PMID- 36111275 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220917 IS - 2472-1972 (Electronic) IS - 2472-1972 (Linking) VI - 6 IP - 10 DP - 2022 Oct 1 TI - Metastatic Grade 3 Neuroendocrine Tumor in Multiple Endocrine Neoplasia Type 1 Expressing Somatostatin Receptors. PG - bvac122 LID - 10.1210/jendso/bvac122 [doi] LID - bvac122 AB - 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 (68)Ga-DOTATATE ((68)Ga-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 (177)Lu-DOTATATE treatment. PRRT (peptide receptor radionuclide therapy) with (177)Lu-DOTATATE ((177)Lu-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. CI - Published by Oxford University Press on behalf of the Endocrine Society 2022. FAU - Graf, Akua AU - Graf A AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Health, Bethesda, MD 20892, USA. FAU - Welch, James AU - Welch J AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Health, Bethesda, MD 20892, USA. FAU - Bansal, Rashika AU - Bansal R AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Health, Bethesda, MD 20892, USA. FAU - Mandl, Adel AU - Mandl A AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Health, Bethesda, MD 20892, USA. FAU - Parekh, Vaishali I AU - Parekh VI AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Health, Bethesda, MD 20892, USA. FAU - Cochran, Craig AU - Cochran C AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Health, Bethesda, MD 20892, USA. FAU - Levy, Elliot AU - Levy E AD - Radiology and Imaging Sciences, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA. FAU - Nilubol, Naris AU - Nilubol N AD - Endocrine Surgery Section, Surgical Oncology Program, National Cancer Institute, Bethesda 20892, MD, USA. FAU - Patel, Dhaval AU - Patel D AD - Endocrine Surgery Section, Surgical Oncology Program, National Cancer Institute, Bethesda 20892, MD, USA. FAU - Sadowski, Samira AU - Sadowski S AD - Endocrine Surgery Section, Surgical Oncology Program, National Cancer Institute, Bethesda 20892, MD, USA. FAU - Jha, Smita AU - Jha S AUID- ORCID: 0000-0001-9201-3340 AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Health, Bethesda, MD 20892, USA. FAU - Agarwal, Sunita K AU - Agarwal SK AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Health, Bethesda, MD 20892, USA. FAU - Millo, Corina AU - Millo C AD - Radiology and Imaging Sciences, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA. FAU - Blau, Jenny E AU - Blau JE AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Health, Bethesda, MD 20892, USA. FAU - Simonds, William F AU - Simonds WF AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Health, Bethesda, MD 20892, USA. FAU - Weinstein, Lee S AU - Weinstein LS AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Health, Bethesda, MD 20892, USA. FAU - Del Rivero, Jaydira AU - Del Rivero J AUID- ORCID: 0000-0001-9710-4030 AD - Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA. LA - eng PT - Case Reports DEP - 20220813 PL - United States TA - J Endocr Soc JT - Journal of the Endocrine Society JID - 101697997 PMC - PMC9469921 OTO - NOTNLM OT - gastroenteropancreatic (GEP) OT - grade 3 (G3) OT - multiple endocrine neoplasia type 1 (MEN1) OT - neuroendocrine tumor (NET) OT - peptide receptor radionuclide therapy (PRRT) EDAT- 2022/09/17 06:00 MHDA- 2022/09/17 06:01 PMCR- 2022/08/13 CRDT- 2022/09/16 02:47 PHST- 2022/03/17 00:00 [received] PHST- 2022/09/16 02:47 [entrez] PHST- 2022/09/17 06:00 [pubmed] PHST- 2022/09/17 06:01 [medline] PHST- 2022/08/13 00:00 [pmc-release] AID - bvac122 [pii] AID - 10.1210/jendso/bvac122 [doi] PST - epublish SO - J Endocr Soc. 2022 Aug 13;6(10):bvac122. doi: 10.1210/jendso/bvac122. eCollection 2022 Oct 1.