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

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.

Cite this paper

@article{Liu2011ACO, title={A case of McCune-Albright syndrome associated with pituitary GH adenoma: therapeutic process and autopsy.}, author={Fuyi Liu and Wenting Li and Yong Yao and Guilin Li and Yi Yang and Wanchen Dou and Dingrong Zhong and Lin Wang and Xiangdong Zhu and Hua Hu and Jianmin Zhang and Renzhi Wang and Gao Chen}, journal={Journal of pediatric endocrinology & metabolism : JPEM}, year={2011}, volume={24 5-6}, pages={283-7} }