Zusammenfassung
Thymome sind selten, repräsentieren jedoch ca. 50 % der Tumoren im vorderen Mediastinum. Es existieren keine typischen Frühsymptome für Thymome, sondern lediglich typische Spätsymptome aufgrund von großen Tumormassen im vorderen Mediastinum. Insgesamt finden sich bei Patienten mit einer Myasthenia gravis in 10 - 15 % der Fälle ein Thymom. Die zwei wichtigsten Klassifikationen im klinischen Alltag sind die Masaoka-Einteilung und die histologische WHO-Klassifikation. Beide sind prognostisch relevant und sollten bei Patienten mit einem Thymom Anwendung finden. Thymome sind immer als maligne anzusehen, eine Metastasierung sowie ein etwaiges Rezidiv nach Resektion finden sich in allen Stadien und sind in ihrer Häufigkeit stadienabhängig. Als einziger weiterer unabhängiger Prognosefaktor hat sich nur die komplette Resektion (R0-Resektion) etabliert. Mehr als 60 % der Patienten sterben an tumor-unabhängigen Ursachen. Die 5-Jahres-Überlebensrate bei Thymomen ist mit etwa 80 % recht hoch, bedeutet jedoch keine Heilung, da die Rezidive im Durchschnitt erst nach 5 Jahren auftreten und die Prognose bestimmen. Die chirurgische Therapie ist als Goldstandard anzusehen und sollte immer angestrebt werden. Der häufigste Zugang ist die mediane Sternotomie. Beim Thymuskarzinom ist die radikale Lymphknotendissektion aus onkologischer Sicht dringend angezeigt. Lediglich für die adjuvante Bestrahlung nach inkompletter Resektion gibt es Hinweise, dass dieses Behandlungskonzept einen Vorteil bringt und die Lokalrezidivrate signifikant senkt. Es existieren keine überzeugenden Daten, die die Anwendung einer adjuvanten Chemotherapie beim Thymom wirklich unterstützen. Generell kann festgehalten werden, dass die alleinige Anwendung der Chemotherapie nur in Einzelfällen erwogen werden sollte. Bei Thymuskarzinomen erscheint eine adjuvante Chemotherapie gerechtfertigt. Durch eine neo-adjuvante Chemotherapie in Kombination mit der Operation und der anschließenden postoperativen Radiochemotherapie kann die Resektabilität in den Stadien III und IV erhöht werden. Dieser Effekt geht mit einem besseren Überleben einher. Bis größere prospektive Studien zu diesem Thema vorliegen, sollte in den Stadien III und IV ein solches multimodales Therapieregime zur Anwendung kommen.
Summary
Thymomas are a rare tumor entity. However, they represent 50 % of all tumors of the anterior mediastinum. There are no specific early symptoms. Overall in 10 - 15 % of patients with myasthenia gravis a thymoma is evident. Two major classifications are relevant in clinical practise: the Masaoka-classification and the WHO-classification. For their clinical and prognostic significance both classifications should be used for patients with thymomas. Additional, only resection status (RO) is known as a significant prognostic factor.
Thymomas are compulsory malignant tumors. Distant metastasis is found as well as local recurrence in all stages of the disease. The 5-year-mortality rate constitutes about 80 %, not meaning any healing because local recurrences occur as late as five years after surgery. 60 % of all patients die from tumor-independent reasons making a clear prognostic statement difficult. Surgical treatment remains the goldstandard and must be performed whenever possible. The most common approach is a median sternotomy. When dealing with a thymuscarcinoma, radical lymph node dissection is advisable. With respect of treatment only adjuvant radiation can possibly improve long term survival and reduces local recurrence rates for incomplete resected patients. There is no evidence for a benefit in patients with thymoma receiving adjuvant chemotherapy. A neo-adjuvant chemotherapy in combination with an adjuvant radiotherapy improves outcome after surgical resection in stage III and IV and goes along with better survival rates. Larger studies have not been performed so far. A multimodal therapy strategy is advised when dealing with thymomas in stage III and IV.
Schlüsselwörter
Thymom - Thymuskarzinom - Prognosefaktoren - Klassifikation - Therapie
Key words
thymoma - thymuscarcinoma - prognostic factor - classification - therapy
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