SUCCESSFUL TREATMENT OF ESTHESIONEUROBLASTOMA AND NEUROENDOCRINE CARCINOMA WITH COMBINED CHEMOTHERAPY AND PROTON RADIATION - RESULTS IN 9 CASES

Objective: To study the efficacy of a newly designed treatment strateg y for esthesioneuroblastoma and neuroendocrine carcinoma of the parana sal 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 unde rwent this treatment protocol. Interventions: After histological diagn osis and detailed imaging, 2 cycles of cisplatin and etoposide chemoth erapy were instituted. Chemotherapy responders were treated with combi ned photon and stereotaxic fractionated proton radiation therapy total ing approximately 68 Gy to the primary site, whereas poor responders w ere treated with surgical resection followed by postoperative radiatio n. In both cases, therapy was then concluded with 2 additional cycles of cisplatin and etoposide chemotherapy. Main Outcomes Measures: Respo nse 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 ex hibited a dramatic response to therapy with remission of their tumor, and resection was not required. One patient failed to respond to induc tion chemotherapy and received surgical therapy to be followed by post operative radiotherapy. There have been no recurrences (mean disease-f ree interval of 14.0 months). Complications were limited and generally transient. Conclusions: The use of combined cisplatin and etoposide c hemotherapy with proton radiation has demonstrated initial success in treatment of these tumors. Dramatic response from chemotherapy is poss ible even in bulky or unresectable disease. This protocol has an accep table complication rate ana conveys less morbidity than craniofacial r esection and conventional radiotherapy. Further follow-up will be requ ired to determine the long-term success rate of this therapeutic proto col.