cases.

  16. Magnetic resonance imaging of benign soft tissue neoplasms in adults.

    PubMed

    Walker, Eric A; Fenton, Michael E; Salesky, Joel S; Murphey, Mark D

    2011-11-01

    This article reviews a spectrum of benign soft tissue tumors found in adults. Rather than presenting a complete review, the focus of this article is on benign tumors for which the diagnosis may be confidently made or strongly suggested on the basis of imaging. Diagnoses presented include nodular fasciitis, superficial and deep fibromatosis, elastofibroma, lipomatous lesions, giant cell tumor of the tendon sheath, pigmented villonodular synovitis, peripheral nerve sheath tumors, Morton neuroma, hemangioma, and myxoma.

  17. Giant ileocolic intussusception in an adult induced by a double ileal lipoma: a case report with pathologic correlation.

    PubMed

    Kraniotis, Pantelis; Pastromas, Georgios; Tsota, Irene; Patsoura, Maria; Petsas, Theodore

    2016-09-01

    Intussusception in adults is rare, accounting for less than 5% of all cases. Unlike the childhood variant, adult intussusception is often associated with a small bowel lesion acting as the "lead point." We herein report an uncommon case of giant intussusception secondary to 2 separate lipomatous lesions located in the ileum, in an adult admitted to our hospital for acute severe abdominal pain. PMID:27594938

  18. Images of liposarcoma using technetium-99m bleomycin and technetium (V)-99m DMSA

    SciTech Connect

    Ohta, H.; Shane, F.I.; Endo, K.; Torizuka, K.; Horiuchi, K.; Yokoyama, A.; Ishii, M.

    1986-12-01

    The effectiveness of Tc-99m bleomycin (BLM) and Tc(V)-99m DMSA are compared with that of Ga-67 citrate, which is currently the most widely used agent. In four patients with lipomatous tumors, the clinical significance of tumor imaging with each of these three agents is discussed and compared. Results indicate that both Tc-99m BLM and Tc(V)-99m DMSA are superior in detecting the extension or localization of liposarcomas.

  19. In vivo MRI characteristics of lipoma arborescens utilizing fat suppression and contrast administration

    SciTech Connect

    Chaljub, G.; Johnson, P.R.

    1996-01-01

    Lipoma arborescens or villous lipomatous proliferation of the synovium is a rare lesion. We describe the in vivo imaging characteristics of lipoma arborescens of the knee utilizing fat suppression techniques and contrast medium administration. As expected, the lesion demonstrated signal loss when utilizing fat suppression. In addition, no contrast enhancement was noted. When a synovial process demonstrates isointensity with subcutaneous fat on all pulse sequences and no contrast enhancement, lipoma arborescens should be the primary diagnosis consideration. 7 refs., 4 figs.

  20. Dermatofibroma: Atypical Presentations.

    PubMed

    Bandyopadhyay, Mousumi Roy; Besra, Mrinal; Dutta, Somasree; Sarkar, Somnath

    2016-01-01

    Dermatofibroma is a common benign fibrohistiocytic tumor and its diagnosis is easy when it presents classical clinicopathological features. However, a dermatofibroma may show a wide variety of clinicopathological variants and, therefore, the diagnosis may be difficult. The typical dermatofibroma generally occurs as a single or multiple firm reddish-brown nodules. We report here two atypical presentations of dermatofibroma - Atrophic dermatofibroma and keloidal presentation of dermatofibroma. Clinical dermal atrophy is a common phenomenon in dermatofibromas as demonstrated by the dimpling on lateral pressure. However, this feature is exaggerated in the atrophic variant of dermatofibroma. Atrophic dermatofibroma is defined by dermal atrophy of more than 50% of the lesion apart from the usual features of common dermatofibroma. The keloidal variant of dermatofibroma should not be overlooked as a simple keloid. The findings of keloidal change in dermatofibromas may support that trauma is a possible cause of dermatofibroma. PMID:26955137

  1. Calcinosis cutis following contact with calcium chloride solution.

    PubMed

    Lim, Penny P L; Kossard, Steven; Stapleton, Karen

    2012-11-01

    Calcinosis cutis is the deposition of insoluble calcium in the cutaneous tissue. Calcinosis cutis can be classified as metastatic, dystrophic, idiopathic or exogenous. We report a 48-year-old white man who was dismantling a portable ice skating rink when calcium chloride solution from the pipes spilt onto his clothing. Several days later, he started to develop mildly pruritic erythematous papules, some studded with white deposits and some with umbilication over the exposed areas corresponding to the spillage of the calcium chloride solution. Histological features revealed interstitial fibrohistiocytic reaction with calcium-encrusted degenerated collagen bundles in the dermis which was further confirmed by von Kossa stain. He was commenced on topical corticosteroid cream twice daily and the lesions cleared completely between 6 to 10 weeks.

  2. Paediatric Primary Pachymeningeal Xanthogranuloma with Scattered Foci Displaying Reticulohistiocytoma-like Features

    PubMed Central

    Salazar, Miguel Fdo.; Estrada Hernández, María del Rocío; Gómez Apo, Erick; Chávez Macías, Laura G.; Rodríguez Álvarez, Carlos Alfonso

    2015-01-01

    We report a unique case of a 4-year-old girl with an intriguing fibrohistiocytic tumour. Magnetic resonance imaging scans showed a dural mass of variegated intensity compressing the left occipital pole and apparently extending toward the superior sagittal sinus. Grossly, the cut surface of the surgical specimen was yellow, pale, and soft with reddish kernel-like crusts. Histologically, the yellow areas resembled cholesterol granulomas with widespread coagulative necrosis, cholesterol clefts, powdery calcification, foreign body-type giant cells, and foamy macrophages, while the scattered red spots contained numerous multinucleated giant cells of foreign-body and Touton types, the former with amphophilic to slightly eosinophilic cytoplasm. Immunoperoxidase reactions confirmed the expression of histiocytic markers and vimentin. As far as we know, no tumour displaying these peculiar morphological features has yet been described. PMID:26081827

  3. Calcinosis cutis following contact with calcium chloride solution.

    PubMed

    Lim, Penny P L; Kossard, Steven; Stapleton, Karen

    2012-11-01

    Calcinosis cutis is the deposition of insoluble calcium in the cutaneous tissue. Calcinosis cutis can be classified as metastatic, dystrophic, idiopathic or exogenous. We report a 48-year-old white man who was dismantling a portable ice skating rink when calcium chloride solution from the pipes spilt onto his clothing. Several days later, he started to develop mildly pruritic erythematous papules, some studded with white deposits and some with umbilication over the exposed areas corresponding to the spillage of the calcium chloride solution. Histological features revealed interstitial fibrohistiocytic reaction with calcium-encrusted degenerated collagen bundles in the dermis which was further confirmed by von Kossa stain. He was commenced on topical corticosteroid cream twice daily and the lesions cleared completely between 6 to 10 weeks. PMID:23157788

  4. Pleomorphic hyalinizing angiectatic tumor of soft parts: case report with unusual ganglion-like cells and review of the literature.

    PubMed

    Changchien, Yi-Che; Bocskai, Pál; Kovács, Ilona; Hargitai, Zoltán; Kollár, Sándor; Török, Miklós

    2014-12-01

    Pleomorphic hyalinizing angiectatic tumor (PHAT) is a recently described, non-metastasizing tumor of uncertain lineage. This tumor distributes equally between the genders and has a predilection for the subcutaneous soft tissue, particularly in lower extremity, other locations are rare. Based on the recent literature, PHAT is suspected to encompass the morphological spectrum with other tumors such as myxoinflammatory fibroblastic sarcoma (MIFS) and hemosiderotic fibrolipomatous tumor (HFLT), although cytogenetic data remain inconsistent. We report a case of PHAT that arose in the upper arm with unusual morphology which showed ganglion-like cells similar to Reed-Sternberg-like cells found in MIFS. The tumor had strong immunohistochemical expression of CD34, CD99, and was negative for S-100. The ganglion-like cells were positive for both CD34 and CD68 but negative for CD30. The translocation between chromosome 1 and 10, a frequent finding of MIFS and HFLT, was not identified by FISH excluding the possibility of hybrid PHAT and MIFS. We conclude FISH can be a potential useful tool to separate PHAT with atypical morphology from hybrid tumor in doubted cases. Due to the rarity of PHAT and lack of consistent pathogenetic signatures, more cases and further studies will be needed to elucidate the pathogenesis and nature of this tumor.

  5. Identification of COL3A1 and RAB2A as novel translocation partner genes of PLAG1 in lipoblastoma.

    PubMed

    Yoshida, Hideki; Miyachi, Mitsuru; Ouchi, Kazutaka; Kuwahara, Yasumichi; Tsuchiya, Kunihiko; Iehara, Tomoko; Konishi, Eiichi; Yanagisawa, Akio; Hosoi, Hajime

    2014-07-01

    Lipoblastoma is a rapidly growing, benign neoplasm in children. Surgical excision is usually curative, with a recurrence rate of about 20%. Because the histology of lipoblastoma is heterogeneous and overlaps with other lipomatous tumors, some lipoblastoma cases have been difficult to diagnose. The detection of PLAG1 gene rearrangement is useful for the diagnosis of lipoblastoma. Three fusion partner genes are known in relation to PLAG1 in lipoblastoma HAS2 at 8q24.1, COL1A2 at 7q22, and RAD51L1 at 14q24. Herein, we describe another two novel fusion genes in lipoblastoma tumor specimens. We checked six tumors for the presence of two known fusion genes, HAS2-PLAG1 and COL1A2-PLAG1. Only HAS2-PLAG1 was found in one of the cases. Next, we attempted to identify potential PLAG1 fusion partners using 5'RACE. Sequence analysis revealed two novel fusion genes, COL3A1-PLAG1 in three cases and RAB2A-PLAG1 in one case, respectively. As a result of the translocations, the constitutively active promoter of the partner gene drives the ectopic expression of PLAG1. We also evaluated whether a high level of PLAG1 expression can be used to help differentiate lipomatous tumors. PLAG1 expression was evaluated by real-time PCR in five lipoblastoma tumor specimens. The expressions were 70-150 times higher in lipoblastomas than in human adipocytes. However, PLAG1 expression was low in one case of lipoma. These results demonstrate that PLAG1 overexpression is a potential marker of lipoblastoma. Our findings, in agreement with previous studies, show that lipoblastoma is a group of lipomatous tumors with PLAG1 rearrangement and overexpression. © 2014 Wiley Periodicals, Inc. PMID:24700772

  6. Liposarcoma of the maxillary antrum: A case report.

    PubMed

    Priyal, G; Shakil, Moidin; Jose, Maji; Hussain, Akther

    2015-01-01

    Atypical Lipomatous Tumor/Well-Differentiated Liposarcoma. (ALT/WDL) is a soft tissue sarcoma of intermediate malignant behavior, which most frequently affects the retroperitoneum and lower extremities. Liposarcomas of head and neck are rare, representing only 2-8% of all sarcomas in this region. The majority of liposarcomas occur in middle-aged adults; however, very uncommonly cases have been reported in infancy and early childhood. We report a case of a 14-year-old girl diagnosed as ALT/WDL of the maxillary antrum. PMID:26881583

  7. Dural arteriovenous fistula coexisting with a lumbar lipomeningocele. Case report.

    PubMed

    Rajeev, Kariyattil; Panikar, Dilip

    2005-11-01

    The authors describe the case of a 44-year-old woman who presented with recent onset of progressive paraparesis and bladder involvement; she had an asymptomatic lumbosacral lipomatous swelling that was present since birth. Magnetic resonance imaging confirmed the diagnosis of a lipomeningocele. It also revealed intramedullary hyperintensity on T2-weighted images and serpiginous flow voids suggestive of a dural arteriovenous fistula (DAVF) at the same level; the lesion was confirmed by spinal angiography. Both lesions were surgically managed, and the patient subsequently experienced neurological improvement. The coexistence of a DAVF and a lipomeningocele at the same level is unusual and can lead to treatment failure if missed.

  8. [Hibernoma: brown fat retroperitoneal tumor. Report of a pediatric case].

    PubMed

    Collado, Laura; Sierre, Sergio; Bosalec, Andrea; Lipsich, José

    2011-12-01

    Hibernoma is a rare benign tumor of soft tissue, composed of brown fat. This tissue is predominant in hibernating animals and hence its name. Because of its rarity in Pediatrics and difficult diagnosis, we report a 3 month-old patient with a diagnosis consistent with an abdominal tumor. Ultrasound and computed tomography exams showed an infiltrative retroperitoneal tumor, with hypervascular and lipomatous features. After tumor excision, histopathological exam confirmed the diagnosis of hibernoma or brown fat tumor. This presentation describes the characteristics of this type of tumor, rare in children, and reviews the fatty tumors, according to their frequency in pediatric patients.

