The Experts below are selected from a list of 1137 Experts worldwide ranked by ideXlab platform
Rana Naous - One of the best experts on this subject based on the ideXlab platform.
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Low-Grade Fibromyxoid Sarcoma: A rare case in an unusual location.
SAGE open medical case reports, 2020Co-Authors: Diandra Perez, Ola El-zammar, Brando Cobanov, Rana NaousAbstract:Low-Grade Fibromyxoid Sarcoma, also known as Evans tumor, is a Low-Grade Sarcoma that most commonly arises in the deep soft tissue of the proximal extremities or trunk in young adults. It is very rare in the viscera as a primary site, with only a few cases reported in the literature. Here, we present a case of Evans tumor occurring in an unusual and rarely reported location; an intrathoracic mass arising from the diaphragmatic pleura.
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Low-Grade Fibromyxoid Sarcoma: A Rare Case in an Unusual Location
American Journal of Clinical Pathology, 2019Co-Authors: Diandra Perez, Ola El-zammar, Rana NaousAbstract:Abstract Introduction Low-Grade Fibromyxoid Sarcoma, also known as Evans tumor, is a Low-Grade Sarcoma that most commonly arises in the deep soft tissue of the proximal extremities or trunk in young adults. It is very rare in the viscera as a primary site with only a few cases reported in the literature. Here we present a case of Evans tumor occurring in an unusual and rarely reported location: the mediastinum. Method A 32-year-old male presented with history of persistent cough, and subsequent imaging revealed a left diaphragmatic tumor measuring around 11 cm with associated chest wall implants. Grossly, the tumor was well circumscribed, arising from the parietal pleura with attachment to both lung and diaphragm and focally involving visceral pleura with no extension into the underlying lung parenchyma. Histologically, the tumor was composed of uniform and bland-appearing spindle cells interspersed among large areas of hyalinized rosettes, so-called giant rosettes. By immunohistochemistry, the tumor was positive for EMA, bcl-2, TLE-1, and MUC-4 and negative for STAT-6, ruling out solitary fibrous tumor. FISH for SYT gene rearrangement was performed and was negative, ruling out synovial Sarcoma. Given the overall morphology and the phenotype, the final diagnosis was Low-Grade Fibromyxoid Sarcoma with giant collagen rosettes (also known as hyalinizing spindle cell tumor with giant rosettes). Conclusion Mediastinal Low-Grade Fibromyxoid Sarcoma is a rare entity that should be considered in the differential diagnosis of visceral soft tissue tumors. Pathologic examination and a thorough immunohistochemical and molecular workup play a vital role in establishing this rare and challenging diagnosis.
Diandra Perez - One of the best experts on this subject based on the ideXlab platform.
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Low-Grade Fibromyxoid Sarcoma: A rare case in an unusual location.
SAGE open medical case reports, 2020Co-Authors: Diandra Perez, Ola El-zammar, Brando Cobanov, Rana NaousAbstract:Low-Grade Fibromyxoid Sarcoma, also known as Evans tumor, is a Low-Grade Sarcoma that most commonly arises in the deep soft tissue of the proximal extremities or trunk in young adults. It is very rare in the viscera as a primary site, with only a few cases reported in the literature. Here, we present a case of Evans tumor occurring in an unusual and rarely reported location; an intrathoracic mass arising from the diaphragmatic pleura.
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Low-Grade Fibromyxoid Sarcoma: A Rare Case in an Unusual Location
American Journal of Clinical Pathology, 2019Co-Authors: Diandra Perez, Ola El-zammar, Rana NaousAbstract:Abstract Introduction Low-Grade Fibromyxoid Sarcoma, also known as Evans tumor, is a Low-Grade Sarcoma that most commonly arises in the deep soft tissue of the proximal extremities or trunk in young adults. It is very rare in the viscera as a primary site with only a few cases reported in the literature. Here we present a case of Evans tumor occurring in an unusual and rarely reported location: the mediastinum. Method A 32-year-old male presented with history of persistent cough, and subsequent imaging revealed a left diaphragmatic tumor measuring around 11 cm with associated chest wall implants. Grossly, the tumor was well circumscribed, arising from the parietal pleura with attachment to both lung and diaphragm and focally involving visceral pleura with no extension into the underlying lung parenchyma. Histologically, the tumor was composed of uniform and bland-appearing spindle cells interspersed among large areas of hyalinized rosettes, so-called giant rosettes. By immunohistochemistry, the tumor was positive for EMA, bcl-2, TLE-1, and MUC-4 and negative for STAT-6, ruling out solitary fibrous tumor. FISH for SYT gene rearrangement was performed and was negative, ruling out synovial Sarcoma. Given the overall morphology and the phenotype, the final diagnosis was Low-Grade Fibromyxoid Sarcoma with giant collagen rosettes (also known as hyalinizing spindle cell tumor with giant rosettes). Conclusion Mediastinal Low-Grade Fibromyxoid Sarcoma is a rare entity that should be considered in the differential diagnosis of visceral soft tissue tumors. Pathologic examination and a thorough immunohistochemical and molecular workup play a vital role in establishing this rare and challenging diagnosis.
