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Published in: BMC Endocrine Disorders 1/2016

Open Access 01-12-2016 | Research article

Natural history and outcome in chinese patients with gastroenteropancreatic neuroendocrine tumours: - a 17-year retrospective analysis

Authors: Doris T. Chan, Andrea O. Y. Luk, W. Y. So, Alice P. S. Kong, Francis C. C. Chow, Ronald C. W. Ma, Anthony W. I. Lo

Published in: BMC Endocrine Disorders | Issue 1/2016

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Abstract

Background

There is rising incidence of gastroenteropancreatic neuroendocrine tumours (GEP- NETs) in many parts of the world, but epidemiological data from Asian populations is rare.

Methods

We conducted a retrospective study in a tertiary medical centre in Hong Kong, using updated diagnostic criteria. The presentation, clinical features, and disease outcome were reviewed for all patients with GEP-NETs confirmed histopathologically at the Prince of Wales Hospital, the Chinese University of Hong Kong, between 1996 and 2013, according to the latest 2010 World Health Organization Classification.

Results

Among 126 patients, GEP- NETs were found in pancreas (34.9 %), rectum (33.3 %), and stomach (8.7 %), and most of them were non- functional GEP- NETs (91.3 %), mostly of grade 1 (G1) (87.3 %), and about 20 % had metastases on presentation. Age under 55 years, G1 tumours and absence of metastases were significant favourable predictors for survival in univariate analysis; whereas G2/3 tumours, size ≥2 cm, and metastases were significant predictors for disease progression (p < 0.05). In multivariate analysis, age and metastases on presentation were significant predictors of mortality (respective hazard ratios [HR] 1.05 [95 % confidence interval {CI} 1.02-1.08] and 6.52 [95 % CI 3.22-13.2]) and disease progression (respective HRs 1.05 [95 % CI 1.02-1.07] and 4.12 [95 % CI 1.96-8.68]), while higher tumour grade also independently predicted disease progression (HR 5.17 [95 % CI 2.05-13.05]) (all p < 0.05).

