title: Ectopic secretion of GHRH by a pancreatic neuroendocrine tumor associated with an empty sella.
abstract: Acromegaly is usually the result of a pituitary growth hormone cell-adenoma or is more rarely due to ectopic secretion of growth hormone releasing hormone (GHRH).We report the case of a 60-year-old woman with acromegaly due to a GH-RH-secreting pancreatic tumor. Laboratory evaluation confirmed the diagnosis of acromegaly. Magnetic resonance imaging revealed a partial empty sella with no signs of adenoma. Ultrasound sonography performed for abdominal pains showed a calcified large heterogeneous infrahepatic mass. Computed tomography scan discovered a heterogeneous pancreatic head mass with a diameter of 10cm. Measurement of fasting plasma GHRH was performed showing a high concentration of 604ng/L (normal 10-60). We therefore concluded that the acromegaly was caused by ectopic overproduction of GHRH likely due to the pancreatic tumor. The patient underwent a cephalic duodenopancreatectomy. Histology revealed a well-circumscribed tumor with organoid architecture. Immunohistochemistry demonstrated diffuse positivity for chromogranin A, neuronal specific enolase and synaptophysin and negative immunoreactivity for prolactin, GH and serotonin. These features were concordant with a well-differentiated neuroendocrine tumor of the pancreas. Surgical resection of this pancreatic tumor was followed by significant amelioration of acromegalic signs and normalization of GHRH and GH levels.
authors.affiliation: Endocrinology Department, Hedi Chaker Hospital, Magida Boulila Avenue, 3029 Sfax, Tunisia. mnifmouna@yahoo.fr
authors.forename: Mouna
authors.initials: M
authors.lastname: Mnif Feki
authors.forename: Fatma
authors.initials: F
authors.lastname: Mnif
authors.forename: Mahdi
authors.initials: M
authors.lastname: Kamoun
authors.forename: Nadia
authors.initials: N
authors.lastname: Charfi
authors.forename: Nabila
authors.initials: N
authors.lastname: Rekik
authors.forename: Basma Ben
authors.initials: BB
authors.lastname: Naceur
authors.forename: Lilia
authors.initials: L
authors.lastname: Mnif
authors.forename: Tahia Sellami
authors.initials: TS
authors.lastname: Boudawara
authors.forename: Mohamed
authors.initials: M
authors.lastname: Abid
mesh_terms: D004652:Empty Sella Syndrome / Q000150:complications* / Q000175:diagnosis; D005260:Female; D013007:Growth Hormone-Releasing Hormone / Q000378:metabolism*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000150:complications / Q000175:diagnosis / Q000378:metabolism*; D010190:Pancreatic Neoplasms / Q000150:complications / Q000175:diagnosis / Q000378:metabolism*; D009384:Paraneoplastic Endocrine Syndromes / Q000150:complications* / Q000175:diagnosis
chemical_list: D013007:Growth Hormone-Releasing Hormone