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      Guidelines for the diagnosis and management of carcinoid tumours. Part 1: The gastrointestinal tract. A statement from a Canadian National Carcinoid Expert Group

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          Abstract

          Carcinoid tumours are relatively rare and, in general, slow growing. They can be “non-functioning” tumours, presenting as a tumour mass, or “functioning” tumours secondary to the production of several biopeptides leading to the carcinoid syndrome. Though these tumours represent 0.25% of an oncology practice, a proper understanding of the clinical course of the disease and of the importance of appropriate diagnostic and therapeutic measures is very important. Proper patient management can lead to cure, particularly if the tumour can be fully resected, or to long-term palliation with medical treatment or cytoreductive surgery, or both, with significant prolongation of survival. A good understanding of the use of somatostatin analogues to achieve effective symptomatic control and of the importance of adequate follow-up and cardiac monitoring to prevent or effectively treat cardiac complications can contribute significantly to optimal control of this complex disease, ultimately improving the quality of life of affected patients. This article, developed by a group of Canadian experts, provides a framework that will assist clinicians in taking an optimal approach to managing their patients with carcinoid tumour.

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          Most cited references49

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          Treatment of neuroendocrine carcinomas with combined etoposide and cisplatin. Evidence of major therapeutic activity in the anaplastic variants of these neoplasms.

          Forty-five patients with metastatic neuroendocrine tumors were treated with a regimen of etoposide 130 mg/m2/d for 3 days plus cisplatin 45 mg/m2/d on days 2 and 3. Both drugs were given by continuous intravenous infusion. Among 27 patients with well-differentiated carcinoid tumors or islet cell carcinomas, only two partial objective tumor regressions were observed (7%). Among 18 patients prospectively classified as having anaplastic neuroendocrine carcinomas, however, there were nine partial regressions and three complete regressions, an overall regression rate of 67%. For anaplastic disease, the median duration of regression was 8 months (range to 21 months). Tumor response was unrelated to primary site, endocrine hyperfunction, or prior therapy experience. The median survival of all patients with anaplastic tumors was 19 months; this seemed favorable when considering the small experiences with these rare tumors reported in the literature. Toxicity, which was severe for most patients, consisted primarily of vomiting, leukopenia, thrombocytopenia, anemia, alopecia, and neuropathy. The anaplastic neuroendocrine tumor is strongly responsive to therapy with combined etoposide and cisplatin. Patients with undifferentiated carcinomas, originating in typical neuroendocrine tumor sites (small and large bowel, pancreas, and stomach) or of unknown origin, who have consistent histologic findings by light microscopy should be evaluated for this possibility with appropriate immune staining or electron microscopy.
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            The diagnosis and medical management of advanced neuroendocrine tumors.

            Neuroendocrine tumors (NETs) constitute a heterogeneous group of neoplasms that originate from endocrine glands such as the pituitary, the parathyroids, and the (neuroendocrine) adrenal, as well as endocrine islets within glandular tissue (thyroid or pancreatic) and cells dispersed between exocrine cells, such as endocrine cells of the digestive (gastroenteropancreatic) and respiratory tracts. Conventionally, NETs may present with a wide variety of functional or nonfunctional endocrine syndromes and may be familial and have other associated tumors. Assessment of specific or general tumor markers offers high sensitivity in establishing the diagnosis and can also have prognostic significance. Imaging modalities include endoscopic ultrasonography, computed tomography and magnetic resonance imaging, and particularly, scintigraphy with somatostatin analogs and metaiodobenzylguanidine. Successful treatment of disseminated NETs requires a multimodal approach; radical tumor surgery may be curative but is rarely possible. Well-differentiated and slow-growing gastroenteropancreatic tumors should be treated with somatostatin analogs or alpha-interferon, with chemotherapy being reserved for poorly differentiated and progressive tumors. Therapy with radionuclides may be used for tumors exhibiting uptake to a diagnostic scan, either after surgery to eradicate microscopic residual disease or later if conventional treatment or biotherapy fails. Maintenance of the quality of life should be a priority, particularly because patients with disseminated disease may experience prolonged survival.
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              Treatment of poorly differentiated neuroendocrine tumours with etoposide and cisplatin

