Interdisziplinäres Management von komplexen tympanojugulären Paragangliomen Fisch Typ C und D

 

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Zusammenfassung

Hintergrund: Tympanojuguläre Paragangliome (TJP) sind benigne gefäßreiche Tumore, die lokal destruktiv wachsen. Trotz der umfangreichen Literatur sind die Behandlungskonzepte speziell bei ausgedehnten TJP nach wie vor umstritten. In der vorliegenden Studie präsentieren wir das interdisziplinäre Management von komplexen TJP.

Material und Methoden: Zwischen 2003 und 2013 wurden insgesamt 17 Patienten mit TJP Fisch Typ C und Typ D durch die Arbeitsgruppe Schädelbasischirurgie am Klinikum Dortmund behandelt. Primärsymptome waren am häufigsten eine Hörminderung, gefolgt von Fazialisparese bzw. kaudaler Hirnnervenläsion. 2 Patienten stellten sich mit einem Rezidivtumor vor. Eine interdisziplinäre mikrochirurgische Tumorresektion über einen retro/infralabyrinthischen Zugang nach vorheriger endovaskulärer Tumorembolisation erfolgte bei 14 Patienten, bei 2 weiteren Patienten erfolgte eine Strahlentherapie.

Ergebnisse: Eine radikale Tumorresektion konnte bei 10 Patienten erreicht werden. Eine temporäre postoperative Fazialisparese trat bei 2 Patienten auf und eine dauerhafte Stimmbandparese bei 3 Patienten. Ein Tumor-Rezidiv trat bei einem Patienten auf. Durch die primäre Strahlentherapie konnte bei 2 Patienten kein Tumorprogress festgestellt werden. Das Outcome des Gesamtkollektivs auf der Basis des Karnofsky-Index betrug bei 12 Patienten 100%, bei den übrigen 5 Patienten betrug er 90%.

Diskussion: Die operative Entfernung von TJP nach vorheriger Embolisation ist das Therapieverfahren 1.Wahl. Bei älteren bzw. multimorbiden Patienten ist die primäre Strahlentherapie eine geeignete Therapieoption und mit einer hohen Tumorkontrollrate assoziiert. Eine präoperative Abklärung mit individueller Risiko-Nutzen-Abwägung sind für den Therapierfolg entscheidend.

Abstract

Background: Tympanojugular paraganglioma (TJP) are benign, high vascularized, local destructive tumors. Despite many studies in the literature, the management of particularly complex TJP (e. g., posterior fossa and/or carotid artery invasion) remains controversial. In the current study we present our treatment strategies for complex TJP and long-term results.

Patients and methods: Between 2003 and 2013, 17 patients with TJP Fisch types C and D were treated in our institution. Primary symptoms were hearing loss, followed by facial nerve palsy and lower cranial nerve impairments. 2 patients presented with recurrent tumors. Surgical treatment after endovascular tumor embolization was performed in 14 patients. 2 patients were treated by radiation therapy.

Results: Gross tumor resection was achieved in 10 patients. A temporary postoperative facial nerve palsy occurred in 2 patients and permanent postoperative vocal cord palsy in 3 patients. During long term follow-up, one patient experienced regrowth of the residual tumor. No tumor progress was observed in both patients treated with radiation therapy. Outcome assessed by Karnofsky scale showed 100% functionality in 12 patients and 90% in 5 patients.

Discussion: Surgical treatment of TJP after endovascular embolization is the treatment of choice in young and healthy patients. In older patients with premorbid conditions, radiation therapy is the main treatment option and is associated with high tumor control rates. Precise preoperative staging together with individualize risk-benefit assessment and interdisciplinary treatment strategy are essential for a favorable outcome.

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