Unilateral adrenalectomy can be an alternative therapy for infantile onset Cushing's syndrome caused by ACTH-independent macronodular adrenal hyperplasia with McCune-Albright syndrome

Takashi Hamajima, Kaori Maruwaka, Keiko Homma, Kumihiro Matsuo, Kenji Fujieda, Tomonobu Hasegawa

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

We report herein the case of a 1-year-old boy with McCune-Albright syndrome (MAS) who presented with infantile-onset Cushing's syndrome caused by ACTH independent macronodular adrenal hyperplasia (AIMAH). Abdominal CT, MRI, and adrenal scintigraphy with 131I-adosterol identified bilateral adrenal involvement with the left adrenal gland being larger and functionally more active. Unilateral adrenalectomy of the left gland was performed and ameliorated many clinical symptoms, such as Cushingoid appearance and height restriction, and it also normalized many endocrinological data, such as diurnal rhythms of ACTH and cortisol, ACTH and cortisol responses to CRH, and urinary 24 hr free cortisol. Glucocorticoid was replaced for the first 1 year and 6 months after the operation. One adrenal crisis episode occurred at 3 weeks after the operation, but none have occurred since. These results suggest that unilateral adrenalectomy of the larger gland can be an alternative therapy for infantile onset Cushing's syndrome caused by AIMAH with MAS, when asymmetric involvement is evident and the smaller gland is not markedly enlarged.

Original languageEnglish
Pages (from-to)819-824
Number of pages6
JournalEndocrine Journal
Volume57
Issue number9
DOIs
Publication statusPublished - 2010

Fingerprint

Polyostotic Fibrous Dysplasia
Cushing Syndrome
Adrenalectomy
Complementary Therapies
Adrenocorticotropic Hormone
Hyperplasia
Hydrocortisone
Adosterol
Adrenal Glands
Circadian Rhythm
Radionuclide Imaging
Glucocorticoids

Keywords

  • Acth-independent macronodular adrenal hyperplasia (AIMAH)
  • Childhood
  • Cushing's syndrome
  • McCune-albright syndrome (MAS)
  • Unilateral adrenalectomy

ASJC Scopus subject areas

  • Endocrinology
  • Endocrinology, Diabetes and Metabolism

Cite this

Unilateral adrenalectomy can be an alternative therapy for infantile onset Cushing's syndrome caused by ACTH-independent macronodular adrenal hyperplasia with McCune-Albright syndrome. / Hamajima, Takashi; Maruwaka, Kaori; Homma, Keiko; Matsuo, Kumihiro; Fujieda, Kenji; Hasegawa, Tomonobu.

In: Endocrine Journal, Vol. 57, No. 9, 2010, p. 819-824.

Research output: Contribution to journalArticle

@article{183caa004abe4f558aed23c252c9eed7,
title = "Unilateral adrenalectomy can be an alternative therapy for infantile onset Cushing's syndrome caused by ACTH-independent macronodular adrenal hyperplasia with McCune-Albright syndrome",
abstract = "We report herein the case of a 1-year-old boy with McCune-Albright syndrome (MAS) who presented with infantile-onset Cushing's syndrome caused by ACTH independent macronodular adrenal hyperplasia (AIMAH). Abdominal CT, MRI, and adrenal scintigraphy with 131I-adosterol identified bilateral adrenal involvement with the left adrenal gland being larger and functionally more active. Unilateral adrenalectomy of the left gland was performed and ameliorated many clinical symptoms, such as Cushingoid appearance and height restriction, and it also normalized many endocrinological data, such as diurnal rhythms of ACTH and cortisol, ACTH and cortisol responses to CRH, and urinary 24 hr free cortisol. Glucocorticoid was replaced for the first 1 year and 6 months after the operation. One adrenal crisis episode occurred at 3 weeks after the operation, but none have occurred since. These results suggest that unilateral adrenalectomy of the larger gland can be an alternative therapy for infantile onset Cushing's syndrome caused by AIMAH with MAS, when asymmetric involvement is evident and the smaller gland is not markedly enlarged.",
keywords = "Acth-independent macronodular adrenal hyperplasia (AIMAH), Childhood, Cushing's syndrome, McCune-albright syndrome (MAS), Unilateral adrenalectomy",
author = "Takashi Hamajima and Kaori Maruwaka and Keiko Homma and Kumihiro Matsuo and Kenji Fujieda and Tomonobu Hasegawa",
year = "2010",
doi = "10.1507/endocrj.K10E-003",
language = "English",
volume = "57",
pages = "819--824",
journal = "Endocrine Journal",
issn = "0918-8959",
publisher = "Japan Endocrine Society",
number = "9",

