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Chirurgische Therapieoptionen bei zervikalen Paragangliomen

Surgical treatment options for cervical paragangliomas

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Zusammenfassung

Die Therapie der seltenen Tumorentität zervikales Paragangliom (PG) durchläuft aktuell einen Paradigmenwechsel. Während bei kleinen Glomus-caroticum-Tumoren, malignen sowie endokrin aktiven Tumoren die chirurgische Resektion die Therapie der Wahl darstellt, sollte eine Operation bei lokal fortgeschrittenen Glomus-caroticum-Tumoren und vagalen PG kritisch diskutiert werden. Aufgrund der unmittelbaren Nähe dieser hypervaskularisierten Tumoren zu den kaudalen Hirnnerven besteht nach einer Resektion insbesondere bei lokal fortgeschrittenen zervikalen PG das Risiko gravierender nervaler Schäden mit einer erheblichen Beeinträchtigung der Lebensqualität, sodass ein restriktives operatives Vorgehen angebracht ist. Die externe Strahlentherapie kann eine gleichwertige primäre Therapieoption hinsichtlich Rezidivfreiheit bei geringerer Morbidität darstellen. Das langsame Tumorwachstum und die Tendenz der hereditären zervikalen PG zur Multifokalität bzw. signifikante Komorbiditäten älterer, asymptomatischer Patienten rechtfertigen ein zunehmend weniger aggressives Vorgehen in der Therapiestrategie dieser Tumoren. Mit dem Wait-and-scan-Management ist eine engmaschige, bildgebende und klinische Reevaluierung verbunden. Die Größe und Lokalisation des Tumors, dessen Wachstumsgeschwindigkeit, Genetik, Patientenalter und Allgemeinzustand sowie therapiebeeinflussende Komorbiditäten, das Vorliegen synchroner PG und/oder vasoaktiver katecholaminproduzierender Tumoren müssen vor dem Therapiebeginn identifiziert und in der multidisziplinären Therapieplanung zwingend berücksichtigt werden. Obwohl Best-practice-Algorithmen zur Therapie der zervikalen PG zur Verfügung stehen, haben die innovativen Entwicklungen der letzten Jahre zu patientenadaptierten, individuellen Therapieansätzen geführt.

Abstract

The therapies available for the rare tumor entity of cervical paraganglioma (PG) are currently undergoing a paradigm shift. The treatment of choice for small carotid body tumors, malignant and active endocrine tumors is surgical resection; however, for locally advanced carotid body tumors and vagal PG, surgical therapy should be critically evaluated. Due to the immediate proximity of these hypervascularized tumors to the caudal cranial nerves, there is a risk of severe nerve damage with a significant impairment of quality of life after resection, particularly for locally advanced cervical PG, emphasizing further the importance of a restrictive surgical strategy. External radiotherapy can provide an equivalent primary therapeutic option with respect to the rate of recurrence and is accompanied by a lower morbidity. The slow rate of tumor progression and the multifocality of the familial variant of cervical PG or significant comorbidities in older, asymptomatic patients warrant a less aggressive treatment strategy for these tumors. When a wait and scan approach is implemented, a closely monitored radiological and clinical re-evaluation is of upmost importance. In a multidisciplinary approach the following critical points require consideration before a therapy is implemented,: size and location of the tumor, progression rate, genetic background, patient age and general condition, relevant comorbidities, the presence of synchronous PG and/or vasoactive catecholamine-producing tumors. Although best practice algorithms for the treatment of cervical PG have already been devised, recent innovative developments have led to more patient-tailored, individualized treatment approaches.

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Authors and Affiliations

  1. Universitätsklinik und Poliklinik für Viszerale, Gefäß und Endokrine Chirurgie, Martin-Luther-Universität Halle-Wittenberg, Ernst-Grube-Str. 40, 06120, Halle/Saale, Deutschland

    R. Schneider, M. Elwerr, K. Lorenz & J. Ukkat

  2. Universitätsklinik und Poliklinik für Hals‑, Nasen‑, Ohrenheilkunde, Kopf- und Halschirurgie, Martin-Luther-Universität, Halle/Saale, Deutschland

    S. Plontke

  3. Sektion Endokrine Chirurgie, Klinik für Allgemeinchirurgie, Viszeral- und Transplantationschirurgie, Universitätsklinikum Essen, Essen, Deutschland

    H. Dralle

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  1. R. Schneider
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Correspondence to R. Schneider.

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Interessenkonflikt

R. Schneider, M. Elwerr, K. Lorenz, S. Plontke, H. Dralle und J. Ukkat geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Schneider, R., Elwerr, M., Lorenz, K. et al. Chirurgische Therapieoptionen bei zervikalen Paragangliomen. Chirurg 90, 29–36 (2019). https://doi.org/10.1007/s00104-018-0734-y

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