Cargando…

Treatment protocols for growth hormone-secreting pituitary adenomas combined with craniofacial fibrous dysplasia: A case report of atypical McCune-Albright syndrome

McCune-Albright syndrome (MAS) is a rare, post-zygotic (non-germline) disorder, characterized by hypersecretory endocrinopathies, fibrous dysplasia of the bone and café-au-lait macules. The most common endocrine dysfunction is gonadal hyperfunction; thus, hypersecretion of growth hormones (GHs) as a...

Descripción completa

Detalles Bibliográficos
Autores principales: XU, JIA, LI, XI, LV, CHANG-SHENG, CHEN, YING, WANG, MENG, LIU, JIAN-FENG, GUI, LAI
Formato: Online Artículo Texto
Lenguaje:English
Publicado: D.A. Spandidos 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4113538/
https://www.ncbi.nlm.nih.gov/pubmed/25120617
http://dx.doi.org/10.3892/etm.2014.1792
_version_ 1782328303784296448
author XU, JIA
LI, XI
LV, CHANG-SHENG
CHEN, YING
WANG, MENG
LIU, JIAN-FENG
GUI, LAI
author_facet XU, JIA
LI, XI
LV, CHANG-SHENG
CHEN, YING
WANG, MENG
LIU, JIAN-FENG
GUI, LAI
author_sort XU, JIA
collection PubMed
description McCune-Albright syndrome (MAS) is a rare, post-zygotic (non-germline) disorder, characterized by hypersecretory endocrinopathies, fibrous dysplasia of the bone and café-au-lait macules. The most common endocrine dysfunction is gonadal hyperfunction; thus, hypersecretion of growth hormones (GHs) as a manifestation of endocrine hyperfunction in MAS is rarely reported. MAS affects both genders, although the majority of cases have been reported in young females. Atypical presentations of MAS, with only one or two of the classic symptoms, have been previously described, but remain particularly challenging due to the lack of a diagnostic phenotype. In patients with atypical MAS, analysis of mutations in the gene of the α-subunit of the stimulatory G-protein is limited; thus, diagnosis is based on clinical judgment. In the present study, a male with polyostotic fibrous dysplasia and GH-secreting pituitary adenomas, diagnosed with atypical MAS, was reported. The pituitary adenoma was effectively treated with radiotherapy and the patient underwent surgery for the polyostotic fibrous dysplasia, with marked improvements observed in appearance.
format Online
Article
Text
id pubmed-4113538
institution National Center for Biotechnology Information
language English
publishDate 2014
publisher D.A. Spandidos
record_format MEDLINE/PubMed
spelling pubmed-41135382014-08-12 Treatment protocols for growth hormone-secreting pituitary adenomas combined with craniofacial fibrous dysplasia: A case report of atypical McCune-Albright syndrome XU, JIA LI, XI LV, CHANG-SHENG CHEN, YING WANG, MENG LIU, JIAN-FENG GUI, LAI Exp Ther Med Articles McCune-Albright syndrome (MAS) is a rare, post-zygotic (non-germline) disorder, characterized by hypersecretory endocrinopathies, fibrous dysplasia of the bone and café-au-lait macules. The most common endocrine dysfunction is gonadal hyperfunction; thus, hypersecretion of growth hormones (GHs) as a manifestation of endocrine hyperfunction in MAS is rarely reported. MAS affects both genders, although the majority of cases have been reported in young females. Atypical presentations of MAS, with only one or two of the classic symptoms, have been previously described, but remain particularly challenging due to the lack of a diagnostic phenotype. In patients with atypical MAS, analysis of mutations in the gene of the α-subunit of the stimulatory G-protein is limited; thus, diagnosis is based on clinical judgment. In the present study, a male with polyostotic fibrous dysplasia and GH-secreting pituitary adenomas, diagnosed with atypical MAS, was reported. The pituitary adenoma was effectively treated with radiotherapy and the patient underwent surgery for the polyostotic fibrous dysplasia, with marked improvements observed in appearance. D.A. Spandidos 2014-09 2014-06-19 /pmc/articles/PMC4113538/ /pubmed/25120617 http://dx.doi.org/10.3892/etm.2014.1792 Text en Copyright © 2014, Spandidos Publications http://creativecommons.org/licenses/by/3.0 This is an open-access article licensed under a Creative Commons Attribution-NonCommercial 3.0 Unported License. The article may be redistributed, reproduced, and reused for non-commercial purposes, provided the original source is properly cited.
spellingShingle Articles
XU, JIA
LI, XI
LV, CHANG-SHENG
CHEN, YING
WANG, MENG
LIU, JIAN-FENG
GUI, LAI
Treatment protocols for growth hormone-secreting pituitary adenomas combined with craniofacial fibrous dysplasia: A case report of atypical McCune-Albright syndrome
title Treatment protocols for growth hormone-secreting pituitary adenomas combined with craniofacial fibrous dysplasia: A case report of atypical McCune-Albright syndrome
title_full Treatment protocols for growth hormone-secreting pituitary adenomas combined with craniofacial fibrous dysplasia: A case report of atypical McCune-Albright syndrome
title_fullStr Treatment protocols for growth hormone-secreting pituitary adenomas combined with craniofacial fibrous dysplasia: A case report of atypical McCune-Albright syndrome
title_full_unstemmed Treatment protocols for growth hormone-secreting pituitary adenomas combined with craniofacial fibrous dysplasia: A case report of atypical McCune-Albright syndrome
title_short Treatment protocols for growth hormone-secreting pituitary adenomas combined with craniofacial fibrous dysplasia: A case report of atypical McCune-Albright syndrome
title_sort treatment protocols for growth hormone-secreting pituitary adenomas combined with craniofacial fibrous dysplasia: a case report of atypical mccune-albright syndrome
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4113538/
https://www.ncbi.nlm.nih.gov/pubmed/25120617
http://dx.doi.org/10.3892/etm.2014.1792
work_keys_str_mv AT xujia treatmentprotocolsforgrowthhormonesecretingpituitaryadenomascombinedwithcraniofacialfibrousdysplasiaacasereportofatypicalmccunealbrightsyndrome
AT lixi treatmentprotocolsforgrowthhormonesecretingpituitaryadenomascombinedwithcraniofacialfibrousdysplasiaacasereportofatypicalmccunealbrightsyndrome
AT lvchangsheng treatmentprotocolsforgrowthhormonesecretingpituitaryadenomascombinedwithcraniofacialfibrousdysplasiaacasereportofatypicalmccunealbrightsyndrome
AT chenying treatmentprotocolsforgrowthhormonesecretingpituitaryadenomascombinedwithcraniofacialfibrousdysplasiaacasereportofatypicalmccunealbrightsyndrome
AT wangmeng treatmentprotocolsforgrowthhormonesecretingpituitaryadenomascombinedwithcraniofacialfibrousdysplasiaacasereportofatypicalmccunealbrightsyndrome
AT liujianfeng treatmentprotocolsforgrowthhormonesecretingpituitaryadenomascombinedwithcraniofacialfibrousdysplasiaacasereportofatypicalmccunealbrightsyndrome
AT guilai treatmentprotocolsforgrowthhormonesecretingpituitaryadenomascombinedwithcraniofacialfibrousdysplasiaacasereportofatypicalmccunealbrightsyndrome