March/April - West Virginia State Medical Association

Scientific Article |Acromegaly Caused by Growth Hormone ReleasingHormone (GHRH) Secreting Tumor in MultipleEndocrine Neoplasia (MEN-1)Tipu Faiz M. Saleem, MDAssociate Professor, Division of Endocrinology,JCESOM, Marshall University, HuntingtonPrasanna Santhanam, MDAssistant Professor, Division of Endocrinology,JCESOM, Marshall University, HuntingtonEyad Hamoudeh, MDFellow, Division of Endocrinology, JCESOM,Marshall University, HuntingtonTamer Hassan, MDResident, Division of Internal Medicine,JCESOM, Marshall University, HuntingtonSaba Faiz MDFellow, Division of Endocrinology, JCESOM,Marshall University, HuntingtonAbbreviationsMEN-1: Multiple endocrineneoplasia type 1, NECT:Neuroendocrine tumor, GHRH:Growth hormone releasinghormone, GH: Growth hormone,IGF-1: Insulin like growth factor.gland and the NECT. Life longsurveillance is needed as recurrencechance is high.IntroductionMultiple endocrine neoplasiatype 1 (MEN-1) is defined aspresence of any two tumors outof the following; parathyroid,neuroendocrine tumor (NECT)and pituitary neoplasia. 1Hypersecretion of growth hormonereleasing hormone (GHRH) is raremanifestation of NECT; howeverhalf of such cases are found withMEN-1. 2 Incidence rate of acromegalywith GH over secretion is up to 15%in MEN-1 pituitary tumors similarto non-MEN-1 pituitary tumors. 3There are two different etiologicmechanisms of acromegaly caused byexcess GH in MEN-1 with differenttreatment implications. Mostly it isdue to pituitary adenomas, whicharise clonally from inactivation ofboth alleles of the MEN-1 gene in atumor precursor cell. 4 The secondmechanism is overproduction ofGHRH by NECT including pancreaticislet 2 or carcinoid tumor leading topolyclonal and hyperplastic pituitarygland with over production of GH.In this report, we describe acase of acromegaly, in a patientFigure 1.Contrast enhanced CT scan of abdomen. Arrow indicating pancreatic head tumor.AbstractWe are presenting the clinicalfeatures, diagnostic work up andtreatment of acromegaly caused byGrowth hormone releasing hormone(GHRH) secreting neuroendocrine tumor(NECT) in a case of multiple endocrineneoplasia type 1(MEN-1).A 36 year old man, known case ofMEN-1 presented with acromegalicfeatures. He has high IGF-1, GH and veryhigh GHRH levels with a pancreatic headtumor and pituitary mass. He had highGHRH arteriovenous gradient acrosspancreatic tumor and underwent tumorresection, Post operative GHRH level felldramatically. Tumor had high GHRHm-RNA level.Acromegalic patients with MEN-1should be screened for ectopic GHRHsecretion. Measurement of GHRHarteriovenous gradient across NECT ormRNA for GHRH in resected tumor canconfirm the ectopic source. Treatment ofchoice is surgical resection of the tumor.Somatostatin analogue is an alternativebecause of its dual action in the pituitary26 West Virginia Medical Journal