PMID- 21415556
OWN - NLM
STAT- MEDLINE
DCOM- 20110902
LR  - 20190907
IS  - 1348-4540 (Electronic)
IS  - 0918-8959 (Linking)
VI  - 58
IP  - 4
DP  - 2011
TI  - A case of ACTH-independent macronodular adrenal hyperplasia associated with 
      multiple endocrine neoplasia type 1.
PG  - 269-77
AB  - Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant neoplasia 
      syndrome characterized by the occurrence of tumors in the parathyroid glands, 
      pancreas, and anterior pituitary. Approximately 30-40% of MEN1 patients also have 
      adrenal lesions, such as hyperplasia, benign adenoma, and adrenocortical 
      carcinoma. Most of the cases are hormonally silent. We describe the case of a 
      60-year-old man with bilateral macronodular adrenal lesions, in addition to 
      parathyroid tumors, multiple insulinomas, and non-functioning pituitary 
      microadenoma. Endocrinological tests revealed subclinical hypercortisolism; 
      midnight cortisol level rose slightly (8.0 microg/dL), although basal plasma ACTH and 
      cortisol levels were within the normal range (19.5 pg/mL and 12.0 microg/dL, 
      respectively). One and 8 mg dexamethasone suppression tests showed cortisol 
      levels of 2.3 and 9.8 microg/dL, respectively. (131)I-adosterol scintigraphy under 
      dexamethasone suppression revealed bilateral adrenal uptake with right-sided 
      predominance. The histological features of the removed right adrenal gland were 
      consistent with ACTH-independent macronodular adrenal hyperplasia (AIMAH): 
      immunoreactivity of 17alpha-hydroxylase was predominantly observed in the small 
      compact cells, while that of 3beta-hydroxysteroid dehydrogenase was exclusively 
      expressed in the large clear cells. The glucose-dependent insulinotropic 
      polypeptide (GIP) receptor was expressed at high levels in compact cells, 
      suggesting that GIP is responsible for the development of AIMAH. Unilateral small 
      adrenal lesions were detected in the patient's 2 children, who also presented 
      with MEN1 symptoms. Genetic abnormalities in the MEN1, p27, and p18 genes were 
      not found, however, the present case may provide a clue to the understanding of 
      the etiology of MEN1 and AIMAH.
FAU - Yoshida, Masanori
AU  - Yoshida M
AD  - Department of Endocrinology and Diabetes, Nagoya Ekisaikai Hospital, Nagoya, 
      Japan. myavp@zm.commufa.jp
FAU - Hiroi, Maiko
AU  - Hiroi M
FAU - Imai, Tsuneo
AU  - Imai T
FAU - Kikumori, Toyone
AU  - Kikumori T
FAU - Himeno, Tatsuhito
AU  - Himeno T
FAU - Nakamura, Yasuhiro
AU  - Nakamura Y
FAU - Sasano, Hironobu
AU  - Sasano H
FAU - Yamada, Masanobu
AU  - Yamada M
FAU - Murakami, Yoshiko
AU  - Murakami Y
FAU - Nakamura, Sigeo
AU  - Nakamura S
FAU - Oiso, Yutaka
AU  - Oiso Y
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20110310
PL  - Japan
TA  - Endocr J
JT  - Endocrine journal
JID - 9313485
RN  - 0 (Receptors, Gastrointestinal Hormone)
RN  - 7S5I7G3JQL (Dexamethasone)
RN  - 9002-60-2 (Adrenocorticotropic Hormone)
RN  - D6H00MV7K8 (gastric inhibitory polypeptide receptor)
SB  - IM
MH  - Adenoma/complications
MH  - Adrenal Hyperplasia, Congenital/*complications/pathology
MH  - Adrenocorticotropic Hormone/blood
MH  - Adult
MH  - Dexamethasone
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*complications/pathology
MH  - Parathyroid Neoplasms/complications
MH  - Pedigree
MH  - Receptors, Gastrointestinal Hormone/metabolism
EDAT- 2011/03/19 06:00
MHDA- 2011/09/03 06:00
CRDT- 2011/03/19 06:00
PHST- 2011/03/19 06:00 [entrez]
PHST- 2011/03/19 06:00 [pubmed]
PHST- 2011/09/03 06:00 [medline]
AID - JST.JSTAGE/endocrj/K10E-218 [pii]
AID - 10.1507/endocrj.k10e-218 [doi]
PST - ppublish
SO  - Endocr J. 2011;58(4):269-77. doi: 10.1507/endocrj.k10e-218. Epub 2011 Mar 10.