A non-acromegalic case of multiple endocrine neoplasia type 1 accompanied by a growth hormone-releasing hormone-producing pancreatic tumor.
Cases of acromegaly due to GHRHproducing pancreatic endocrine tumors have been reported. Here we present a case of a 31-yr-old nonacromegalic man with hyperparathyroidism and elevated serum IGF-I with normal serum GH levels. Serum GH was not suppressed below 1 ng/ml by the glucose tolerance test and increased in response to TR H and GHRH administration. Magnetic resonance imaging (MRI) revealed pituitary hyperplasia and an abdominal computed tomography (CT ) scan showed a tumor in the pancreatic tail. Plasma concentration of GHRH was elevated. Based on these clinical data, multiple endocrine neoplasia (MEN) type 1 was suspected. Three enlarged parathyroid glands were removed and a distal pancreatectomy was performed. Pathological examination of the parathyroid glands and pancreatic tumor showed nodular hyperplasia and a well-differentiated endocrine tumor, respectively, both compatible with MEN features. Immunohistochemistry revealed positive immunoreactivity for GHRH, SS , insulin, glucagon, chromogranin A, and pancreatic polypeptide in the pancreatic tumor. After pancreatic surgery, elevated levels of GHRH and IGF-I were normalized and pituitary hyperplasia definitely decreased in size. In cases of pituitary hyperplasia with elevated IGF-I, ectopic GHRH syndrome must be considered even if physical features of acromegaly are absent. It is also important to measure plasma GHRH concentrations in order to give a diagnosis.
Sugihara
H
H
Department of Medicine, Nippon Medical School, Sendagi, Bunkyo-ku, Tokyo, Japan. hitoshi@nms.ac.jp
Shibasaki
T
T
Tatsuguchi
A
A
Okajima
F
F
Wakita
S
S
Nakajima
Y
Y
Tanimura
K
K
Tamura
H
H
Ishii
S
S
Kamegai
J
J
Akasu
H
H
Kitagawa
W
W
Shimizu
K
K
Nakamura
Y
Y
Uchida
E
E
Tajiri
T
T
Naito
Z
Z
Katakami
H
H
Oikawa
S
S
D000172:Acromegaly; D000328:Adult; D013007:Growth Hormone-Releasing Hormone / Q000378:metabolism*; D019382:Human Growth Hormone / Q000097:blood; D006801:Humans; D006965:Hyperplasia; D006980:Hyperthyroidism / Q000150:complications / Q000473:pathology; D007334:Insulin-Like Growth Factor I / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D008297:Male; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000378:metabolism*; D010900:Pituitary Diseases / Q000473:pathology; D014057:Tomography, X-Ray Computed
D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I; D013007:Growth Hormone-Releasing Hormone