Recurrent Esthesioneuroblastoma Presenting as an ACTH Paraneoplastic Syndrome

The authors report the case of a 51-year-old male that presented with hypertension and arm pain 5 years after combined transcranial and transfacial resection followed by radiotherapy for esthesioneuroblastoma. Laboratory studies revealed hypokalemia and elevated serum cortisol and ACTH levels. Brain magnetic resonance imaging (MRI) revealed a left-sided contrast-enhancing extra-axial dural-based lesion along the convexity. After resection of the convexity tumor, histological examination identified the lesion as recurrent esthesioneuroblastoma and serum ACTH and cortisol levels returned to near normal. Ectopic ACTH production from esthesioneuroblastoma is extremely rare and only six cases have been reported. This case report will describe the clinical presentation and pathology of recurrent esthesioneuroblastoma and review the literature.