PMID- 1352309
OWN - NLM
STAT- MEDLINE
DCOM- 19920731
LR  - 20161123
IS  - 0021-972X (Print)
IS  - 0021-972X (Linking)
VI  - 75
IP  - 1
DP  - 1992 Jul
TI  - Clinical and genetic features of adrenocortical lesions in multiple endocrine 
      neoplasia type 1.
PG  - 76-81
AB  - In multiple endocrine neoplasia type 1 (MEN-1), benign enlargement of the adrenal 
      cortex has been found in about one third of necropsy cases. To elucidate the 
      clinical and genetic characteristics of the MEN-1 adrenal lesion, we have 
      investigated 33 MEN-1 patients. Twelve individuals (37%) demonstrated adrenal 
      enlargement, which was bilateral in 7 of them. Histopathology revealed diffuse 
      and nodular cortical hyperplasia, adenomas, and a single case of adrenocortical 
      carcinoma. The apparently benign adrenal enlargements were not associated with 
      presently ascertainable biochemical disturbances in the 
      hypothalamic-pituitary-adrenocortical axis, and they were without radiological 
      signs of progression during follow-up. The individual developing unilateral 
      adrenocortical carcinoma showed rapid adrenal expansion, feminization, and an 
      abnormal urinary steroid profile after 4 yr of observation for bilateral minor 
      adrenal enlargements. Pancreatic endocrine tumors were significantly 
      overrepresented and present in all MEN-1 individuals with adrenal involvement. In 
      agreement with findings in sporadic cases, the MEN-1 adrenocortical carcinoma 
      genome showed loss of constitutional heterozygosity for alleles at 17p, 13q, 11p, 
      and 11q. The benign adrenal lesions retained heterozygosity for the MEN-1 locus 
      at chromosome 11 q 13. Despite its prevalence and malignant potential, the 
      pituitary-independent adrenocortical proliferation does not appear to be a 
      primary lesion in MEN-1, but might represent a secondary phenomenon, perhaps 
      related to the pancreatic endocrine tumor.
FAU - Skogseid, B
AU  - Skogseid B
AD  - Department of Internal Medicine, University Hospital, Sweden.
FAU - Larsson, C
AU  - Larsson C
FAU - Lindgren, P G
AU  - Lindgren PG
FAU - Kvanta, E
AU  - Kvanta E
FAU - Rastad, J
AU  - Rastad J
FAU - Theodorsson, E
AU  - Theodorsson E
FAU - Wide, L
AU  - Wide L
FAU - Wilander, E
AU  - Wilander E
FAU - Oberg, K
AU  - Oberg K
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Clin Endocrinol Metab
JT  - The Journal of clinical endocrinology and metabolism
JID - 0375362
RN  - 3XMK78S47O (Testosterone)
RN  - 459AG36T1B (Dehydroepiandrosterone)
RN  - 4964P6T9RB (Aldosterone)
RN  - 9002-60-2 (Adrenocorticotropic Hormone)
RN  - 9015-71-8 (Corticotropin-Releasing Hormone)
RN  - WI4X0X7BPJ (Hydrocortisone)
SB  - IM
MH  - Adrenal Cortex/pathology
MH  - Adrenal Cortex Neoplasms/diagnostic imaging/*genetics/*pathology
MH  - Adrenocorticotropic Hormone/blood
MH  - Adult
MH  - Aged
MH  - Aldosterone/blood
MH  - Chromosomes, Human, Pair 11
MH  - Corticotropin-Releasing Hormone/blood
MH  - Dehydroepiandrosterone/blood
MH  - Female
MH  - Heterozygote
MH  - Humans
MH  - Hydrocortisone/blood/urine
MH  - Hypertrophy/diagnostic imaging
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia/diagnostic imaging/*genetics/*pathology
MH  - Polymorphism, Restriction Fragment Length
MH  - Testosterone/blood
MH  - Tomography, X-Ray Computed
MH  - Ultrasonography
EDAT- 1992/07/01 00:00
MHDA- 1992/07/01 00:01
CRDT- 1992/07/01 00:00
PHST- 1992/07/01 00:00 [pubmed]
PHST- 1992/07/01 00:01 [medline]
PHST- 1992/07/01 00:00 [entrez]
AID - 10.1210/jcem.75.1.1352309 [doi]
PST - ppublish
SO  - J Clin Endocrinol Metab. 1992 Jul;75(1):76-81. doi: 10.1210/jcem.75.1.1352309.