Rare Fibroblastic and Myofibroblastic Tumors

This review discusses the clinical presentation, histopathologic characteristics, and diagnostic and treatment algorithms for several distinct fibroblastic and myofibroblastic tumor subtypes. Low-grade fibromyxoid sarcomas typically arise in the deep soft tissues of the proximal extremities or trunk in young adults. They tend to have a high local recurrence rate, although some may have distant metastases. Histologically, they consist of bland fibroblasts with a whorled or linear arrangement alternating with hypocellular myxoid stroma. Sclerosing epithelioid fibrosarcomas typically arise in the deep soft tissues of the lower extremities of middle-aged adults. They tend to be slow growing but clinically aggressive, with a high propensity for local recurrence and distant metastases. Classic histologic features include the presence of small, uniform, ovoid epithelioid cells with sparse cytoplasm, arranged in cords within a sclerotic fibrous stroma. Myxoinflammatory fibroblastic sarcomas mainly affect the hands and feet. They are prone to local recurrence, with a very low likelihood of distant metastasis. Histologically, they have nodular architecture and highly cellular areas with an a