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Dermatofibrosarcoma Protuberans NOS
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
Dermatofibrosarcoma is a rare tumor that can be present in all age groups, but many of the reported cases arise in children.[39-41] A review of 451 cases in children younger than 20 years in the SEER Program database found that the incidence was 1 case per 1 million. The incidence was highest among Black patients aged 15 to 19 years. The most common sites were the trunk and extremities, which is similar to what is found in adults.
Ninety-five percent of patients underwent surgery. The overall survival (OS) rate was 100% at 5 years, 98% at 15 years, and 97% at 30 years. Male patients had decreased survival compared with female patients (P < .05).[42][Level of evidence C1]
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Preserved source evidence · Not medical advice