Fibrohistiocytic Neoplasms of Borderline Malignancy International Society of Bone and Soft Tissue Pathology

Dr. Enzinger was the primary editor of the first soft tissue World Health Organization (WHO) book in 1969, entitled “Histological Typing of Soft Tissue Tumours.” Although he had not yet described angiomatoid “malignant” fibrous histiocytoma (A(M)FH), giant cell fibroblastoma (GCF), or plexiform fibrohistiocytic tumor (PFHT), he had already considered the concept of a fibrous tumor of intermediate malignant potential category. Therefore, dermatofibrosarcoma protuberans (DFSP) was independently described and separated from benign tumors of fibrous tissue and from fibrosarcoma. Even then Dr. Enzinger classified DFSP as we know this tumor today: “A cellular tumor of disputed histiocytic or fibroblastic origin composed of small uniform cells arranged in a cartwheel pattern. It usually forms a protruding nodular or multinodular mass by infiltration of the entire dermis and the subcutaneous fat. The tumor has a tendency to recur locally after simple excision. Cases with metastases have been recorded.”