Metastatic Grade 3 Neuroendocrine Tumor in Multiple Endocrine Neoplasia Type 1 Expressing Somatostatin Receptors

Akua Graf; James Welch; Rashika Bansal; Adel Mandl; Vaishali I Parekh; Craig Cochran; Elliot Levy; Naris Nilubol; Dhaval Patel; Samira Sadowski; Smita Jha; Sunita K Agarwal; Corina Millo; Jenny E Blau; William F Simonds; Lee S Weinstein; Jaydira Del Rivero

doi:10.1210/jendso/bvac122

Metastatic Grade 3 Neuroendocrine Tumor in Multiple Endocrine Neoplasia Type 1 Expressing Somatostatin Receptors

J Endocr Soc. 2022 Aug 13;6(10):bvac122. doi: 10.1210/jendso/bvac122. eCollection 2022 Oct 1.

Authors

Akua Graf¹, James Welch¹, Rashika Bansal¹, Adel Mandl¹, Vaishali I Parekh¹, Craig Cochran¹, Elliot Levy², Naris Nilubol³, Dhaval Patel³, Samira Sadowski³, Smita Jha¹, Sunita K Agarwal¹, Corina Millo², Jenny E Blau¹, William F Simonds¹, Lee S Weinstein¹, Jaydira Del Rivero⁴

Affiliations

¹ Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Health, Bethesda, MD 20892, USA.
² Radiology and Imaging Sciences, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA.
³ Endocrine Surgery Section, Surgical Oncology Program, National Cancer Institute, Bethesda 20892, MD, USA.
⁴ Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA.

Abstract

Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) may occur in 30% to 90% of patients with multiple endocrine neoplasia type 1 (MEN1). However, only 1% of GEP-NETs are grade 3 (G3). Given the rarity of these aggressive tumors, treatment of advanced G3 GEP-NETs in MEN1 is based on the treatment guidelines for sporadic GEP-NETs. We report a 43-year-old male with germline MEN1 followed at our institution, with clinical features including hyperparathyroidism, a nonfunctional pancreatic NET, and Zollinger-Ellison syndrome. On routine surveillance imaging at age 40, computed tomography/positron emission tomography imaging showed 2 arterially enhancing intraluminal masses on the medial aspect of the gastric wall. Anatomical imaging confirmed 2 enhancing masses within the pancreas and a rounded mass-like thickening along the lesser curvature of the stomach. The gastric mass was resected, and pathology reported a well-differentiated G3 NET with a Ki-67 >20%. The patient continued active surveillance. Eighteen months later cross-sectional imaging studies showed findings consistent with metastatic disease within the right hepatic lobe and bland embolization was done. On follow-up scans, including ⁶⁸Ga-DOTATATE (⁶⁸Ga-DOTA(0)-Tyr(3)-octreotate) imaging, interval increase in number and avidity of metastatic lesions were compatible with disease progression. Given a paucity of treatment recommendations for G3 tumors in MEN1, the patient was counseled based on standard NET treatment guidelines and recommended ¹⁷⁷Lu-DOTATATE treatment. PRRT (peptide receptor radionuclide therapy) with ¹⁷⁷Lu-DOTATATE (¹⁷⁷Lu-tetraazacyclododecanetetraacetic acid-octreotide) is an important therapeutic modality for patients with somatostatin receptor-positive NETs. However, prospective studies are needed to understand the role of PRRT in G3 NETs.

Keywords: gastroenteropancreatic (GEP); grade 3 (G3); multiple endocrine neoplasia type 1 (MEN1); neuroendocrine tumor (NET); peptide receptor radionuclide therapy (PRRT).

Published by Oxford University Press on behalf of the Endocrine Society 2022.

Publication types

Case Reports