Three-Quarters Adrenalectomy for Infantile-Onset Cushing Syndrome due to Bilateral Adrenal Hyperplasia in McCune-Albright Syndrome

Tomoyo Itonaga, Hironori Goto, Manabu Toujigamori, Yasuharu Ohno, Seigo Korematsu, Tatsuro Izumi, Satoshi Narumi, Tomonobu Hasegawa, Kenji Ihara

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Background: Bilateral adrenalectomy is performed in cases with infantile-onset Cushing syndrome due to bilateral adrenal hyperplasia in McCune-Albright syndrome (MAS) because severe Cushing syndrome with heart failure and liver dysfunction can have a lethal outcome. This procedure can completely ameliorate hypercortisolism, although lifetime steroid replacement therapy and steps to prevent adrenal crisis are necessary. Recently, the efficacy of unilateral adrenalectomy has been reported in adult cases of bilateral macronodular adrenal hyperplasia, but there is no consensus regarding the appropriate surgical treatment for bilateral adrenal hyperplasia in MAS. Objective: A 6-month-old girl presented with café-au-lait spots, short stature, central obesity, a moon face, and hypertension. Endocrinological tests and imaging studies led to the diagnosis of ACTH-independent Cushing syndrome due to bilateral adrenal hyperplasia induced by MAS. “Three-quarters adrenalectomy”, namely right-sided total adrenalectomy and left-sided half adrenalectomy, was carried out. An activating mutation of the GNAS1 gene (p.Arg201Cys) was identified in the adrenal tissues. Since the operation, our patient has been in a state of clinical remission for more than 2 years. Conclusion: Our original surgical intervention, three-quarters adrenalectomy, may be a new treatment option for Cushing syndrome associated with MAS.

Original languageEnglish
JournalHormone Research in Paediatrics
DOIs
Publication statusAccepted/In press - 2017 May 19

Fingerprint

Polyostotic Fibrous Dysplasia
Cushing Syndrome
Adrenalectomy
Hyperplasia
Abdominal Obesity
Adrenocorticotropic Hormone
Liver Diseases
Therapeutics
Heart Failure
Steroids
Hypertension
Mutation
Genes

Keywords

  • Adrenalectomy
  • Bilateral adrenal hyperplasia
  • Cushing syndrome
  • McCune-Albright syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

Cite this

Three-Quarters Adrenalectomy for Infantile-Onset Cushing Syndrome due to Bilateral Adrenal Hyperplasia in McCune-Albright Syndrome. / Itonaga, Tomoyo; Goto, Hironori; Toujigamori, Manabu; Ohno, Yasuharu; Korematsu, Seigo; Izumi, Tatsuro; Narumi, Satoshi; Hasegawa, Tomonobu; Ihara, Kenji.

In: Hormone Research in Paediatrics, 19.05.2017.

Research output: Contribution to journalArticle

Itonaga, Tomoyo ; Goto, Hironori ; Toujigamori, Manabu ; Ohno, Yasuharu ; Korematsu, Seigo ; Izumi, Tatsuro ; Narumi, Satoshi ; Hasegawa, Tomonobu ; Ihara, Kenji. / Three-Quarters Adrenalectomy for Infantile-Onset Cushing Syndrome due to Bilateral Adrenal Hyperplasia in McCune-Albright Syndrome. In: Hormone Research in Paediatrics. 2017.
@article{15cd7dcbfebe452392488c13c059b14c,
title = "Three-Quarters Adrenalectomy for Infantile-Onset Cushing Syndrome due to Bilateral Adrenal Hyperplasia in McCune-Albright Syndrome",
abstract = "Background: Bilateral adrenalectomy is performed in cases with infantile-onset Cushing syndrome due to bilateral adrenal hyperplasia in McCune-Albright syndrome (MAS) because severe Cushing syndrome with heart failure and liver dysfunction can have a lethal outcome. This procedure can completely ameliorate hypercortisolism, although lifetime steroid replacement therapy and steps to prevent adrenal crisis are necessary. Recently, the efficacy of unilateral adrenalectomy has been reported in adult cases of bilateral macronodular adrenal hyperplasia, but there is no consensus regarding the appropriate surgical treatment for bilateral adrenal hyperplasia in MAS. Objective: A 6-month-old girl presented with caf{\'e}-au-lait spots, short stature, central obesity, a moon face, and hypertension. Endocrinological tests and imaging studies led to the diagnosis of ACTH-independent Cushing syndrome due to bilateral adrenal hyperplasia induced by MAS. “Three-quarters adrenalectomy”, namely right-sided total adrenalectomy and left-sided half adrenalectomy, was carried out. An activating mutation of the GNAS1 gene (p.Arg201Cys) was identified in the adrenal tissues. Since the operation, our patient has been in a state of clinical remission for more than 2 years. Conclusion: Our original surgical intervention, three-quarters adrenalectomy, may be a new treatment option for Cushing syndrome associated with MAS.",
keywords = "Adrenalectomy, Bilateral adrenal hyperplasia, Cushing syndrome, McCune-Albright syndrome",
author = "Tomoyo Itonaga and Hironori Goto and Manabu Toujigamori and Yasuharu Ohno and Seigo Korematsu and Tatsuro Izumi and Satoshi Narumi and Tomonobu Hasegawa and Kenji Ihara",
year = "2017",
month = "5",
day = "19",
doi = "10.1159/000473878",
language = "English",
journal = "Hormone Research in Paediatrics",
issn = "1663-2818",
publisher = "S. Karger AG",