Conclusion

Non-functional tumours with non-specific symptoms account for the vast majority of GEP-NETs in this Chinese series. Multidisciplinary approach in the management of patients with GEP-NETs may help improve the treatment efficacy and outcome.
Literature
1.
go back to reference Langley K. The neuroendocrine concept today. Ann N Y Acad Sci. 1994;15(733):1–17.CrossRef Langley K. The neuroendocrine concept today. Ann N Y Acad Sci. 1994;15(733):1–17.CrossRef
2.
go back to reference Modlin IM, Oberg K, Chung DC, Jensen RT, de Herder WW, Thakker RV, et al. Gastroenteropancreatic neuroendocrine tumours. Lancet Oncol. 2008;9:61–72.CrossRefPubMed Modlin IM, Oberg K, Chung DC, Jensen RT, de Herder WW, Thakker RV, et al. Gastroenteropancreatic neuroendocrine tumours. Lancet Oncol. 2008;9:61–72.CrossRefPubMed
3.
go back to reference Rindi G, Wiedenmann B. Neuroendocrine neoplasms of the gut and pancreas: new insights. Nat Rev Endocrinol. 2012;8:54–64.CrossRef Rindi G, Wiedenmann B. Neuroendocrine neoplasms of the gut and pancreas: new insights. Nat Rev Endocrinol. 2012;8:54–64.CrossRef
4.
go back to reference Ong SL, Garcea G, Pollard CA, Furness PN, Steward WP, Rajesh A, et al. A fuller understanding of pancreatic neuroendocrine tumours combined with aggressive management improves outcome. Pancreatology. 2009;9:583–600.CrossRefPubMed Ong SL, Garcea G, Pollard CA, Furness PN, Steward WP, Rajesh A, et al. A fuller understanding of pancreatic neuroendocrine tumours combined with aggressive management improves outcome. Pancreatology. 2009;9:583–600.CrossRefPubMed
5.
go back to reference Yao JC, Hassan M, Phan A, Dagohoy C, Leary C, Mares JE, et al. One hundred years after “carcinoid”: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol. 2008;26:3063–72.CrossRefPubMed Yao JC, Hassan M, Phan A, Dagohoy C, Leary C, Mares JE, et al. One hundred years after “carcinoid”: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol. 2008;26:3063–72.CrossRefPubMed
6.
go back to reference Fraenkel M, Kim MK, Faggiano A, Valk GD. Epidemiology of gastroenteropancreatic neuroendocrine tumours. Best Pract Res Clin Gastroenterol. 2012;26:691–703.CrossRefPubMed Fraenkel M, Kim MK, Faggiano A, Valk GD. Epidemiology of gastroenteropancreatic neuroendocrine tumours. Best Pract Res Clin Gastroenterol. 2012;26:691–703.CrossRefPubMed
7.
go back to reference Lawrence B, Gustafsson BI, Chan A, Svejda B, Kidd M, Modlin IM. The epidemiology of gastroenteropancreatic neuroendocrine tumors. Endocrinol Metab Clin North Am. 2011;40:1–18. vii.CrossRefPubMed Lawrence B, Gustafsson BI, Chan A, Svejda B, Kidd M, Modlin IM. The epidemiology of gastroenteropancreatic neuroendocrine tumors. Endocrinol Metab Clin North Am. 2011;40:1–18. vii.CrossRefPubMed
8.
go back to reference Amarapurkar DN, Juneja MP, Patel ND, Amarapurkar AD, Amarapurkar PD. A retrospective clinico-pathological analysis of neuroendocrine tumors of the gastrointestinal tract. Trop Gastroenterol. 2010;31:101–4.PubMed Amarapurkar DN, Juneja MP, Patel ND, Amarapurkar AD, Amarapurkar PD. A retrospective clinico-pathological analysis of neuroendocrine tumors of the gastrointestinal tract. Trop Gastroenterol. 2010;31:101–4.PubMed
9.
go back to reference Gunavathy M, Ghani RA, Sukor N, Kamaruddin NA. A ten-year retrospective analysis of gastroenteropancreatic-neuroendocrine tumors (GEP-NETs) in Malaysia. Med J Malaysia. 2014;69:133–7. Gunavathy M, Ghani RA, Sukor N, Kamaruddin NA. A ten-year retrospective analysis of gastroenteropancreatic-neuroendocrine tumors (GEP-NETs) in Malaysia. Med J Malaysia. 2014;69:133–7.
10.
go back to reference Li AF, Hsu CY, Li A, Tai LC, Liang WY, Li WY, et al. A 35-year retrospective study of carcinoid tumors in Taiwan: differences in distribution with a high probability of associated second primary malignancies. Cancer. 2008;112:274–83.CrossRefPubMed Li AF, Hsu CY, Li A, Tai LC, Liang WY, Li WY, et al. A 35-year retrospective study of carcinoid tumors in Taiwan: differences in distribution with a high probability of associated second primary malignancies. Cancer. 2008;112:274–83.CrossRefPubMed
11.
go back to reference Lim T, Lee J, Kim JJ, Lee JK, Lee KT, Kim YH, et al. Gastroenteropancreatic neuroendocrine tumors: incidence and treatment outcome in a single institution in Korea. Asia Pac J Clin Oncol. 2011;7:293–9.CrossRefPubMed Lim T, Lee J, Kim JJ, Lee JK, Lee KT, Kim YH, et al. Gastroenteropancreatic neuroendocrine tumors: incidence and treatment outcome in a single institution in Korea. Asia Pac J Clin Oncol. 2011;7:293–9.CrossRefPubMed
12.
