Intestinal neurofibromatosis and small-bowel adenocarcinoma: a single case study

European Journal of Cancer Care
Charalabos StratopoulosE Hadjiyannakis

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.

References

Jan 1, 1991·Genes, Chromosomes & Cancer·T W GloverS Johnson
Apr 5, 2002·Journal of Gastroenterology·Young-Eun JooSei-Jong Kim

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Citations

Mar 26, 2011·Histopathology·Monika Verma, Maurice B Loughrey
Apr 29, 2014·Colorectal Disease : the Official Journal of the Association of Coloproctology of Great Britain and Ireland·B Trilling, J-L Faucheron
Jun 10, 2015·Cirugia y cirujanos·René Francisco Candia-de la RosaRaúl Candia-García
Jan 30, 2019·Indian Journal of Cancer·Reetu KunduRavinder Kaur

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