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Publications

Case Reports Journal Article Epublish

PubMed: 42007244 DOI: 10.1210/jcemcr/luag002

GHRH and insulin hypersecretion from a pancreatic neuroendocrine tumor in multiple endocrine neoplasia type 1.

JCEM Case Rep 4(4):luag002

Authors: Elisa Lamback, Daniel Alves Bulzico, Delmar Muniz Lourenço, Alexandre Vasiljevic, Gerald Raverot, Mônica R Gadelha

Affiliations

  • Neuroendocrinology Research Center, Endocrinology Section, Medical School and Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro 21941-617, Brazil.
  • Neuropathology and Molecular Genetics Laboratory, Instituto Estadual do Cérebro Paulo Niemeyer, Rio de Janeiro 20231-092, Brazil.
  • Neuroendocrine Unit, Instituto Estadual do Cérebro Paulo Niemeyer, Rio de Janeiro 20231-092, Brazil.
  • Endocrine Oncology Unit, Brazilian National Institute of Cancer, Rio de Janeiro 20230-130, Brazil.
  • Endocrine Genetics Unit, Laboratory of Cellular and Molecular Endocrinology (LIM-25), Division of Endocrinology and Metabolism, Hospital das Clínicas, University of São Paulo School of Medicine, São Paulo 05403-010, Brazil.
  • Division of Endocrine Oncology, Institute of Cancer of the State of São Paulo, University of São Paulo School of Medicine, São Paulo 01246-000, Brazil.
  • Centre de Pathologie Est, Groupement Hospitalier Est, Hospices Civils de Lyon, Lyon 69003, France.
  • Cancer Research Center of Lyon, Inserm U1052, Claude Bernard Lyon 1 University, Lyon 69373, France.
  • Endocrinology Department, Reference Center for Rare Pituitary Diseases HYPO, "Groupement Hospitalier Est" Hospices Civils de Lyon, Bron 69500, France.

Abstract

Acromegaly caused by ectopic growth hormone-releasing hormone (GHRH)-secreting neuroendocrine tumor (NET) is extremely rare, with cosecreting NETs even more seldom. We report a case of a female patient who presented with primary hyperparathyroidism (pHPT) and a GHRH- and insulin cosecreting pancreatic NET (pNET) and genetically confirmed multiple endocrine neoplasia type 1 (MEN1), within an undiagnosed family with various MEN1-related NETs. Due to the diagnosis of MEN1, screening for pituitary tumor was performed with biochemical evidence of acromegaly. Sellar magnetic resonance imaging revealed a sellar lesion, which was excised and compatible with somatotroph hyperplasia. Postoperatively, the patient developed severe hypoglycemia requiring hospitalization and the pNET was removed. Histopathology confirmed GHRH and insulin secreting pNET. Ectopic acromegaly in MEN1 is exceedingly rare. Patients with MEN1 often present with multiple pNETs, which may exhibit multihormonal secretion and frequently cosecrete GHRH and insulin in MEN1, while hypoglycemia may not be manifested possibly due to GH and insulin's counteractive effects on glucose metabolism.

MeSH

Insulin, Multiple Endocrine Neoplasia Type 1, Neoplasms, Multiple Endocrine Neoplasia, Insulinoma, Hormones, Acromegaly, Neuroendocrine Tumors, Neuroectodermal Tumors, Primitive, Hypoglycemia