PMID- 37409236
OWN - NLM
STAT- MEDLINE
DCOM- 20230707
LR  - 20230718
IS  - 1664-2392 (Print)
IS  - 1664-2392 (Electronic)
IS  - 1664-2392 (Linking)
VI  - 14
DP  - 2023
TI  - Expressions of Cushing's syndrome in multiple endocrine neoplasia type 1.
PG  - 1183297
LID - 10.3389/fendo.2023.1183297 [doi]
LID - 1183297
AB  - Cushing's syndrome (CS) resulting from endogenous hypercortisolism can be 
      sporadic or can occur in the context of familial disease because of pituitary or 
      extra-pituitary neuroendocrine tumors. Multiple endocrine neoplasia type 1 (MEN1) 
      is unique among familial endocrine tumor syndromes because hypercortisolism in 
      this context can result from pituitary, adrenal, or thymic neuroendocrine tumors 
      and can therefore reflect either ACTH-dependent or ACTH-independent 
      pathophysiologies. The prominent expressions of MEN1 include primary 
      hyperparathyroidism, tumors of the anterior pituitary, gastroenteropancreatic 
      neuroendocrine tumors, and bronchial carcinoid tumors along with several common 
      non-endocrine manifestations such as cutaneous angiofibromas and leiomyomas. 
      Pituitary tumors are present in about 40% of MEN1 patients, and up to 10% of such 
      tumors secrete ACTH that can result in Cushing's disease. Adrenocortical 
      neoplasms occur frequently in MEN1. Although such adrenal tumors are mostly 
      clinically silent, this category can include benign or malignant tumors causing 
      hypercortisolism and CS. Ectopic tumoral ACTH secretion has also been observed in 
      MEN1, almost exclusively originating from thymic neuroendocrine tumors. The range 
      of clinical presentations, etiologies, and diagnostic challenges of CS in MEN1 
      are reviewed herein with an emphasis on the medical literature since 1997, when 
      the MEN1 gene was identified.
CI  - Copyright (c) 2023 Simonds.
FAU - Simonds, William F
AU  - Simonds WF
AD  - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and 
      Kidney Diseases, National Institutes of Health, Bethesda, MD, United States.
LA  - eng
GR  - ZIA DK043012/ImNIH/Intramural NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Intramural
PT  - Review
DEP - 20230620
PL  - Switzerland
TA  - Front Endocrinol (Lausanne)
JT  - Frontiers in endocrinology
JID - 101555782
RN  - 9002-60-2 (Adrenocorticotropic Hormone)
SB  - IM
MH  - Humans
MH  - *Cushing Syndrome/complications/diagnosis
MH  - *Multiple Endocrine Neoplasia Type 1/complications/genetics
MH  - *Pituitary ACTH Hypersecretion/complications
MH  - *Pituitary Neoplasms/complications
MH  - *ACTH Syndrome, Ectopic/complications
MH  - *Neuroendocrine Tumors/complications/genetics
MH  - *Thymus Neoplasms
MH  - Adrenocorticotropic Hormone
PMC - PMC10319112
OTO - NOTNLM
OT  - ACTH - independent CS
OT  - Cushing's adenoma
OT  - MEN1 = multiple endocrine neoplasia Type 1
OT  - corticotropinoma
OT  - familial neoplasia syndrome
OT  - pituitary tumor
COIS- The author declares that the research was conducted in the absence of any 
      commercial or financial relationships that could be construed as a potential 
      conflict of interest.
EDAT- 2023/07/06 06:42
MHDA- 2023/07/07 06:42
PMCR- 2023/01/01
CRDT- 2023/07/06 04:23
PHST- 2023/03/09 00:00 [received]
PHST- 2023/06/05 00:00 [accepted]
PHST- 2023/07/07 06:42 [medline]
PHST- 2023/07/06 06:42 [pubmed]
PHST- 2023/07/06 04:23 [entrez]
PHST- 2023/01/01 00:00 [pmc-release]
AID - 10.3389/fendo.2023.1183297 [doi]
PST - epublish
SO  - Front Endocrinol (Lausanne). 2023 Jun 20;14:1183297. doi: 
      10.3389/fendo.2023.1183297. eCollection 2023.