2020 Volume 56 Issue 7 Pages 1144-1149
A boy of 4 years and 3 months of age was found to have a tumor in the upper abdominal wall. Further studies revealed that the tumor (4×2 cm) was in the muscle layer and had an unclear boundary. Open biopsy of the tumor led to the diagnosis of desmoid fibromatosis. The tumor was resected with a resection margin of over 1 cm, while preserving the rib bones. Histopathological examination of the resected specimen revealed desmoid fibromatosis with a positive margin. Four months after the first surgery, a local recurrent tumor was found and was resected widely again. Histopathological examination of the resected specimen revealed desmoid fibromatosis with a positive margin again. Therefore, he received adjuvant chemotherapy, low-dose vinblastine and methotrexate. Five months later, local recurrence was found again. Tumor excision, chemotherapy (vincristine, actinomycin D, cyclophosphamide: VAC), and radiotherapy (60 Gy) were performed. The multidisciplinary treatment was completed without major complications, and the residual tumor gradually decreased in size. Seven years later, the tumor had grown to a size of 2 cm. The tumor was resected and radiotherapy (60 Gy) was administered again. However, recurrence was observed outside the irradiation field and additional radiotherapy (50 Gy) was performed.