              The purpose of this study was to evaluate by a retrospective analysis of 53 patients the efficacy of chemotherapy combining etoposide and cisplatin in the treatment of neuroendocrine tumours. The regimen was a combination of etoposide 100 mg m–2 day–1 for 3 days and cisplatin 100 mg m–2 on day 1, given by 2-h intravenous infusion, administered every 21 days. Twelve patients had a well-differentiated and 41 a poorly differentiated neuroendocrine tumour. Toxicity of treatment was assessed in 50 patients and efficacy in 52 patients. Among the 11 patients with a well-differentiated tumour evaluable for tumoural response, only one (9.4%) had a partial response for 8.5 months. Forty-one patients with a poorly differentiated tumour showed an objective response rate of 41.5% (four complete and 13 partial responses); the median duration of response was 9.2 months, the median overall survival 15 months and the median progression-free survival 8.9 months. Haematological grade 3–4 toxicity was observed in 60% of the cases with one treatment-related death, digestive grade 3–4 toxicity in 40% and grade 3 alopecia was constant. No severe renal, hearing and neurological toxicities were observed (grade 1 in 6%, 14%, 72% respectively and no grade >1). We confirm that poorly differentiated neuroendocrine tumours are chemosensitive to the etoposide plus cisplatin combination. However, the prognosis remains poor with a 2-year survival lower than 20% confirming that new therapeutic strategies have to be developed. © 1999 Cancer Research Campaign
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                Author and article information

                Journal
                Curr Oncol
                CO
                Current Oncology
                Multimed Inc. (66 Martin St. Milton, ON, Canada L9T 2R2 )
                1198-0052
                April 2006
                : 13
                : 2
                : 67-76
                Affiliations
                [* ] Ottawa Hospital Regional Cancer Centre, Ottawa, Ontario
                [] London Regional Cancer Centre, London, Ontario
                [] H. Lee Moffitt Cancer Centre, Tampa, Florida, U.S.A
                [§ ] Toronto–Sunnybrook Regional Cancer, Toronto, Ontario
                [|| ] University Health Network, Princess Margaret Hospital, Toronto, Ontario
                [# ] Sudbury Regional Hospital–Laurentian, Sudbury, Ontario
                [** ] Sunnybrook and Women’s College Health Sciences Centre, Toronto, Ontario
                [†† ] CHUM–Hospital St-Luc, Montreal, Quebec
                [‡‡ ] Queen Elizabeth II Health Sciences Centre, Halifax, Nova Scotia
                [§§ ] London Health Sciences Centre–Victoria Campus, London, Ontario
                [|||| ] MUHC–Royal Victoria Hospital, Montreal, Quebec
                [## ] B.C. Cancer Agency–Vancouver Cancer Centre, Vancouver, British Columbia
                [*** ] Hôpital Maisonneuve–Rosemont, Montreal, Quebec
                [††† ] St. Boniface General Hospital, Winnipeg, Manitoba
                Author notes
                Corresponding author: Jean A. Maroun, Ottawa Hospital Regional Cancer Centre, 501 Smyth Road, Ottawa, Ontario K1H 8L6. E-mail: jmaroun@ 123456ottawahospital.on.ca
                Article
                co13_2p067
                10.3390/curroncol13020006
                1891174
                17576444
                798079b5-966c-4092-ab37-d07d3e38cad3
                2006 Multimed Inc.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Categories
                Practice Guidelines Series

                Oncology & Radiotherapy
                guidelines,carcinoid syndrome,clinical management,carcinoid tumour
                Oncology & Radiotherapy
                guidelines, carcinoid syndrome, clinical management, carcinoid tumour

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