Primary sclerosing epithelioid fibrosarcoma of the sacrum: a case report and review of the literature

• Published in: Journal of clinical pathology 2004-01, Vol.57 (1), p.90-94
• Main Authors: Chow, L T C, Lui, Y H, Kumta, S M, Allen, P W
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; bone; Case Reports; Cytoplasm; EMA; epithelial membrane antigen; Fatal Outcome; Female; Fibrosarcoma - diagnostic imaging; Fibrosarcoma - pathology; Follow-Up Studies; Humans; Investigative techniques, diagnostic techniques (general aspects); LCA; leucocyte common antigen; Medical sciences; Metastasis; Middle Aged; Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques; Radiography; Sacrum; sclerosing epithelioid fibrosarcoma; Spinal Neoplasms - diagnostic imaging; Spinal Neoplasms - pathology; spine; Tomography; Tumors
• ISSN: 0021-9746; 1472-4146
• DOI: 10.1136/jcp.57.1.90

Sclerosing epithelioid fibrosarcoma is a rare tumour characterised histologically by a predominant population of epithelioid cells arranged in strands and nests, embedded in a fibrotic and hyalinised stroma. It is a low grade tumour with an indolent course. A 48 year old woman presented with a painful swelling over her back for six months. Investigation and biopsy revealed features of sclerosing epithelioid fibrosarcoma involving the left half of the sacrum, left sacro-iliac joint, and posterior part of the left ilium. Preoperative radiotherapy and wide location excision of the tumour were followed by metastatic recurrence of the tumour in the lung and scalp six years after initial presentation. The tumour showed typical histology of sclerosing epithelioid fibrosarcoma. The radiological features confirmed its primary location in the sacrum. The patient declined chemotherapy and died of disseminated disease eight years after initial presentation. Review of the literature confirms the fact that sclerosing epithelioid fibrosarcoma, despite its low grade, is a clinicopathologically distinct tumour with full malignant potential, the recurrence, metastasis, and mortality rate being 48%, 60%, and 35%, respectively. Sclerosing epithelioid fibrosarcoma can occur as a primary bone tumour, the clinical behaviour of which is probably similar to its soft tissue counterpart.