Journal ArticleDOI
Angiomatoid malignant fibrous histiocytoma. A distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm
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TLDR
Most likely it is a tumor of fibroblast‐ and histiocyte‐like cells, akin to malignant fibrous histiocytoma, but different in its age incidence, microscopic appearance, and behavior.Abstract:
This article describes 41 examples of an unusual fibrohistiocytic sarcoma which occurred primarily in the extremities of young individuals between the ages of 5 and 25 years (median 13 years). It manifested as a nodular subcutaneous growth that seldom caused tenderness or pain, and clinically was often mistaken for a hematoma or a hemangioma. Grossly, the tumor presented as a circumscribed, multinodular or multicystic, hemorrhagic mass that ranged in size from 0.7 to 10 cm (median 2.5 cm). On microscopic examination, it consisted principally of 1) solid arrays or nests of fibroblast- and histiocyte-like cells, not infrequently containing varying amounts of intracellular hemosiderin or lipid, 2) focal areas of hemorrhage or hemorrhagic cyst-like spaces, sometimes occupying the major portion of the tumor, and 3) aggregates of chronic inflammatory cells, chiefly lymphocytes and plasmacytes, a feature that caused confusion with a lymph node metastasis in several cases. Follow-up information, available in 24 patients, revealed a variable clinical course. Twenty-one patients were alive, 11 with recurrence (including one with 9 recurrences in a 21-year period) one with recurrence and metastasis and one with metastasis. Three patients had died of metastasis 1, 3, and 13 years respectively, after the initial surgical therapy. The exact histogenesis is still obscure. Most likely it is a tumor of fibroblast- and histiocyte-like cells, akin to malignant fibrous histiocytoma, but different in its age incidence, microscopic appearance, and behavior.read more
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Sarcomas: genetics, signalling, and cellular origins. Part 1: The fellowship of TET.
TL;DR: The mechanisms whereby specific fusion/mutant gene products participate in sarcoma development and the cellular context that may provide the necessary permissiveness for their expression and oncogenicity are covered.
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The diversity of soft tissue tumours with EWSR1 gene rearrangements: a review
TL;DR: The diversity of the several soft tissue tumour types that are associated with rearrangement of the EWSR1 gene is reviewed.
Journal ArticleDOI
The fibroma‐like variant of epithelioid sarcoma
TL;DR: The clinical presentation, ultrastructural features, and presence of vimentin and low molecular weight keratin within the tumor cells justifies their designation as an epithelioid sarcoma variant.
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The role of genetic testing in soft tissue sarcoma.
TL;DR: This review focuses on the applicability of genetic testing in the diagnosis and prognosis of soft tissue sarcomas, and gives a realistic appraisal of the ancillary role of molecular techniques, including its advantages and limitations.
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Fusion of the EWSR1 and ATF1 genes without expression of the MITF-M transcript in angiomatoid fibrous histiocytoma
Karolin H. Hallor,Fredrik Mertens,Yuesheng Jin,Jeanne M. Meis-Kindblom,Lars-Gunnar Kindblom,Mikael Behrendtz,Anders Kalén,Nils Mandahl,Ioannis Panagopoulos +8 more
TL;DR: It is demonstrated that the EWSR1–ATF1 chimera represents a fusion gene that can be associated with different tumor types, and is probably an early step in the transformation process, but the overall gene expression patterns are likely to vary considerably between AFH and CCS, in keeping with their clinicopathologic differences.
References
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Journal ArticleDOI
Malignant fibrous histiocytoma: an analysis of 200 cases.
TL;DR: The clinicopathologic findings in 200 cases of malignant fibrous histiocytoma (MFH) with follow‐up information are presented and it is felt it is best regarded as a primitive and pleomorphic sarcoma showing partial fibroblastic and Histiocytic differentiation, as reflected by collagen production and occasional phagocytosis.
Journal ArticleDOI
Hemangiopericytoma. An analysis of 106 cases.
Franz M. Enzinger,Bruce H. Smith +1 more
TL;DR: The close resemblance of hemangiopericytoma to richly vascular forms of fibrous histiocytomas and synovial sarcoma is emphasized, and the morphologic differences from other mesenchymal tumors showing a hemango-like vascular pattern are discussed.
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Histiocytic tumors (fibrous xanthoma and histiocytoma) in children.
Journal ArticleDOI
Aneurysmal Sclerosing Hemangioma of Skin
TL;DR: Although both lesions were clinically alarming, they were histologically benign and no recurrence or metastasis has developed in either case in follow-up periods of 9 and 2½ years respectively.
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