Abstract

Several types of tumors affect adolescents. Despite being rare, the Ewing sarcoma family of tumors represents the second most common primary bone malignancy in children and adolescents. Paraneoplastic Cushing syndrome is a well-known condition characterized by an ectopic secretion of ACTH (or CRH), and in turn of cortisol by tumor cells. Paraneoplastic Cushing syndrome is however very rare in Ewing sarcoma, and to our knowledge, it was only described once. Diagnosis, treatment and adherence to treatment may be especially difficult in children and adolescents. Here we describe a case of severe paraneoplastic Cushing syndrome in a young adult with Ewing sarcoma. It was associated with psychosis and complicated by hemodynamic instability due to the unexpectedly rapid apparition of iatrogenic adrenal insufficiency.

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