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September 1963

Intestinal Hemorrhage in von Recklinghausen's Disease: Neurogenic Tumors as Cause

Author Affiliations

Resident in Surgery, The Johns Hopkins Hospital.; From the Department of Surgery, The Johns Hopkins University School of Medicine and Hospital.

Arch Surg. 1963;87(3):374-378. doi:10.1001/archsurg.1963.01310150010003

Patients with multiple neurofibromatosis are prone to develop neurogenic tumors within the peritoneal and retroperitoneal spaces, but such a tumor arising in the wall of the bowel is extremely unusual. In a collected review of 1,399 patients with a benign small intestinal tumor only 90 had a neurogenic tumor, and in only 14 were there cutaneous manifestations of von Recklinghausen's disease.1 Intestinal bleeding produced by such a tumor has occasionally been observed,2-5 and in some instances hemorrhage has been massive.2-4 Perforation6 as well as intestinal obstruction7 from these tumors has been reported. During a period of seven years there have been two patients with von Recklinghausen's disease who have undergone surgery at The Johns Hopkins Hospital for bleeding neurogenic tumor of the intestinal tract. Because of the rarity of intestinal bleeding from a neurogenic tumor in patients with multiple neurofibromatosis and the difficulty in establishing