go back to reference Wang YH, Lin Y, Xue L, Wang JH, Chen MH, Chen J. Relationship between clinical characteristics and survival of gastroenteropancreatic neuroendocrine neoplasms: A single-institution analysis (1995-2012) in South China. BMC Endocr Disord. 2012;12:30.PubMedCentralCrossRefPubMed Wang YH, Lin Y, Xue L, Wang JH, Chen MH, Chen J. Relationship between clinical characteristics and survival of gastroenteropancreatic neuroendocrine neoplasms: A single-institution analysis (1995-2012) in South China. BMC Endocr Disord. 2012;12:30.PubMedCentralCrossRefPubMed
13.
go back to reference Capelli P, Fassan M, Scarpa A. Pathology - grading and staging of GEP-NETs. Best Pract Res Clin Gastroenterol. 2012;26:705–17.CrossRefPubMed Capelli P, Fassan M, Scarpa A. Pathology - grading and staging of GEP-NETs. Best Pract Res Clin Gastroenterol. 2012;26:705–17.CrossRefPubMed
14.
go back to reference Ellis L, Shale MJ, Coleman MP. Carcinoid tumors of the gastrointestinal tract: trends in incidence in England since 1971. Am J Gastroenterol. 2010;105:2563–9.CrossRefPubMed Ellis L, Shale MJ, Coleman MP. Carcinoid tumors of the gastrointestinal tract: trends in incidence in England since 1971. Am J Gastroenterol. 2010;105:2563–9.CrossRefPubMed
15.
go back to reference Hemminki K, Li X. Incidence trends and risk factors of carcinoid tumors: a nationwide epidemiologic study from Sweden. Cancer. 2001;92:2204–10.CrossRefPubMed Hemminki K, Li X. Incidence trends and risk factors of carcinoid tumors: a nationwide epidemiologic study from Sweden. Cancer. 2001;92:2204–10.CrossRefPubMed
16.
go back to reference Newton JN, Swerdlow AJ, dos Santos Silva IM, Vessey MP, Grahame-Smith DG, Primatesta P, et al. The epidemiology of carcinoid tumours in England and Scotland. Br J Cancer. 1994;70:939–42.PubMedCentralCrossRefPubMed Newton JN, Swerdlow AJ, dos Santos Silva IM, Vessey MP, Grahame-Smith DG, Primatesta P, et al. The epidemiology of carcinoid tumours in England and Scotland. Br J Cancer. 1994;70:939–42.PubMedCentralCrossRefPubMed
17.
go back to reference Niederle MB, Hackl M, Kaserer K, Niederle B. Gastroenteropancreatic neuroendocrine tumours: the current incidence and staging based on the WHO and European Neuroendocrine Tumour Society classification: an analysis based on prospectively collected parameters. Endocr Relat Cancer. 2010;17:909–18.CrossRefPubMed Niederle MB, Hackl M, Kaserer K, Niederle B. Gastroenteropancreatic neuroendocrine tumours: the current incidence and staging based on the WHO and European Neuroendocrine Tumour Society classification: an analysis based on prospectively collected parameters. Endocr Relat Cancer. 2010;17:909–18.CrossRefPubMed
18.
go back to reference Quaedvlieg PF, Visser O, Lamers CB, Janssen-Heijen ML, Taal BG. Epidemiology and survival in patients with carcinoid disease in The Netherlands. An epidemiological study with 2391 patients. Ann Oncol. 2001;12:1295–300.CrossRefPubMed Quaedvlieg PF, Visser O, Lamers CB, Janssen-Heijen ML, Taal BG. Epidemiology and survival in patients with carcinoid disease in The Netherlands. An epidemiological study with 2391 patients. Ann Oncol. 2001;12:1295–300.CrossRefPubMed
19.
go back to reference Westergaard T, Frisch M, Melbye M. Carcinoid tumors in Denmark 1978-1989 and the risk of subsequent cancers. A population-based study. Cancer. 1995;76:106–9.CrossRefPubMed Westergaard T, Frisch M, Melbye M. Carcinoid tumors in Denmark 1978-1989 and the risk of subsequent cancers. A population-based study. Cancer. 1995;76:106–9.CrossRefPubMed
20.
21.
go back to reference Kloppel G. Tumour biology and histopathology of neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab. 2007;21:15–31.CrossRefPubMed Kloppel G. Tumour biology and histopathology of neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab. 2007;21:15–31.CrossRefPubMed
22.
go back to reference Ito T, Sasano H, Tanaka M, Osamura RY, Sasaki I, Kimura W, et al. Epidemiological study of gastroenteropancreatic neuroendocrine tumours in Japan. J Gastroenterol. 2010;45:234–43.CrossRefPubMed Ito T, Sasano H, Tanaka M, Osamura RY, Sasaki I, Kimura W, et al. Epidemiological study of gastroenteropancreatic neuroendocrine tumours in Japan. J Gastroenterol. 2010;45:234–43.CrossRefPubMed
23.
go back to reference Rothenstein J, Cleary SP, Pond GR, Dale D, Gallinger S, Moore MJ, et al. Neuroendocrine tumors of the gastrointestinal tract: a decade of experience at the Princess Margaret Hospital. Am J Clin Oncol. 2008;31:64–70.CrossRefPubMed Rothenstein J, Cleary SP, Pond GR, Dale D, Gallinger S, Moore MJ, et al. Neuroendocrine tumors of the gastrointestinal tract: a decade of experience at the Princess Margaret Hospital. Am J Clin Oncol. 2008;31:64–70.CrossRefPubMed
