Hintergrund: Das Ästhesioneuroblastom stellt als seltener und klinisch sehr variabler Tumor der Nasenhaupt- und -nebenhöhlen und der Schädelbasis eine Herausforderung an eine moderne multidisziplinäre Therapie dar. Allgemein anerkannte Prognosefaktoren existieren ebenso wenig wie eine Standardtherapie. Patienten und Methoden: Zwischen 1975 und 2001 haben wir 26 Patienten mit einem Ästhesioneuroblastom diagnostiziert und therapiert. Nach der Klassifikation von Kadish hatten 4 % das Stadium A, 53 % das Stadium B und 43 % das Stadium C. Nach dem Hyam-Grading handelte es sich in 52 % um low-grade- und in 48 % um high-grade-Tumoren. Bei 23 aller Patienten (88,5 %) wurde eine operative Therapie durchgeführt. Bei 5 von diesen Patienten war die Operation die einzige Therapie. In den übrigen 18 Fällen erfolgte eine kombinierte Therapie. Ergebnisse: Bei Datenerhebung waren 16 von 26 therapierten Patienten (61,5 %) am Leben. Die krankheitsspezifische 15-Jahres-Überlebensrate nach Kaplan-Meier betrug jeweils 76,2 %. Die 15-Jahres-Überlebensrate betrug für kleinere Tumoren (Kadish A/B) 86,7 %, für fortgeschrittene Tumoren (Kadish C) 63,6 %. 26,9 % aller therapierten Patienten entwickelten ein Rezidiv. Die Salvage-Therapie war in 60 % erfolgreich und die 15-Jahres-Überlebensrate nach Salvage-Therapie beträgt 60 %. Zusammenfassung: Bei der Therapie des Ästhesioneuroblastoms bedarf es einer interdisziplinären, multimodalen Therapiestrategie vor allem für fortgeschrittene Tumoren. Das Tumorstadium sowie das histopathologische Grading nach Hyams sind wichtige Faktoren für das Überleben und die Prognose. Eine aggressive Salvage-Therapie kann zu einer deutlichen Verbesserung der Langzeitüberlebensrate führen.
Abstract
Background: Esthesioneuroblastoma is a rare and clinically variable tumor of nasal sinus and skull base and challenging for modern multidisciplinary therapy. There are no generally known prognostic factors or generally accepted standard therapy regimens. Patients and methods: Between 1975 and 2001 26 patients were treated after the diagnosis of esthesioneuroblastoma was established. The cases were evaluated retrospectively. According to the classification of Kadish 1 patient (4 %) had stage A, 16 patients (53 %) stage B and 11 cases (43 %) had stage C tumors. Hyams grading could be obtained in 22 cases (81 %). Tumors were in 12 patients (52 %) graded I or II and in 10 cases tumors had grade III or IV (48 %). Operative therapy was performed in 23 patients (88.5 %), being in 5 patients a single mode therapy. In 18 cases combined therapy was performed (surgery and radiotherapy or radiotherapy and chemotherapy). Results: Actuarial survival was 61.5 % (16/26). Disease specific 10- and 15-year-survival according to the estimation of Kaplan-Meier was 76.2 %. Patients with small tumors (Kadish A/B) had a 15-year-survival of 86.7 % compared to 63.6 % in cases with advanced tumors (Kadish C). In 7 cases (26.9 %) recurrences developed. Salvage therapy was performed in 5 cases (71.4 %) with a success rate and a 15-year survival each of 60 %. Conclusions: Therapy of esthesioneuroblastoma is challenging because of rarity and biologic variability of the tumor and and because of lack of a standard therapy. An interdisciplinary multimodal therapeutic approach is necessary especially in case of advanced tumors with promising results. Histopathological grading according to Hyams and tumor stage are important factors for survival and prognosis. Although recurrence can occur with high frequency even after prolonged time interval, long time survival can be improved after aggressive salvage therapy. Therefore longterm follow up is mandatory.
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