}

TY - JOUR

T1 - Three-Quarters Adrenalectomy for Infantile-Onset Cushing Syndrome due to Bilateral Adrenal Hyperplasia in McCune-Albright Syndrome

AU - Itonaga, Tomoyo

AU - Goto, Hironori

AU - Toujigamori, Manabu

AU - Ohno, Yasuharu

AU - Korematsu, Seigo

AU - Izumi, Tatsuro

AU - Narumi, Satoshi

AU - Hasegawa, Tomonobu

AU - Ihara, Kenji

PY - 2017/5/19

Y1 - 2017/5/19

N2 - Background: Bilateral adrenalectomy is performed in cases with infantile-onset Cushing syndrome due to bilateral adrenal hyperplasia in McCune-Albright syndrome (MAS) because severe Cushing syndrome with heart failure and liver dysfunction can have a lethal outcome. This procedure can completely ameliorate hypercortisolism, although lifetime steroid replacement therapy and steps to prevent adrenal crisis are necessary. Recently, the efficacy of unilateral adrenalectomy has been reported in adult cases of bilateral macronodular adrenal hyperplasia, but there is no consensus regarding the appropriate surgical treatment for bilateral adrenal hyperplasia in MAS. Objective: A 6-month-old girl presented with café-au-lait spots, short stature, central obesity, a moon face, and hypertension. Endocrinological tests and imaging studies led to the diagnosis of ACTH-independent Cushing syndrome due to bilateral adrenal hyperplasia induced by MAS. “Three-quarters adrenalectomy”, namely right-sided total adrenalectomy and left-sided half adrenalectomy, was carried out. An activating mutation of the GNAS1 gene (p.Arg201Cys) was identified in the adrenal tissues. Since the operation, our patient has been in a state of clinical remission for more than 2 years. Conclusion: Our original surgical intervention, three-quarters adrenalectomy, may be a new treatment option for Cushing syndrome associated with MAS.

AB - Background: Bilateral adrenalectomy is performed in cases with infantile-onset Cushing syndrome due to bilateral adrenal hyperplasia in McCune-Albright syndrome (MAS) because severe Cushing syndrome with heart failure and liver dysfunction can have a lethal outcome. This procedure can completely ameliorate hypercortisolism, although lifetime steroid replacement therapy and steps to prevent adrenal crisis are necessary. Recently, the efficacy of unilateral adrenalectomy has been reported in adult cases of bilateral macronodular adrenal hyperplasia, but there is no consensus regarding the appropriate surgical treatment for bilateral adrenal hyperplasia in MAS. Objective: A 6-month-old girl presented with café-au-lait spots, short stature, central obesity, a moon face, and hypertension. Endocrinological tests and imaging studies led to the diagnosis of ACTH-independent Cushing syndrome due to bilateral adrenal hyperplasia induced by MAS. “Three-quarters adrenalectomy”, namely right-sided total adrenalectomy and left-sided half adrenalectomy, was carried out. An activating mutation of the GNAS1 gene (p.Arg201Cys) was identified in the adrenal tissues. Since the operation, our patient has been in a state of clinical remission for more than 2 years. Conclusion: Our original surgical intervention, three-quarters adrenalectomy, may be a new treatment option for Cushing syndrome associated with MAS.

KW - Adrenalectomy

KW - Bilateral adrenal hyperplasia

KW - Cushing syndrome

KW - McCune-Albright syndrome

UR - http://www.scopus.com/inward/record.url?scp=85020193250&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85020193250&partnerID=8YFLogxK

U2 - 10.1159/000473878

DO - 10.1159/000473878

M3 - Article

JO - Hormone Research in Paediatrics

JF - Hormone Research in Paediatrics

SN - 1663-2818

ER -