Malignant carcinoid tumors. An analysis of 103 patients with regard to tumor localization, hormone production, and survival

Ann Surg. 1987 Aug;206(2):115-25. doi: 10.1097/00000658-198708000-00001.


In a prospective study of 103 patients with carcinoid tumors consecutively referred for medical treatment, the most common sites of the primary tumors were the ileum (73%), bronchi (7%), and jejunum (4%). All patients had local metastases, and 96 (93%) also had liver metastases. The most common initial symptoms were diarrhea (32%), ileus (25%), and flush (23%). The overall frequency of diarrhea was 84% and of flush was 75%. Heart insufficiency caused by cardiac valve disease was seen in 33% of the patients. The carcinoid syndrome, including flush, diarrhea, and elevated urinary 5-hydroxyindole acetic acid (5-HIAA) concentrations, was manifested by 69 patients (67%), 64 of whom (93%) had carcinoid tumors of mid-gut origin. Elevated urinary 5-HIAA was found in 91 patients (88%), of which 89 displayed liver metastases. The plasma concentration of the tachykinin neuropeptide K (NPK) was elevated in 67 patients (66%), 63 of whom had tumors of the mid-gut region. Serum pancreatic polypeptide (PP) and human chorionic gonadotrophin alpha levels were elevated in 43% and 28% of the patients, respectively, and the highest levels were found in patients with metastatic bronchial carcinoid tumors. Thirty-nine of the 103 patients are now dead; 18 died of tumor progression, whereas 14 patients died of heart failure secondary to a carcinoid tricuspidal valve insufficiency. The estimated median survival from the time of histologic diagnosis was 14 years, and from the time of carcinoid syndrome was 8 years.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Bronchial Neoplasms / pathology
  • Diarrhea / complications
  • Female
  • Flushing / complications
  • Gastrointestinal Neoplasms / pathology
  • Heart Arrest / complications
  • Humans
  • Hydroxyindoleacetic Acid / urine
  • Male
  • Malignant Carcinoid Syndrome* / complications
  • Malignant Carcinoid Syndrome* / diagnostic imaging
  • Malignant Carcinoid Syndrome* / pathology
  • Malignant Carcinoid Syndrome* / urine
  • Middle Aged
  • Neuropeptides / metabolism
  • Prospective Studies
  • Tachykinins*
  • Tomography, X-Ray Computed


  • Neuropeptides
  • Tachykinins
  • neuropeptide K
  • Hydroxyindoleacetic Acid