McCune-Albright syndrome combined with gigantism: clinical characteristics and long-term follow-up

doi:10.7504/nk2014050403

Abstract：Objective To investigate the clinical characteristics,causes of disease and responses to treatment in McCuneAlbright syndrome patients combined with gigantism.Methods Long-term follow-up were performed on two cases of McCune-Albright syndrome combined with gigantism,and in combination with literatures the clinical characteristics were analyzed and discussed.Results Both patients underwent neuronavigator assisted transsphenoidal resection of pituitary tumor.After operation,the levels of GH and IGF-1 were significantly decreased.In case 2,GH and IGF-1 levels returned to normal without recurrence during 3-year follow-up.In case 1,treatment of bromocriptine and long-acting somatostatin were performed;growth was notably inhibited and the levels of GH and IGF-1 were significantly decreased.Both patients received bisphosphonate therapy,and bone lesion progression was effectively controlled.Both patients were diagnosed with hypogonadism at the follow-up.Conclusion Treatment of this disease is a long procedure.Clinician should select individualized and comprehensive treatment measures according to the characteristics of patients.Neuronavigation in transsphenoidal resection of pituitary adenoma operation,long-acting somatostatin and bisphosphonate therapy are important measures to control the disease.