  9. Peripheral nerve lipoma: Case report of an intraneural lipoma of the median nerve and literature review

    PubMed Central

    Teles, Alisson Roberto; Finger, Guilherme; Schuster, Marcelo N.; Gobbato, Pedro Luis

    2016-01-01

    Adipose lesions rarely affect the peripheral nerves. This can occur in two different ways: Direct compression by an extraneural lipoma, or by a lipoma originated from the adipose cells located inside the nerve. Since its first description, many terms have been used in the literature to mention intraneural lipomatous lesions. In this article, the authors report a case of a 62-year-old female who presented with an intraneural median nerve lipoma and review the literature concerning the classification of adipose lesions of the nerve, radiological diagnosis and treatment. PMID:27695575

  10. Clinics in diagnostic imaging (169)

    PubMed Central

    Venkatesh, Sandeep Halagatti; Wong, Bak Siew Steven

    2016-01-01

    A 54-year-old woman presented with a large mass on her right hand that was progressively enlarging over a period of a few years. She had cosmetic concerns about the enlarging mass and experienced interference with routine activity. Physical examination revealed a soft, mobile, non-tender, lobulated mass with well-defined margins. Magnetic resonance imaging showed the soft lump to be a lipomatous mass in the deep palmar space of the hand, which was subsequently surgically resected. The imaging features of deep palmar lipomas of the hand and other common benign lesions at this location are discussed. PMID:27549159

  11. Peripheral nerve lipoma: Case report of an intraneural lipoma of the median nerve and literature review

    PubMed Central

    Teles, Alisson Roberto; Finger, Guilherme; Schuster, Marcelo N.; Gobbato, Pedro Luis

    2016-01-01

    Adipose lesions rarely affect the peripheral nerves. This can occur in two different ways: Direct compression by an extraneural lipoma, or by a lipoma originated from the adipose cells located inside the nerve. Since its first description, many terms have been used in the literature to mention intraneural lipomatous lesions. In this article, the authors report a case of a 62-year-old female who presented with an intraneural median nerve lipoma and review the literature concerning the classification of adipose lesions of the nerve, radiological diagnosis and treatment.

  12. Sialolipoma of the parotid gland.

    PubMed

    Michaelidis, Ioannis George-Akrivos; Stefanopoulos, Panagiotis Konstantinos; Sambaziotis, Demetrios; Zahos, Marios Alexandros; Papadimitriou, George Alexandros

    2006-01-01

    A case of slow-enlarging mass of the parotid region in a 44-year-old male is presented, which proved to be a lipomatous tumour of the parotid gland. The clinical impression was that of a benign salivary gland tumour. The tumour was situated in the deep lobe of the gland, thus a total parotidectomy was performed, with preservation of the facial nerve. Microscopically the lesion was described as consisting of mature adipose tissue, which, however, encompassed both glandular elements and nerve bundles within it. This tumour has been recently recognized as a separate entity under the term 'sialolipoma'.

  13. Combined liposuction and excision of lipomas: long-term evaluation of a large sample of patients.

    PubMed

    Copeland-Halperin, Libby R; Pimpinella, Vincenza; Copeland, Michelle

    2015-01-01

    Background. Lipomas are benign tumors of mature fat cells. They can be removed by liposuction, yet this technique is seldom employed because of concerns that removal may be incomplete and recurrence may be more frequent than after conventional excision. Objectives. We assessed the short- and long-term clinical outcomes and recurrence of combined liposuction and limited surgical excision of subcutaneous lipomas. Methods. From 2003 to 2012, 25 patients with 48 lipomas were treated with liposuction followed by direct excision through the same incision to remove residual lipomatous tissue. Initial postoperative follow-up ranged from 1 week to 3 months, and long-term outcomes, complications, and recurrence were surveyed 1 to 10 years postoperatively. Results. Lipomas on the head, neck, trunk, and extremities ranged from 1 to 15 cm in diameter. Early postoperative hematoma and seromas were managed by aspiration. Among 23 survey respondents (92%), patients were uniformly pleased with the cosmetic results; none reported recurrent lipoma. Conclusions. The combination of liposuction and excision is a safe alternative for lipoma removal; malignancy and recurrence are uncommon. Liposuction performed through a small incision provides satisfactory aesthetic results in most cases. Once reduced in size, residual lipomatous and capsular tissue can be removed without expanding the incision. These favorable outcomes support wider application of this technique in appropriate cases. PMID:25694827

  14. Chordoma Cutis – A Diagnosis not to be Missed

    PubMed Central

    Mishra, Kiran

    2014-01-01

    Chordomas are rare midline tumors of the bone usually arising from sacrum, skull bones and spine, close to neuraxis. However an extensive involvement of the soft tissues can simulate a soft tissue subcutaneous tumour of the gluteal region –a presentation called chordoma cutis. Our patient presented with a gluteal mass and a trucut biopsy was done suspecting a soft tissue tumour. The hematoxylin and eosin stained section of the biopsy closely simulated a lipomatous tumour. However, on closer inspection the clear cells were found to have very fine vacuolations.The usual myxoid background and characteristic physaliferous cells seen in chordomas were not seen. Still a differential of chordoma was entertained in view of the site and age of the patient. Immunohistochemistry for cytokeratin and S-100 was performed and both were unambiguously positive. On literature search, we came across a soft tissue tumour called parachordoma which mimics chordoma both morphologically and immunohistochemically and has also been reported in the gluteal region. An MRI was performed which showed the tumour to be arising from the sacrum and secondarily involving the gluteal soft tissues. This case highlights the importance of considering chordoma in the differential diagnosis of gluteal masses with clear cell morphology even in the absence of physaliferous cells and myxoid background before signing them out as lipomatous tumours. PMID:25120995

  15. Symmetric Lipomatosis Arising in the Tongue Presenting as Macroglossia and Articulatory Disorder.

    PubMed

    Kudoh, Masanori; Omura, Ken; Satsukawa, Arata; Matsumoto, Koshi; Taguchi, Takahide; Harada, Hiroyuki; Ishii, Yoshimasa

    2016-01-01

    Symmetric lipomatosis is definitively characterized by symmetric, tumorous lipomatous proliferation of adipose tissue that often develops in the head and neck, shoulders, and upper trunk. However, in the oral region, symmetric lipomatosis of the tongue (SLT) is an extremely rare condition related to generalized lipidosis that is often caused by chronic alcoholism. It is characterized by multiple symmetric lipomatous nodules and diffuse bilateral swelling located within the tongue. We report an extremely rare case of SLT arising in an 80-year-old man with a long history of alcoholic liver cirrhosis. He exhibited multiple soft nodular protrusions on the bilateral margin of the tongue presenting as macroglossia for years. Although MR imaging showed multiple fatty masses on both sides of the tongue, there was no elevated tumor mass on the bilateral margin. The patient underwent bilateral partial glossectomy under general anesthesia. Histopathologically, the resected tumor exhibited diffuse infiltration with mature adipose tissue lacking a fibrous capsule. Due to the lipidosis and the unusual presentation of multiple lesions, the lesion was ultimately diagnosed as SLT. At present, after surgery, the patient wears a full-denture and is in excellent condition, with no sign of recurrence, improved QOL, and recovery of masticatory, articulatory, and speech intelligibility functions. PMID:27413566

  16. Auditory Hallucinosis as a Presenting Feature of Interpeduncular Lipoma with Proximal P1 Segment Fenestration: Report of a Rare Case and Review of Literature on Peduncular Hallucinosis

    PubMed Central

    Kulhari, Ashish; Manjila, Sunil; Singh, Gagandeep; Kumar, Kunal; Tarr, Robert W; Bambakidis, Nicholas

    2016-01-01

    The authors present a unique case of intracranial lipoma in the interpeduncular cistern associated with proximal P1 segment fenestration. This patient is a 20-year-old male with extensive psychiatric history and complaints of recent auditory hallucinations. Cranial magnetic resonance imaging (MRI) (T1, T2, and FLAIR) showed a hyperintense lesion in the left aspect of interpeduncular cistern with a prominent flow void within the hyperintense lesion suggestive of a combined vascular–lipomatous lesion. Computed tomography (CT) angiography showed a high-riding large tortuous P1 segment on the left side with proximal fenestration, the ectatic posteromedial limb harboring a fusiform dilated segment. Since there are anecdotal cases of cerebral aneurysms associated with intracranial lipomas, a conventional angiography was done, which confirmed a proximal left P1 fenestration and a fusiform-dilated segment, and no aneurysm. There are few cases of hallucinations associated with a vascular midbrain pathology reported in literature, but hallucinations associated with a combination of lipoma and arterial ectasia have never been reported. This article not only demonstrates the MRI and angiographic appearance of this rare lipomatous lesion but also highlights this unique association and significance of auditory hallucinations as a clinical presentation, akin to peduncular hallucinosis. PMID:27403217

  17. Symmetric Lipomatosis Arising in the Tongue Presenting as Macroglossia and Articulatory Disorder

    PubMed Central

    Omura, Ken; Satsukawa, Arata; Taguchi, Takahide; Harada, Hiroyuki; Ishii, Yoshimasa

    2016-01-01

    Symmetric lipomatosis is definitively characterized by symmetric, tumorous lipomatous proliferation of adipose tissue that often develops in the head and neck, shoulders, and upper trunk. However, in the oral region, symmetric lipomatosis of the tongue (SLT) is an extremely rare condition related to generalized lipidosis that is often caused by chronic alcoholism. It is characterized by multiple symmetric lipomatous nodules and diffuse bilateral swelling located within the tongue. We report an extremely rare case of SLT arising in an 80-year-old man with a long history of alcoholic liver cirrhosis. He exhibited multiple soft nodular protrusions on the bilateral margin of the tongue presenting as macroglossia for years. Although MR imaging showed multiple fatty masses on both sides of the tongue, there was no elevated tumor mass on the bilateral margin. The patient underwent bilateral partial glossectomy under general anesthesia. Histopathologically, the resected tumor exhibited diffuse infiltration with mature adipose tissue lacking a fibrous capsule. Due to the lipidosis and the unusual presentation of multiple lesions, the lesion was ultimately diagnosed as SLT. At present, after surgery, the patient wears a full-denture and is in excellent condition, with no sign of recurrence, improved QOL, and recovery of masticatory, articulatory, and speech intelligibility functions. PMID:27413566

  18. Combined Liposuction and Excision of Lipomas: Long-Term Evaluation of a Large Sample of Patients

    PubMed Central

    Copeland-Halperin, Libby R.; Pimpinella, Vincenza

    2015-01-01

    Background. Lipomas are benign tumors of mature fat cells. They can be removed by liposuction, yet this technique is seldom employed because of concerns that removal may be incomplete and recurrence may be more frequent than after conventional excision. Objectives. We assessed the short- and long-term clinical outcomes and recurrence of combined liposuction and limited surgical excision of subcutaneous lipomas. Methods. From 2003 to 2012, 25 patients with 48 lipomas were treated with liposuction followed by direct excision through the same incision to remove residual lipomatous tissue. Initial postoperative follow-up ranged from 1 week to 3 months, and long-term outcomes, complications, and recurrence were surveyed 1 to 10 years postoperatively. Results. Lipomas on the head, neck, trunk, and extremities ranged from 1 to 15 cm in diameter. Early postoperative hematoma and seromas were managed by aspiration. Among 23 survey respondents (92%), patients were uniformly pleased with the cosmetic results; none reported recurrent lipoma. Conclusions. The combination of liposuction and excision is a safe alternative for lipoma removal; malignancy and recurrence are uncommon. Liposuction performed through a small incision provides satisfactory aesthetic results in most cases. Once reduced in size, residual lipomatous and capsular tissue can be removed without expanding the incision. These favorable outcomes support wider application of this technique in appropriate cases. PMID:25694827

  19. Intra-articular knee mass in a 51-year-old woman.

    PubMed

    Bergin, Patrick F; Milchteim, Charles; Beaulieu, Gregory P; Brindle, Kathleen A; Schwartz, Arnold M; Faulks, Craig R

    2011-03-01

    A 51-year-old woman presented with moderate knee pain refractory to conservative measures. Radiographs revealed a well-defined, but irregularly-shaped ovoid soft tissue density at the posterior superior aspect of the infrapatellar fat pad of Hoffa. On magnetic resonance imaging, the mass was mildly heterogeneous and had intermediate signal in the proton density series and mixed signal intensity on the T2-weighted images. A low-signal rim could be seen around a portion of the lesion. Arthroscopic resection was performed and a vascular stalk was encountered. The differential diagnosis included: ganglion cyst, meniscal cyst, intra-articular lipoma, villous proliferation of the synovial membrane, Hoffa disease, and intracapsular chondroma. Histologic examination revealed a fibrous capsule partially surrounding a benign lipomatous neoplasm containing an abundance of thin- and thick-walled blood vessels with periadventitial myxoid stroma. To our knowledge, this is the first reported case of an intra-articular angiomyxolipoma in the literature. At 8-month follow-up, the patient was asymptomatic with no sign of recurrence. We postulate a low recurrence rate based on the insidious growth rate and benign histological appearance of such lesions. To our knowledge, no other intra-articular lipomatous lesion of the knee has been removed solely with arthroscopic techniques. Further follow-up information is needed to better understand the natural course of these lesions.

