PMID- 17235589
OWN - NLM
STAT- MEDLINE
DCOM- 20071106
LR  - 20220716
IS  - 1435-2443 (Print)
IS  - 1435-2443 (Linking)
VI  - 392
IP  - 4
DP  - 2007 Jul
TI  - Adrenal involvement in multiple endocrine neoplasia type 1: results of 7 years 
      prospective screening.
PG  - 437-43
AB  - BACKGROUND: Adrenal tumors are a common manifestation of the multiple endocrine 
      neoplasia type 1 (MEN-1) syndrome. Prevalence in recent studies varies between 9 
      and 45%. A genotype-phenotype correlation has been described as well as the 
      development of adrenocortical carcinomas. Long-term prospective data are still 
      lacking. MATERIALS AND METHODS: Thirty-eight MEN-1 patients with proven germline 
      mutations have been prospectively observed in a regular screening program in our 
      hospital. Adrenal glands have been screened by biochemical analysis and either by 
      endoscopic ultrasound (EUS) or computed tomography (CT) or both. Median follow-up 
      was 48 months (12-108 months). Age at diagnosis of MEN-1, type of adrenal tumor, 
      genotype, therapy, and clinical characteristics have been analyzed. RESULTS: In 
      21 (55%) patients, adrenal involvement of the disease was detected. Adrenal 
      lesions were detected in average 6.9 years after the initial diagnosis of MEN-1. 
      Median tumor size was 12 mm (5-40 mm). Tumor size smaller than 10 mm was observed 
      in 11 patients. Twelve patients had unilateral while nine had bilateral adrenal 
      lesions. EUS detected all adrenal tumors, whereas CT failed in seven cases. In 
      three patients, functioning tumors (one pheochromocytoma, one bilateral Cushing 
      adenoma, and one adrenocortical carcinoma) and one nonfunctioning adenoma were 
      diagnosed by histology and biochemical assessment. Two laparoscopic 
      adrenalectomies and one laparoscopic subtotal resection were performed. 
      Nonfunctioning adrenal lesions, not characterized by histology yet, were found in 
      18 patients. There was no statistical difference with regard to adrenal 
      involvement between patients with germline mutations in exons 2 and 10 (12/21) 
      and those with mutations in exons 3-9 (6/11). CONCLUSION: MEN-1-associated 
      adrenal tumors are mostly small, benign, and nonfunctioning and much more common 
      than previously reported. EUS was the most sensitive imaging procedure. The 
      genotype-pheotype correlation previously suggested by our group could not be 
      confirmed.
FAU - Waldmann, J
AU  - Waldmann J
AD  - Department of General Surgery, Philipps-University, Marburg, Germany.
FAU - Bartsch, D K
AU  - Bartsch DK
FAU - Kann, P H
AU  - Kann PH
FAU - Fendrich, V
AU  - Fendrich V
FAU - Rothmund, M
AU  - Rothmund M
FAU - Langer, P
AU  - Langer P
LA  - eng
PT  - Journal Article
DEP - 20070119
PL  - Germany
TA  - Langenbecks Arch Surg
JT  - Langenbeck's archives of surgery
JID - 9808285
SB  - IM
MH  - Adolescent
MH  - *Adrenal Gland Neoplasms/diagnostic imaging/epidemiology
MH  - Adrenalectomy
MH  - Adult
MH  - Aged
MH  - DNA Mutational Analysis
MH  - Endosonography
MH  - Female
MH  - Genotype
MH  - Germ-Line Mutation
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - *Multiple Endocrine Neoplasia Type 1/diagnostic imaging/epidemiology/genetics
MH  - Phenotype
MH  - Prevalence
MH  - Prospective Studies
EDAT- 2007/01/20 09:00
MHDA- 2007/11/07 09:00
CRDT- 2007/01/20 09:00
PHST- 2006/06/22 00:00 [received]
PHST- 2006/11/03 00:00 [accepted]
PHST- 2007/01/20 09:00 [pubmed]
PHST- 2007/11/07 09:00 [medline]
PHST- 2007/01/20 09:00 [entrez]
AID - 10.1007/s00423-006-0124-7 [doi]
PST - ppublish
SO  - Langenbecks Arch Surg. 2007 Jul;392(4):437-43. doi: 10.1007/s00423-006-0124-7. 
      Epub 2007 Jan 19.