Esthesioneuroblastoma (Olfactory Neuroblastoma) with Ectopic ACTH Syndrome: A Multidisciplinary Case Presentation from the Joan Karnell Cancer Center of Pennsylvania Hospital

Abstract

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

Low-Grade Esthesioneuroblastoma Presenting as SIADH: A Review of Atypical Manifestations

“…Most of the 8 reported cases of ectopic ACTH were from primary ENB tumors. It has also been found to first appear with recurrent disease, as in one pediatric case of late cervical lymph node recurrence [41] and in two adult cases of local recurrence despite aggressive surgical management [42, 48]. Although one patient died of recurrent disease, treatment of the ENB and/or recurrence led to resolution of the associated Cushing's syndrome in all other cases.…”

Neuroendocrine Neoplasms of the Sinonasal Tract: Neuroendocrine Carcinomas and Olfactory Neuroblastoma

“…The most common symptoms at presentation are nasal obstruction, epistaxis, and anosmia. Rare patients have presented with production of ectopic adrenocorticotropic hormone (ACTH) and inappropriate secretion of antidiuretic hormone secretion [27,28]. A complete physical and radiological examination-including CT and MRI-are essential for diagnosis, staging, and proper treatment planning [10].…”

Intraoperative hypertensive crisis due to a catecholamine‐secreting esthesioneuroblastoma

“…There are previous reports describing secreting esthesioneuroblastomas, but few reports describing catecholamine‐producing esthesioneuroblastomas . Other studies have reported secretion of adrenocorticotropic hormone from esthesioneuroblastomas . Syndrome of inappropriate antidiuretic hormone has also been reported in patients with esthesioneuroblastoma , …”