  20. The pathogenesis of experimentally induced amebic liver abscess in the gerbil (Meriones unguiculatus).

    PubMed Central

    Chadee, K.; Meerovitch, E.

    1984-01-01

    Sequential development and pathology of experimentally induced amebic liver abscess in the gerbil (Meriones unguiculatus) were studied from 1 to 60 days after inoculation. Early lesions were characterized by an acute inflammatory response, which became granulomatous at 5 days. Early granulomas were discrete, with well-defined fibrohistiocytic walls. Trophozoite dissemination as a result of fibrolysis of granuloma wall was confined to the liver parenchyma. The granulomatous cellular infiltrate (less than 20 days) was characterized by granulocytes and histiocytes; older granulomas (greater than 30 days) were composed of lymphocytic infiltrate, plasma cells, and a few granulocytes, and were characterized by the absence of epithelioid histiocytes. The degree of pathologic change adjacent to liver granulomas followed the sequential development of the amebic liver abscess. Severe changes observed were portal canal lymphocytic infiltration, the presence of foreign body giant cells, periportal fibrosis, proliferation of bile duct epithelium, and hepatocyte anisonucleosis and ballooning degeneration. The pathogenesis of the infection and the usefulness of the gerbil model for the study of human amebiasis are discussed. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 Figure 8 Figure 9 Figure 10 Figure 11 Figure 12 Figure 13 Figure 14 Figure 15 Figure 16 Figure 17 Figure 18 PMID:6385727

  1. A Case of Cellular Fibrous Histiocytoma on the Right Elbow with Repeated Relapse within a Short Period.

    PubMed

    Tsunoda, Kanako; Oikawa, Hiroki; Maeda, Fumihiko; Takahashi, Kazuhiro; Akasaka, Toshihide

    2015-01-01

    Cellular fibrous histiocytoma, a variant of fibrous histiocytoma, is a designation used for lesions showing increased cellularity with a fascicular growth pattern and frequent extension into the subcutis. Here we describe a case of cellular fibrous histiocytoma showing repeated recurrence in a 36-year-old woman who initially presented with a 2-cm cutaneous tumor on her right elbow. Histopathologically, the first resected specimen demonstrated irregularly arranged collagen fibers mixed with scattered proliferating plump to spindle-shaped fibrohistiocytes. However, examination of the resected specimens obtained after recurrence showed that the cellularity had increased, the spindle-shaped cells showing monomorphic proliferation with a fascicular and storiform growth pattern extending into the subcutis, as well as an increase of Ki-67 positivity. Since the lesion showed repeated relapse within a short period, we performed wide-field resection of the tumor with a 3-cm margin. Currently, 48 months after surgery, there has been no local recurrence or metastasis, but continuous strict follow-up will be necessary. PMID:25759652

  2. A Case of Cellular Fibrous Histiocytoma on the Right Elbow with Repeated Relapse within a Short Period

    PubMed Central

    Tsunoda, Kanako; Oikawa, Hiroki; Maeda, Fumihiko; Takahashi, Kazuhiro; Akasaka, Toshihide

    2015-01-01

    Cellular fibrous histiocytoma, a variant of fibrous histiocytoma, is a designation used for lesions showing increased cellularity with a fascicular growth pattern and frequent extension into the subcutis. Here we describe a case of cellular fibrous histiocytoma showing repeated recurrence in a 36-year-old woman who initially presented with a 2-cm cutaneous tumor on her right elbow. Histopathologically, the first resected specimen demonstrated irregularly arranged collagen fibers mixed with scattered proliferating plump to spindle-shaped fibrohistiocytes. However, examination of the resected specimens obtained after recurrence showed that the cellularity had increased, the spindle-shaped cells showing monomorphic proliferation with a fascicular and storiform growth pattern extending into the subcutis, as well as an increase of Ki-67 positivity. Since the lesion showed repeated relapse within a short period, we performed wide-field resection of the tumor with a 3-cm margin. Currently, 48 months after surgery, there has been no local recurrence or metastasis, but continuous strict follow-up will be necessary. PMID:25759652

  3. Solitary fibrous tumor with malignant potential arising in sublingual gland.

    PubMed

    Ogawa, Ikuko; Sato, Sunao; Kudo, Yasusei; Miyauchi, Mutsumi; Sugiyama, Masaru; Suei, Yoshikazu; Takata, Takashi

    2003-01-01

    A rare case is described of a solitary fibrous tumor (SFT) with malignant potential arising in the sublingual gland. A 59-year-old man presented with a 4-month history of a slowly enlarging painless mass in the center of the floor of the mouth. The tumor was a well-demarcated, firm mass with a multicystic lesion. The tumor exhibited highly cellular areas of spindle cells with patternless architecture alternating with hypocellular areas. The tumor cells were positive for CD34 and bcl-2 as well as vimentin, and negative for epithelial, myogenic, neurogenic and histiocytic markers. The tumor cells formed multiple satellite nodules around dilated ducts in the multicystic lesion, indicating infiltrative growth. In addition, areas exhibiting higher cellularity with increased mitoses were noticed in the satellite nodules, although cellular atypia was not obvious. These findings led to a final diagnosis of SFT with malignant potential. There has been no recurrence or metastasis for 27 months after the surgery. Solitary fibrous tumor of the salivary gland must be differentiated from various spindle cell neoplasms including myogenic, peripheral nerve sheath, fibroblastic and fibro-histiocytic spindle cell neoplasms, hemangiopericytoma and myoepithelioma. In addition to characteristic morphological features, an immunohistochemical positivity for CD34 and bcl-2 may aid in the diagnosis of SFT.

  4. Tenosynovial giant cell tumour (pigmented villonodular synovitis-)-like changes in periprosthetic interface membranes.

    PubMed

    Söder, Stephan; Sesselmann, Stefan; Aigner, Thomas; Oehler, Stephan; Agaimy, Abbas

    2016-02-01

    Tenosynovial giant cell tumour (TSGCT; synonym, pigmented villonodular synovitis (PVNS)) is a rare low-grade mesenchymal neoplasm of either intra-articular or extra-articular origin. The etiopathogenesis of TSGCT is still uncertain, but recent studies showed a translocation involving colony-stimulating factor 1 (CSF-1) gene in a subset of cases. Histological features mimicking TSGCT can sometimes be encountered in periprosthetic interface membranes. To investigate the frequency and morphologic spectrum of this phenomenon, we conducted a systematic analysis of 477 periprosthetic interface membranes and performed immunohistochemical analysis on a subset of lesions compared to genuine TSGCT. In 26 of 477 periprosthetic membrane samples (5 %), at least some TSGCT-like features were found and 18 cases (4 %) strongly resembled it. Wear particles were detected in 100 % of the TSGCT-like lesions but only in 63.3 % of the whole cohort of periprosthetic membranes (p value <0.001). Immunohistochemistry comparing true TSGCT and TSGCT-like membranes showed similar inflammatory infiltrates with slightly elevated CD3+/CD8+ T lymphocytes and a slightly higher proliferation index in TSGCT samples. In conclusion, TSGCT-like changes in periprosthetic membranes likely represent exuberant fibrohistiocytic inflammatory response induced by wear particles and should be distinguished from genuine (neoplastic) TSGCT. Although TSGCT and TSGCT-like periprosthetic membranes represent different entities, their comparable morphology might reflect analogous morphogenesis.

  5. [About a case of calcifying fibrous tumor of the pleura].

    PubMed

    Rocas, Delphine; Thivolet-Béjui, Françoise; Tronc, François; Chalabreysse, Lara

    2015-12-01

    Calcifying fibrous tumor is a rare soft tissue benign tumor (OMS 2002). Some pleural localisations are described, which affect slightly older individuals than the other soft tissue forms. The calcifying fibrous tumor is included in the 2004 World Health Organization classification of pleural tumors. A pleural tumor located in the right inferior pulmonary lobe is diagnosed in a 59-year-old man. This pleural tumor is macroscopically well-circumscribed. Histologically, the rare spindle tumoral cells are located between bundles of a collagenous tissue, sometimes hyalinized, with psammomatous or dystrophic calcifications. The tumoral cells have a fibrohistiocytic origin. They stain positively for antibodies against vimentin, factor XIIIa, CD68, CD163, CD34. Antibodies against smooth muscle actin, desmin, PS100, ALK1 and EBV are negative. Main differencial diagnoses are other benign pleural tumors (solitary fibrous tumor, inflammatory myofibroblastique tumor), some malignant tumors (desmoplastic malignant pleural mesothelioma) and pleural pseudotumors (calcified pleural plaques, chronic fibrous pleuritis, amylose, hyalinizing granuloma). Our case is the 15th pleural calcifying fibrous tumor being reported. PMID:26608111

  6. High-pressure paint-gun injury of the finger simulating giant cell tumor of tendon sheath.

    PubMed

    Stefanato, Catherine M; Turner, Matthew S; Bhawan, Jag

    2005-02-01

    High-pressure paint guns deliver paint at approximately 3000 pounds per square inch. At this pressure, paint will penetrate the skin and spread quickly through fascial planes and tendon sheaths. The present case is that of a lesion from the finger of a 35-year-old white male in whom a history was initially unavailable. Histologic examination revealed diffuse fibrohistiocytic proliferation and giant cells, with numerous darkly pigmented, uniformly small-sized particles throughout the lesion. The initial impression was that of a giant cell tumor of tendon sheath. However, the pigment particles were negative for Perls stain, and polariscopic examination revealed clear refractile fragments. These findings raised the possibility that the lesion was the result of a traumatic event. On further inquiry, it was revealed that the patient had sustained a high-pressure paint-gun injury 1 year earlier. The simulation, histopathologically, of a giant cell tumor of tendon sheath by a high-pressure paint-gun injury has not, to our knowledge, been reported previously, nor has the histologic finding of small, uniformly sized pigment particles and polarizable refractile fragments in this particular type of injury.

  7. Dedifferentiated liposarcoma involving the spleen and splenic hilum: a report of a case with a rare growth pattern.

    PubMed

    Nishikawa, Gen; Minamiguchi, Sachiko; Hata, Hiroaki; Ogiso, Satoshi; Yamaguchi, Takashi; Otani, Tethushi; Ikai, Iwao

    2015-01-01

    We present a rare case of dedifferentiated liposarcoma confined to the spleen and splenic hilum. An 81-year-old man was referred to our hospital with a large asymptomatic splenic tumor. The patient underwent splenectomy, and the adipose tissue surrounding the splenic hilum was also resected. Microscopically, the tumor mainly consisted of high-grade spindle cells similar to those seen in undifferentiated pleomorphic liposarcoma. In the splenic hilum, scattered atypical cells were detected in the sclerosing component and adipose tissue. Immunohistochemically, both the spindle cells in the spleen and the atypical cells in the splenic hilum were positive for MDM2 and CDK4. The histopathologic diagnosis was dedifferentiated liposarcoma derived from an atypical lipomatous tumor/well-differentiated liposarcoma of the adipose tissue in the splenic hilum with extension into the spleen. Dedifferentiated liposarcoma in the spleen and splenic hilum should be considered as a differential diagnosis of splenic tumors.

  8. Enlarged pancreas: not always a cancer.

    PubMed

    Calculli, Lucia; Festi, Davide; Pezzilli, Raffaele

    2015-02-01

    Pancreatic fat accumulation has been described with various terms including pancreatic lipomatosis, pancreatic steatosis, fatty replacement, fatty infiltration, fatty pancreas, lipomatous pseudohypertrophy and nonalcoholic fatty pancreas disease. It has been reported to be associated with type 2 diabetes mellitus, acute pancreatitis, pancreatic cancer and the formation of pancreatic fistula. The real incidence of this condition is still unknown. We report a case of pancreatic steatosis in a non-obese female patient initially diagnosed with a mass in the head of the pancreas. Magnetic resonance imaging (MRI) was carried out to define the characteristics of the pancreatic mass. MRI confirmed the diagnosis of fat pancreas. Enlarged pancreas is not always a cancer, but pancreatic steatosis is characterized by pancreatic enlargement. MRI could give a definite diagnosis of pancreatic steatosis or cancer.

