Behandlungsstrategie bei sinunasalen adenoidzystischen Karzinomen – Ein Fall mit Literaturübersicht

Zusammenfassung

Hintergrund: Adenoidzystische Karzinome (ACC) im sinunasalen Bereich sind häufig nicht komplett zu resezieren. Vor diesem Hintergrund ist sowohl präoperativ über Resektionsgrenzen und funktionelle Ergebnisse zu diskutieren, als auch postoperativ über adjuvante Maßnahmen nachzudenken. Die frühere Meinung einer Strahlen- bzw. Chemoresistenz der ACC scheint überholt, obgleich kein einheitlicher Konsens über die therapeutische Strategie besteht.

Material und Methoden: Wir diskutieren die therapeutischen Optionen und vergleichen Literaturdaten bezüglich der optimalen adjuvanten Therapie an dem Fall einer 25-jährigen Patientin mit einem fortgeschrittenen adenoidzystischen Karzinom der Fossa pterygopalatina links. Daneben stellen wir alternative primäre oder adjuvante Therapieoptionen wie Neutronenbestrahlung, Chemotherapie oder „targeted” Therapie vor. Nach den Empfehlungen aus der Literatur haben wir eine augerhaltende Tumorresektion mittels transmaxilloethmoidaler Sphenoidektomie links über einen kombinierten trans- und extranasalen Zugang durchgeführt. Unter Inkaufnahme fehlender Randschnittfreiheit zugunsten des Verzichts auf verstümmelnde Maßnahmen wurde eine adjuvante Radiochemotherapie mit Taxol und Carboplatin durchgeführt.

Ergebnisse: Dadurch gelang bis dato (36 Monate postoperativ) eine optimale Lokalkontrolle bei anhaltendem uneingeschränktem Visus ohne funktionelle und kosmetische Einschränkun­gen. Regelmäßig durchgeführte Staginguntersuchungen ergaben bislang keinen Hinweis auf einen lokalen Tumorprogress oder Metastasen.

Schlussfolgerung: Funktionserhaltende Chirurgie und adjuvante Radio-chemotherapie auch in Kombination mit Taxol und Carboplatin ist nach unserer Ansicht eine geeignete Therapieoption zur Behandlung eines fortgeschrittenen ACC, und scheint keine Nachteile gegenüber radikalen chirurgischen Maßnahmen zu haben.

Abstract

Therapeutic Options in Sinunasal Adenoid Cystic ­Carcinomas – A Case Report and Review

Background: Adenoid cystic carcinomas (ACC) in sinunasal compartments are often not completely resectable. We discuss both, surgical margins and functional results preoperatively and postoperative options for adjuvant therapy. The former opinion of a resistance of ACC towards chemotherapy or irradiation seems to be outdated, even though consensus about therapeutic strategies is still missing.

Material and methods: We discuss therapeutic options and compare data from literature regarding the best adjuvant therapy with the case of a 25-year-old patient with an advanced ACC of the left fossa pterygopalatina. Further we discuss alternative therapeutic options like neutron irradiation, chemotherapy or targeted therapy. Following the advice from literature, we performed an eye saving tumor resection by a left transmaxilloethmoidale sphenoidectomy via combined trans- und extranasal approach. We accepted close surgical margins for the benefit of the abandonment of dismembering measures and performed an adjuvant radiochemotherapy with taxol and carboplatin.

Results: With the described therapeutic strategy we reached an optimal local tumor control with unlimited visus and without functional and cosmetic restrictions up to now. Periodic staging did not show any local tumor progress or metastatic spread hitherto.

Conclusion: Organ preserving surgery and adjuvant radiochemotherapy even in combination with taxol and carboplatin seems to be a sufficient therapeutic option in treating advanced sinunasal ACC, and might not have any prognostic disadvantages to radical surgery.

Schlüsselwörter

adenoidzystisches Karzinom - Fossa pterygopalatina - chirurgische Therapie - adjuvante Therapie

Key words

adenoid cystic carcinoma - fossa pterygopalatina - surgical options - adjuvant therapy

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