Esthesioneuroblastoma: the Johns Hopkins experience.
BACKGROUND
Esthesioneuroblastoma (ENB) is an uncommon malignant neoplasm of the upper nasal cavity. Therapeutic management approaches for this neoplasm lack uniformity and there is no universally accepted staging system.


METHODS
A retrospective review of 27 patients with histologically confirmed ENB managed at The Johns Hopkins Hospital.


RESULTS
Eighty-five percent of patients had surgical resection as part of their disease management. Complete surgical resection was achieved in 62% of patients who had a craniofacial resection. Eighty percent of patients with negative surgical margins remain with no evidence of disease, with a median follow-up of 5.6 years. Adjuvant radiation therapy was beneficial to 62% of patients with positive surgical margins. Clinical responses were observed with cisplatin- and etoposide-containing chemotherapy regimens in patients with advanced disease. A revised staging system based on our experience is proposed.


CONCLUSIONS
ENB is best managed by craniofacial resection with complete tumor resection. Adjuvant radiation therapy is warranted in patients that remain with positive histologic margins of resection. Chemotherapy with cisplatin- and etoposide-containing regimens may be useful for palliation of advanced disease.
Resto
V A
VA
Department of Otolaryngology-Head and Neck Surgery, The Johns Hopkins Hospital, 601 North Caroline Street, Baltimore, Maryland 21287, USA.
Eisele
D W
DW
Forastiere
A
A
Zahurak
M
M
Lee
D J
DJ
Westra
W H
WH
D000293:Adolescent; D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002945:Cisplatin / Q000627:therapeutic use; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D018304:Esthesioneuroblastoma, Olfactory / Q000401:mortality / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D005047:Etoposide / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009296:Nasal Cavity; D009367:Neoplasm Staging; D009669:Nose Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies
D000970:Antineoplastic Agents; D005047:Etoposide; D002945:Cisplatin