Results of a prospective multicenter neuroendocrine tumor registry reporting on clinicopathologic characteristics of Greek patients

BMC Endocrine Disorders

Research Article

Philippos Kaldrymidis¹  Dimitrios Dimitroulopoulos²  Georgios Alexandrakis³  Anna Koumarianou⁴  Vyron Markussis⁵  George C. Nikou⁶  Michalis Vaslamatzis⁷  Pavlos Papakostas⁸  Kalliopi Pazaitou-Panayiotou⁹ 

[1] Department of Endocrinology, Metaxa Cancer Hospital, Piraeus, Greece;Gastroenterology Department, Agios Savas Cancer Hospital, Αthens, Greece;Gastroenterology Department, ΝΙΜΤS Hospital, Αthens, Greece;Hematology-Oncology Unit, Fourth Department of Internal Medicine, Attikon University Hospital, Medical School, National and Kapodestrian University of Athens, Rimini 1, 12462, Haidari, Athens, Greece;Ipsen epe, Athens, Greece;Neuroendocrinology Section, 1st Department of Propaedeutic Internal Medicine, Laiko University Hospital, Athens, Greece;Oncology Department, Evangelismos Hospital, Athens, Greece;Oncology Department, Hippokrateion Hospital, Athens, Greece;Unit of Endocrinology and Endocrine Oncology, Theagenio Cancer Hospital, Thessaloniki, Greece;

关键词: Neuroendocrine tumors;    NET;    Neuroendocrine neoplasms;    NEN;    Neuroendocrine carcinomas;    NEC;    Gastric;    Enteric;    Pancreatic;    Head and neck;    Imaging;    Therapy;    Registry;   

DOI  :  10.1186/s12902-016-0089-7

 received in 2015-04-28, accepted in 2016-02-05,  发布年份 2016

来源: Springer

【 摘 要 】

BackgroundThe rare incidence of neuroendocrine neoplasms (NENs) has contributed to a paucity of large epidemiologic studies of patients with this condition. We investigated the occurrence and clinicopathologic features of NENs in Greece.MethodsBetween October 2010 and November 2012 we collected data on 246 newly diagnosed patients from a broad-based multi-institutional registry that comprises eight academic and hospital sites in Greece. The WHO 2010 pathologic classification and the 7th AJCC Staging system was applied in all cases.ResultsOf all patients 94 % had a sporadic and 6 % a multiple endocrine neoplasia tumor; 63.4 % were gastroenteropancreatic-(GEP)-NENs, 17.9 % Head & Neck NENs, 9.8 % NENs of Unknown Primary, 6.5 % Lung NENs and 2.4 % Pheochromocytomas. Gastric and pancreatic NENs were the most common primary sites. Poorly differentiated neuroendocrine carcinomas (NEC) were 9.3 %, all sporadic. Fifteen percent of patients were asymptomatic at presentation, 24 % had a first symptom of the disease related to endocrine syndrome and 61 % had symptoms related to locally advanced or metastatic disease. Metastatic disease was established in 25 % of tumors most frequently in the GEP NEN group. Findings are presented according to Ki-67 distribution. MRI had a higher diagnostic positive yield than Octreoscan. Somatostatin analogs, lanreotide and octreotide acetate, were prescribed at 38.5 & 61.5 % of NEN patients respectively and were found to be equally effective at providing symptomatic relief.ConclusionsThis is to our knowledge the first study of a Greek tumor registry and one of the few European Registries providing information regarding clinicopathologic characteristics and therapies in patients with neuroendocrine tumors of various origin sites, beyond GEP NENs.

【 授权许可】

CC BY   
© Nikou et al. 2016