PMID- 20713338
OWN - NLM
STAT- MEDLINE
DCOM- 20110801
LR  - 20210204
IS  - 1934-2403 (Electronic)
IS  - 1530-891X (Linking)
VI  - 17
IP  - 1
DP  - 2011 Jan-Feb
TI  - Ectopic acromegaly due to a pancreatic neuroendocrine tumor producing growth 
      hormone-releasing hormone.
PG  - 79-84
LID - 10.4158/EP10165.CR [doi]
AB  - OBJECTIVE: To present a case of acromegaly due to ectopic growth 
      hormone-releasing hormone (GHRH) secretion from a pancreatic neuroendocrine tumor 
      in the context of multiple endocrine neoplasia type 1 (MEN 1). METHODS: We 
      describe the clinical, imaging, and pathologic findings of the study patient. 
      RESULTS: A 46-year-old woman presented with clinical and biochemical findings 
      diagnostic of acromegaly. Magnetic resonance imaging showed a 1.2-cm sellar mass. 
      Following resection of the macroadenoma, serum insulin-like growth factor 1 
      (IGF-1) and growth hormone (GH) levels remained unchanged. Pathologic examination 
      revealed adenomatous changes, including a nonsecretory focus and a prolactin 
      immunopositive area (GH stain negative in both). Octreotide long-acting release 
      was ineffective. Search for an ectopic tumor included normal octreoscan and 
      abdominal computed tomography. GHRH was greater than 1000 pg/mL. Repeated 
      abdominal computed tomography documented a 6.2-cm mass in the tail and body of 
      the pancreas. Distal pancreatectomy revealed a pancreatic neuroendocrine tumor 
      that stained positive for GHRH. Postoperatively, serum GHRH and IGF-1 normalized. 
      Re-evaluation of the initial pituitary pathologic specimen revealed additional 
      somatotroph hyperplasia of the adjacent, normal pituitary gland. Primary 
      hyperparathyroidism was diagnosed, and multigland parathyroid hyperplasia was 
      noted at surgery. Genetic testing was positive for a mutation in the MEN1 gene. 
      CONCLUSION: This patient's acromegaly was resistant to somatostatin analogue 
      therapy, reflecting the negative octreoscan imaging. In addition, this case is 
      novel because the patient presented with pituitary adenomatous changes, which 
      were presumably associated with MEN 1 and/or possibly the elevated GHRH levels.
FAU - Weiss, Danielle E
AU  - Weiss DE
AD  - Department of Medicine, Stanford University School of Medicine, Stanford, 
      California, USA.
FAU - Vogel, Hannes
AU  - Vogel H
FAU - Lopes, M Beatriz S
AU  - Lopes MB
FAU - Chang, Steven D
AU  - Chang SD
FAU - Katznelson, Laurence
AU  - Katznelson L
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Endocr Pract
JT  - Endocrine practice : official journal of the American College of Endocrinology 
      and the American Association of Clinical Endocrinologists
JID - 9607439
RN  - 12629-01-5 (Human Growth Hormone)
RN  - 67763-96-6 (Insulin-Like Growth Factor I)
RN  - 9034-39-3 (Growth Hormone-Releasing Hormone)
SB  - IM
MH  - Acromegaly/blood/*etiology/metabolism
MH  - Female
MH  - Growth Hormone-Releasing Hormone/*metabolism
MH  - Human Growth Hormone/blood
MH  - Humans
MH  - Insulin-Like Growth Factor I/metabolism
MH  - Middle Aged
MH  - Neuroendocrine Tumors/blood/*complications/*metabolism
MH  - Pancreatic Neoplasms/blood/*complications/*metabolism
EDAT- 2010/08/18 06:00
MHDA- 2011/08/02 06:00
CRDT- 2010/08/18 06:00
PHST- 2010/08/18 06:00 [entrez]
PHST- 2010/08/18 06:00 [pubmed]
PHST- 2011/08/02 06:00 [medline]
AID - S1530-891X(20)40877-8 [pii]
AID - 10.4158/EP10165.CR [doi]
PST - ppublish
SO  - Endocr Pract. 2011 Jan-Feb;17(1):79-84. doi: 10.4158/EP10165.CR.