  9. Fat-forming variant of solitary fibrous tumour of the pleura: CT findings.

    PubMed

    Park, C Y; Rho, J Y; Yoo, S M; Jung, H K

    2011-11-01

    The fat-forming variant of solitary fibrous tumour (SFT) was previously called lipomatous haemangiopericytoma and is a rare variant of solitary fibrous tumour. It predominantly occurs in the deep soft tissues of the retroperitoneum and thigh. Only a handful of cases involving the perineum, spine, thoracic wall and pelvic cavity have been reported in the radiological literature and the fat-forming variant of SFT involving the pleura has not been previously reported. Herein, we report the CT findings of a case of the fat-forming variant of SFT involving the pleura that was treated by excision. Chest CT showed a large lobulated heterogeneous fatty mass with a multifocal enhancing soft-tissue component in the left lower hemithorax. Although rare, the fat-forming variant of SFT of the pleura should be added to the differential diagnosis of fat-containing pleural soft-tissue tumours.

  10. Solitary fibrous tumour of the thyroid: clinicopathological, immunohistochemical and ultrastructural study of three cases.

    PubMed

    Taccagni, G; Sambade, C; Nesland, J; Terreni, M R; Sobrinho-Simões, M

    1993-01-01

    We describe three cases of solitary fibrous tumour (SFT) arising from thyroid stroma. Grossly, the tumours were clearly delimited but only partly encapsulated. The following histomorphological growth patterns were observed: bundles of cells in storiform configuration; non-structured bundles; prevalence of fibrous matrix; highly cellular, non-structured; prevalence of loose, non-structured extracellular substance; cellular proliferation and vascular spaces in a haemangiopericytic configuration and a lipomatous component. Immunohistochemical investigation demonstrated intense, diffuse vimentin positivity and focal, less intense actin positivity in all three cases. At electron microscopy we observed a primitive cell of mesenchymal type, with cytoplasm poor in organelles and rich in filaments; this cell sometimes presented differentiation characteristics. SFT is at present the most correct term for the lesions presented here despite some morphological characteristics which differ from cases reported in the literature.

  11. Paratesticular liposarcoma-masquerading as a testicular tumour.

    PubMed

    Vinayagam, Kalaivani; Hosamath, Vijayakumar; Honnappa, Sridhar; Rau, Aarathi Ranga

    2014-02-01

    Paratesticular liposarcomas are rare tumours which account for 12% of all liposarcomas. Probably there are about 186 cases which have been reported till date. They must be differentiated from tumours of testicular origin which have extension to the spermatic cord. We are reporting a case of a 50-year-old male who had presented with a painless swelling in the right hemiscrotum, which was of 20 years' duration. Inititally, a clinical diagnosis of testicular tumour was made; however, CT of the scrotum revealed paratesticular tumour? liposarcoma and testis being normal and displaced postero-inferiorly. Metastatic work-up, which included CT of the abdomen and pelvis, thorax and whole body scan, did not reveal any distant metastasis. Patient underwent high orchidectomy, hemiscrotectomy. Histopathological studies confirmed the diagnosis of well-differentiated liposarcoma (atypical lipomatous tumour of sclerosing type). PMID:24701520

  12. Liposarcoma in clownfish, Amphiprion ocellaris Cuvier, produced in indoor aquaculture.

    PubMed

    Sharon, G; Benharroch, D; Kachko, L; Reis-Hevlin, N; Zilberg, D

    2015-06-01

    Clownfish, Amphiprion ocellaris Cuvier, produced and grown in an experimental indoor aquaculture facility, presented with lipomatous tumours. A total of 14 affected fish were examined. Based on the total number of fish at the aquaculture facility at the time of outbreak of this pathology, the scope of the incident is estimated to be 1 of 300 fish. The tumours were characterized by the presence of mature adipocytes of variable sizes, lipoblasts and by an invasive behaviour, which affected internal organs, muscle, central nervous system and, in one case, an eye. Detailed macroscopic and histopathological features are presented. The suggested diagnosis is that of a well-differentiated liposarcoma, a diagnosis so far never applied to fish. The limited outbreak of the neoplasm lasted a few months in 2011 and did not recur. Possible factors leading to this phenomenon, notably the metastasis, are discussed. PMID:24917512

  13. Neoplasms involving the heart, their simulators, and adverse consequences of their therapy

    PubMed Central

    2001-01-01

    Primary cardiac tumors involving the heart may be either benign or malignant. Most of the benign tumors are myxomas, which are most commonly located in the left atrium. Primary malignant neoplasms usually involve the myocardium and the interior of the cardiac cavities, whereas neoplasms metastatic to the heart most commonly involve pericardium, and pericardial effusion and constriction are the most common consequences. Computed tomography and magnetic resonance imaging are becoming the most useful instruments of precision for the diagnosis of cardiac tumors. Pericardial cysts, teratomas, lipomatous hypertrophy of the atrial septum, papillary fibroelastomas, thrombi, and sarcoid are frequently mistaken for cardiac neoplasms. There are a number of cardiac consequences of malignancy, including radiation heart disease, cardiac hemorrhages, cardiac infection, cardiac adiposity or the corticosteroidtreated heart, cardiac hemosiderosis, and toxicity due to anthracycline chemotherapy. PMID:16369647

  14. Role of adipose tissue in the pathogenesis of cardiac arrhythmias.

    PubMed

    Samanta, Rahul; Pouliopoulos, Jim; Thiagalingam, Aravinda; Kovoor, Pramesh

    2016-01-01

    Epicardial adipose tissue is present in normal healthy individuals. It is a unique fat depot that, under physiologic conditions, plays a cardioprotective role. However, excess epicardial adipose tissue has been shown to be associated with prevalence and severity of atrial fibrillation. In arrhythmogenic right ventricular cardiomyopathy and myotonic dystrophy, fibrofatty infiltration of the myocardium is associated with ventricular arrhythmias. In the ovine model of ischemic cardiomyopathy, the presence of intramyocardial adipose or lipomatous metaplasia has been associated with increased propensity to ventricular tachycardia. These observations suggest a role of adipose tissue in the pathogenesis of cardiac arrhythmias. In this article, we review the role of cardiac adipose tissue in various cardiac arrhythmias and discuss the possible pathophysiologic mechanisms.

  15. Duodenal Lipomatosis as a Curious Cause of Upper Gastrointestinal Bleed: A Report with Review of Literature

    PubMed Central

    Wani, Majid; Tiwari, Priyanka; Ramaswamy, Palaniswamy Kalipatti; Kumar, Reddy Prasanna

    2016-01-01

    Lipomas of the gastrointestinal tract are rare. Duodenal lipomas are incidental and mostly asymptomatic. Tumours may produce symptoms of abdominal pain and discomfort or cause bleeding due to ulceration or intestinal obstruction due to intussusception. We describe a 45-year-old man presenting in emergency with 3 days history of melena with normal gastroduodenoscopy and contrast enhanced computed tomography revealing multiple polypoid lesion in duodenum and proximal jejunum suggestive of lipoma. Due to ongoing bleed, he underwent laparotomy with duodenectomy and uneventful postoperative recovery. Our review of cases published in last 67 years indicate that duodenal lipomas are rare to occur but commonly found in second part, they may be seen in third and fourth part of duodenum which may be missed on endoscopy. They can be multiple and may present as severe UGI bleeding which could be managed surgically. Though CT is diagnostic, histopathology confirms the diagnosis which shows lipomatous lesion composed of mature adipose arranged in lobules. PMID:27437304

  16. Lumbar spinal angiolipoma: case report and review of the literature.

    PubMed

    Konya, Deniz; Ozgen, Serdar; Kurtkaya, Ozlem; Pamir, Necmettin M

    2006-06-01

    Spinal angiolipomas are extremely rare benign tumors composed of mature lipomatous and angiomatous elements. Most are symptomatic due to progressive spinal cord or root compression. This article describes the case of a 60-year-old woman who presented with a 6-month history of low back pain radiating to her right leg. The pain was multisegmental. The condition had worsened with time. Lumbar magnetic resonance imaging revealed a dorsal epidural mass at L5 and erosion of the lamina of the L5 vertebra. Laminectomy was performed, and an extradural tumor was totally excised. Neuropathologic examination identified it as a lumbar spinal angiolipoma. There was no evidence of recurrence in follow-up 12 months later. This rare clinical entity must be considered in the differential diagnosis for any spinal epidural lesion.

  17. Paratesticular liposarcoma and contralateral angiolipoma in a 60-year-old patient.

    PubMed

    Christodoulidou, Michelle; Khetrapal, Pramit; Edmunds, Lilian; Muneer, Asif

    2015-10-05

    We present the case of a 60-year-old man who was referred with a 1-year history of a slow-growing right scrotal lump. Following surgical excision together with a radical orchidectomy, the histological diagnosis was of a paratesticular dedifferentiated liposarcoma. Radiological staging showed no metastatic disease. Six months later the patient presented with a new left scrotal lump, which was felt to be separate from the testis. Owing to the previous history of a liposarcoma and indeterminate ultrasound findings, this was surgically excised without orchidectomy. Histological analysis showed this to be an angiolipoma. The patient remained on clinical and radiological surveillance. Paratesticular tumours are rare neoplasms and liposarcomas of the spermatic cord only represent 7% of these lesions. Any link between lipomatous tumours and liposarcomas remains controversial and is still under investigation. This is the first case to report a paratesticular liposarcoma and a contralateral angiolipoma in the same patient.

  18. Intraosseous angiolipoma of the frontal bone with a unique location: a clinical and pathological case illustration and review of the literature.

    PubMed

    Atilgan, Alev Ok; Terzi, Aysen; Agildere, Muhtesem; Caner, Hakan; Ozdemir, B Handan

    2014-01-01

    Here, we report a case of a 16-year-old female patient was referred with scalp swelling and headache. Her neurological examination was normal and imaging of the skull revealed a well-defined lytic lesion measuring 15 mm × 6 mm to the right of the frontal bone. She was operated on with a prediagnosis of Langerhans cell histiocytosis. A wide excision with negative margins was made and the defect was reconstructed with a titanium plate. Subsequently, the lesion was histopathologically diagnosed as an angiolipoma of the frontal bone. The postoperative period was uneventful and she remained well during 1-year follow-up with no evidence of recurrence. Angiolipomas are rare benign lipomatous lesions located mostly in subcutaneous tissue of the forearm or trunk and frequently occur before puberty or in young adults. They are not common in bones. To the best of our knowledge, this is the first angiolipoma of the frontal bone reported.

  19. Spindle Cell Lipoma of the Neck: Review of the Literature and Case Report

    PubMed Central

    Machol, Jacques A.; Cusic, Jenna G.; O'Connor, Elizabeth A.; Sanger, James R.

    2015-01-01

    Summary: Spindle cell lipomas (SCL) are benign, slow growing tumors arising most frequently in the subcutaneous tissue of the upper back, posterior neck, and shoulders in males aged 40–70 years. Local excision is generally curative. Classification of lipomatous tumors has progressed recently, and tumors of similar morphology and unusual presentation are increasingly reported, thereby making correct diagnosis even more vital. SCL require pathologic differentiation from liposarcoma, other spindle cell neoplasms, and myxoid lesions for treatment purposes. Cytology, histology, and cytogenetics, in conjunction with clinical presentation, are paramount in arriving at the correct diagnosis of spindle cell lipoma. We present a case report with characteristics typical of an SCL along with a literature review to further elucidate the diagnosis and surgical treatment of this soft tissue tumor. PMID:26893975

  20. Parapharyngeal space lipomatosis with secondary dyspnea, disphagia and disphonia

    PubMed Central

    Garcia-Ortega, Dorian Yarih; Gomez-Pedraza, Antonio; Luna-Ortiz, Kuauhyama

    2015-01-01

    Introduction Parapharyngeal space tumors are a small percentage of all head and neck neoplasms. Due to their anatomic location, they represent a therapeutic challenge. To our knowledge, 11 cases of parapharyngeal lipomatous tumors have been reported in the literature. Case A 48 year old male with chief complaints of dyspnea, dysphagia and dysphonia was found to have a parapharyngeal space tumor. He was scheduled to undergo lumpectomy and neck exploration. Discussion Benign tumors represented 70% of all cases. Open neck surgery is considered the gold-standard of treatment. Conclusion It is important to bear in mind the lipomas of the parapharyngeal space to establish an accurate diagnosis and implement timely, appropriate treatment in order to avoid future complications and reduce morbidity and mortality. PMID:26318127

  1. Intramuscular Lipoma: A Review of the Literature

    PubMed Central

    McTighe, Shane; Chernev, Ivan

    2014-01-01

    Lipomas are the most common type of soft tissue mesenchymal tumors. They are typically located subcutaneously and consist of mature fatty tissue. When they occur under the enclosing fascia, they are called deep-seated lipomas. Infrequently, lipomas can arise inside the muscle and are called intramuscular lipomas. Intramuscular lipomas have been commonly investigated and categorized in the same group as other deep-seated and superficial lipomatous lesions. Their clinical, histological and imaging characteristics may resemble well-differentiated liposarcomas, further adding to the difficulties in the differential diagnosis. This article summarizes the available literature and describes the typical epidemiological, pathological and clinical features of intramuscular lipomas, as well as delineating their treatment and prognosis. PMID:25568733

  2. Liposarcoma in clownfish, Amphiprion ocellaris Cuvier, produced in indoor aquaculture.

    PubMed

    Sharon, G; Benharroch, D; Kachko, L; Reis-Hevlin, N; Zilberg, D

    2015-06-01

    Clownfish, Amphiprion ocellaris Cuvier, produced and grown in an experimental indoor aquaculture facility, presented with lipomatous tumours. A total of 14 affected fish were examined. Based on the total number of fish at the aquaculture facility at the time of outbreak of this pathology, the scope of the incident is estimated to be 1 of 300 fish. The tumours were characterized by the presence of mature adipocytes of variable sizes, lipoblasts and by an invasive behaviour, which affected internal organs, muscle, central nervous system and, in one case, an eye. Detailed macroscopic and histopathological features are presented. The suggested diagnosis is that of a well-differentiated liposarcoma, a diagnosis so far never applied to fish. The limited outbreak of the neoplasm lasted a few months in 2011 and did not recur. Possible factors leading to this phenomenon, notably the metastasis, are discussed.

