THE malignant carcinoid syndrome has challenged the skills of the clinician, biochemist, and pharmacologist since its original description by Biörck et al1 22 years ago.
Although attention originally centered on a symptom complex and a serotonin-producing midintestinal neoplasm that had metastasized to the liver, the carcinoid syndrome has now been expanded to include a spectrum of biochemical, anatomic, and histologic variants. These embrace such diverse sites as stomach, rectum, bronchus, ovary, pancreas, biliary tract, and Meckel diverticulum, and are expressed biochemically by the tryptophan-serotonin pathway, histamine, the kinin-kallikrien system, and probably other vasoactive amines.2 The malignant carcinoid syndrome has been associated with tumor production of immunoreactive insulin, secondary hypoglycemia, Cushing syndrome via corticotropin, and hyperpigmentation via melanotropin secretion.3
In addition, the malignant carcinoid syndrome may present as a paraneoplastic syndrome of such noncarcinoid malignant neoplasms as oat cell cancer of the lung, medullary cancer of the thyroid,
Ureles AL. Diagnosis and Treatment of Malignant Carcinoid Syndrome. JAMA. 1974;229(10):1346–1348. doi:10.1001/jama.1974.03230480062036
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