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. 2021 Nov;56(11):1033-1044.
doi: 10.1007/s00535-021-01827-7. Epub 2021 Sep 29.

JNETS clinical practice guidelines for gastroenteropancreatic neuroendocrine neoplasms: diagnosis, treatment, and follow-up: a synopsis

Tetsuhide Ito  1   2 , Toshihiko Masui  3   4 , Izumi Komoto  3   4 , Ryuichiro Doi  3   4 , Robert Y Osamura  3   4 , Akihiro Sakurai  3   4 , Masafumi Ikeda  3   4 , Koji Takano  3   4 , Hisato Igarashi  3   4 , Akira Shimatsu  3   4 , Kazuhiko Nakamura  3   4 , Yuji Nakamoto  3   4 , Susumu Hijioka  3   4 , Koji Morita  3   4 , Yuichi Ishikawa  3   4 , Nobuyuki Ohike  3   4 , Atsuko Kasajima  3   4 , Ryoji Kushima  3   4 , Motohiro Kojima  3   4 , Hironobu Sasano  3   4 , Satoshi Hirano  3   4 , Nobumasa Mizuno  3   4 , Taku Aoki  3   4 , Takeshi Aoki  3   4 , Takao Ohtsuka  3   4 , Tomoyuki Okumura  3   4 , Yasutoshi Kimura  3   4 , Atsushi Kudo  3   4 , Tsuyoshi Konishi  3   4 , Ippei Matsumoto  3   4 , Noritoshi Kobayashi  3   4 , Nao Fujimori  3   4 , Yoshitaka Honma  3   4 , Chigusa Morizane  3   4 , Shinya Uchino  3   4 , Kiyomi Horiuchi  3   4 , Masanori Yamasaki  3   4 , Jun Matsubayashi  3   4 , Yuichi Sato  3   4 , Masau Sekiguchi  3   4 , Shinichi Abe  3   4 , Takuji Okusaka  3   4 , Mitsuhiro Kida  3   4 , Wataru Kimura  3   4 , Masao Tanaka  3   4 , Yoshiyuki Majima  3   4 , Robert T Jensen  3   4 , Koichi Hirata  3   4 , Masayuki Imamura  3   4 , Shinji Uemoto  3   4
Affiliations

JNETS clinical practice guidelines for gastroenteropancreatic neuroendocrine neoplasms: diagnosis, treatment, and follow-up: a synopsis

Tetsuhide Ito et al. J Gastroenterol. 2021 Nov.

Abstract

Neuroendocrine neoplasms (NENs) are rare neoplasms that occur in various organs and present with diverse clinical manifestations. Pathological classification is important in the diagnosis of NENs. Treatment strategies must be selected according to the status of differentiation and malignancy by accurately determining whether the neoplasm is functioning or nonfunctioning, degree of disease progression, and presence of metastasis. The newly revised Clinical Practice Guidelines for Gastroenteropancreatic Neuroendocrine Neoplasms (GEP-NENs) comprises 5 chapters-diagnosis, pathology, surgical treatment, medical and multidisciplinary treatment, and multiple endocrine neoplasia type 1 (MEN1)/von Hippel-Lindau (VHL) disease-and includes 51 clinical questions and 19 columns. These guidelines aim to provide direction and practical clinical content for the management of GEP-NEN preferentially based on clinically useful reports. These revised guidelines also refer to the new concept of "neuroendocrine tumor" (NET) grade 3, which is based on the 2017 and 2019 WHO criteria; this includes health insurance coverage of somatostatin receptor scintigraphy for NEN, everolimus for lung and gastrointestinal NET, and lanreotide for GEP-NET. The guidelines also newly refer to the diagnosis, treatment, and surveillance of NEN associated with VHL disease and MEN1. The accuracy of these guidelines has been improved by examining and adopting new evidence obtained after the first edition was published.

Keywords: Clinical practice guideline; Gastroenteropancreatic neuroendocrine neoplasm; Japanese Neuroendocrine Tumor Society.

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Conflict of interest statement

Any financial relationship with enterprises, businesses, or academic institutions in the subject matter or materials discussed in the manuscript are listed as follows; Tetsuhide Ito received speaker’s honoraria from Teijin Pharma Limited, Novartis Pharma and Mylan EPD. Robert Y. Osamura received speaker’s honoraria from Teijin Pharma Limited and Novartis Pharma. Akihiro Sakurai received speaker’s honoraria from AstraZeneca. Masafumi Ikeda received speaker’s honoraria from Novartis Pharma, Chugai Pharmaceutical Co. Ltd. and Bayer Pharma. Susumu Hijioka received speaker’s honoraria from Teijin Pharma Limited, Novartis Pharma and Novel Pharma. Taku Aoki received speaker’s honoraria from Novartis Pharma. Atsuko Kasajima received speaker’s honoraria from Teijin Pharma Limited. Ryoji Kushima received speaker’s honoraria from Takeda Pharmaceutical Co. Ltd. Hironobu Sasano received speaker’s honoraria from Novartis Pharma. Tsuyoshi Konishi received speaker’s honoraria from Olympus and Johnson & Johnson. The authors, the spouse, partner or immediate relatives of the authors report no other conflicts of interest in this work.

Figures

Fig. 1
Fig. 1
Surgical approach for nonfunctioning pancreatic NETs. (Superscript a) Check the swelling and firmness of the regional lymph nodes and dissect if lymph node metastases are suspected; if the tumor is discovered incidentally and there is no radiographic evidence of metastasis or invasion, follow-up may be an option with adequate explanation. PanNEC, pancreatic neuroendocrine carcinoma; PanNEN, pancreatic neuroendocrine neoplasm; PanNET, pancreatic neuroendocrine tumor
Fig. 2
Fig. 2
Surgical approach for NENs of the colon and rectum. NEC, neuroendocrine carcinoma; NEN, neuroendocrine neoplasm; NET, neuroendocrine tumor
Fig. 3
Fig. 3
Treatment strategy for metastatic/recurrent GEP-NEN. GEP-NEN: gastroenteropancreatic neuroendocrine neoplasm; NEC: neuroendocrine carcinoma; NET: neuroendocrine tumor; PPI: proton pump inhibitor; RFA: radiofrequency ablation; SSA: somatostatin analogue; TACE: transarterial chemoembolization; TAE: transcatheter arterial embolization
See this image and copyright information in PMC

References

    1. Imanura M, Ito T, Masui T, et al. Japanese Neuroendocrine Tumor Society. 1. Tokyo: Kanehara syuppan; 2015. Clinical practice guidelines for gastroenteropancreatic neuroendocrine tumors (NET)
    1. Ito T, Masui T, Komoto I, et al. Japanese Neuroendocrine Tumor Society. 2. Tokyo: Kanehara syuppan; 2019. Clinical practice guidelines for gastroenteropancreatic neuroendocrine neoplasms (NEN) 2019.
    1. Ito T, Hijioka S, Masui T, et al. Advances in the diagnosis and treatment of pancreatic neuroendocrine neoplasms in Japan. J Gastroenterol. 2017;52:9–18. doi: 10.1007/s00535-016-1250-9. - DOI - PubMed
    1. Ito T, Jensen RT. Molecular imaging in neuroendocrine tumors: recent advances, controversies, unresolved issues, and roles in management. Curr Opin Endocrinol Diabetes Obes. 2017;24:15–24. - PMC - PubMed
    1. Lloyd RV, Osamura RY, Klöppel G, et al. WHO classification of tumours of endocrine organs. In: Lloyd RV, et al., editors. WHO classification of tumours. 4. Lyon: International Agency for Research on Cancer; 2017.

MeSH terms

Supplementary concepts