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← Childhood Soft Tissue Sarcoma

HEALTH PROFESSIONAL · SOURCE READING

Clinical presentation

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 Fibroblastic and Myofibroblastic Tumors / Inflammatory Myofibroblastic Tumor and Epithelioid Inflammatory Myofibroblastic Sarcoma

Inflammatory myofibroblastic tumors are rare tumors that affect soft tissues and visceral organs of children and young adults.[55] They rarely metastasize but tend to be locally invasive. Usual anatomical sites of disease include soft tissue, lungs, spleen, colon, and breast.[52] A review of 42 cases of pediatric inflammatory myofibroblastic tumor of the bladder was published in 2015.[56]

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Epithelioid inflammatory myofibroblastic sarcoma is an uncommon subtype of inflammatory myofibroblastic tumors that shows a male predominance and can present from infancy through adulthood.[57-59] This subtype shows epithelioid morphology and a perinuclear or nuclear membrane pattern of immunostaining for ALK.[57] The most common site of presentation is the abdomen, although other primary sites have been reported.[57-59]

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