Low grade fibromyxoid sarcoma of the perineum: a surgical challenge

Abstract

Low-grade fibromyxoid sarcoma is a rare, slow growing and deceptively benign-appearing neoplasm. It is presently diagnosed on the basis of histopathology (typical fibro-myxoid appearance), immunohistochemistry (vimentin staining) and cytogenetics [chimeric FUS/CREB3L2 gene produced by t (7;16), (q33;p11)]. This tumour should be differentiated from other resembling tumours like myxofibrosarcoma, sclerosing epitheloid fibrosarcoma, desmoid fibromatosis and others. It has the potential for local recurrence and late metastasis, if not treated adequately. Although surgical excision is the only hope for treatment, reporting of details of surgical management have often been neglected for this primarily pathological entity. We report a case of a 30-year female who had a perineal mass which was managed by wide surgical excision. It was diagnosed as low-grade fibromyxoid sarcoma on the basis of histopathology and immunohistochemistry. This extremely rare presentation is, thus, discussed in context of challenging nature of its surgical excision. Patient is in follow-up and has no recurrence even after 5 years.