Fibrohistiocytic Tumors and Related Neoplasms in Children and Adolescents

Abstract

Fibrohistiocytic tumors (FHTs) in children and adolescents range from the benign fibrous histiocytoma, or dermatofibroma, to a variety of intermediate and malignant neoplasms, such as dermatofibrosarcoma protruberans and high-grade undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma). Those tumors as a group are comprised of fibroblasts, myofibroblasts, and histiocytes-dendritic cells with a variably prominent inflammatory infiltrate consisting of lymphocytes and eosinophils. Dendritic cells are also a major constituent of another group of neoplasms that include Langerhans cell histiocytosis, follicular and interdigitating cell sarcomas, and juvenile xanthogranuloma. These latter tumors are considered in this discussion for the sake of differential diagnosis and their possible histogenetic relationship to FHTs. Recent studies have suggested that the relationship between the fibroblast and histiocyte in the FHTs may reflect the intrinsic capacity to transdifferentiate from one to the other morphologic and functional state. The so-called “facultative fibroblast,” as a cell with fibroblastic and histiocytic properties, was discussed in the context of the fibrous xanthoma 50 years ago. Possibly the entire histogenetic concept of FHTs should be reconsidered in light of current studies.