The case of a patient with recurrent esthesioneuroblastoma complicated by ectopic adrenocorticotropic hormone production is presented, including the workup and management of this uncommon complication of an uncommon disease. The Oncologist 2010;15: 51-58
CASE PRESENTATIONIn 1992, a 55-year-old white man presented with epistaxis and was diagnosed with esthesioneuroblastoma. He underwent a craniofacial resection with postoperative radiation therapy to his tumor site. In 1996, he relapsed in the cervical lymph nodes and underwent a bilateral cervical lymph node dissection. In total, eight positive lymph nodes were removed. He was then treated with chemotherapy with etoposide and cisplatin for four cycles. Although radiation therapy to the neck was recommended, the patient refused. In 1998, he relapsed again in the left submandibular area, and two involved lymph nodes were resected. He then agreed to and received radiation therapy to both sides of the neck. In 2002, he had evidence of further disease progression in the posterior nasopharynx and a left preauricular node. In 2004, he was treated with etoposide and carboplatin for four cycles with a minor response. Following that, he was observed without further therapy, given the indolent nature of his disease and minimal symptoms. He did, however, experience focal seizures, occasional epistaxis, hyponatremia, hypothyroidism, and an episode of pneumococcal meningitis.In August 2007, he developed fatigue, confusion, severe hypertension, and proximal muscle weakness. Laboratory studies revealed a serum glucose level of 228 mg/dl (normal, 70 -99 mg/dl), serum potassium of 2.9 mmol/l (normal, 3.5-5.1 mmol/l), pH of 7.570 (normal, 7.350 -7.450