Hironori Fujisawa - One of the best experts on this subject based on the ideXlab platform.
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Intracranial Low-Grade Fibromyxoid Sarcoma: Findings on Electron Microscopy and Histologic Analysis
World neurosurgery, 2019Co-Authors: Yu Shimizu, Katsuhiro Tsuchiya, Hironori FujisawaAbstract:Background Low-Grade Fibromyxoid Sarcoma is a rare tumor that manifests as a deep soft tissue mass characterized by a benign histology, but with potentially aggressive clinical behavior and a high rate of recurrence; primary intracranial Sarcomas are even rarer. We present a case of primary intracranial Low-Grade Fibromyxoid Sarcoma, emphasizing its clinical, radiologic, and histologic features. Case Description A 39-year-old woman presented with a recent history of headache and seizures. A right parietal mass was detected on computed tomography and magnetic resonance imaging, and she was subsequently operated on to remove the intracranial mass. Histologic examination of the resected tumor revealed mildly atypical fibroblastic cells embedded within a myxoid matrix. The diagnosis was confirmed by electron microscopy and cytogenetic analyses. Conclusions This report describes electron microscopic evaluation of intracranial Low-Grade Fibromyxoid Sarcoma, which has an extremely rare occurrence.
Brian Osullivan - One of the best experts on this subject based on the ideXlab platform.
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sclerosing epithelioid fibroSarcoma of the jaw late recurrence from a low grade Fibromyxoid Sarcoma
Head and Neck Pathology, 2018Co-Authors: Catherine Laliberte, Iona Leong, Howard I Holmes, Eric Monteiro, Brian OsullivanAbstract:Sclerosing epithelioid fibroSarcoma (SEF) is an uncommon variant of fibroSarcoma that is characterized by a distinct morphology. It most frequently presents in the deep soft tissues of the lower extremities, often in intimate association with fascia and periosteum, although reports of the head and neck involvement have been reported. A minority of cases show morphological, immunohistochemical and molecular overlap with low grade Fibromyxoid Sarcoma (LG-FMS). Herein, we describe a case of a bland spindle cell neoplasm presenting in the jaw that was initially incompletely excised. Over the course of 20 years the tumor subsequently recurred with a SEF morphology. Molecular testing performed on both specimens subsequently confirmed the presence of an EWSR1-CREB3L1 gene fusion. This report highlights the diagnostic difficulty with LG-FMS, particularly in unusual anatomic locations; reiterates the potential for the uncommon EWSR1-CREB3L1 fusion product in LG-FMS; and, reaffirms the potential for progression and/or overlap between LG-FMS to SEF over time.
John X. O'connell - One of the best experts on this subject based on the ideXlab platform.
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Low-Grade Fibromyxoid Sarcoma of the perineum with heterotopic ossification: case report and review of the literature.
Human pathology, 2011Co-Authors: Anna F. Lee, Stephen Yip, Adam C. Smith, Malcolm Hayes, Torsten O. Nielsen, John X. O'connellAbstract:Summary Low-Grade Fibromyxoid Sarcoma was first described more than 20 years ago. Subsequently, it was discovered to carry the recurrent chromosomal translocation t(7;16)(q33;p11) encoding a FUS-CREB3L2 fusion oncoprotein. Molecular tests for this pathognomonic gene fusion can confirm the identity of histologic variants (such as hyalinizing spindle cell tumor with giant rosettes) and suggest that some cases of sclerosing epithelioid fibroSarcoma may represent a high-grade version of this entity. We present a case of an ossifying tumor of the perineum that required an open biopsy and fluorescent in situ hybridization testing for FUS and CREB3L2 for diagnosis as a variant of Low-Grade Fibromyxoid Sarcoma. Subsequent excision revealed characteristic areas with collagen rosettes as well as foci of heterotopic ossification. Significant ossification, which is well documented in entities such as synovial Sarcoma, ossifying Fibromyxoid tumor, and extraskeletal osteoSarcoma, has not been reported previously in Low-Grade Fibromyxoid Sarcoma. This case demonstrates the value of having a distinctive confirmatory molecular pathology test for diagnosis and expands our knowledge of the histologic variants possible in Low-Grade Fibromyxoid Sarcoma.
