title: Intestinal neurofibromatosis and small-bowel adenocarcinoma: a single case study.
abstract: Patients with Von Recklinghausen's disease (neurofibromatosis type 1) are at increased risk of developing various tumours. However, the coexistence of neurofibromatosis with small-bowel adenocarcinoma is exceedingly rare. We present an uncommon case of neurofibromatosis type 1, involving the small bowel in a 73-year-old man, who was admitted to our department with signs of acute abdomen. At laparotomy, multiple mesenteric and intramural nodules were seen in the distal ileum. These nodules obstructed ileal lumen, while the intestine wall was perforated in one point. A wide resection of the affected ileum together with all visible nodules in the adjacent mesentery was performed. Histology revealed neurofibromatosis type 1 with malignant transformation to small-bowel adenocarcinoma. The patient had no additional therapy. In a follow-up of 2 years, the patient is very well and there was no recurrence of the disease. We suggest that adenocarcinoma of small bowel should be considered in the evaluation of acute abdominal pain in neurofibromatosis patients.
authors.affiliation: First Surgical Department and Transplant Unit, Evagelismos Hospital, Athens, Greece. harstrat@otenet.gr
authors.forename: Charalabos
authors.initials: C
authors.lastname: Stratopoulos
authors.forename: A
authors.initials: A
authors.lastname: Papakonstantinou
authors.forename: G
authors.initials: G
authors.lastname: Anagnostopoulos
authors.forename: I
authors.initials: I
authors.lastname: Terzis
authors.forename: G
authors.initials: G
authors.lastname: Tzimas
authors.forename: S
authors.initials: S
authors.lastname: Gourgiotis
authors.forename: C
authors.initials: C
authors.lastname: Vamvouka
authors.forename: E
authors.initials: E
authors.lastname: Hadjiyannakis
mesh_terms: D000006:Abdomen, Acute / Q000209:etiology; D000230:Adenocarcinoma / Q000473:pathology*; D000368:Aged; D005500:Follow-Up Studies; D006801:Humans; D007078:Ileal Neoplasms / Q000473:pathology*; D007415:Intestinal Obstruction / Q000209:etiology / Q000473:pathology; D007421:Intestine, Small / Q000473:pathology*; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000473:pathology*; D014057:Tomography, X-Ray Computed