Skull sclerosing epithelioid fibrosarcoma: A case report and review of the literature

Abstract

Abstract. Sclerosing epithelioid fibrosarcoma (SEF) is an unusual variant of fibrosarcoma that was previously considered to be a low-grade tumor with an indolent course. The tumor occurs most commonly in the soft tissue of the limb, trunk, head and neck, and occasionally in the bone and visceral organs. The skull is a rare primary site for SEF, with only 3 cases reported to date. The current study reports a case of SEF occurring in the occipital bone of a 24-year-old man, who lacked neurological symptoms. Imaging revealed a large mass emanating from the occipital bone and involving the superior sagittal sinus, torcular herophili and adjacent brain tissue. Histological and immunohistochemical characteristics confirmed the diagnosis of SEF. The patient experienced local recurrence and distant metastasis at 10 and 15 months, respectively, subsequent to the resection of the primary mass. The current case and review of the literature suggest that skull SEF may behave clinically as an aggressive malignant sarcoma. Radiological findings indicated the biological and histopathological characteristics of the tumor. Thus, its clinical behavior and certain imaging features may suggest this diagnosis.

Clinical and molecular characterization of primary sclerosing epithelioid fibrosarcoma of bone and review of the literature

“…[4][5][6][7][8][9] Similar to a previous report, 5 one of our cases showed a poor response to Ewing sarcoma chemotherapy regimen. A literature review of previously reported cases showed a similar incidence of metastatic disease, occurring in 50% of the patients.…”

“…1 A pathogenetic link with low grade fibromyxoid sarcoma (LGFMS) has been suggested, due to cases with hybrid morphology as well as overlapping genetic signature. [4][5][6][7][8][9][10] Only in a handful of these reports, the presence of EWSR1 [4][5][6][7][8][9][10] Only in a handful of these reports, the presence of EWSR1…”

Sclerosing Epithelioid Fibrosarcoma – A Rare Sacral Lesion and Literature Review

“…This case report emphasizes the rarity of SEF, and necessity of diagnosis alertness, especially when presenting bone involvement [9,10]. Like it's presented by Chow et al [10], we expect a similar behaviour when compared for the soft-tissue counterpart.…”

“…However, concerning this controversial definition, recently, WHO "Classification of Tumours of Soft Tissue and Bone" review (2013), includes sclerosing epithelioid fibrosarcoma, on malignant Fibroblastic/Myofibroblastic tumours, along with adult fibrosarcoma, myxofibrosarcoma and low-grade fibromyxoid sarcoma [3]. A propensity for local recurrence and late metastasis, findings of necrosis and large anaplastic figures, and an accumulation of p53 by immunohistochemistry that is paradoxically always found, corroborate this classification [9,13,14].…”

“…The first description of SEF with bone origin was found in 2002 by Abdulkader et al [4] that reported a case of an iliac SEF as a primary bone tumour. To our knowledge, on literature (Embase®, NCBI-Pubmed® and Google Scholar®), from around 20 cases of SEF arising from bone [7][8][9], five cases were allocated to the axial skeleton, but there is only one report of sacrum involvement, by Chow et al [10]. Here we describe a rare case of SEF arising from sacral bone tissue, an unusual origin and location for such a relatively rare lesion.…”