Esthesioneuroblastoma: cerebrospinal fluid rhinorrhea and widespread metastasis

Esthesioneuroblastoma, a tumor of neural crest origin arising in the nasal cavity, is uncommon, may be difficult to diagnose, and frequently is not cured. We report a case that was originally diagnosed as an inflammatory polyp and recurred with extension into the anterior cranial fossa. Surgery and radiotherapy were followed by local recurrence and cerebrospinal fluid rhinorrhea. Despite initial negative results of investigation for metastases, recurrence appeared in cervical nodes and was followed by widespread bone, soft tissue, and cerebral metastasis. There was no response to multiple-agent chemotherapy. The results of surgery, radiation, and chemotherapy in this rare and lethal tumor are reviewed.