24.
go back to reference Klöppel G, Perren A, Heitz PU. The gastroenteropancreatic neuroendocrine cell system and its tumours - The WHO classification. Ann NY Acad Sci. 2004;1014:13–27.CrossRefPubMed Klöppel G, Perren A, Heitz PU. The gastroenteropancreatic neuroendocrine cell system and its tumours - The WHO classification. Ann NY Acad Sci. 2004;1014:13–27.CrossRefPubMed
25.
go back to reference Pape UF, Berndt U, Muller-Nordhorn J, Bohmig M, Roll S, Koch M, et al. Prognostic factors of long-term outcome in gastroenteropancreatic neuroendocrine tumours. Endocr Relat Cancer. 2008;15:1083–97.CrossRefPubMed Pape UF, Berndt U, Muller-Nordhorn J, Bohmig M, Roll S, Koch M, et al. Prognostic factors of long-term outcome in gastroenteropancreatic neuroendocrine tumours. Endocr Relat Cancer. 2008;15:1083–97.CrossRefPubMed
27.
go back to reference Sundin A. Radiological and nuclear medicine imaging of gastroenteropancreatic neuroendocrine tumours. Best Pract Res Clin Gastroenterol. 2012;26:803–18.CrossRefPubMed Sundin A. Radiological and nuclear medicine imaging of gastroenteropancreatic neuroendocrine tumours. Best Pract Res Clin Gastroenterol. 2012;26:803–18.CrossRefPubMed
28.
go back to reference Rufini V, Calcagni ML, Baum RP. Imaging of neuroendocrine tumors. Semin Nucl Med. 2006;36:228–47.CrossRefPubMed Rufini V, Calcagni ML, Baum RP. Imaging of neuroendocrine tumors. Semin Nucl Med. 2006;36:228–47.CrossRefPubMed
29.
go back to reference Balon HR, Goldsmith SJ, Siegel BA, Silberstein EB, Krenning EP, Lang O, et al. Procedure guideline for somatostatin receptor scintigraphy with (111)In-pentetreotide. J Nucl Med. 2001;42:1134–8.PubMed Balon HR, Goldsmith SJ, Siegel BA, Silberstein EB, Krenning EP, Lang O, et al. Procedure guideline for somatostatin receptor scintigraphy with (111)In-pentetreotide. J Nucl Med. 2001;42:1134–8.PubMed
30.
go back to reference Modlin IM, Gustafsson BI, Moss SF, Pavel M, Tsolakis AV, Kidd M. Chromogranin A--biological function and clinical utility in neuro endocrine tumor disease. Ann Surg Oncol. 2010;17:2427–43.CrossRefPubMed Modlin IM, Gustafsson BI, Moss SF, Pavel M, Tsolakis AV, Kidd M. Chromogranin A--biological function and clinical utility in neuro endocrine tumor disease. Ann Surg Oncol. 2010;17:2427–43.CrossRefPubMed
31.
go back to reference Wiedenmann B, Franke WW, Kuhn C, Moll R, Gould VE. Synaptophysin: a marker protein for neuroendocrine cells and neoplasms. Proc Natl Acad Sci U S A. 1986;83:3500–4.PubMedCentralCrossRefPubMed Wiedenmann B, Franke WW, Kuhn C, Moll R, Gould VE. Synaptophysin: a marker protein for neuroendocrine cells and neoplasms. Proc Natl Acad Sci U S A. 1986;83:3500–4.PubMedCentralCrossRefPubMed
32.
go back to reference Pavel M, Baudin E, Couvelard A, Krenning E, Oberg K, Steinmuller T, et al. ENETS Consensus Guidelines for the management of patients with liver and other distant metastases from neuroendocrine neoplasms of foregut, ion, hindgut, and unknown primary. Neuroendocrinology. 2012;95:157–76.CrossRefPubMed Pavel M, Baudin E, Couvelard A, Krenning E, Oberg K, Steinmuller T, et al. ENETS Consensus Guidelines for the management of patients with liver and other distant metastases from neuroendocrine neoplasms of foregut, ion, hindgut, and unknown primary. Neuroendocrinology. 2012;95:157–76.CrossRefPubMed
33.
go back to reference Caplin ME, Pavel M, Cwikla JB, Phan AT, Raderer M, Sedlackova E, et al. Lanreotide in metastatic enteropancreatic neuroendocrine tumors. N Engl J Med. 2014;371:224–33.CrossRefPubMed Caplin ME, Pavel M, Cwikla JB, Phan AT, Raderer M, Sedlackova E, et al. Lanreotide in metastatic enteropancreatic neuroendocrine tumors. N Engl J Med. 2014;371:224–33.CrossRefPubMed
34.
go back to reference Metz DC, Choi J, Strosberg J, Heaney AP, Howdend CW, Klimstra D, et al. A rationale for multidisciplinary care in treating neuroendocrine tumours. Curr Opin Endocrinol Diabetes Obes. 2012;19:306–13.CrossRefPubMed Metz DC, Choi J, Strosberg J, Heaney AP, Howdend CW, Klimstra D, et al. A rationale for multidisciplinary care in treating neuroendocrine tumours. Curr Opin Endocrinol Diabetes Obes. 2012;19:306–13.CrossRefPubMed
Metadata
Title
Natural history and outcome in chinese patients with gastroenteropancreatic neuroendocrine tumours: - a 17-year retrospective analysis
Authors
Doris T. Chan
Andrea O. Y. Luk
W. Y. So
Alice P. S. Kong
Francis C. C. Chow
Ronald C. W. Ma
Anthony W. I. Lo
Publication date
01-12-2016
Publisher
BioMed Central
Published in
BMC Endocrine Disorders / Issue 1/2016
Electronic ISSN: 1472-6823
DOI
https://doi.org/10.1186/s12902-016-0087-9

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