Fibrosarcoma: a review and update

Abstract

Adult fibrosarcoma, defined by the World Health Organization as a 'malignant neoplasm composed of fibroblasts with variable collagen production and, in classical cases, a "herringbone" architecture', is a very rare soft tissue sarcoma. Once considered the most common adult sarcoma, the incidence of adult fibrosarcoma has declined dramatically over the past several decades. This is due to (i) evolution in the classification of soft tissue tumours (ii) recognition of clinically, morphologically and genetically distinctive subtypes of fibrosarcoma and (iii) increased understanding of the many other mesenchymal and non-mesenchymal tumours that may mimic fibrosarcoma. This review article will summarize the current state of our knowledge about strictly defined adult fibrosarcoma and discuss important entities in its differential diagnosis, including various fibrosarcoma variants, monophasic synovial sarcoma and other potential mesenchymal and non-mesenchymal mimics.

Keywords: dermatofibrosarcoma protuberans; fibrosarcoma; immunohistochemistry; low-grade fibromyxoid sarcoma; molecular genetics; sclerosing epithelioid fibrosarcoma.