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← Childhood Soft Tissue Sarcoma

HEALTH PROFESSIONAL · SOURCE READING

Intracranial Mesenchymal Tumor

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

Intracranial mesenchymal tumor, with the FET::CREB gene fusion, has previously been called angiomatoid fibrous histiocytoma (AFP) or intracranial myxoid mesenchymal tumor. The molecular findings suggest that these tumors are histological variants of intracranial mesenchymal tumor.[156] In one study, the tumors of 20 patients were separated into two epigenetic subgroups. Group A tumors clustered nearest to but independent of solitary fibrous tumor and occurred in adolescents and young adults. Group B tumors clustered nearest to but independent of clear cell sarcoma and occurred in children. Patients with group B tumors had an inferior survival compared with patients with group A tumors (4.5 vs. 49 months; P = .001).[157]

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