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Esthesioneuroblastoma. Long term outcome and patterns of failure—the University of Virginia experience
Author(s) -
Eden Bernard V.,
Debo Richard F.,
Larner James M.,
Kelly Maria D.,
Levine Paul A.,
Stewart F. Marc,
Cantrell Robert W.,
Constable William C.
Publication year - 1994
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19940515)73:10<2556::aid-cncr2820731017>3.0.co;2-s
Subject(s) - esthesioneuroblastoma , medicine , surgery , radiation therapy , vincristine , chemotherapy , stage (stratigraphy) , salvage therapy , retrospective cohort study , cyclophosphamide , paleontology , biology
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 cyclophos‐phamide 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 locore‐gional. 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.