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Langenbecks Archiv für Chirurgie

, Volume 382, Issue 6, pp 349–358 | Cite as

Merkel-Zell-Tumor oder neuroendokrines Hautkarzinom

  • U. Meyer-Pannwitt
  • K. Kummerfeldt
  • P. Boubaris
  • J. Caselitz
Originalarbeit
  • 106 Downloads

Zusammenfassung

Der Merkel-Zell-Tumor ist ein maligner Hauttumor von neuroendokriner Differenzierung. Er befällt vorwiegend ältere Menschen. 78,6% der Patienten sind älter als 59 Jahre. Bis zum 60. Lebensjahr sind beide Geschlechter gleichermaßen betroffen. Ab dem 60. Lebensjahr überwiegt das weibliche Geschlecht. Der Tumor ist am häufigsten im Kopf-Hals-Bereich (50,8%) und an den Extremitäten (33,7%) lokalisiert und hat zum Zeitpunkt der Diagnosestellung eine durchschnittliche Größe von 29 mm. Bei 30% der Patienten liegen bereits bei der Erstvorstellung klinisch positive regionale Lymphknoten vor. Klinisch ist eine definitive Diagnosestellung nicht möglich. Eine lichtmikroskopisch geäußerte Verdachtsdiagnose muß durch immunzytochemische Untersuchungen bestätigt werden. Immunhistologisch werden die Differenzierungsmarker (Intermediärfilamente, speziell Neurofilamente und neuroendokrine Marker) zur Abgrenzung gegen andere Hauttumoren und undifferenzierte Karzinome eingesetzt. Als Primärtherapie wird vorwiegend eine Tumorexzision im Gesunden durchgeführt. Dabei scheint die Kombinationsbehandlung von Tumorexzision und konsekutiver Radiatio auch bei Patienten ohne Befall der regionalen Lymphknoten das Risiko eines Rezidivs oder einer Metastasierung zu senken. Bei Patienten mit ungünstigen prognostischen Indikatoren sollte immer auch eine Strahlenbehandlung durchgeführt werden. Die Ergänzung der chirurgische Primärtherapie (Exzision im Gesunden) durch eine radikale Lymphadenektomie sollte zumindest bei jüngeren Patienten erwogen werden. Eine regelmäßige, engmaschige, langjährige Tumornachsorge ist erforderlich. Treten Fernmetastasen auf, können durch eine Polychemotherapie nur kurzfristige Remissionen erreicht werden. Eine bereits eingetretene Lymphknotenmetastasierung, eine Tumorgröße über 2 cm sowie männliche Geschlechtszugehörigkeit stellen prognostisch ungünstige Indikatoren dar, welche bei der Therapieplanung berücksichtigt werden sollten.

Schlüsselwörter

Merkel-Zell-Karzinom Chirurgie Strahlentherapie Chemotherapie Pathologie Prognose Therapieergebnis Rezidiv 

Merkel cell carcinoma

Abstract

Merkel cell carcinoma is a rare malignant tumor of the skin with predominance in older patients; 78.6% of patients are older than 59 years. Female and male patients are equally involved in the age group below 60 years. After 60 years, Merkel cell carcinomas are more often observed in female patients. The tumor is most often located in the head and neck region (50.8%) or the extremities (33.7%). The average size is 29 mm at presentation. Clinically, only a presumptive diagnosis of Merkel cell carcinoma can be established. The definite diagnosis is made by histological, especially immunohistological methods (detection of intermediate filaments and neuroendocrine markers). The therapy of choice is local excision. Secondary therapy may be a combination of operation and radiation or chemotherapy. Since this combination may reduce the risk of recurrences it should be applied for patients with poor prognostic features. Especially in young patients, additional lymphadenectomy should be discussed. Clinical control is necessary. Distant metastases should be treated by chemotherapy. Bad prognostic features are: lymph node metastasis, size larger than 2 cm, male sex.

Key words

Merkel cell carcinoma Surgery Radiotherapy Chemotherapy Pathology Prognosis Therapeutic outcome Recurrence 

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Copyright information

© Springer-Verlag 1997

Authors and Affiliations

  • U. Meyer-Pannwitt
    • 1
  • K. Kummerfeldt
    • 2
  • P. Boubaris
    • 3
  • J. Caselitz
    • 4
  1. 1.Chirurgische KlinikUniversitäts-Krankenhaus EppendorfHamburg
  2. 2.Frauenklinik, Universitäts-Krankenhaus EppendorfHamburg
  3. 3.Innere AbteilungKreiskrankenhaus StadeStadeGermany
  4. 4.Abteilung für PathologieAllgemeines Krankenhaus AltonaHamburg

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