  3. Detection of MDM2-CDK4 amplification by fluorescence in situ hybridization in 200 paraffin-embedded tumor samples: utility in diagnosing adipocytic lesions and comparison with immunohistochemistry and real-time PCR.

    PubMed

    Sirvent, Nicolas; Coindre, Jean-Michel; Maire, Georges; Hostein, Isabelle; Keslair, Frédérique; Guillou, Louis; Ranchere-Vince, Dominique; Terrier, Philippe; Pedeutour, Florence

    2007-10-01

    Atypical lipomatous tumor/well-differentiated liposarcomas and dedifferentiated liposarcomas are characterized by the amplification of MDM2 and CDK4 genes. To evaluate the accuracy of fluorescence in situ hybridization (FISH) analysis in the differential diagnosis of adipose tissue tumors, we investigated MDM2-CDK4 status by FISH, real-time polymerase chain reaction (PCR) [quantitative PCR (Q-PCR)] and immunohistochemistry (IHC) in a series of 200 adipose tumors. First, we evaluated MDM2-CDK4 amplification and expression in a series of 94 well-defined adipose tissue tumors. Results showed that FISH was interpretable in 45 of 50 cases (90%), and was more specific and sensitive than Q-PCR and IHC. We then used the same techniques as complementary diagnostic tools in a series of 106 adipose and soft tissue tumors of unclear diagnosis to distinguish between (i) lipomas and atypical lipomatous tumor/well-differentiated liposarcomas, (ii) malignant undifferentiated tumors and dedifferentiated liposarcomas, and (iii) a variety of benign tumors and liposarcomas. Our results indicate that although helpful, IHC alone is often insufficient to solve diagnostic problems. FISH and Q-PCR methods gave concordant results and were equally informative in most cases. However, the proportion of noninterpretable cases was slightly higher with FISH than with Q-PCR. When tumor cells represented a minor component of the tumor tissue, such as with inflammatory tumors, FISH was more powerful than Q-PCR by allowing visualization of individual cells. In conclusion, we recommend that the evaluation of MDM2-CDK4 amplification using FISH or Q-PCR be used to supplement IHC analysis when diagnosis of adipose tissue tumors is not possible based on clinical and histologic information alone.

  4. Spindle cell lipoma-like tumor, solitary fibrous tumor and myofibroblastoma of the breast: a clinico-pathological analysis of 13 cases in favor of a unifying histogenetic concept.

    PubMed

    Magro, Gaetano; Bisceglia, Michele; Michal, Michal; Eusebi, Vincenzo

    2002-03-01

    We reviewed the clinico-pathological features of a series of 13 cases of benign spindle stromal tumors (BSSTs) of the breast relating to a basic common theme consisting of a well-circumscribed proliferation of vimentin+/CD34+/BCL-2+/CD99+ spindly to oval-epithelioid cells, variably arranged in haphazard to short fascicular growth pattern, with interspersed thick or thin collagen bands. Morphological variations included atypical mono- or multi-nucleated cells in five cases and a mature lipomatous tumor component, varying from focal to prominent, in eight cases. Based on morphological and immunophenotypical features, a distinction was made between two main subtypes of these tumors--fibroblastic and myofibroblastic. The former subtype included two cases respectively represented by a typical solitary fibrous tumor (SFT) and a neoplasm labeled "spindle-cell lipoma (SCL)-like tumor", closely reminiscent of soft tissue SCL. Both tumors had cells with fibroblastic-like appearance, haphazardly arranged and immunoreactive for vimentin, CD34, BCL-2, and CD99. The latter subtype, comprised nine cases exhibiting evidence of myofibroblastic differentiation (desmin and alpha-smooth muscle actin) which were classified as myofibroblastomas (MFBs). The remaining two cases were defined as "mixed BSSTs", having typical features of diverse neoplasms, respectively represented by a case of MFB with focal SFT and pleomorphic/SCL-like areas, and SFT with focal MFB-like component. The common basic morpho-immunophenotypical features, the possibility that both fibroblastic and myofibroblastic tumors may contain an additional mature lipomatous component, and the existence of hybrid stages (mixed BSSTs) strongly support the view that such tumors belong to the same category of lesions. We postulate that the precursor of all these neoplasms is the vimentin+/CD34+ cells of the mammary stroma, the well-known inherent plasticity of which to differentiate toward several mesenchymal lines, provides

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

    PubMed Central

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

    2016-01-01

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

  6. Rare case of a liposarcoma in the brachial plexus.

    PubMed

    Kosutic, D; Gajanan, K

    2016-09-01

    Introduction A liposarcoma is a rare cancer of connective tissues that resemble fat cells under light microscopy. Case History A 73-year old female patient presented to our tertiary cancer centre with an eight-year history of a large, slow-growing painless mass in the right axilla. Magnetic resonance imaging showed a lipomatous, well-circumscribed mass of dimension 30 × 16 × 10cm extending towards the right clavicle and causing deformation to the right chest wall and right breast. Surgery revealed a large tumour that had stretched all three cords of the brachial plexus. Histopathology was consistent with a diagnosis of a low-grade liposarcoma. After a period of neuropraxia, the patient returned to normal activities 4 months after surgery. Conclusions Although extremely rare, low-grade liposarcomas of the brachial plexus should be considered in the differential diagnosis of a slow-growing axillary mass. Referral to a tertiary sarcoma centre is essential for an appropriate diagnosis, adequate treatment, and long-term follow-up. PMID:27241607

  7. Mullerian choristoma in a case of spinal dysraphism.

    PubMed

    Kwatra, Kanwardeep Singh; Paul, Preethi A M; Calton, Nalini; George, Shubhra

    2015-01-01

    It is extremely rare to find mullerian choristomas in association with spinal dysraphism, with <10 cases published in English literature. We report a case of heterotopic uterus and fallopian tube-like tissue within a lumbar subcutaneous lipoma associated with spina bifida and tethered cord. A 21-year-old lady presented with lumbar swelling since birth and dull pain in the lower back. Magnetic resonance imaging showed spina bifida at level L3 and L4, tethering of the cord and a subcutaneous lipomatous swelling. Biopsy revealed lobules of fibroadipose tissue embedded in which were seen organoid cystic structures containing prominent smooth muscle coats in their wall. These cystic structures were lined by the endometrium and showed fallopian tube-like papillary infoldings. Immunohistochemistry showed estrogen receptor positivity in the epithelium, stroma, and smooth muscles. The epithelial cells were also positive for cancer antigen 125 and cytokeratin 7 while the stromal cells showed CD10 positivity, supporting mullerian derivation. The pathogenesis and differential diagnosis of such lesions is discussed.

  8. Solitary fibrous tumour and haemangiopericytoma: evolution of a concept.

    PubMed

    Gengler, C; Guillou, L

    2006-01-01

    Haemangiopericytoma (HPC) was described in 1942 by Stout and Murray as a distinctive soft tissue neoplasm, presumably of pericytic origin, exhibiting a characteristic well-developed "staghorn" branching vascular pattern. Over the years, it appeared that this growth pattern was a non-specific one, shared by numerous, unrelated benign and malignant lesions, and that HPC was better considered as a diagnosis of exclusion. Three categories of lesion may now be individualized within the heterogeneous group of HPC-like neoplasms. The first category corresponds to those non-HPC neoplasms that occasionally display HPC-like features (e.g. synovial sarcoma). Lesions belonging to the second category show clear evidence of myoid/pericytic differentiation and correspond to true HPCs. They generally show a benign clinical course, and include glomangiopericytoma/myopericytoma, infantile myofibromatosis (previously called infantile HPC), and a subset of sinonasal HPCs. The third category is the solitary fibrous tumour (SFT) lesional group, which includes fibrous-to-cellular SFTs, and related lesions such as giant cell angiofibromas and lipomatous HPCs. In practice, any HPC-like lesion can be allocated to one of these categories, leaving the ill-defined "haemangiopericytoma" category empty.

  9. Spinal extradural angiolipoma: report of two cases and review of the literature

    PubMed Central

    García-Allut, Alfredo

    2009-01-01

    Spinal angiolipomas are benign uncommon neoplasm composed of mature lipocytes admixed with abnormal blood vessels. They account for only 0.04–1.2% of all spinal tumors. We report two cases of lumbar extradural angiolipoma and review previously reported cases. We found 118 cases of spinal epidural angiolipoma (70 females and 48 males; age range 1.5–85 years, mean 44.03) spanning from 1890 to 2006. Prior to diagnosis 40.6% of the patients had weakness of the lower limbs. The interval between the initial symptoms and tumor diagnosis ranged from 1 day to 17 years (mean 20.2 months). Except for four cases diagnosed at autopsy, 109 patients underwent surgery and gross-total resection was performed in 79 cases (72.4%). Spinal angiolipomas are tumors containing angiomatous and lipomatous tissue, predominantly located in the mid-thoracic region. All angiolipomas show iso- or hyperintensity on T1-weighted images and hyperintensity on T2-weighted images and most lesions enhance with gadolinium administration. The treatment for spinal extradural angiolipomas is total surgical resection and no adjuvant therapy should be administered. PMID:19127373

  10. Molecular and Functional Characterization of Three Different Postzygotic Mutations in PIK3CA-Related Overgrowth Spectrum (PROS) Patients: Effects on PI3K/AKT/mTOR Signaling and Sensitivity to PIK3 Inhibitors

    PubMed Central

    Forte, Giovanna; Bagnulo, Rosanna; Stella, Alessandro; Lastella, Patrizia; Cutrone, Mario; Benedicenti, Francesco; Susca, Francesco C.; Patruno, Margherita; Varvara, Dora; Germani, Aldo; Chessa, Luciana; Laforgia, Nicola; Tenconi, Romano; Simone, Cristiano; Resta, Nicoletta

    2015-01-01

    Background PIK3CA-related overgrowth spectrum (PROS) include a group of disorders that affect only the terminal portion of a limb, such as type I macrodactyly, and conditions like fibroadipose overgrowth (FAO), megalencephaly-capillary malformation (MCAP) syndrome, congenital lipomatous asymmetric overgrowth of the trunk, lymphatic, capillary, venous, and combined-type vascular malformations, epidermal nevi, skeletal and spinal anomalies (CLOVES) syndrome and Hemihyperplasia Multiple Lipomatosis (HHML). Heterozygous postzygotic PIK3CA mutations are frequently identified in these syndromes, while timing and tissue specificity of the mutational event are likely responsible for the extreme phenotypic variability observed. Methods We carried out a combination of Sanger sequencing and targeted deep sequencing of genes involved in the PI3K/AKT/mTOR pathway in three patients (1 MCAP and 2 FAO) to identify causative mutations, and performed immunoblot analyses to assay the phosphorylation status of AKT and P70S6K in affected dermal fibroblasts. In addition, we evaluated their ability to grow in the absence of serum and their response to the PI3K inhibitors wortmannin and LY294002 in vitro. Results and Conclusion Our data indicate that patients’ cells showed constitutive activation of the PI3K/Akt pathway. Of note, PI3K pharmacological blockade resulted in a significant reduction of the proliferation rate in culture, suggesting that inhibition of PI3K might prove beneficial in future therapies for PROS patients. PMID:25915946

  11. Fine-Needle Aspiration Cytology of Soft Tissue Sarcoma: Benefits and Limitations

    PubMed Central

    1998-01-01

    Purpose. Examine the benefits and limitations of fine-needle aspiration cytology (FNA) used as the definitive diagnostic method before treatment. Method. Review of the 25 year experience at a multidisciplinary musculo-skeletal centre where FNA is the primary diagnostic approach to soft tissue sarcoma in the extremities and trunk wall and the experience of various experts in the field. Results. FNA has several benefits compared with coarse needle or open surgical biopsy. The most important are rapid preliminary diagnosis, no need for hospitalization and anaesthesia, negligible complications and fear for tumour cell spread. With the collected experience gained during the years a reliable diagnosis of sarcoma is the rule in general and specific-type diagnoses are possible in many histotypes, especially when the cytologic examination is supplemented with ancillary diagnostics. The most important limitations are inability to hit small deep-seated sarcoma and some diagnostic pitfalls such as the correct diagnosis of spindle cell neoplasms, variants of benign lipomatous tumours and ‘new soft tissue tumour entities’. Discussion. Optimal use of FNA calls for certain requirements such as centralization, experience in soft tissue tumour cytology–histopathology, the FNA technique and close co-operation between the orthopaedic surgeon and cytopathologist. PMID:18521248

  12. Pleomorphic malignant fibrous histiocytoma/undifferentiated high-grade pleomorphic sarcoma of the scrotum in a patient presenting as fournier gangrene: a case report.

