PMID- 18753103
OWN - NLM
STAT- MEDLINE
DCOM- 20090114
LR  - 20210204
IS  - 1934-2403 (Electronic)
IS  - 1530-891X (Linking)
VI  - 14
IP  - 5
DP  - 2008 Jul-Aug
TI  - Gonadotroph adenoma in multiple endocrine neoplasia type 1.
PG  - 592-4
AB  - OBJECTIVE: To describe a case of an in vivo follicle-stimulating hormone 
      (FSH)-secreting gonadotroph adenoma in a man with multiple endocrine neoplasia 
      type 1 (MEN 1) syndrome. METHODS: We present a retrospective case description and 
      a discussion of the related literature. RESULTS: A 48-year-old man had 
      progressively deteriorating visual acuity and bitemporal hemianopia, found to be 
      attributable to a macroadenoma encircling both carotid arteries and compressing 
      the optic chiasm. His libido and erectile function had been reduced for 2 years. 
      The serum FSH level was substantially elevated at 31.1 IU/L (reference range, 1 
      to 10); alpha-subunit was elevated at 2.25 IU/L (reference range, 0.09 to 0.4); 
      luteinizing hormone was normal at 4 IU/L (reference range, 1 to 10); total 
      testosterone was low at 6.8 nmol/L (reference range, 9.5 to 35); and prolactin 
      was slightly increased at 433 mU/L (reference range, 50 to 300). Transsphenoidal 
      hypophysectomy was performed. Pituitary histopathologic examination showed a 
      tumor with cytoplasmic granular FSH immunoreactivity. The patient had undergone 
      parathyroidectomy for primary hyperparathyroidism 2 years before the current 
      intervention. A family history of endocrine neoplasia was obtained of one sibling 
      with a nonfunctioning pituitary adenoma, another sibling who had died of 
      pancreatic carcinoma, and a third sibling, along with her son, who has primary 
      hyperparathyroidism. Performance of genetic testing for MEN 1 revealed a nonsense 
      mutation--R460X (nt7605C>T)--located on exon 10 of the MEN1 gene. CONCLUSION: We 
      report an unusual case of clearly high circulating immunoreactive FSH due to a 
      functioning FSH-secreting gonadotroph adenoma in a man with the MEN 1 syndrome.
FAU - Sztal-Mazer, Shoshana
AU  - Sztal-Mazer S
AD  - Department of Endocrinology & Diabetes, The Alfred Hospital, Melbourne, 
      Australia.
FAU - Topliss, Duncan J
AU  - Topliss DJ
FAU - Simpson, Richard W
AU  - Simpson RW
FAU - Hamblin, P Shane
AU  - Hamblin PS
FAU - Rosenfeld, Jeffrey V
AU  - Rosenfeld JV
FAU - McLean, Catriona A
AU  - McLean CA
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  - 3XMK78S47O (Testosterone)
RN  - 9002-67-9 (Luteinizing Hormone)
RN  - 9002-68-0 (Follicle Stimulating Hormone)
SB  - IM
MH  - Adenoma/*blood/complications/therapy
MH  - Follicle Stimulating Hormone/blood/metabolism
MH  - Humans
MH  - Luteinizing Hormone/blood
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*blood/complications/therapy
MH  - Pituitary Neoplasms/*blood/etiology/therapy
MH  - Testosterone/blood
EDAT- 2008/08/30 09:00
MHDA- 2009/01/15 09:00
CRDT- 2008/08/30 09:00
PHST- 2008/08/30 09:00 [pubmed]
PHST- 2009/01/15 09:00 [medline]
PHST- 2008/08/30 09:00 [entrez]
AID - S1530-891X(20)41096-1 [pii]
AID - 10.4158/EP.14.5.592 [doi]
PST - ppublish
SO  - Endocr Pract. 2008 Jul-Aug;14(5):592-4. doi: 10.4158/EP.14.5.592.