Carcinoid Syndrome

10/14/98 (K. Kokko)

Question: What is carcinoid syndrome?

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Unique Identifier 93312582
Authors: Kvols LK. Reubi JC.
Institution: Department of Oncology, Mayo Clinic and Foundation, Rochester, Minnesota 55905.
Title: Metastatic carcinoid tumors and the malignant carcinoid syndrome. [Review] [10 refs]
Source: Acta Oncologica. 32(2)197-201, 1993.
Abstract: Patients with metastatic carcinoid tumors and the malignant carcinoid syndrome have benefited immensely from diagnostic and therapeutic advances during the past decade. Magnetic resonance imaging and whole body scintigraphy with radiolabelled analogues of somatostatin have improved our ability to diagnose, detect, stage and follow response to therapy. Surgical, medical, and radiation therapy may all contribute to the management of these patients. This disease is variable in its presenting symptoms and the biologic behavior of the tumor. The spectrum of clinical manifestations varies depending upon the type and quantity of polypeptide hormones or biogenic amines being produced. Although the tumors are usually indolent in their growth, the more dedifferentiated or anaplastic tumors can be quite aggressive. Thanks to new treatments that are very effective in the subgroup of anaplastic neuroendocrine carcinomas it is vital to recognize this subset. As research scientists and clinicians we must be aware of the natural history of the disease in order to optimize each patient's treatment. This highly selective review focuses on studies performed in collaboration with Dr. Charles Moertel along with other colleagues at the Mayo Clinic, have done in the past few years. [References: 10]

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Unique Identifier 86311083
Authors: Kvols LK. Moertel CG. O'Connell MJ. Schutt AJ. Rubin J. Hahn RG.
Institution: Division of Medical Genetics, University of Birmingham, Birmingham Women's Hospital, UK.
Title: Treatment of the malignant carcinoid syndrome. Evaluation of a long-acting somatostatin analogue.
Source: New England Journal of Medicine. 315(11)663-6, 1986 Sep 11.
Abstract: We studied the effects of a long-acting analogue of somatostatin (SMS 201-995, Sandoz) in 25 patients with histologically proved metastatic carcinoid tumors and the carcinoid syndrome. This drug was self-administered by subcutaneous injection at a dose of 150 micrograms three times daily. Flushing and diarrhea associated with the syndrome were promptly relieved in 22 patients. All 25 patients had an elevated 24-hour urinary excretion of 5-hydroxyindoleacetic acid (5-HIAA) (mean, 265 mg per 24 hours; range, 14 to 1079), which served as an objective indicator of disease activity. Eighteen of the 25 patients (72 percent) had a decrease of 50 percent or more in their urinary 5-HIAA levels, as compared with the pretreatment values. The median duration of this biochemical response was more than 12 months (range, 1 to greater than 18). Since no serious toxicity was observed, we conclude that SMS 201-995 may be appropriate for use as early therapy in patients with symptoms due to the carcinoid syndrome who have not responded to simpler measures.

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