    PubMed

    Guo, Juan; Zhou, Shengmei; Rao, Nagesh P; Pez, Gholam H

    2010-10-01

    Pleomorphic malignant fibrous histiocytoma (MFH), also known as undifferentiated high-grade pleomorphic sarcoma according to the latest World Health Organization classification, is a diagnosis of exclusion and extremely rare in adult scrotal/paratesticular region. Clinical presentation of scrotal/paratesticular pleomorphic MFH is usually a painless and gradual scrotal swelling. We report a case of scrotal MFH in a 63-year-old man who presented as Fournier gangrene after 10-month painful scrotal swelling and multiple procedures. The specimen of emergent debridement was submitted for pathologic and bacteriologic examination. Microscopically, the lesion had marked architectural and cytologic pleomorphism. The neoplastic cells were positive for vimentin, but negative for all lineage-specific markers. Fluorescence in-situ hybridization showed an aneuploid karyotype and negative results for lipomatous tumor abnormalities. Bacterial cultures of the specimen showed extensive growth of virulent polymicrobes. The diagnosis of scrotal/paratesticular pleomorphic MFH with concurrent Fournier gangrene was made. Thoracic computed tomography scan showed bilateral multiple pulmonary nodules. The patient died 1 month later. PMID:20505510

  13. Angiolipoma of the female breast: clinicomorphological correlation of 52 cases.

    PubMed

    Kryvenko, Oleksandr N; Chitale, Dhananjay A; VanEgmond, Eve M; Gupta, Nilesh S; Schultz, Daniel; Lee, Min W

    2011-02-01

    The authors analyzed 52 cases of female breast angiolipoma (AL). Age distribution was 25 to 80 years of age (56.81 ± 12.78). Most cases showed vascularity below 50%, and 14 cases had vascularity >50%. Cellular and low-vascularity ALs had different clinical and radiological presentations. The mean size was 7.00 ± 3.62 mm for cellular ALs and 19.61 ± 7.58 mm for low-vascularity ALs. In any paucicellular area, the authors could identify a cluster of at least 3 interconnected vessels. The endothelium was mostly flat with uniform, hyperchromatic nuclei, and mitoses and nucleoli were absent. Fibrin thrombi in proliferating capillaries were noted in 96% of cases. Low-vascularity AL can be reliably distinguished on needle core biopsy from other lipomatous and vascular tumors of the breast. Tortuosity and proliferation of capillaries with at least 3 interconnected capillary channels in 1 focus with associated fibrin thrombi constitute a very strong clue for the diagnosis of AL on a breast needle core biopsy. Definite diagnosis of cellular AL is not always feasible because of rare cases with mitotic activity and cellular atypia. Excision is often recommended for cellular AL.

  14. Sudden onset of paraplegia caused by hemorrhagic spinal epidural angiolipoma. A case report.

    PubMed

    Akhaddar, Ali; Albouzidi, Abderrahmane; Elmostarchid, Brahim; Gazzaz, Miloudi; Boucetta, Mohamed

    2008-09-01

    Spinal epidural angiolipoma is a rare benign tumor containing vascular and mature adipose elements. A slow progressive clinical course was mostly presented and rarely a fluctuating course during pregnancy. The authors report the original case of spontaneous spinal epidural bleeding resulting from thoracic epidural angiolipoma who presented with hyperacute onset of paraplegia, simulating an extradural hematoma. The patient was admitted with sudden non-traumatic hyperacute paraplegia during a prolonged walk. Neurologic examination showed sensory loss below T6 and bladder disturbances. Spinal MRI revealed a non-enhanced heterogeneous thoracic epidural lesion, extending from T2 to T3. A bilateral T2-T4 laminectomy was performed to achieve resection of a lipomatous tumor containing area of spontaneous hemorrhage. The postoperative course was uneventful with complete neurologic recovery. Histologic examination revealed the tumor as an angiolipoma. Because the prognosis after rapid surgical management of this lesion is favorable, the diagnosis of spinal angiolipoma with bleeding should be considered in the differential diagnosis of hyperacute spinal cord compression.

  15. Spinal extradural angiolipoma: report of two cases and review of the literature.

    PubMed

    Gelabert-González, Miguel; García-Allut, Alfredo

    2009-03-01

    Spinal angiolipomas are benign uncommon neoplasm composed of mature lipocytes admixed with abnormal blood vessels. They account for only 0.04-1.2% of all spinal tumors. We report two cases of lumbar extradural angiolipoma and review previously reported cases. We found 118 cases of spinal epidural angiolipoma (70 females and 48 males; age range 1.5-85 years, mean 44.03) spanning from 1890 to 2006. Prior to diagnosis 40.6% of the patients had weakness of the lower limbs. The interval between the initial symptoms and tumor diagnosis ranged from 1 day to 17 years (mean 20.2 months). Except for four cases diagnosed at autopsy, 109 patients underwent surgery and gross-total resection was performed in 79 cases (72.4%). Spinal angiolipomas are tumors containing angiomatous and lipomatous tissue, predominantly located in the mid-thoracic region. All angiolipomas show iso- or hyperintensity on T1-weighted images and hyperintensity on T2-weighted images and most lesions enhance with gadolinium administration. The treatment for spinal extradural angiolipomas is total surgical resection and no adjuvant therapy should be administered.

  16. Oncocytic Lipoadenoma of Submandibular Gland: A Case Report

    PubMed Central

    2015-01-01

    Lipomatous tumours of salivary glands are very uncommonly diagnosed and reported tumours. Majority of these tumours present as painless masses which grow slowly over many years. On the histological grounds, they can be subclassified into “monophasic” (lipoma component only) and “biphasic” (lipoma component+epithelial component) tumours. A handful of biphasic tumours contain oncocytic component and they have been omitted from the WHO classification of salivary gland tumours so far and this has remained a matter of persistent confusion and controversy over many years. Other rare variants of monophasic tumours include fibrolipoma, sialolipoma, angiolipoma, sialadenosis and very rarely a liposarcoma can also occur in salivary glands. Pre-operative imaging can help in picking up the fatty component of the tumours and guide in accurate classification. FNAC may not be accurate in diagnosis of these tumours. We report a case of 45-year-old gentleman presenting with submandibular gland swelling for which submandibular gland excision was done. A final diagnosis of oncocytic lipoadenoma was made. We present this rare entity to add to the few cases reported to date and hence, to increase recognisation and understanding of these rare tumours, which may help in establishing a reproducible subclassification. We have discussed the pathological aspects with review of literature of this very rare entity. PMID:25954628

  17. Spinal epidural angiolipomas: Clinical characteristics, management and outcomes

    PubMed Central

    Bouali, Sofiene; Maatar, Nidhal; Bouhoula, Asma; Abderrahmen, Khansa; Said, Imed Ben; Boubaker, Adnen; Kallel, Jalel; Jemel, Hafedh

    2016-01-01

    Purpose: The spinal epidural angiolipomas are rare expansive processes made of mature lipomatous and angiomatous elements. They often have a benign character. Their etiology, pathogenesis remains uncertain, and it is a cause of spinal cord compression. The magnetic resonance imaging is the most important neuroradiological examination. Histological examination is the only examination to confirm the diagnosis. Surgery is the treatment of choice. Methods: A retrospective study of all patients operated on for a spinal epidural angiolipoma at the Department of Neurosurgery at the National Institute of Neurology of Tunis between January 2000 and December 2014 (15 years) was performed. The aim of this study is to describe the clinical, radiological, histological characteristics and the treatment of this tumor. Results: A total of nine patients were operated from January 01, 2000 to November 30, 2014. The average age of our patients was 51 years with ages that ranged from 29 to 65 with a male predominance. The period between onset of symptoms and diagnosis ranged from 24 months with an average 12 months. Posterior localization of the tumor was seen in all patients. Surgical resection was performed for all cases. The postoperative course has been satisfactory, with a complete recovery of neurological functions in all patients. Conclusions: The spinal epidural angiolipomas is rare expansive process causing spinal cord compression. Treatment is exclusively surgical resection. The functional outcome of spinal epidural angiolipomas is particularly favorable with a complete neurological recovery is if the patient was quickly operated. PMID:27695535

  18. Spinal epidural angiolipomas: Clinical characteristics, management and outcomes

    PubMed Central

    Bouali, Sofiene; Maatar, Nidhal; Bouhoula, Asma; Abderrahmen, Khansa; Said, Imed Ben; Boubaker, Adnen; Kallel, Jalel; Jemel, Hafedh

    2016-01-01

    Purpose: The spinal epidural angiolipomas are rare expansive processes made of mature lipomatous and angiomatous elements. They often have a benign character. Their etiology, pathogenesis remains uncertain, and it is a cause of spinal cord compression. The magnetic resonance imaging is the most important neuroradiological examination. Histological examination is the only examination to confirm the diagnosis. Surgery is the treatment of choice. Methods: A retrospective study of all patients operated on for a spinal epidural angiolipoma at the Department of Neurosurgery at the National Institute of Neurology of Tunis between January 2000 and December 2014 (15 years) was performed. The aim of this study is to describe the clinical, radiological, histological characteristics and the treatment of this tumor. Results: A total of nine patients were operated from January 01, 2000 to November 30, 2014. The average age of our patients was 51 years with ages that ranged from 29 to 65 with a male predominance. The period between onset of symptoms and diagnosis ranged from 24 months with an average 12 months. Posterior localization of the tumor was seen in all patients. Surgical resection was performed for all cases. The postoperative course has been satisfactory, with a complete recovery of neurological functions in all patients. Conclusions: The spinal epidural angiolipomas is rare expansive process causing spinal cord compression. Treatment is exclusively surgical resection. The functional outcome of spinal epidural angiolipomas is particularly favorable with a complete neurological recovery is if the patient was quickly operated.

  19. Multiple benign symmetric lipomatosis -- a differential diagnosis of obesity.

    PubMed

    Ardeleanu, V; Chicos, S; Georgescu, C; Tutunaru, D

    2013-01-01

    Benign symmetrical lipomatosis (BSL), or Madelung's disease, is a rare disease characterized by the progressive growth of diffuse, painless, non-enveloped symmetric lipomas. The etiology of this disease remains unknown, although it was associated in the medical literature with alcohol and nicotine abuse, metabolic disorders and a number of malignancies. It is assumed that there is a genetic predisposition for this affliction. The management in such cases is surgical removal of the lipomas, most times in several sessions, but this is often followed by relapse. However, surgical removal of the lipomas can provide satisfactory patient functionality and cosmetic results. The differential diagnosis is made with morbid obesity, Cushing syndrome, angiolipomatosis, encapsulated lipomas, neurofibromatosis, myxoid liposarcoma, lymphoma, salivary gland disease, Frolich and lipomatosis syndrome in patients infected with HIV. The current paper reports the case of a 55 year-old man, who presented with several large lipomatous masses, arranged symmetrically on the front and back of the trunk, and several smaller lipomas in the upper and lower limbs. Treatment consisted of resection of these lipomas in several sessions, without recurrence at one year after the last operation.

  20. Sialolipoma in minor salivary gland: case report and review of the literature.

    PubMed

    de Moraes, Maiara; de Matos, Felipe Rodrigues; de Carvalho, Cyntia Pereira; de Medeiros, Ana Miryam Costa; de Souza, Lélia Batista

    2010-09-01

    Sialolipoma is a rare benign neoplasm characterized by a well-circumscribed mass composed of neoplastic mature adipose tissue and non-neoplastic salivary gland elements. A 72-year-old woman presented with a painless swelling located in the hard palate, which had been identified 15 days earlier. Microscopically, the tumor was well-circumscribed consisting of lobular proliferation of the lipomatous tissue with thin fibrous tissue septa containing clustered salivary gland elements. Both the glandular and adipose components were found in almost equal proportion. No atypia in the adipose tissue was observed. The definitive diagnosis was sialolipoma. The patient showed no signs of recurrence 8 months after surgical excision. Including the present case, 35 cases of sialolipoma have been reported in the English literature. Of these 35 cases, 16 cases were located in minor salivary glands. Gender was identified in 14 of these cases with 4 males (28.5%) and 10 females (71.5%). The age distribution was from 27 to 84 years (mean, 61.6 years) and the tumor size ranged from 0.9 to 4 cm (mean, 1.7 cm). The most frequently reported clinical presentation was of a painless swelling (56.3%).

