Nationwide registry for patients with neuroendocrine neoplasm of pancreas, gastrointestinal tract, lungs, bronchi, or thymus in Japan

Susumu Hijioka,Norihiro Kokudo,Yasushi Ichikawa,Masayuki Imamura,Toshihiko Masui,Hiro‐O Yamano ,Akihiro Sakurai,Shinsuke Kojima,Yusuke Kinugasa,Yosuke Kasai,Hiroshi Date,Izumi Komoto,Kenichi Sugihara,Koichi Hirata,Ken Haruma,Akira Kudo ,Shinji Üemoto ,Minoru Tanabe,Takuji Okusaka,Kazuo Hara,Satoshi Hirano,Masanori Fukushima,Motohiro Sakamine,Tetsuhide Ito,Takashi Kikuchi

doi:10.1007/s10147-022-02130-y

Abstract

BackgroundNeuroendocrine neoplasm (NEN) is a comparatively rare tumor that has been considered indolent. Due to these characteristics, detailed epidemiological data have not been analyzed in Japan. To elucidate the present status of NEN diagnosis and treatment in Japan, we started a registry cohort study in January 2015.MethodsPatients pathologically diagnosed with NENs of the pancreas, gastrointestinal tract, lungs, bronchi, or thymus after January 2012 were enrolled in this registry after the date of ethics review committee approval in each hospital or institute. Follow-up was continued for enrolled patients.ResultsDuring 5 years of enrollment between January 2015 and December 2019, a total of 1526 participants from 63 departments were enrolled in this registry (mean, 305.2 participants/year), covering approximately 5.8% of the annual incidence of NENs in Japan. For pancreatic NEN, 41.9% of patients had metastasis and the dominant metastatic site was the liver, at twice the rate of lymph node metastasis in the current registry. In contrast, the frequency of lymph node metastasis from gastrointestinal (GI)-NEN was similar to that of the liver. The distribution of WHO 2019-based grades varied according to the primary site. Low-to-intermediate grade (G1–G2) was dominant for duodenal, jejunal/ileal, rectal, and pancreatic NENs, whereas high grade (G3 or NEC) was dominant for esophageal, stomach, and colon NENs. For PanNENs, G3 and NEC accounted only for 1.6% and 2.9%, respectively.ConclusionsThese cohort data provide crucial information for clinical research to clarify the characteristics of NENs in Japan.

Highlights

Neuroendocrine neoplasm (NEN) was defined by the World Health Organization (WHO) in 2000 as a tumor displaying positive immunostaining for chromogranin A or synaptophysin and specific histological features
Extended author information available on the last page of the article with gastroenteropancreatic NEN (GEP-NEN) has been increasing; the annual incidence rose from 1.09/100,000 population in 1973 to 5.25/100,000 population in 2004 [1]
A recent populationbased study reported that the annual incidence of GEP-NEN was 3.56/100,000 population in 2016 in Japan [5]

Summary

Introduction

Neuroendocrine neoplasm (NEN) was defined by the World Health Organization (WHO) in 2000 as a tumor displaying positive immunostaining for chromogranin A or synaptophysin and specific histological features. Bronchopulmonary neuroendocrine tumors (BP-NETs) comprise malignant carcinomas such as large-cell neuroendocrine carcinoma (LCNEC) and small cell lung cancer (SCLC), and typical and atypical carcinoid Such tumors form a pathologically and clinically heterogeneous group [6]. The clinicopathological characteristics of LCNEC and thymic NEC in Japan remain poorly understood, so detailed analysis of data for these pathological entities is needed With these backgrounds, we established a registry-based survey of patients with NENs of the pancreas, gastrointestinal tract, lungs, bronchi, or thymus in Japan to explore the status quo of clinical outcomes for NENs and to analyze the resulting data with the aim of contributing to future guidelines for the diagnosis and/or treatment of these entities in Japan

Patients and methods

Results

Conclusion