Zusammenfassung
Das Merkelzellkarzinom (MZK) ist ein seltener, schnell wachsender, hochmaligner dermaler Tumor, der bevorzugt auf lichtexponierter Haut in höherem Lebensalter entsteht. Der Verlauf ist oft durch Ausbildung von Lymphknotenmetastasen und Lokalrezidiven bereits im ersten Jahr nach Entfernung des Primärtumors geprägt. Die 5-Jahres-Überlebensrate liegt auch bei konsequenter Therapie nur bei etwa 65 %. Das histologische Bild ist gekennzeichnet durch trabekuläre Stränge kleiner uniformer Zellen mit großen basophilen Kernen und typischen neuroendokrinen Granula. Die Diagnose wird immunhistochemisch durch neuroendokrine und epitheliale Marker gesichert. Therapie der Wahl ist die Exzision des Primärtumors. Die Indikation zur adjuvanten Strahlentherapie ist fast stets gegeben und sollte auch drainierende Lymphknotenstationen einbeziehen. Beim Vorliegen von Lymphknotenmetastasen sollten diese exzidiert und eine Nachbestrahlung eingeleitet werden. Eine adjuvante Chemotherapie in Anlehnung an das kleinzellige Bronchialkarzinom kann in diesem Stadium erfolgen. Der prognostische Vorteil ist jedoch nicht gesichert. Trotz guten Ansprechens auf Strahlen- und Chemotherapie mit zumindest verlängertem rezidivfreien Intervall ist das MZK im Stadium der Fernmetastasierung derzeit kaum kurabel.
Ein aggressives und gleichwohl individuell abgestimmtes Behandlungskonzept unter Einbeziehung der Strahlentherapie könnte die Prognose für die betroffenen Patienten vor allem in den ersten Erkrankungsstadien in Zukunft wesentlich verbessern.
Summary
Merkel cell carcinoma is a rare, rapidly growing, highly malignant dermal tumor which occurs preferentially on light-exposed skin in advanced age. The course of the disease is frequently characterized by the occurrence of lymph node metastases and local recurrences, even in the first year after removal of the primary tumour. The five-year overall survival rate is only about 65 %, despite rigorous therapy. The histological pattern is characterized by trabecular strands of small, uniform cells with large basophilic nuclei and typical neuroendocrine granules. The diagnosis is confirmed immunohistochemically by neuroendocrine and epithelial markers. The excision of the primary tumor is regarded as first-line therapy. Adjuvant radiatiotherapy is almost always indicated and should also include lymph node drainage. In the stage of nodal disease, a combination of excision and radiotherapy is recommended. Adjuvant chemotherapy can be applied in this stage, as in small-cell bronchial carcinoma. The prognostic advantage has, however, not been proven. Despite good response to radiatiotherapy and chemotherapy, with at least prolonged recurrence-free intervals, Merkel cell carcinoma is rarely curable at the distant metastasizing stage.
Individually defined, aggressive treatment,including radiatiotherapy, may in future considerably improve the prognosis, especially in the early stages of the disease.
Schlüsselwörter
Merkelzellkarzinom - Radiotherapie
Key words
Merkel cell carcinoma - radiotherapy
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Prof. Dr. med. Peter Helmbold
Hautklinik der Ruprecht-Karls-Universität Heidelberg
Voßstraße 2
69115 Heidelberg
Phone: 06221/56 8505
Fax: 06221/56 8563
Email: peter.helmbold@med.uni-heidelberg.de