  1. Oncocytic lipoadenoma of submandibular gland: a case report.

    PubMed

    Parmar, Harshad V

    2015-03-01

    Lipomatous tumours of salivary glands are very uncommonly diagnosed and reported tumours. Majority of these tumours present as painless masses which grow slowly over many years. On the histological grounds, they can be subclassified into "monophasic" (lipoma component only) and "biphasic" (lipoma component+epithelial component) tumours. A handful of biphasic tumours contain oncocytic component and they have been omitted from the WHO classification of salivary gland tumours so far and this has remained a matter of persistent confusion and controversy over many years. Other rare variants of monophasic tumours include fibrolipoma, sialolipoma, angiolipoma, sialadenosis and very rarely a liposarcoma can also occur in salivary glands. Pre-operative imaging can help in picking up the fatty component of the tumours and guide in accurate classification. FNAC may not be accurate in diagnosis of these tumours. We report a case of 45-year-old gentleman presenting with submandibular gland swelling for which submandibular gland excision was done. A final diagnosis of oncocytic lipoadenoma was made. We present this rare entity to add to the few cases reported to date and hence, to increase recognisation and understanding of these rare tumours, which may help in establishing a reproducible subclassification. We have discussed the pathological aspects with review of literature of this very rare entity.

  2. Hepatic angiomyolipoma: a series of six cases with emphasis on pathological-radiological correlations and unusual variants diagnosed by core needle biopsy

    PubMed Central

    Agaimy, Abbas; Vassos, Nikolaos; Croner, Roland S; Strobel, Deike; Lell, Michael

    2012-01-01

    Hepatic angiomyolipoma is rare and may pose differential diagnostic difficulty, particularly if encountered in core needle biopsy. We studied 6 cases from 5 males and one female (median age, 48.6 yrs). All presented with non-specific symptoms or an incidentally discovered tumor mass. Two patients had a remote history of chemotherapy for hematological neoplasms (acute lymphoblastic leukemia and Hodgkin lymphoma respectively) and another had clear cell renal cell carcinoma and anaplastic pancreatic carcinoma diagnosed at autopsy without definable syndrome. None of the patients had evidence of the tuberous sclerosis complex or renal or other extra-renal angiomyolipoma. Three tumors were resected completely and three have been only biopsied and followed up. None of the resected cases recurred at a mean follow-up of 35 months. Histologically, tumors were classified as classical triphasic (1), lipomatous (2), epithelioid/oncocytoid (1), epithelioid trabecular (1) and myelolipoma-like (1). The adjacent liver parenchyma was normal in 3 cases, showed pigment cirrhosis in one case and mild fatty change in another case. One case had clinically diagnosed but histologically unverified cirrhosis. The initial diagnostic impression/frozen section was misleading in 5 of the cases and included vascular lesion, focal fatty change, myelolipoma, hepatocellular tumor and oncocytic neoplasm. All tumors expressed HMB45 and variably desmin. One epithelioid lesion expressed HMB45 and TFE3, but lacked desmin expression. In conclusion, hepatic angiomyolipomas are increasingly recognized as incidental findings during surveillance for cirrhosis or investigations for unrelated conditions. Awareness of their diverse morphological spectrum in liver biopsy is necessary to avoid misdiagnosis as hepatocellular carcinoma, metastatic melanoma or other malignant neoplasms. PMID:22949933

  3. Cystic Fibrosis below the Diaphragm: Abdominal Findings in Adult Patients.

    PubMed

    Lavelle, Lisa P; McEvoy, Sinead H; Ni Mhurchu, Elaine; Gibney, Robert G; McMahon, Colm J; Heffernan, Eric J; Malone, Dermot E

    2015-01-01

    Cystic fibrosis (CF) is the most common lethal autosomal recessive disease in the white population. Mutation of the CF transmembrane conductance regulator gene on chromosome 7 results in production of abnormally viscous mucus and secretions in the lungs of patients with CF. A similar pathologic process occurs in the gastrointestinal tract, pancreas, and hepatobiliary system. Inspissated mucus causes luminal obstruction and resultant clinical and radiologic complications associated with the disease process. Pancreatic involvement can result in exocrine and endocrine insufficiency, pancreatic atrophy, fatty replacement, or lipomatous pseudohypertrophy. Acute and chronic pancreatitis, pancreatic calcification, cysts, and cystosis also occur. Hepatic manifestations include hepatic steatosis, focal biliary and multilobular cirrhosis, and portal hypertension. Biliary complications include cholelithiasis, microgallbladder, and sclerosing cholangitis. The entire digestive tract can be involved. Distal ileal obstruction syndrome, intussusception, appendicitis, chronic constipation, colonic wall thickening, fibrosing colonopathy, pneumatosis intestinalis, gastroesophageal reflux, and peptic ulcer disease have been described. Renal manifestations include nephrolithiasis and secondary amyloidosis. The educational objectives of this review are to reveal the abdominal manifestations of CF to facilitate focused analysis of cross-sectional imaging in adult patients. Life expectancy in patients with CF continues to improve because of a combination of aggressive antibiotic treatment, improved emphasis on nutrition and physiotherapy, and development of promising new CF transmembrane conductance regulator modulators. As lung function and survival improve, extrapulmonary conditions, including hepatic and gastrointestinal malignancy, will be an increasing cause of morbidity and mortality. Awareness of the expected abdominal manifestations of CF may assist radiologists in identifying

  4. Managing Liposarcomas: Cutting Through the Fat.

    PubMed

    Manji, Gulam A; Schwartz, Gary K

    2016-03-01

    Liposarcomas are one of the most common of more than 50 histologic subtypes of soft tissue sarcomas that, themselves, are heterogeneous. Liposarcomas fall into four distinct histologic subtypes: atypical lipomatous tumor/well-differentiated liposarcoma, dedifferentiated liposarcoma, myxoid (round cell) liposarcoma, and pleomorphic liposarcoma. Definitive treatment remains surgical resection with negative margins for resectable disease. However, well-differentiated liposarcomas that are large or difficult to operate upon should be followed with close surveillance as long as there is no radiologic concern for a dedifferentiated component. In contrast, first-line chemotherapy with anthracycline with or without ifosfamide, or gemcitabine and docetaxel should be used for inoperable myxoid (round cell) or pleomorphic liposarcomas, which are relatively responsive to chemotherapy. In the second- and third-line setting, myxoid liposarcomas, in particular, seem to be sensitive to trabectedin, which was recently approved by the US Food and Drug Administration (FDA). Eribulin offered a survival benefit when compared with dacarbazine in the third-line setting in liposarcomas (other than the well-differentiated subtype) and is now FDA approved. Recent studies have identified distinct genetic aberrations that not only aid in the diagnosis of liposarcoma subtypes but represent actionable targets. Cyclin-dependent kinase 4 and murine double minute 2 are overexpressed in well-differentiated and dedifferentiated liposarcomas and offer opportunities that are being pursued in clinical trials. It is critical that liposarcomas are not approached by oncologists as one disease entity but rather subclassified into distinct subtypes using histologic and molecular tools before formalizing a treatment plan. PMID:26962163

  5. Challenging dedifferentiated liposarcoma identified by MDM2-amplification, a report of two cases

    PubMed Central

    2014-01-01

    Background Liposarcoma is the most frequent soft tissue sarcoma. Well differentiated liposarcoma may progress into dedifferentiated liposarcoma with pleomorphic histology. A minority additionally features myogenic, osteo- or chondrosarcomatous heterologous differentiation. Genomic amplification of the Mouse double minute 2 homolog (MDM2) locus is characteristic for well differentiated and dedifferentiated liposarcomas. Detection of MDM2 amplification may supplement histopathology and aid to distinguish liposarcoma from other soft tissue neoplasia. Case presentation Here we present two cases of dedifferentiated liposarcoma with challenging presentation. Case 1 features a myogenic component. As the tumour infiltrated the abdominal muscles and showed immunohistochemical expression of myogenic proteins, rhabdomyosarcoma had to be ruled out. Case 2 has an osteosarcomatous component resembling extraosseous osteosarcoma. The MDM2 status was determined in both cases and helped making the correct diagnosis. Overexpression of MDM2 and co-overexpression of Cyclin-dependent kinase 4 is demonstrated by immunohistochemistry. The underlying MDM2 amplification is shown by fluorescence in situ hybridisation. Since low grade osteosarcoma may also harbour MDM2 amplification it is emphasised that the amplification has to be present in the lipomatous parts of the tumour to distinguish liposarcoma from extraosseous osteosarcoma. Conclusions The two cases exemplify challenges in the diagnoses of dedifferentiated liposarcoma. Liposarcoma often has pleomorphic histology and additionally may feature heterologous components that mimic other soft tissue neoplasms. Amplification of MDM2 is characteristic for well differentiated and dedifferentiated liposarcomas. Determination of the MDM2 status by in situ hybridisation may assist histopathology and help to rule out differential diagnoses. PMID:25126005

  6. PIK3CA-Related Overgrowth Spectrum (PROS): Diagnostic and Testing Eligibility Criteria, Differential Diagnosis, and Evaluation

    PubMed Central

    Keppler-Noreuil, Kim M.; Rios, Jonathan J.; Parker, Victoria E.R.; Semple, Robert K.; Lindhurst, Marjorie J.; Sapp, Julie C.; Alomari, Ahmad; Ezaki, Marybeth; Dobyns, William; Biesecker, Leslie G.

    2015-01-01

    Somatic activating mutations in the phosphatidylinositol-3-kinase/AKT/mTOR pathway underlie heterogeneous segmental overgrowth phenotypes. Because of the extreme differences among patients, we sought to characterize the phenotypic spectrum associated with different genotypes and mutation burdens, including a better understanding of associated complications and natural history. Historically, the clinical diagnoses in patients with PIK3CA activating mutations have included Fibroadipose hyperplasia or Overgrowth (FAO), Hemihyperplasia Multiple Lipomatosis (HHML), Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi, Scoliosis/Skeletal and Spinal (CLOVES) syndrome, macrodactyly, Fibroadipose Infiltrating Lipomatosis, and the related megalencephaly syndromes, Megalencephaly-Capillary Malformation (MCAP or M-CM) and Dysplastic Megalencephaly (DMEG). A workshop was convened at the National Institutes of Health (NIH) to discuss and develop a consensus document regarding diagnosis and treatment of patients with PIK3CA-associated somatic overgrowth disorders. Participants in the workshop included a group of researchers from several institutions who have been studying these disorders and have published their findings, as well as representatives from patient-advocacy and support groups. The umbrella term of “PIK3CA-Related Overgrowth Spectrum (PROS)” was agreed upon to encompass both the known and emerging clinical entities associated with somatic PIK3CA mutations including, macrodactyly, FAO, HHML, CLOVES, and related megalencephaly conditions. Key clinical diagnostic features and criteria for testing were proposed, and testing approaches summarized. Preliminary recommendations for a uniform approach to assessment of overgrowth and molecular diagnostic testing were determined. Future areas to address include the surgical management of overgrowth tissue and vascular anomalies, the optimal approach to thrombosis risk, and the testing of potential

  7. Clinical Delineation and Natural History of the PIK3CA-Related Overgrowth Spectrum**

    PubMed Central

    Keppler-Noreuil, Kim M; Sapp, Julie C; Lindhurst, Marjorie J; Parker, Victoria ER; Blumhorst, Cathy; Darling, Thomas; Tosi, Laura L; Huson, Susan M; Whitehouse, Richard W; Jakkula, Eveliina; Grant, Ian; Balasubramanian, Meena; Chandler, Kate E; Fraser, Jamie L; Gucev, Zoran; Crow, Yanick J; Brennan, Leslie Manace; Clark, Robin; Sellars, Elizabeth A; Pena, Loren DM; Krishnamurty, Vidya; Shuen, Andrew; Braverman, Nancy; Cunningham, Michael L; Sutton, V Reid; Tasic, Velibor; Graham, John M; Geer, Joseph; Henderson, Alex; Semple, Robert K; Biesecker, Leslie G

