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

Xu,Jingjing; Wang,Jiawei; Zhang,Minming; Li,Baizhou

doi:10.3892/ol.2016.4385

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

Authors:
- Jingjing Xu
- Jiawei Wang
- Minming Zhang
- Baizhou Li
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Affiliations: Department of Radiology, The Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, Zhejiang 310009, P.R. China, Department of Pathology, The Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, Zhejiang 310009, P.R. China
Published online on: March 29, 2016 https://doi.org/10.3892/ol.2016.4385
Pages: 3417-3420

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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.

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