PMID- 21916912
OWN - NLM
STAT- MEDLINE
DCOM- 20120622
LR  - 20211020
IS  - 1365-2265 (Electronic)
IS  - 0300-0664 (Print)
IS  - 0300-0664 (Linking)
VI  - 76
IP  - 3
DP  - 2012 Mar
TI  - Cushing's syndrome in multiple endocrine neoplasia type 1.
PG  - 379-86
LID - 10.1111/j.1365-2265.2011.04220.x [doi]
AB  - OBJECTIVE:   In patients with multiple endocrine neoplasia type 1 (MEN1), 
      Cushing's syndrome (CS) from endogenous hypercortisolism can result from 
      pituitary, adrenal or other endocrine tumours. The purpose of this study was to 
      characterize the range of presentations of CS in a large series of MEN1 patients. 
      DESIGN:   Retrospective review of NIH Clinical Center inpatient records over an 
      approximately 40-year period. PATIENTS:   Nineteen patients (eight males, 11 
      females) with CS and MEN1. MEASUREMENTS:   Biochemical, imaging, surgical and 
      pathological findings. RESULTS:   An aetiology was determined for 14 of the 19 
      patients with CS and MEN1: 11 (79%) had Cushing's disease (CD) and three (21%) 
      had ACTH-independent CS owing to adrenal tumours, frequencies indistinguishable 
      from sporadic CS. Three of 11 MEN1 patients with CD (27%) had additional 
      non-ACTH-secreting pituitary microadenomas identified at surgery, an incidence 
      10-fold higher than in sporadic CD. Ninety-one per cent of MEN1 patients with CD 
      were cured after surgery. Two of three MEN1 patients with ACTH-independent CS 
      (67%) had adrenocortical carcinoma. One patient with adrenal cancer and another 
      with adrenal adenoma were cured by unilateral adrenalectomy. No case of ectopic 
      ACTH secretion was identified in our patient cohort. The aetiology of CS could 
      not be defined in five patients; in three of these, hypercortisolism appeared to 
      resolve spontaneously. CONCLUSIONS:   The tumour multiplicity of MEN1 can be 
      reflected in the anterior pituitary, MEN1-associated ACTH-independent CS may be 
      associated with aggressive adrenocortical disease and an aetiology for CS in MEN1 
      may be elusive in a substantial minority of patients.
CI  - Published 2012. This article is a US Government work and is in the public domain 
      in the USA.
FAU - Simonds, William F
AU  - Simonds WF
AD  - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and 
      Kidney Diseases, National Institutes of Health, 10 Center Drive, Bethesda, MD 
      20892, USA. wfs@helix.nih.gov
FAU - Varghese, Sarah
AU  - Varghese S
FAU - Marx, Stephen J
AU  - Marx SJ
FAU - Nieman, Lynnette K
AU  - Nieman LK
LA  - eng
GR  - ZIA DK043012-09/Intramural NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Intramural
PL  - England
TA  - Clin Endocrinol (Oxf)
JT  - Clinical endocrinology
JID - 0346653
RN  - 9002-60-2 (Adrenocorticotropic Hormone)
SB  - IM
MH  - Adolescent
MH  - Adrenal Gland Neoplasms/blood/complications/surgery
MH  - Adrenocortical Adenoma/blood/complications/surgery
MH  - Adrenocorticotropic Hormone/blood
MH  - Adult
MH  - Child
MH  - Cushing Syndrome/blood/*complications/surgery
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/blood/*complications/surgery
MH  - Pituitary ACTH Hypersecretion/blood/*complications/surgery
MH  - Pituitary Neoplasms/blood/complications/surgery
MH  - Retrospective Studies
MH  - Time Factors
MH  - Young Adult
PMC - PMC3243821
MID - NIHMS323053
COIS- None of the authors has a conflict of interest to declare.
EDAT- 2011/09/16 06:00
MHDA- 2012/06/23 06:00
PMCR- 2013/03/01
CRDT- 2011/09/16 06:00
PHST- 2011/09/16 06:00 [entrez]
PHST- 2011/09/16 06:00 [pubmed]
PHST- 2012/06/23 06:00 [medline]
PHST- 2013/03/01 00:00 [pmc-release]
AID - 10.1111/j.1365-2265.2011.04220.x [doi]
PST - ppublish
SO  - Clin Endocrinol (Oxf). 2012 Mar;76(3):379-86. doi: 
      10.1111/j.1365-2265.2011.04220.x.