    2014-01-01

    Somatic mutations in the phosphatidylinositol/AKT/mTOR pathway cause segmental overgrowth disorders. Diagnostic descriptors associated with PIK3CA mutations include fibroadipose overgrowth (FAO), Hemihyperplasia multiple Lipomatosis (HHML), Congenital Lipomatous Overgrowth, Vascular malformations, Epidermal nevi, Scoliosis/skeletal and spinal (CLOVES) syndrome, macrodactyly, and the megalencephaly syndrome, Megalencephaly-Capillary malformation (MCAP) syndrome. We set out to refine the understanding of the clinical spectrum and natural history of these phenotypes, and now describe 35 patients with segmental overgrowth and somatic PIK3CA mutations. The phenotypic data show that these previously described disease entities have considerable overlap, and represent a spectrum. While this spectrum overlaps with Proteus syndrome (sporadic, mosaic, and progressive) it can be distinguished by the absence of cerebriform connective tissue nevi and a distinct natural history. Vascular malformations were found in 15/35 (43%) and epidermal nevi in 4/35 (11%) patients, lower than in Proteus syndrome. Unlike Proteus syndrome, 31/35 (89%) patients with PIK3CA mutations had congenital overgrowth, and in 35/35 patients this was asymmetric and disproportionate. Overgrowth was mild with little postnatal progression in most, while in others it was severe and progressive requiring multiple surgeries. Novel findings include: adipose dysregulation present in all patients, unilateral overgrowth that is predominantly left-sided, overgrowth that affects the lower extremities more than the upper extremities and progresses in a distal to proximal pattern, and in the most severely affected patients is associated with marked paucity of adipose tissue in unaffected areas. While the current data are consistent with some genotype–phenotype correlation, this cannot yet be confirmed. © The Authors. American Journal of Medical Genetics Part A published by Wiley Periodicals, Inc. PMID:24782230

  8. Diagnostic dilemmas in enlarged and diffusely hemorrhagic adrenal glands.

    PubMed

    Diolombi, Mairo L; Khani, Francesca; Epstein, Jonathan I

    2016-07-01

    We have noted an increasing number of cases of enlarged adrenal glands where the underlying diagnosis was masked by a diffusely hemorrhagic process. We identified from our database 59 cases (32 consults, 27 routine) of adrenal glands with diffuse (>25%) hemorrhage received between 2000 and 2014. Fifty-three adrenalectomies and 6 biopsies were identified. The diagnoses after central review were 41 adrenocortical adenomas, 1 nodular adrenocortical hyperplasia with associated myelolipoma, 1 benign adrenocortical cyst, and 10 nonneoplastic adrenal glands with hemorrhage. A definitive diagnosis for the 6 biopsies was precluded by the sample size. The adrenocortical adenomas (size, 1-13 cm; 25%-95% hemorrhage) showed clear cell change in the neoplastic area (10%-80% of the tumor), 19 showed focal calcification (1 with ossification), 11 showed areas of papillary endothelial hyperplasia, 10 showed scattered lymphoplasmacytic inflammation, 6 showed benign cortical tissue extending beyond the adrenal capsule into soft tissue, 1 showed necrosis in the form of ghost cells, 2 showed lipomatous change, and 6 were associated with incidental benign lesions (1 cortical cyst, 1 schwannoma, and 4 myelolipomas). Twenty-four of the adrenocortical adenomas were consults where the referring pathologist had trouble classifying the lesion. Of the 10 nonneoplastic adrenals (4.5-22 cm; 40%-80% hemorrhage), 2 were consults. In summary, pathologists have difficulties recognizing adrenocortical adenomas in the setting of a massively enlarged and hemorrhagic adrenal gland. Although there is a correlation between adrenocortical malignancy and size, hemorrhage into nonmalignant adrenal glands can result in markedly enlarged adrenals.

  9. Three dosimetry models of lipoma arborescens treated by {sup 90}Y synovectomy

    SciTech Connect

    O’Doherty, Jim; Clauss, Ralf; Scuffham, James; Khan, Aman; Petitguillaume, Alice; Desbrée, Aurélie

    2014-05-15

    Purpose: Lipoma arborescens (LA) is a benign intra-articular lipomatous proliferation of the synovial membrane. This extremely rare condition has previously been treated by intra-articular{sup 90}Y radiosynoviorthesis but dosimetry literature on this form of radionuclide therapy is nonexistent. The authors detail methodology for successful treatment of LA and provide for the first time estimates of radiation dosimetry. The authors also analyze the biodistribution of the radiopharmaceutical over the course of the patient's treatment through sequential imaging. Methods: A patient with bilateral LA underwent intracavity injection of{sup 90}Y citrate colloid to the right and left knee joint spaces (181 and 198 MBq, respectively). SPECT/CT datasets were acquired over 9 days to quantify the biodistribution and kinetics of the radiopharmaceutical. Radiation dosimetry was performed using the MIRD schema (through OLINDA software), a custom voxel-based method, and a direct Monte Carlo calculation (OEDIPE). Results: Follow-up MRI showed marked reduction in LA size in both knees. Mean absorbed doses to the LA were 21.2 ± 0.8 and 42.9 ± 2.3 Gy using OLINDA, 8.1 ± 0.3 and 16.7 ± 0.5 Gy using voxel based methodology, and 8.2 ± 0.3 and 15.7 ± 0.5 Gy for OEDIPE in the right and left LA, respectively. Distribution of the radiopharmaceutical within the joint space alters over the imaging period, with less than 1% of the remaining activity having moved posteriorly in the knee cavity. No uptake was detected outside of the joint space after assessment with whole-body scintigraphy. Conclusions: An activity of approximately 185 MBq successfully relieved clinical symptoms of LA. There was good correlation between direct Monte Carlo and voxel based techniques, but OLINDA was shown to overestimate the absorbed dose to the tumor. Accurate dosimetry may help select an activity more tailored to the specific size and location of the LA.

  10. Scope of FNAC in the diagnosis of soft tissue tumors-A study from a tertiary cancer referral center in India

    PubMed Central

    Rekhi, Bharat; Gorad, Biru D; Kakade, Anagha C; Chinoy, RF

    2007-01-01

    Background Fine needle aspiration cytology (FNAC) forms one of the first diagnostic tools in the evaluation of tumors. Its role in diagnosing soft tissue tumors (STT) has been fairly documented, as well as debated. Present study was aimed at evaluating its scope in diagnosing 127 cases of soft tissue tumors. Methods Conventional Pap and MGG staining was available in all the cases. Immunocytochemistry (ICC) was performed in 15 cases. Histopathological details were available in 115 cases. Results 50% cases were referred for a primary diagnosis, while 26.8% & 22.8% cases were evaluated for recurrent and metastatic lesions, respectively. Extremities were the commonest sites. On FNAC, 101 cases (79.5%) were labeled as malignant, whereas 10 cases (7.9%) were labeled as benign. The remaining 16 cases (11%) were not categorized and were labeled as 'unsure/not specified'. Histopathological confirmation in 115 cases, gave a diagnostic accuracy of 98%, with a positive predictive value of 98% in malignant cases and a negative predictive value of 100% in benign cases. Two cases were false positive. Among the various cytological categories, 60 cases (47.2%) were of spindle cell type, followed by 32 (25.2%) of round cell type and 14 cases (11%) of lipomatous type. Other 12 cases (9.4%) were of pleomorphic type; 7 (5.5%) cases of epithelioid type and remaining 2 cases were of myxoid type. All the round cell, pleomorphic and myxoid type of tumors were sarcomas, whereas 73.3% cases of spindle cell type were labeled as 'malignant'. Exact cytological sub typing was offered in 58 cases, with rhabdomyosarcoma (RMS) as the most frequently sub typed tumor. The two false positive malignant cases were of fibromatosis and a pigmented schwannoma, on biopsy. Out of 28 metastatic lesions, lymph nodes were the commonest site for metastasis, with epithelioid tumors that formed highest percentage of metastatic cases. Conclusion FNAC is fairly specific and sensitive in STT diagnoses for primary

  11. A clinicopathologic comparison of malignant fibrous histiocytoma and liposarcoma.

    PubMed

    Spanier, S S; Floyd, J

    1989-01-01

    The diagnosis of MFH depends on the demonstration of histiocytic and fibroblastic functions. MFHs may phagocytose fat; therefore, lipid stains are useless. By electron microscopy, cytoplasmic lipid is membrane-bound. Immunohistochemical staining for vimentin and histiocytic markers may be helpful. Liposarcoma is diagnosed only when there is convincing evidence of synthesis and storage of fat by the tumor cells. By electron microscopy, cytoplasmic lipid is nonmembrane-bound. Both MFH and liposarcoma have subtypes. In MFH, the pleomorphic forms are the most common. Myxoid MFH is less common; all other types are rare. In liposarcoma, the myxoid types are by far the most common. The myxoid types of both MFH and liposarcoma may contain other elements that vary in degree and geographic distribution and that can raise the histologic grade. About 50% of liposarcomas are low-grade tumors; these are almost always purely myxoid. Low-grade myxoid liposarcoma has a much better prognosis than other types. Myxoid liposarcoma has a better prognosis than myxoid MFH. The peak incidence of MFH is in the seventh decade of life whereas that of liposarcoma is in the fifth decade. A substantial number (roughly 25% to 30%) of MFHs occur in the subcutaneous tissue. Clinically they are almost invariably mistaken for ganglion cysts. Liposarcoma, however, is likely to occur in or below the buttocks. Most are in the anterior thigh. Subcutaneous liposarcoma is extremely rare. A tumor in this area is likely to be either a more malignant myxoid MFH or one of the benign atypical lipomatous tumors. In both MFH and liposarcoma, the development of distant lesions is related to the tumor's histologic grade and size and to local recurrence. Favored metastatic sites of MFH are lung and lymph nodes. Favored sites of distant lesions in the myxoid/round cell types of liposarcoma are intra-abdominal, retroperitoneal, other soft-tissue areas (especially in the neck), and bone. Lymph node involvement is very

  12. In vivo verification of proton beam path by using post-treatment PET/CT imaging

    SciTech Connect

    Hsi, Wen C.; Indelicato, Daniel J.; Vargas, Carlos; Duvvuri, Srividya; Li Zuofeng; Palta, Jatinder

    2009-09-15

    studies, referred as motion-after-Tx cases, also show large misalignment between the field edge and the positron emission distribution in lipomatous tissues around the prostate. These motion-after-Tx cases correspond to patients with large changes in volume of rectal gas between the post-Tx and the post-PET CTs. The standard deviations for D{sub AP} and D{sub SI} are 5.0 and 3.0 mm, respectively, for these motion-after-Tx cases. The final seven studies, referred to as position-error cases, which had a large discordance but no misalignment, were found to have deviations of 4.6 and 3.6 mm in D{sub AP} and D{sub SI}, respectively. The position-error cases correspond to a large discrepancy on the relative location between the centroid and pelvic bones seen in post-Tx CT and recorded x-ray radiographs. Conclusions: Systematic analyses of proton-activated positron emission distributions provide patient-specific information on prostate motion ({sigma}{sub M}) and patient position variability ({Sigma}{sub p}) during daily proton beam delivery. The less than 2 mm of displacement variations in the good cases indicates that population-based values of {Sigma}{sub p} and {sigma}{sub M} used in margin algorithms for treatment planning at the authors' institution are valid for the majority of cases. However, a small fraction of PET/CT studies (approximately 14%) with {approx}4 mm displacement variations may require different margins. Such data are useful in establishing patient-specific planning target volume margins.

  13. Morphologic characterization of spontaneous nervous system tumors in mice and rats.

    PubMed

    Krinke, G J; Kaufmann, W; Mahrous, A T; Schaetti, P

    2000-01-01

    . Primitive neuroectodermal tumors of rats, such as pineal tumors or medulloblastomas, appear to have features similar to those found in man. In mice, the meningeal tumors are mostly devoid of granular cells and the astrocytomas are similar to those occurring in rats, whereas spontaneous oligodendrogliomas are observed extremely rarely. Tumorlike lesions, such as lipomatous hamartomas or epidermoid cysts, are occasionally encountered in the mouse CNS. It is suggested that we classify rodent CNS lesions as "low grade" and "high grade" rather than as "benign" and "malignant." The size of CNS tumors is generally related to their malignancy. Tumors of the peripheral nervous system are schwannomas and neurofibromas or neurofibrosarcomas consisting of Schwann cells, fibroblasts, and perineural cells. Well-differentiated schwannomas are characterized by S-100 positivity and the presence of basement membrane. They show either Antoni A pattern with fusiform palisading cells or Antoni B pattern, which is sparsely cellular and has a clear matrix. The rat develops specific forms of schwannomas in the areas of the submandibular salivary gland, the external ear, the orbit, and the endocardium. Spontaneous ganglioneuromas occur in the rat adrenal medulla or thyroid gland. Compared to experimentally induced neoplasms, the spontaneous tumors of the rodent nervous system are poor and impractical models of human disease, although they may serve as general indicators of the carcinogenic potential of tested chemicals.