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Genomic alterations
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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Roughly one-half of inflammatory myofibroblastic tumors exhibit a clonal variant that activates the ALK gene (encodes a receptor tyrosine kinase) at chromosome 2p23.[60]
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Most cases of epithelioid inflammatory myofibroblastic sarcoma have RANBP2::ALK gene fusions. RRBP1::ALK gene fusions have also been reported.[57-59] Because RANBP2 localizes to the nuclear pore, this likely explains the perinuclear or nuclear membrane pattern of staining noted for ALK in epithelioid inflammatory myofibroblastic sarcoma.
ROS1 and PDGFRB kinase fusions were identified in 8 of 11 patients (73%) who were negative for ALK by immunohistochemistry.[61][Level of evidence C3]
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Preserved source evidence · Not medical advice