MAS associated with pituitary GH adenoma:therapeutic process,necropsy and papers review

Abstract

Objective To observe the disease development and treatment process of McCune-Albright syndrome(MAS)associated with pituitary growth hormone(GH)adenoma by a continuous prospective following of a patient.To analyze the clinical and pathological recordings from the following and necropsy and obtain conclusions about the appropriate treatment for this disease.Method A prospective following of a patient of MAS associated with pituitary GH adenoma was performed continuously by recording the disease development and the treatment process until his death,after which a necropsy was performed.Combined with the recordings,necropsy revealing and literature study,the case was analyzed and discussed.Results MAS is a disease with progressive development,which can be accelerated by the associated pituitary GH adenoma and thus impair the life quality and shorten the life span of the patients.Although radiation could efficaciously controll GH hypersecretion of pituitary,it also may cause malignant transformation of the dysplastic bone tissue.The direct cause of death of this case,demonstrated by necropsy and pathology,was that the malignant transformed dysplastic bone tissue caused compression of the brain tissue,which eventually caused brain hernia and death.Conclusions CH adenoma,present in patient with MAS,is difficult to treat because the skull base bone dysplasia often prevents traditionary surgical removal of the tumor,while navigation-assisted transsphenoidal operation may be considered.Radiation therapy(RT)is in controversy because of the risk of sarcomatous transformation of the dysplastic bone tissue. Key words: Fibrous dysplasia,polyostotic; Growth hormone-secreting pituitary adenoma; Therapy