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Maligne Speicheldrüsentumoren

Malignant salivary gland tumors

  • Leitthema
  • Published:
Der MKG-Chirurg Aims and scope

Zusammenfassung

Die malignen Speicheldrüsentumoren sind eine extrem heterogene Gruppe seltener Tumoren mit großer klinischer, pathologischer und molekularbiologischer Variabilität. Die chirurgische Resektion, oftmals mit adjuvanter Radio(Chemo)-Therapie, stellt die Behandlung der Wahl dar, während herkömmliche Chemotherapeutika kaum eine Wirksamkeit besitzen. Im Fokus der aktuellen Forschung steht die histologische und die molekularbiologische Charakterisierung der Tumoren. Neue Entitäten wie das „mammary analogue secretory carcinoma“ wurden beschrieben; darüber hinaus wurde der Begriff der High-Grade-Transformation mit Verlust der tumorspezifischen histologischen Merkmale geprägt. Ätiopathogenetisch zeigen verschiedene Subtypen wie z. B. das adenoidzystische Karzinom oder das Mukoepidermoidkarzinom spezifische chromosomale Aberrationen, die mit der Bildung von sog. Fusionsgenen einhergehen und für die Ausbildung sowie das Wachstum der entsprechenden Tumoren verantwortlich gemacht werden. Die zunehmende Erkenntnis dieser genomischen Alterationen und tumorspezifischen Signalwege eröffnet neue vielversprechende Möglichkeiten zielgerichteter molekularer Therapien im fortgeschrittenen Tumorstadium.

Abstract

Malignant salivary gland tumors represent a heterogeneous group of rare tumors with distinct clinical, pathological and genetic features. Surgical resection often with adjuvant radio(chemo)therapy remains the first option of treatment while conventional chemotherapy has very little effect. Recent investigations focused on new insights into the genetic and histological characterization of tumors. For example new entities, such as mammary analogue secretory carcinoma were described. Moreover, high-grade transformations with dedifferentiation of the original tumor-specific malignant histology were found in several tumor types implicating aggressive tumor growth and worse prognosis. Etiopathogenetically, various subtypes, such as adenoid cystic carcinoma and mucoepidermoid carcinoma have specific chromosomal aberrations with formation of fusion genes which are responsible for driving cancer growth. Increasing knowledge of genomic alterations and tumor-specific signaling pathways provide promising evidence for molecularly targeted therapies in advanced tumor stages.

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  1. Klinik und Poliklinik für MKG-Chirurgie, Universitätsklinikum Regensburg, Franz-Josef-Strauß-Allee 11, 93053, Regensburg, Deutschland

    T. Ettl

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Ettl, T. Maligne Speicheldrüsentumoren. MKG-Chirurg 8, 151–166 (2015). https://doi.org/10.1007/s12285-015-0016-3

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