Acromegaly caused by growth hormone releasing hormone (GHRH) secreting tumor in multiple endocrine neoplasia (MEN-1).
We are presenting the clinical features, diagnostic work up and treatment of acromegaly caused by Growth hormone releasing hormone (GHRH) secreting neuroendocrine tumor (NECT) in a case of multiple endocrine neoplasia type 1 (MEN-1). A 36 year old man, known case of MEN-1 presented with acromegalic features. He has high IGF-1, GH and very high GHRH levels with a pancreatic head tumor and pituitary mass. He had high GHRH arteriovenous gradient across pancreatic tumor and underwent tumor resection, Post operative GHRH level fell dramatically. Tumor had high GHRH m-RNA level. Acromegalic patients with MEN-1 should be screened for ectopic GHRH secretion. Measurement of GHRH arteriovenous gradient across NECT or mRNA for GHRH in resected tumor can confirm the ectopic source. Treatment of choice is surgical resection of the tumor. Somatostatin analogue is an alternative because of its dual action in the pituitary gland and the NECT. Life long surveillance is needed as recurrence chance is high.
Division of Endocrinology, JCESOM, Marshall University Huntington, USA.
Tipu Faiz M
TF
Saleem
Prasanna
P
Santhanam
Eyad
E
Hamoudeh
Tamer
T
Hassan
Saba
S
Faiz
D000172:Acromegaly / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism; D000328:Adult; D013007:Growth Hormone-Releasing Hormone / Q000378:metabolism*; D006801:Humans; D007334:Insulin-Like Growth Factor I; D008297:Male; D009377:Multiple Endocrine Neoplasia / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism / Q000601:surgery; D018358:Neuroendocrine Tumors / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism / Q000601:surgery
D007334:Insulin-Like Growth Factor I; D013007:Growth Hormone-Releasing Hormone