Successful treatment of esthesioneuroblastoma and neuroendocrine carcinoma with combined chemotherapy and proton radiation. Results in 9 cases

Abstract

Objective: To study the efficacy of a newly designed treatment strategy for esthesioneuroblastoma and neuroendocrine carcinoma of the paranasal sinuses.

Design and setting: Nonrandomized prospective study of a case series in a tertiary referral center.

Patients: Nine consecutive patients with newly diagnosed esthesioneuroblastoma or neuroendocrine carcinoma of the paranasal sinuses from June 1992 to October 1995 underwent this treatment protocol.

Interventions: After histological diagnosis and detailed imaging, 2 cycles of cisplatin and etoposide chemotherapy were instituted. Chemotherapy responders were treated with combined photon and stereotaxic fractionated proton radiation therapy totaling approximately 68 Gy to the primary site, whereas poor responders were treated with surgical resection followed by postoperative radiation. In both cases, therapy was then concluded with 2 additional cycles of cisplatin and etoposide chemotherapy.

Main outcomes measures: Response to therapy, survival, disease-free survival, and complications of therapy were examined.

Results: Nine patients with a median Dulguerov T stage of T3 (range, T2 to T4) completed the treatment protocol, with mean follow-up after diagnosis of 20.5 months. Eight of 9 patients exhibited a dramatic response to therapy with remission of their tumor, and resection was not required. One patient failed to respond to induction chemotherapy and received surgical therapy to be followed by postoperative radiotherapy. There have been no recurrences (mean disease-free interval of 14.0 months). Complications were limited and generally transient.

Conclusions: The use of combined cisplatin and etoposide chemotherapy with proton radiation has demonstrated initial success in treatment of these tumors. Dramatic response from chemotherapy is possible even in bulky or unresectable disease. This protocol has an acceptable complication rate and conveys less morbidity than craniofacial resection and conventional radiotherapy. Further follow-up will be required to determine the long-term success rate of this therapeutic protocol.