Zusammenfassung
Das Merkel-Zell-Karzinom ist eines der aggressivsten Malignome der Haut mit einer hohen Rate an Lokal-, regionären Lymphknoten- und Fernmetastasen. Der Tumor ist zumeist gekennzeichnet durch eine rötlich livide Farbe mit unauffälliger Epidermis und ein rasches, schmerzloses Wachstum mit einer eisbergartigen Verbreiterung zur Tiefe. Am Stamm oder gluteal können Merkel-Zell-Karzinome aber auch als tief liegende, nur palpatorisch erfassbare Knoten in Erscheinung treten. Die klinische Differenzialdiagnose umfasst in erster Linie Hautmetastasen, maligne Lymphome, maligne Adnextumoren und bei tiefer Lokalisation (z. B. gluteal) auch Zysten. Die Diagnose wird histologisch und immunhistologisch gesichert. Dabei kommt dem Nachweis von Zytokeratin 20 in dem typischen globulären Verteilungsmuster eine besondere Bedeutung zu. Aufgrund seines seltenen Vorkommens wird das Merkel-Zell-Karzinom trotz seines recht charakteristischen klinischen Bildes nur selten primär diagnostiziert. Im Anschluss an die Exzision mit 3 cm Sicherheitsabstand ist nach dem derzeitigen Kenntnisstand eine postoperative Bestrahlung des Operationsgebiets und der abfließenden Lymphwege zu empfehlen. Auch über den Vorteil einer gleichzeitigen Exzision des ersten drainierenden Lymphknotens gibt es mittlerweile Berichte. Bei Fernmetastasen bleibt neben der möglichen Operation und Bestrahlung oft nur eine palliative Chemotherapie. Aufgrund der hohen Rezidivrate des Merkel-Zell-Karzinoms wird besonders im 1. Jahr eine engmaschige Nachsorge im Abstand von 4–6 Wochen empfohlen.
Abstract
Merkel cell carcinomas are rare tumors of the skin with an aggressive behavior and frequent regional and distant metastases. Typically, the primary is a fast-growing, painless, reddish nodule with an iceberg-like effect, broadening in the depth. On the trunk and the buttocks, deep clinically rather inconspicuous nodules can occur. The clinical differential diagnosis of the Merkel cell carcinoma includes skin metastases, malignant lymphomas, malignant adnexal tumors and cysts when the tumor is located deep in the soft tissue (e.g. on the buttocks). Histological and immunohistochemical analysis is necessary for the diagnosis. The demonstration of cytokeratin 20 in the typical globular distribution pattern is of main importance in the diagnosis of Merkel cell carcinoma. Because they are very rare, Merkel cell carcinomas are infrequently diagnosed clinically, in spite of the rather characteristic picture. Diagnostic excision with a safety margin of 3 cm is recommended followed by an adjuvant radiotherapy. The radiation field should include the area of the draining vessels and the first regional lymph nodes. There are some reports concerning the advantage of sentinel lymph node biopsy. In distant metastases, the therapy is multimodal and palliative including surgery, radiation and chemotherapy. Because of the high incidence of regional and distant metastases, regular follow-up is important.
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Khan Durani, B., Hartschuh, W. Merkel-Zell-Karzinom. Hautarzt 54, 1171–1176 (2003). https://doi.org/10.1007/s00105-003-0633-z
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DOI: https://doi.org/10.1007/s00105-003-0633-z