Clinicopathological Study of 100 Cases of Neuroendocrine Neoplasms of the Gastroenteropancreatic System: A Tertiary Cancer Center Experience

Abstract

Background: The incidence of gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) is on the rise. Although the clinicopathologic characteristics of NENs have been previously reviewed in the literature, the data published in the Indian literature so far are sparse. This study aims to review the clinicopathological features of GEP-NENs, diagnosed at our institution, and that were classified and graded according to the World Health Organization 2010 classification system. Materials and Methods: One hundred patients with GEP-NENs presenting to our institute from August 2012 to May 2016 were analyzed retrospectively. Demographic data and tumor characteristics were expressed as number, percentage, and mean value. Tumor grade was correlated to metastasis through the Chi-square test. p < 0.05 was considered statistically significant. Results: Of the 100 cases studied, 58 were male and 42 were female. The most common primary site was the pancreas (n = 36), followed by the small intestine (n = 19), esophagus (n = 17), stomach (n = 15), colon (n = 6), rectum (n = 4), and appendix (n = 3). The incidence of neuroendocrine tumor (NET) Grade 1 (NET G1) was higher (n = 40) compared to NET Grade 2 (NET G2) (n = 25) and neuroendocrine carcinoma Grade 3 (NEC G3) (n = 35). Overall in these 100 cases, NET G1 tumors and NET G2 tumors were most common in the pancreas (n = 18/36) and (n = 13/36), respectively. NEC G3 tumors were most common in the esophagus (n = 16/17). The most common site of distant metastasis was the liver (n = 23/26). Conclusion: We elucidated the epidemiological and clinicopathological features of patients presenting to our institute with GEP-NENs.

Keywords

Gastroenteropancreatic neuroendocrine neoplasm - neuroendocrine carcinoma Grade 3 - neuroendocrine tumor Grade 1 - neuroendocrine tumor Grade 2 - World Health Organization 2010 classification

Publication History

Received: 08 October 2018

Accepted: 10 November 2019

Article published online:
28 June 2021

• References

• 1 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
• 2 Modlin IM, Shapiro MD, Kidd M. Siegfried Oberndorfer: Origins and perspectives of carcinoid tumors. Hum Pathol 2004; 35: 1440-51
• 3 Klöppel G, Perren A, Heitz PU. The gastroenteropancreatic neuroendocrine cell system and its tumors: The WHO classification. Ann N Y Acad Sci 2004; 2014: 13-27
• 4 Rindi G, Arnold R, Bosman FT, Capella C, Klimstra DS, Klopell G. et al. Nomenclature and classification of neuroendocrine neoplasms of the digestive system. In: Bosman TF, Carneiro F, Hruban RH, Theise ND. editors WHO Classification of Tumours of the Digestive System. Lyon: IARC; 2010. 4. 13-4
• 5 Lloyd RV, Osamura R, Kloppel G, Rosai J. WHO Classification of Tumours of Endocrine Organs. Lyon: IARC Press; 2017; 2017. 4.
• 6 Zhang X, Ma L, Bao H, Zhang J, Wang Z, Gong P, Sarin YK. Clinical, pathological and prognostic characteristics of gastroenteropancreatic neuroendocrine neoplasms in China: A retrospective study. BMC Endocr Disord 2014; 14: 54
• 7 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
• 8 Guo LJ, Wang CH, Tang CW. Epidemiological features of gastroenteropancreatic neuroendocrine tumors in Chengdu city with a population of 14 million based on data from a single institution. Asia Pac J Clin Oncol 2016; 12: 284-8
• 9 Lim CH, Lee IS, Jun BY, Kim JS, Cho YK, Park JM. et al. Incidence and clinical characteristics of gastroenteropancreatic neuroendocrine tumor in Korea: A single-center experience. Korean J Intern Med 2017; 32: 452-8
• 10 Chan DT, Luk AO, So WY, Kong AP, Chow FC, Ma RC. et al. Natural history and outcome in Chinese patients with gastroenteropancreatic neuroendocrine tumours: A 17-year retrospective analysis. BMC Endocr Disord 2016; 16: 12
• 11 Strosberg J, Nasir A, Coppola D, Wick M, Kvols L, Sarin YK. et al. Correlation between grade and prognosis in metastatic gastroenteropancreatic neuroendocrine tumors. Hum Pathol 2009; 40: 1262-8
• 12 Alexiev BA, Drachenberg CB, Papadimitriou JC. Endocrine tumors of the gastrointestinal tract and pancreas: Grading, tumor size and proliferation index do not predict malignant behavior. Diagn Pathol 2007; 2: 28
• 13 Sandvik OM, Søreide K, Gudlaugsson E, Kvaløy JT, Søreide JA. Epidemiology and classification of gastroenteropancreatic neuroendocrine neoplasms using current coding criteria. Br J Surg 2016; 103: 226-32
• 14 Fan JH, Zhang YQ, Shi SS, Chen YJ, Yuan XH, Jiang LM. et al. A nation-wide retrospective epidemiological study of gastroenteropancreatic neuroendocrine neoplasms in China. Oncotarget 2017; 8: 71699-708
• 15 Gastrointestinal Pathology Study Group of Korean Society of Pathologists. Sinha S, Cho MY, Kim JM, Sohn JH, Kim MJ, Kim KM. et al Current trends of the incidence and pathological diagnosis of gastroenteropancreatic neuroendocrine tumors (GEP-NETs) in Korea 2000-2009: Multicenter study. Cancer Res Treat 2012; 44: 157-65
• 16 Uppin MS, Uppin SG, Sunil CS, Hui M, Paul TH, Bheerappa N. et al. Clinicopathologic study of neuroendocrine tumors of gastroenteropancreatic tract: A single institutional experience. J Gastrointest Oncol 2017; 8: 139-47
• 17 Capella C, Solcia E, Sobin LH, Arnold R. Endocrine tumours of the oesophagus. In: Hamilton SR, Aaltonen LA. editors World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System. Lyon: IARC; 2000. p. 26-7
• 18 Arnold R, Capella C, Klimstra DS, Kloppel G, Komminoth P. et al. Neuroendocrine neoplasms of the oesophagus. In: Bosman TF, Carneiro F, Hruban RH, Theise ND. editors WHO Classification of Tumours of the Digestive system. Lyon: IARC; 2000. p. 26-7
• 19 Lloyd RV. Practical markers used in the diagnosis of neuroendocrine tumors. Endocr Pathol 2003; 14: 293-301
• 20 Lombard-Bohas C, Mitry E, O'Toole D, Louvet C, Pillon D, Cadiot G, Sarin YK. et al. Thirteen-month registration of patients with gastroenteropancreatic endocrine tumours in France. Neuroendocrinology 2009; 89: 217-22
• 21 Falconi M, Bettini R, Scarpa A, Capelli P, Pederzoli P. Surgical strategy in the treatment of gastrointestinal neuroendocrine tumours. Ann Oncol 2001; 12 Suppl 2: S101-3
• 22 Zhang M, Zhao P, Shi X, Zhao A, Zhang L, Zhou L. Clinicopathological features and prognosis of gastroenteropancreatic neuroendocrine neoplasms in a Chinese population: A large, retrospective single-centre study. BMC Endocr Disord 2017; 17: 39