A 15-year-old male patient with palpable abdominal tumor presented to our hospital. CT scan revealed a giant tumor, 15 cm in diameter, with infiltration to the pancreas body. In addition, the tumor invaded to the greater curvature of the stomach and the transverse colon. We performed distal gastrectomy, distal pancreatectomy, splenectomy and transverse colectomy. The final diagnosis of histopathology was desmoid tumor. The tumor recurred locally 9 months after the surgery. Recurrent legion was unresectable because of the invasion to the orifice of SMV and weekly combination chemotherapy of VLB and MTX was started. Although a partial response was achieved for a local recurrent legion after 20 courses, CT scan showed other new recurrent multiple lesions in the abdominal cavity. Due to the severe abdominal discomfort and intestinal obstruction, a reduction surgery was performed 5 times in total. Endocrine therapy and administration of NSAID were not effective. Tumor progression was uncontrollable, and the patient died 5 years and 8 months after the initial surgery.

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