Early abdominal manifestation of neurofibromatosis type 1 (NF 1) in a 12-year-old boy

Neurofibromatosis type 1 (NF 1) is a neuroectodermal tumor in which abdominal manifestations occur in about 25 % of cases in addition to the typical cutaneous lesions. A child with a severe farm of an abdominal manifestation of NF 1 was treated in the Hanover Medical School by tumorresection. Pre-operative diagnosis using CT, angiography and ultrasound showed an enormous abdominal tumor constricting the mesenteric vessels. The tumor and the affected portion of the small intestine (150 ems) were removed surgically. No tumor recurrence was observed nine months after operation. Surgical removal is the only currently available therapy for treating tumors of this size.