Extrakranielle Schwannome im Kopf-Hals-Bereich: Klinische Implikationen anhand von 20 Fällen

Zusammenfassung

Hintergrund Schwannome sind seltene, benigne Tumore, die von den Gliazellen des peripheren Nervensystems (Schwannsche Zellen) ausgehen. Während es für intrakranielle Schwannome, insbesondere die des N. vestibularis, etablierte Therapiestrategien gibt, liegen zu den extrakraniellen Schwannomen weniger belastbare Daten vor.

Methode Es wurde eine retrospektive Analyse von 20 Patienten mit histopathologisch gesicherten extrakraniellen Schwannomen im Kopf-Hals-Bereich mit einem Schwerpunkt auf Tumorlokalisation und postoperativem funktionellem Therapieergebnis durchgeführt.

Ergebnisse Die Schwannome im vorliegenden Kollektiv entstammten vornehmlich dem N. facialis (n = 4), dem N. vagus (n = 4) oder dem sympathischen Grenzstrang (n = 3). Die häufigsten Primärsymptome waren Schwellungen (n = 12) und Schmerzen (n = 3). MRT (n = 13), Ultraschall (n = 12) und CT (n = 3) wurden zur Diagnostik eingesetzt. In 18 Fällen wurde ein chirurgischer Behandlungsansatz gewählt (n = 14 komplette Exstirpation, n = 3 Teilexstirpation, n = 1 unbekannter Resektionsumfang). Komplette Schwannomexstirpationen von motorisch-efferentenNerven (n = 10) machten ein Absetzen des Nervens häufiger erforderlich, wenn bereits präoperativ ein motorisches Defizit bestand (3 von 4 = 75 % bei präoperativem Defizit vs. 2 von 6 = 33 % ohne präoperatives Defizit).

Schlussfolgerung Extrakranielle Schwannome stellen seltene, meist vom N. facialis, dem sympathischen Grenzstrang oder den kaudalen Hirnnerven ausgehende, benigne Tumore des HNO-Trakts dar, die in der Regel chirurgisch therapiert werden. Mit postoperativen motorischen Defiziten bei kompletten Exstirpationen muss insbesondere bei bereits präoperativ bestehenden Funktionsstörungen gerechnet werden.

Abstract

Background Schwannomas are rare benign tumors originating from the perineural cells forming the myelin layer in the peripheral nervous system (PNS). While well established therapeutic concepts exist for intracranial schwannomas, there is a lack of consistent clinical standards for extracranial schwannomas.

Method This retrospective study describes the clinical pathway of 20 patients with histologically proven extracranial schwannomas of the head and neck. The diagnostic and therapeutic strategies for schwannomas are discussed with special emphasis on localization and functional outcome.

Results Extracranial schwannomas of the head and neck region mostly originated from the facial nerve (n = 4), vagal nerve (n = 4) or sympathetic chain (n = 3). Most common symptoms were swelling (n = 12) and pain (n = 3). Preoperative imaging included MRI (n = 13), ultrasound (n = 12) and CT (n = 3). Surgical intervention was performed in 18 cases (n = 14 complete extirpation, n = 3 partial extirpation, n = 1 unknown). Regarding completely extirpated schwannomas of motor nerves (n = 10) severing the nerve of origin was more often required in patients with a preexisting functional deficit (3 out of 4 = 75 %) than in patients without preexisting deficits (2 out of 6 = 33 %).

Conclusion Representing rare tumors of the head and neck region mostly originating from the facial nerve, sympathetic chain or caudal cranial nerves extracranial schwannomas require a systematic diagnostic and therapeutic approach. Postoperative functional deficits after complete extirpation must especially be anticipated in patients with a preexisting functional deficit.

Schlüsselwörter

Extrakranielle Schwannome - Lokalisation - Ursprungsnerv - Exstirpation - Funktionelles Therapieergebnis

Key words

Extracranial schwannomas - localization - functional outcome

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