Esthesioneuroblastoma. Long-term outcome and patterns of failure--the University of Virginia experience.
BACKGROUND
Esthesioneuroblastoma is a rare tumor arising from olfactory epithelium. This retrospective review analyzed the patterns of failure and long term outcome of patients with esthesioneuroblastoma evaluated at a single institution.


METHODS
Forty patients with esthesioneuroblastoma were evaluated at the University of Virginia, with a median follow-up of 130 months. In most cases, treatment consisted of combined-modality therapy, including radiotherapy and surgery for Stages A and B disease and the addition of chemotherapy for Stage C disease. Fifteen patients received chemotherapy that included cyclophosphamide plus vincristine. Thirty-eight patients received radiotherapy, with a median dose of 50 Gy. Initial surgery for 23 patients included craniofacial resection, whereas the remainder had less extensive surgery (3 had no initial surgery). Five patients were salvaged with high dose chemotherapy and autologous bone marrow transplantation (CTX/BMT).


RESULTS
Actuarial survivals at 5, 10, and 15 years are 78%, 71%, and 65% respectively. Fifty-five percent of patients failed therapy, and 68% of the failures were locoregional. Thirty-nine percent of recurrences occurred later than 5 years from diagnosis. Three of the five patients were successfully salvaged with CTX/BMT compared with four of seventeen patients who underwent conventional salvage therapy.


CONCLUSIONS
Esthesioneuroblastoma is associated with long term survival and late recurrences. Multimodality therapy should be used initially. Durable remissions of failures can be achieved with CTX/BMT:
Eden
B V
BV
Department of Radiology, University of Virginia Health Sciences Center, Charlottesville 22908.
Debo
R F
RF
Larner
J M
JM
Kelly
M D
MD
Levine
P A
PA
Stewart
F M
FM
Cantrell
R W
RW
Constable
W C
WC
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016026:Bone Marrow Transplantation; D002648:Child; D003131:Combined Modality Therapy; D004359:Drug Therapy, Combination; D018304:Esthesioneuroblastoma, Olfactory / Q000401:mortality / Q000628:therapy*; D005260:Female; D006801:Humans; D008206:Lymphatic Diseases / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009296:Nasal Cavity; D009362:Neoplasm Metastasis; D009364:Neoplasm Recurrence, Local; D009378:Neoplasms, Multiple Primary; D009669:Nose Neoplasms / Q000401:mortality / Q000628:therapy*; D011878:Radiotherapy; D016879:Salvage Therapy; D017211:Treatment Failure; D016896:Treatment Outcome