Jump to ContentJump to Main Navigation
Show Summary Details
More options …

Journal of Pediatric Endocrinology and Metabolism

Editor-in-Chief: Kiess, Wieland

Ed. by Bereket, Abdullah / Darendeliler, Feyza / Dattani, Mehul / Gustafsson, Jan / Luo, Fei Hong / Mericq, Veronica / Toppari, Jorma


IMPACT FACTOR 2018: 1.239

CiteScore 2018: 1.22

SCImago Journal Rank (SJR) 2018: 0.507
Source Normalized Impact per Paper (SNIP) 2018: 0.562

Online
ISSN
2191-0251
See all formats and pricing
More options …
Volume 24, Issue 5-6

Issues

A case of McCune-Albright syndrome associated with pituitary GH adenoma: therapeutic process and autopsy

Liu Fuyi
  • Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hang Zhou 310009, China
  • Liu Fuyi, Li Wenting and Yao Yong contributed equally to this work
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Li Wenting
  • Department of Anatomy, Histology and Embryology, School of Basic Medicine, Peking Union Medical College, Institute of Basic Medical Science, Chinese Academy of Medical Sciences, Beijing 100005, China
  • Liu Fuyi, Li Wenting and Yao Yong contributed equally to this work
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Yao Yong
  • Department of Neurosurgery, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China
  • Liu Fuyi, Li Wenting and Yao Yong contributed equally to this work
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Li Guilin
  • Department of Neurosurgery, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Yang Yi
  • Department of Neurosurgery, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Dou Wanchen
  • Department of Neurosurgery, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Zhong Dingrong
  • Department of Pathology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Wang Lin
  • Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hang Zhou 310009, China
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Zhu Xiangdong
  • Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hang Zhou 310009, China
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Hu Hua
  • Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hang Zhou 310009, China
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Zhang Jianmin
  • Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hang Zhou 310009, China
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Wang Renzhi
  • Department of Neurosurgery, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Chen Gao
  • Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hang Zhou 310009, China
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
Published Online: 2011-05-24 | DOI: https://doi.org/10.1515/jpem.2011.178

Abstract

Background: McCune-Albright syndrome (MAS) is a clinical syndrome with low incidence, and its concurrence with pituitary GH adenoma is rare. Little of the history, treatment and outcome has been studied.

Method: Follow-up of a 37-year-old male patient of MAS associated with pituitary GH adenoma was performed continuously recording the disease development and the treatment process until death, after which an autopsy was performed.

Results: Radiation therapy (RT) efficaciously controlled GH hypersecretion, however, it may have been the cause of the malignant transformation of the dysplastic bone tissue, which eventually caused brain hernia and death; autopsy demonstrated that the cranium had significant thickening (as much as 10 cm), the pathological diagnosis was fibrous dysplasia of bone associated with chondrosarcoma; and undifferentiated chondrosarcoma with malignant fibrous histocytoma subtype in the sellar region; nodular goiter with the thyroid gland, one nodus was pathologically demonstrated as papillary carcinoma.

Conclusion: GH adenoma, present in a patient with MAS, might be cured by RT; but the risk of malignant transformation of the dysplastic bone tissue in the field of irradiation make it controversial. Lessons from the case reported here told us that we should take great caution when recommending RT for patients like this.

Keywords: McCune-Albright syndrome; papillary carcinoma of the thyroid gland; pituitary GH adenoma; radiation therapy

About the article

Corresponding authors: Wang Renzhi, Department of Neurosurgery, Peking Union Medical College Hospital, Peking Union Medical College, Beijing 100730, China Chen Gao, Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hang Zhou 310009, China Phone: +86-10-65296071, Fax: +86-10-65124875


Published Online: 2011-05-24

Published in Print: 2011-06-01


Citation Information: Journal of Pediatric Endocrinology and Metabolism, Volume 24, Issue 5-6, Pages 283–287, ISSN (Online) 2191-0251, ISSN (Print) 0334-018X, DOI: https://doi.org/10.1515/jpem.2011.178.

Export Citation

Comments (0)

Please log in or register to comment.
Log in