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A 32-year-old female patient visited the emergency room because of sudden onset of abdominal pain, which was found to be induced by hemorrhagic infarction of a plexiform neurofibroma arising from the lesser omentum. No other classic clinical characteristics of systemic von Recklinghausen's disease had been seen up until now. The tumor, classified as sporadic plexiform neurofibroma, rarely occurs in the digestive tract. This diagnosis was confirmed by exploratory laparotomy with complete resection of the tumor. Here, we describe the rare case of a sporadic intra-abdominal plexiform neurofibroma and discuss the clinical characteristics and management of plexiform neurofibroma.

We experienced the case of an 11-year-old boy diagnosed as having type 1 neurofibromatosis with intraabdominal and pelvic masses. On physical examination, there were multiple café-au-lait spots larger than 15 mm in diameter scattered over the patient's entire body and axillary freckling, but no cutaneous neurofibromas were present. Lisch nodules were detected in the iris by a slit lamp. A large, firm mobile mass was palpated in the lower abdomen. Abdominal computed tomographic scan showed the hypodense masses in the lower abdomen and pelvic cavity. At laparotomy, a 16 x 9-cm, firm nodular mass along the mesentery of the terminal ileum and ascending colon was found. The mesenteric mass was encasing the superior mesenteric vessels and extending into the serosa of the intestine. The mass was incompletely excised together with affected intestine. However, resection of the rectum could not be performed because of the extensive involvement of the entire mesorectum. Histopathologic study revealed a plexiform neurofibroma involving the mesentery and intestine without evidence of malignant transformation. The postoperative course was uneventful. Ten months after the operation, a magnetic resonance imaging showed no interval change of the mesorectal mass.

Gastrointestinal involvement in von Recklinghausen's disease is usually in the form of neurofibromas and leiomyomas. Very rarely, plexiform neurofibromas may be seen involving the bowel wall and myenteric plexus with secondary minor involvement of the mesentery. This report describes the computed tomography morphology of a plexiform neurofibroma extensively involving the mesentery with minor involvement of the bowel wall.

We present a case of plexiform neurofibromatosis involving the ileum and its mesentery. Computed tomography (CT) scans showed a cluster of small soft-tissue density nodules, which represented a cross-sectional image of the enlarged peripheral nerves and wall thickening of the distal ileum. Trapped fat tissue was demonstrated between these enlarged nerves. Histopathologic studies of the resected specimen correlated well with CT findings.