FUS rearrangements are rare in ‘pure' sclerosing epithelioid fibrosarcoma

Wang, Wei Lien; Evans, Harry L.; Meis, Jeanne M.; Liegl‐Atzwanger, Bernadette; Bovée, Judith; Goldblum, John R.; Billings, Steven D.; Rubin, Brian P.; López‐Terrada, Dolores; Lazar, Alexander J.

doi:10.1038/modpathol.2011.214

Cited by 69 publications

(41 citation statements)

References 29 publications

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“…To provide further comparative information, I selected cases with 5 or more years of follow-up from a concurrent study of sclerosing epithelioid fibrosarcoma being carried out at U.T.M.D. Anderson Cancer Center by Wang et al 11 and obtained clinical information on these ( Table 3). As Table 3 demonstrates, sclerosing epithelioid fibrosarcoma occurred in an older age group compared with low-grade fibromyxoid sarcoma, had more frequent metastases, and was associated with a higher death rate and much shorter survival.…”

Section: Discussionmentioning

confidence: 99%

Low-Grade Fibromyxoid Sarcoma

Evans¹

2011

American Journal of Surgical Pathology

235

141

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Cases listed in the U.T.M.D Anderson Cancer Center files as low-grade fibromyxoid sarcoma and originally diagnosed before 2004 were reviewed. They were included in the study if the diagnosis was confirmed and if there was adequate histologic material and clinical information with at least 5 years of follow-up. Thirty-three cases met the study criteria. The patients were 6 to 52 years old at the time of diagnosis (median, 29 y); 19 were male and 14 were female. The most common tumor locations were the shoulder area (5), thigh (5), and inguinal area (4). Tumor size varied from 1.5 to 16 cm (median, 9.4 cm) in those cases in which it was known. The typical histologic findings were contrasting fibrous and myxoid areas, moderate to low cellularity, bland-appearing spindle cells with no or slight nuclear pleomorphism and rare mitotic figures, and a swirling, whorled growth pattern. Prominent vascularity in myxoid areas and perivascular hypercellularity were fairly common, whereas larger hypercellular zones were sometimes seen in primary tumors but were more frequent in recurrences (local) and metastases. Hypercellular regions sometimes had round rather than spindle cells, a diffuse sheet-like cell arrangement, and/or a somewhat increased mitotic rate. Very hypocellular fibrotic areas were also observed and sometimes had thick collagen bundles. Pericollagenous rosettes were present in 6 cases but not in all specimens from these. Other growth pattern variations included storiform, fascicular-herringbone, and patternless areas; uncommonly noted were cell clusters, strands, palisades, and a retiform network. Additional unusual features were moderate nuclear pleomorphism (seen mostly in recurrent and metastatic tumors), cysts, osseous metaplasia, and a tigroid pattern with alternating fibrous and myxoid strips. One patient had a recurrence with features of sclerosing epithelioid fibrosarcoma, whereas 2 had dedifferentiated recurrences with anaplastic predominantly round cells and numerous mitotic figures. Fourteen patients died of tumor after 3 (this patient's tumor became dedifferentiated) to 42 years (median, 15 y). Nineteen patients were alive at last follow-up of 5½ to 70 years (median, 13 y), 6 with tumor and 13 without. Twenty-one patients had recurrence after intervals of up to 15 years (median, 3½ y), and 15 had metastases (mostly in the lungs and pleura) after periods of up to 45 years (median, 5 y). Except for dedifferentiation, which led to short survival after it occurred, histologic differences were not related to tumor behavior or patient survival. The 4 patients whose neoplasms measured <3.5 cm were all tumor free at last follow-up.

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Section: Discussionmentioning

confidence: 99%

Low-Grade Fibromyxoid Sarcoma

Evans¹

2011

American Journal of Surgical Pathology

235

141

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“…It has also been appreciated that one may see tumours showing hybrid morphological features of SEF and LGFMS (Figure ). The precise nosological status of cases of SEF which lack FUS gene rearrangement remains to be determined …”

Section: Introductionmentioning

confidence: 99%

The evolving classification of soft tissue tumours – an update based on the new 2013 WHO classification

2013

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The new World Health Organization (WHO) classification of soft tissue tumours was published in early 2013, almost 11 years after the previous edition. While the number of newly recognized entities included for the first time is fewer than that in 2002, there have instead been substantial steps forward in molecular genetic and cytogenetic characterization of this family of tumours, leading to more reproducible diagnosis, a more meaningful classification scheme and providing new insights regarding pathogenesis, which previously has been obscure in most of these lesions. This brief overview summarizes changes in the classification in each of the broad categories of soft tissue tumour (adipocytic, fibroblastic, etc.) and also provides a short summary of newer genetic data which have been incorporated in the WHO classification.

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“…[3][4][5] MUC4 is less useful as a marker in other spindle cell tumours, as it is expressed with a patchy distribution in only 21% of ossifying fibromyxoid tumours 6 and 30% of synovial sarcomas. 1 A review of our archive revealed that we had reported a primary bone tumour with morphological features typical for LFS in the scapula of a 41-yearold female (case 1, Figure 1A) and a second tumour in the femur of a 62-year-old female (case 2, not shown), exhibiting features of SEF.…”

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confidence: 99%