}

TY - JOUR

T1 - Unilateral adrenalectomy can be an alternative therapy for infantile onset Cushing's syndrome caused by ACTH-independent macronodular adrenal hyperplasia with McCune-Albright syndrome

AU - Hamajima, Takashi

AU - Maruwaka, Kaori

AU - Homma, Keiko

AU - Matsuo, Kumihiro

AU - Fujieda, Kenji

AU - Hasegawa, Tomonobu

PY - 2010

Y1 - 2010

N2 - We report herein the case of a 1-year-old boy with McCune-Albright syndrome (MAS) who presented with infantile-onset Cushing's syndrome caused by ACTH independent macronodular adrenal hyperplasia (AIMAH). Abdominal CT, MRI, and adrenal scintigraphy with 131I-adosterol identified bilateral adrenal involvement with the left adrenal gland being larger and functionally more active. Unilateral adrenalectomy of the left gland was performed and ameliorated many clinical symptoms, such as Cushingoid appearance and height restriction, and it also normalized many endocrinological data, such as diurnal rhythms of ACTH and cortisol, ACTH and cortisol responses to CRH, and urinary 24 hr free cortisol. Glucocorticoid was replaced for the first 1 year and 6 months after the operation. One adrenal crisis episode occurred at 3 weeks after the operation, but none have occurred since. These results suggest that unilateral adrenalectomy of the larger gland can be an alternative therapy for infantile onset Cushing's syndrome caused by AIMAH with MAS, when asymmetric involvement is evident and the smaller gland is not markedly enlarged.

AB - We report herein the case of a 1-year-old boy with McCune-Albright syndrome (MAS) who presented with infantile-onset Cushing's syndrome caused by ACTH independent macronodular adrenal hyperplasia (AIMAH). Abdominal CT, MRI, and adrenal scintigraphy with 131I-adosterol identified bilateral adrenal involvement with the left adrenal gland being larger and functionally more active. Unilateral adrenalectomy of the left gland was performed and ameliorated many clinical symptoms, such as Cushingoid appearance and height restriction, and it also normalized many endocrinological data, such as diurnal rhythms of ACTH and cortisol, ACTH and cortisol responses to CRH, and urinary 24 hr free cortisol. Glucocorticoid was replaced for the first 1 year and 6 months after the operation. One adrenal crisis episode occurred at 3 weeks after the operation, but none have occurred since. These results suggest that unilateral adrenalectomy of the larger gland can be an alternative therapy for infantile onset Cushing's syndrome caused by AIMAH with MAS, when asymmetric involvement is evident and the smaller gland is not markedly enlarged.

KW - Acth-independent macronodular adrenal hyperplasia (AIMAH)

KW - Childhood

KW - Cushing's syndrome

KW - McCune-albright syndrome (MAS)

KW - Unilateral adrenalectomy

UR - http://www.scopus.com/inward/record.url?scp=77957743225&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=77957743225&partnerID=8YFLogxK

U2 - 10.1507/endocrj.K10E-003

DO - 10.1507/endocrj.K10E-003

M3 - Article

C2 - 20543510

AN - SCOPUS:77957743225

VL - 57

SP - 819

EP - 824

JO - Endocrine Journal

JF - Endocrine Journal

SN - 0918-8959

IS - 9

ER -