University of Oulu

Koivikko, Minna, et al. “Acromegaly Caused by a GHRH-Producing Pancreatic Neuroendocrine Tumor: A Rare Manifestation of MEN1 Syndrome.” Endocrinology, Diabetes & Metabolism Case Reports, vol. 2022, Feb. 2022, pp. 21–0079.

Acromegaly caused by a GHRH-producing pancreatic neuroendocrine tumor : a rare manifestation of MEN1 syndrome

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Author: Koivikko, Minna1; Ebeling, Tapani1; Mäkinen, Markus2;
Organizations: 1Department of Internal Medicine, University of Oulu and Oulu University Hospital, Oulu, Finland
2Department of Pathology, University of Oulu, Oulu, Finland
3Institute of Biomedicine, University of Oulu, Oulu, Finland
4Department of Otorhinolaryngology, University of Oulu and Oulu University Hospital, Oulu, Finland
5Department of Internal Medicine, Central Finland Central Hospital, Jyväskylä, Finland
Format: article
Version: published version
Access: open
Online Access: PDF Full Text (PDF, 0.9 MB)
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Language: English
Published: Bioscientifica, 2022
Publish Date: 2022-09-28


Multiple endocrine neoplasia type 1 NM_001370259.2(MEN1):c.466G>C(p.Gly156Arg) is characterized by tumors of various endocrine organs. We report on a rare, growth hormone-releasing hormone (GHRH)-releasing pancreatic tumor in a MEN1 patient with a long-term follow-up after surgery. A 22-year-old male with MEN1 syndrome, primary hyperparathyroidism and an acromegalic habitus was observed to have a pancreatic tumor on abdominal CT scanning, growth hormone (GH) and insulin-like growth factor 1 (IGF1) were elevated and plasma GHRH was exceptionally high. GHRH and GH were measured before the treatment and were followed during the study. During octreotide treatment, IGF1 normalized and the GH curve was near normal. After surgical treatment of primary hyperparathyroidism, a pancreatic tail tumor was enucleated. The tumor cells were positive for GHRH antibody staining. After the operation, acromegaly was cured as judged by laboratory tests. No reactivation of acromegaly has been seen during a 20-year follow-up. In conclusion, an ectopic GHRH-producing, pancreatic endocrine neoplasia may represent a rare manifestation of MEN1 syndrome.

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Series: Endocrinology, diabetes & metabolism case reports
ISSN: 2052-0573
ISSN-E: 2052-0573
ISSN-L: 2052-0573
Volume: 2022
Issue: 1
Article number: 21-0079
DOI: 10.1530/edm-21-0079
Type of Publication: A1 Journal article – refereed
Field of Science: 3121 General medicine, internal medicine and other clinical medicine
3111 Biomedicine
3122 Cancers
Copyright information: © 2022 The authors. This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.