Fibrohistiocytic tumors

Abstract

Fibroblastic, myofibroblastic, and fibrohistiocytic tumors and tumor-like lesions of the orbit are mesenchymal lesions that are divided into two major categories in this chapter as fibromatoses and histiocytoses and their morphologic, radiologic, and clinical features are reviewed. About 25 % of fibrohistiocytic masses of the entire body are located in the head and neck region and 10 % involve the orbit. Approximately 75 % of these tumors are considered benign and 10 % are malignant; the rest may show a benign or malignant behavior depending on the type of neoplasm and clinical setting. Fibrous histiocytoma, solitary fibrous tumor, dermatofibrosarcoma protuberans and aggressive fibromatoses of the fibromatoses group and necrobiotic xanthogranuloma, Langerhans cell, and non-Langerhans proliferations of the histiocytoses group are discussed in detail including their management and prognostic trends. Association of these tumors to systemic disorders such as Erdheim–Chester disease and asthma is discussed.