Anterior abdominal wall malignant peripheral nerve sheath tumor in an infant

Malignant peripheral nerve sheath tumors (MPNST), also known as malignant s chwannomas, are highly malignant lesions that tend to arise in the head and neck region or on the extremities, and only rarely in the retroperitoneum. These tumors are not common in children. Almost 50% of cases are associate d with neurofibromatosis. When these neoplasms arise in the retroperitoneum , they usually are difficult to diagnose and to treat. Although various rad iologic imaging methods are helpful for identifying some features of the ma ss, definitive diagnosis requires histologic examination and immunohistoche mical staining. After treatment, the tumor recurs in 25% of patients. Five- year survival rates as high as 80% have been reported. Total excision, lack of invasion of surrounding tissues and vessels, and absence of neurofibrom atosis, are features associated with better outcome. The authors present th e case of a 1-month-old boy who had malignant schwannoma diagnosed in a uni que location over the peritoneum of the anterior abdomen. The location and rapid growth of the tumor permitted early identification and total excision . Copyright (C) 2001 by WB. Saunders Company.