The effects of synthetic cyclic somatostatin 14 were studied in two patients with the carcinoid syndrome. The 3-hour intravenous administration of somatostatin (250 micrograms X h-1), a) resulted in the disappearance of flushing in the first patient but was without any clinical effect in the second subject who remained chronically colored; b) lowered plasma levels of motilin, prostaglandins (E1, E2 and F2 alpha) and to a lesser extent of catecholamines in both patients whereas the serotonin level was not altered; c) was followed by a rebound effect with recurrence of severe flushing in the first patient and was associated with a dramatic increase of prostaglandin, substance P and catecholamine levels in both patients. The inhibitory effect of somatostatin and the occurrence of a rebound effect at the end of infusion were confirmed by infusing somatostatin (6 mg per day) during 48 h in the first patients. These results: a) show that somatostatin is an effective drug in carcinoid syndrome with severe flushing; b) confirm that several mediators are affected in carcinoid syndrome. However it could not be excluded that increased circulating levels of prostaglandins, substance P and catecholamines may represent unrelated secondary events; c) suggest that somatostatin primarily inhibits the release rather than the synthesis of tumor products. Owing to the severity of the rebound effect, treatment of the carcinoid syndrome with somatostatin must be undertaken with precaution until specific long-acting analogs are available.
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J Neuroendocrinol
January 2025
Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida, USA.
Colonic neuroendocrine tumors (NETs), excluding rectal NETs, are often described as relatively common and aggressive, with inferior median survival compared with other gastrointestinal (GI) primary sites. However, epidemiological databases may conflate well-differentiated NETs with poorly differentiated neuroendocrine carcinomas (NECs), leading to a lack of precise data on the prevalence, clinical behavior, and prognosis of well-differentiated colonic NETs. We analyzed a large institutional database to identify patients with well-differentiated NETs originating in the colon, excluding rectal NETs.
View Article and Find Full Text PDFEndokrynol Pol
December 2024
Department of Endocrinology and Neuroendocrine Tumours, Medical University of Silesia, Katowice, Poland.
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View Article and Find Full Text PDFAACE Clin Case Rep
August 2024
Division of Endocrinology, Department of Medicine, Duke University, Durham, North Carolina.
Background/objective: Pulmonary carcinoid tumors are a rare cause of Cushing's syndrome and usually present with an indolent course. Here, we present a case of rapid onset and severe Cushing's syndrome due to a typical pulmonary carcinoid tumor.
Case Report: A 32-year-old woman developed diabetes, hypertension, and weight gain of 50 pounds over 3 months.
G Ital Cardiol (Rome)
January 2025
U.O. Cardiologia, Dipartimento di Specialità Medico-Chirurgiche, Scienze Radiologiche e Sanità Pubblica, Università degli Studi, Brescia.
Tricuspid regurgitation can be due to different causes and mechanisms. Among these, cardiac involvement in carcinoid disease is a rare cause of tricuspid valve disease with a peculiar echocardiographic aspect. We report the case of a 59-year-old woman, with no past medical history, who was recently found to have a heart murmur and signs of right heart failure.
View Article and Find Full Text PDFCancers (Basel)
November 2024
Endocrinology, Abdominal Center, Helsinki University Hospital and University of Helsinki, ENDO-ERN (European Reference Network on Rare Endocrine Conditions), 00280 Helsinki, Finland.
Small intestinal neuroendocrine tumors (SI-NETs) are characterized by carcinoid syndrome and carcinoid heart disease (CHD). The aim of the present study was to identify early risk markers for carcinoid heart disease and survival in a prospective median-term follow-up setting. We measured 5-HIAA and cumulative 5-HIAA exposure (Cum-5-HIAA) based on repeated measurements, proBNP, vascular function, hepatic tumor load, and transthoracic echocardiography (TTE) at baseline and during the median 5-year follow-up.
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