Somatostatin-producing duodenal carcinoids in patients with von Recklinghausen's neurofibromatosis. A predilection for black patients

Cancer. 1990 Apr 1;65(7):1591-5. doi: 10.1002/1097-0142(19900401)65:7<1591::aid-cncr2820650723>;2-n.


Eight patients with von Recklinghausen's disease (VRD) and duodenal carcinoids are presented. Seven patients were black, and one white. Six of the eight were women. The presenting symptom was either jaundice or abdominal pain. All tumors were located in the second portion of the duodenum, and three were multiple. Associated tumors other than neurofibromas included multiple leiomyomas, meningioma, neurofibrosarcoma, and prostatic sarcoma. Seven tumors had psammoma bodies, and in three they were numerous. Somatostatin-positive cells were demonstrated in all cases. Two tumors had spread to regional lymph nodes at the time of surgery. There appears to be a predilection for black patients among those with VRD and duodenal carcinoids.

MeSH terms

  • Adult
  • Black People
  • Carcinoid Tumor / analysis
  • Carcinoid Tumor / ethnology*
  • Carcinoid Tumor / pathology
  • Duodenal Neoplasms / analysis
  • Duodenal Neoplasms / ethnology*
  • Duodenal Neoplasms / pathology
  • Female
  • Humans
  • Male
  • Middle Aged
  • Neoplasms, Multiple Primary / ethnology*
  • Neurofibromatosis 1 / ethnology*
  • Somatostatin / analysis


  • Somatostatin