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Pleomorphic Sarcoma, Undifferentiated (Malignant Fibrous Histiocytoma)

Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: April 21, 2025 · Captured 2026-09-09.

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Context: Treatment of Tumors of Uncertain Differentiation

At one time, malignant fibrous histiocytoma was the single most common histotype among adults with soft tissue sarcomas. Since it was first recognized in the early 1960s, malignant fibrous histiocytoma has been controversial, in terms of both its histogenesis and its validity as a clinico-pathological entity. The World Health Organization (WHO) classification no longer includes malignant fibrous histiocytoma as a distinct diagnostic category but rather as a subtype of an undifferentiated pleomorphic sarcoma.[149,150]

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This entity accounts for 2% to 6% of all childhood soft tissue sarcomas.[151]

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Clinical presentation →Risk factors →Molecular features →Treatment of newly diagnosed pleomorphic sarcoma →Treatment of recurrent or refractory pleomorphic sarcoma →

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