Plexiform Fibrohistiocytic Tumor: A Brief Review

Abstract

Abstract Plexiform fibrohistiocytic tumor is a rare mesenchymal neoplasm of intermediate malignancy, first reported by Enzinger and Zhang in 1988. It has a predilection for children and young adults but can occur at any age. The tumor usually involves the upper limbs as a slow-growing, painless mass. The tumor has a high local recurrence rate but metastasizes only rarely. Histologically, the tumor is characterized by poorly demarcated dermal or subcutaneous mass with multinodular plexiform growth and fibrohistiocytic cytomorphology. There are three distinct recognized growth patterns: fibrohistiocytic, fibroblastic, and mixed types. The tumor displays uniform immunoreactivity for vimentin and CD68. Ultrastructurally, the tumor cells have features of myofibroblasts and histiocyte-like cells. Complete surgical resection of the tumor, preferably with wider margins, is required to prevent local recurrence. Long-term follow-up is necessary to detect any nodal or pulmonary metastasis.