Das Mukoepidermoidkarzinom – Bedeutung der Molekularpathologie

Zusammenfassung

Das Mukoepidermoidkarzinom ist das häufigste primäre Speicheldrüsenmalignom im Kopf-Hals-Bereich. Das Tumorgrading hat eine erhebliche prognostische Bedeutung. Die 5-Jahres-Überlebensrate ist für Low-grade-Mukoepidermoidkarzinome deutlich höher als für Intermediate-grade- oder High-grade-Mukoepidermoidkarzinome. Für das Mukoepidermoidkarzinom scheint die Translokation von t(11;19)(q21;p13) mit der resultierenden CRTC1-MAML2-Transfusion ebenfalls von prognostischer Relevanz zu sein. Diese Translokation ist in 38–82 % aller Mukoepidermoidkarzinome nachweisbar. In Studien konnte für Patienten mit CRTC1-MAML2-positiven Mukoepidermoidkarzinomen eine deutlich bessere Prognose als für Patienten mit Translokations-negativen Mukoepidermoidkarzinomen nachgewiesen werden. Die t(11;19)(q21;p13) -Translokation wird deutlich häufiger bei Low- und Intermediate-grade-Mukoepidermoidkarzinomen detektiert als bei High-grade-Tumoren derselben Entität. Darüber hinaus wurden Translokations-positive Mukoepidermoidkarzinome häufiger bei jüngeren Patienten, kleineren Tumoren und niedrigeren Tumorstadien diagnostiziert sowie seltener Lymphknoten- und Fernmetastasierung gefunden. Bislang ist die Translokation nicht von therapeutischer Bedeutung. Jedoch sollte aufgrund der prognostischen Relevanz eine Diagnostik regelhaft erfolgen. Bei Grenzfällen kann der fehlende Nachweis der Translokation eine Entscheidungshilfe zu einer Therapieeskalation geben. Um den individuellen prognostischen und therapeutischen Wert des Fusionsgens genauer zu beurteilen, werden weitere Untersuchungen notwendig sein.

Abstract

Mucoepidermoid carcinoma is the most common primary salivary gland malignancy and its tumor grading has an important prognostic significance. The 5 year overall survival rate is significantly higher for low grade mucoepidermoid carcinomas than for intermediate grade and high grade mucoepidermoid carcinomas. The translocation of t(11;19)(q21;p13) with the resulting CRTC1-MAML2 transfusion appears to be of prognostic relevance in patients with mucoepidermoid carcinoma. The translocation is detectable in 38–82 % of all mucoepidermoid carcinomas. Study results have shown a significantly better prognosis for patients with fusion-positive mucoepidermoid carcinomas than fusion-negative mucoepidermoid carcinomas. The t(11;19)(q21;p13) translocation can be found more often in low and intermediate grade mucoepidermoid carcinomas than in high grade tumors of the same entity. Moreover, fusion positive mucoepidermoid carcinoma were found more frequently in younger patients, smaller tumors, lower tumor stages and less frequently lymph node and distant metastases. Up to now, the translocation has not been of therapeutic importance. In selected cases, the lack of t(11;19)(q21;p13) translocation might facilitate the decision towards further escalation of therapy. More studies will be necessary to evaluate the individual prognostic and therapeutic value of CRTC1-MAML2 transfusion.

Schlüsselwörter

Glandula parotidea - Speicheldrüsenkarzinom - Translokation - Transfusion - CRTC1 - MAML2 - Prognose - Therapie

Key words

salivary gland carcinoma - CRTC1 - MAML2 - transfusion - prognosis - therapy

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