Metastatic Adrenocortical Carcinoma as an Unusual Cause of Hypercortisolism in McCune-Albright Syndrome: a Case Report

Abstract

McCune-Albright Syndrome (MAS) is a mosaic genetic disorder characterized by polyostic fibrous dysplasia, precocious puberty, and café-au-lait skin spots. Due to the affection of various organs, now the clinical spectrum of presentation extends beyond the classical triad. Multiple associated endocrinopathies contribute majorly to the morbidity and mortality of patients. We present a case of a 9-year-old female child affected with MAS with hypercortisolism secondary to a neoplastic adrenal mass lesion with hepatic, pulmonary, and cerebral metastasis. The initial presenting symptom was altered mental sensorium with imaging features suggesting posterior reversible encephalopathy syndrome. Other associated endocrinopathies were also detected. The outcome, unfortunately, was fatal within 22 days of initial presentation. Association of endocrinopathies are common occurrence on cases of MAS; hence, patients must be adequately evaluated for them to prevent morbidity and mortality.