title: Malignant carcinoid tumors. An analysis of 103 patients with regard to tumor localization, hormone production, and survival.
abstract: 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.
mesh_terms: D000328:Adult; D000368:Aged; D001984:Bronchial Neoplasms / Q000473:pathology; D003967:Diarrhea / Q000150:complications; D005260:Female; D005483:Flushing / Q000150:complications; D005770:Gastrointestinal Neoplasms / Q000473:pathology; D006323:Heart Arrest / Q000150:complications; D006801:Humans; D006897:Hydroxyindoleacetic Acid / Q000652:urine; D008297:Male; D008303:Malignant Carcinoid Syndrome / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology / Q000652:urine; D008875:Middle Aged; D009479:Neuropeptides / Q000378:metabolism; D011446:Prospective Studies; D015320:Tachykinins; D014057:Tomography, X-Ray Computed
chemical_list: D009479:Neuropeptides; D015320:Tachykinins; C045206:neuropeptide K; D006897:Hydroxyindoleacetic Acid