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J Pediatr Endocrinol Metab. 2011;24(5-6):283-7.

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

Author information

1
Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hang Zhou 310009, China.

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.

PMID:
21823524
[Indexed for MEDLINE]

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