Combination chemotherapy (cyclophosphamide, doxorubicin, and vincristine with continuous-infusion cisplatin and etoposide) and radiotherapy with stem cell support can be beneficial for adolescents and adults with estheisoneuroblastoma.

Abstract

Adolescent-onset and adult-onset esthesioneuroblastoma is a rare disease and is considered incurable. In many patients, local resection and radiation are chosen as clinical therapy with or without chemotherapy. It was reported previously that local resection and radiotherapy led to temporary remission and, in many patients, recurrent disease. Although combination with chemotherapy has been regarded as promising, an effective regimen has not been established. In the current study, the authors investigated the effect and tolerability of the combination of chemotherapy, radiotherapy, and peripheral blood stem cell transplantation (PBSCT). The study population included 12 patients with adolescent-onset and adult-onset esthesioneuroblastoma classified as Kadish Stage A-D. The patients received two cycles of combination chemotherapy, which consisted of cyclophosphamide, doxorubicin, and vincristine (CAD) with continuous-infusion cisplatin and etoposide (CVP). This was combined with radiotherapy with or without PBSCT. Nine of 12 patients (75%) obtained more than a partial response after only 2 cycles of chemotherapy. After radiation with or without PBSCT, six patients obtained a complete remission (CR). The longest survival was > 3 years. All patients who underwent PBSCT obtained a CR. The most severe side effects were loss of sodium and potassium induced by cisplatin-related renal tubular distress. Those abnormalities were temporary, and all patients recovered. The chemotherapy regimen with CADO and CVP does not require continuation for a long time and is very effective and tolerable for patients with adolescent-onset and adult-onset esthesioneuroblastoma. The combination with radiotherapy and PBSCT may lead to a CR without facial disfigurement. In this report, the authors discuss the feasibility and efficacy of this multidisciplinary approach.