Gastroenteropancreatic endocrine tumors

Weil, Claude

doi:10.1007/bf01731493

Cited by 40 publications

(3 citation statements)

References 148 publications

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“…The condition may not be as rare as the above data suggest. Postmortem studies have found the incidence of malignant carcinoid tumors to be as high as 2.1 cases per 100,000 population per year [4]; and in another series Weil [5] found carcinoid tumors to be present in 1 of every 400 postmortem examinations. It is not known if any of the patients reported in these two studies had symptoms caused by their carcinoid tumor.…”

mentioning

confidence: 95%

Clinical Manifestations of Carcinoid Disease

et al. 1996

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Carcinoid tumors are relatively uncommon tumors and their presentation is varied. For these reasons, a high index of suspicion is necessary in order to consider the diagnosis. It is important to separate the "syndrome" from the primary tumor. It is obviously more effective to diagnose the tumor itself before the syndrome manifests itself, usually as a result of metastatic disease. Since the tumors are characteristically slow-growing, the physician may be misled into thinking the patient has functional problems rather than a tumor. Some data and guidelines are given for focusing on the signs and symptoms of carcinoid disease.

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mentioning

confidence: 95%

Clinical Manifestations of Carcinoid Disease

et al. 1996

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“…The typical features of VHL disease include retinal and brain hemangioblastoma, renal cell carcinoma, renal cysts, pheochromocytoma, and pancreatic tumors and cysts (Lonser et al 2003). Pancreatic Data compiled from original articles and reviews (Grimelius et al 1975, Creutzfeldt 1980, Weil 1985, Harris et al 1987, Klö ppel & Heitz 1988, Eriksson et al 1989, Watson et al 1989, Demeure et al 1991, Kimura et al 1991, Service et al 1991, Soga & Yakuwa 1994, 1998b, 1999, Wermers et al 1996, Lam & Lo 1997, Solcia et al 1997, Smith et al 1998, Jensen 1999 www.endocrinology-journals.org endocrine tumors are found in 9.5-17% of patients with VHL disease (Binkovitz et al 1990, Libutti et al 1998, Hammel et al 2000, Blansfield et al 2007. The PETs associated with VHL disease are virtually always nonfunctional (Blansfield et al 2007).…”

Section: Pets Associated With Hereditary Syndromesmentioning

confidence: 99%

Pancreatic endocrine neoplasms: epidemiology and prognosis of pancreatic endocrine tumors

Halfdanarson¹,

Rubin²,

Farnell³

et al. 2008

Endocrine Related Cancer

343

233

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Pancreatic endocrine tumors (PETs) are uncommon tumors with an annual incidence !1 per 100 000 person-years in the general population. The PETs that produce hormones resulting in symptoms are designated as functional. The majority of PETs are non-functional. Of the functional tumors, insulinomas are the most common, followed by gastrinomas. The clinical course of patients with PETs is variable and depends on the extent of the disease and the treatment rendered. Patients with completely resected tumors generally have a good prognosis, and aggressive surgical therapy in patients with advanced disease may also prolong survival. The epidemiology, prognosis, and established and novel prognostic markers of PETs are reviewed.

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“…Although carcinoids are classically tumors of enterochromaffin and argentaffin cells of the digestive tract, the term "carcinoid tumor" can be expanded to cover "gut" tumors of paracrine and endocrine-like cells of unknown function (3,4). It is now established that these tumors are of neuroendocrine origin and derive from a primitive stem cell that may differentiate into any one of a variety of adult endocrine secreting cells: B cell and insulinoma; A cell and glucagonoma; D cell and somatostatinoma; and the PP cell and PPoma, or cells capable of producing ACTH, GHRH, VIP, substance P, GRF, calcitonin, and the EC cell with its ability to cosecrete amines such as serotonin and the peptide motilin.…”

Section: Smentioning

confidence: 99%

Use of somatostatin analog in management of carcinoid syndrome

Vinik

Moattari

1989

Digest Dis Sci

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Carcinoid tumors are the most frequent gut neuroendocrine tumors accounting for more than 50% of all tumors of the gastroenteropancreatic (GEP) axis. These tumors appear to derive from a stem cell line capable of differentiating into a variety of malignant cells that secrete many different peptides and amines. The symptoms of carcinoid tumors are often non-specific, vague abdominal pain that may precede the diagnosis by a median of 9 years. Carcinoid syndrome occurs in less than 10% of patients. We evaluated the effects of SMS 201-995 in 14 such patients, 12 with diarrhea, 8 with flushing, 3 with wheezing, one with tricuspid valve incompetence, 6 with facial telangiectasia, 3 with a pellagra type dermatosis and one with myopathy. Diarrhea was abolished or significantly reduced in 83%, flushing in 100%, wheezing in 100%, and myopathy improved in the one patient. Blood serotonin was resistant to change, urine 5HIAA fell in 75%, and most gut neuropeptide hormones apart from somatostatin were suppressed. Tumor growth appeared to be slowed in 2/3 of cases treated for up to 4 years. The analog of somatostatin appears to be a useful addition to the therapeutic armamentarium for carcinoid tumors and the symptom complex.

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Gastroenteropancreatic endocrine tumors

Cited by 40 publications

References 148 publications

Clinical Manifestations of Carcinoid Disease

Clinical Manifestations of Carcinoid Disease

Pancreatic endocrine neoplasms: epidemiology and prognosis of pancreatic endocrine tumors

Use of somatostatin analog in management of carcinoid syndrome

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