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DOI: 10.1055/s-0030-1269932
© Georg Thieme Verlag KG Stuttgart · New York
Aktuelles Update zur Tumorbiologie und Behandlung des Merkelzell-Karzinoms
Recent Update on Tumour Biology and Treatment of Merkel Cell CarcinomaPublication History
eingereicht 07.09.2010
akzeptiert 03.12.2010
Publication Date:
12 January 2012 (online)
![](https://www.thieme-connect.de/media/hamipla/201106/lookinside/thumbnails/10.1055-s-0030-1269932-1.jpg)
Zusammenfassung
Das Merkelzell-Karzinom (MCC) geht aus Merkelzellen hervor, die in der Basalschicht der Epidermis lokalisiert sind. Es imponiert am häufigsten im Kopf- und Halsbereich älterer hellhäutiger Patienten und wird als tödlichster Hautkrebs angesehen. Die radikale chirurgische Exzision verbunden mit der pathologischen Beurteilung des gesamten entnommenen Tumorgewebes stellt die empfohlene Behandlungsmethode dar. Frühe MCC-Stadien können durch chirurgische Maßnahmen mit oder ohne postoperative Radiotherapie geheilt werden, fortgeschrittene Stadien gelten hingegen als unheilbar. 2008 wurde ein neues humanes Polyoma-Virus im Genom des MCC Tumors entdeckt, das Merkelzell-Polyoma-Virus (MCPyV). Das MCPyV ist das erste Beispiel eines humanen onkogenen Polyoma-Virus. Die spezifischen Mutationen im viralen Genom und die klonale Integration in das Tumorgenom sind starke Beweise dafür, dass MCPyV kein sekundär infizierender Passagier-Virus ist. Ziel dieses Review-Artikels ist es, diesen seltenen Hautkrebs im Detail zu beschreiben und auf neueste Fortschritte in der Forschung zum MCC aufmerksam zu machen.
Abstract
Merkel cell carcinoma (MCC) is a carcinoma arising from Merkel cells located in the basal layer of the epidermis. The skin of the head and neck is a common site for MCC, occurring generally in fair-skinned elderly patients. MCC is considered as the most lethal skin cancer. Radical surgical excision with pathological verification of complete removal of the tumour is the recommended treatment. Early MCC can be cured by surgery with or without postoperative radiation therapy, whereas advanced MCC is currently considered to be incurable. In the year 2008, a new polyoma virus was found in the tumour genome of the MCC tumours. MCPyV (Merkel cell polyoma virus) appears to be the first example of a human oncogenic polyoma virus. Specific mutations in the viral genome and its clonal integration to the tumour genome are strong evidence against MCPyV as being a passenger virus that secondarily infects MCC tumours. The purpose of this review article is to shed light on this rare skin cancer and introduce the latest advances in research on MCC.
Schlüsselwörter
Merkelzell-Karzinom - Merkelzellen - Polyomavirus - Haut
Key words
Merkel cell carcinoma - Merkel cells - polyoma virus - skin
Literatur
- 1 Koljonen V. Merkel cell carcinoma. World J Surg Oncol. 2006; 4 7
- 2 Halata Z, Grim M, Bauman KI. Friedrich Sigmund Merkel and his “Merkel cell”, morphology, development, and physiology: Review and new results. Anat Rec. 2003; 271A (1) 225-239
- 3 Heath M, Jaimes N, Lemos B. et al . Clinical characteristics of Merkel cell carcinoma at diagnosis in 195 patients: the AEIOU features. J Am Acad Dermatol. 2008; 58 (3) 375-381
- 4 Nakamura T, Katano H. [Merkel cell polyomavirus and Merkel cell carcinoma]. Uirusu. 2009; 59 (1) 37-42
- 5 Landis SH, Murray T, Bolden S. et al . Cancer statistics, 1998. CA Cancer J Clin. 1998; 48 (1) 6-29
- 6 Albores-Saavedra J, Batich K, Chable-Montero F. et al . Merkel cell carcinoma demographics, morphology, and survival based on 3 870 cases: a population based study. J Cutan Pathol. 2009;
- 7 Howard RA, Dores GM, Curtis RE. et al . Merkel cell carcinoma and multiple primary cancers. Cancer Epidemiol Biomarkers Prev. 2006; 15 (8) 1545-1549
- 8 Gupta SG, Wang LC, Penas PF. et al . Sentinel lymph node biopsy for evaluation and treatment of patients with Merkel cell carcinoma: The Dana-Farber experience and meta-analysis of the literature. Arch Dermatol. 2006; 142 (6) 685-690
- 9 Dancey AL, Rayatt SS, Soon C. et al . Merkel cell carcinoma: a report of 34 cases and literature review. J Plast Reconstr Aesthet Surg. 2006; 59 (12) 1294-1299
- 10 Ralston J, Chiriboga L, Nonaka D. MASH1: a useful marker in differentiating pulmonary small cell carcinoma from Merkel cell carcinoma. Mod Pathol. 2008;
- 11 Van Gele M, Boyle GM, Cook AL. et al . Gene-expression profiling reveals distinct expression patterns for Classic versus Variant Merkel cell phenotypes and new classifier genes to distinguish Merkel cell from small-cell lung carcinoma. Oncogene. 2004; 23 (15) 2732-2742
- 12 Anderson SE, Beer KT, Banic A. et al . MRI of Merkel cell carcinoma: histologic correlation and review of the literature. AJR Am J Roentgenol. 2005; 185 (6) 1441-1448
- 13 Fernandez-Figueras MT, Puig L, Musulen E. et al . Expression profiles associated with aggressive behavior in Merkel cell carcinoma. Mod Pathol. 2007; 20 (1) 90-101
- 14 Poulsen M. Merkel-cell carcinoma of the skin. Lancet Oncol. 2004; 5 (10) 593-599
- 15 Andea AA, Coit DG, Amin B. et al . Merkel cell carcinoma: histologic features and prognosis. Cancer. 2008; 113 (9) 2549-2558
- 16 Ng L, Beer TW, Murray K. Vascular density has prognostic value in Merkel cell carcinoma. Am J Dermatopathol. 2008; 30 (5) 442-445
- 17 Beer TW, Ng LB, Murray K. Mast cells have prognostic value in Merkel cell carcinoma. Am J Dermatopathol. 2008; 30 (1) 27-30
- 18 Goldberg SR, Neifeld JP, Frable WJ. Prognostic value of tumor thickness in patients with Merkel cell carcinoma. J Surg Oncol. 2007;
- 19 Sandel HDt, Day T, Richardson MS. et al . Merkel cell carcinoma: does tumor size or depth of invasion correlate with recurrence, metastasis, or patient survival?. Laryngoscope. 2006; 116 (5) 791-795
- 20 Strub B, Weindel S, Witt P. et al . [The interdisciplinary treatment of Merkel cell carcinoma: a retrospective case analysis and review of the current literature]. Zentralbl Chir. 2009; 134 (5) 468-473
- 21 Bajetta E, Celio L, Platania M. et al . Single-Institution Series of Early-Stage Merkel Cell Carcinoma: Long-Term Outcomes in 95 Patients Managed with Surgery Alone. Ann Surg Oncol. 2009;
- 22 Mehrany K, Otley CC, Weening RH. et al . A Meta-analysis of the Prognostic Significance of Sentinel Lymph Node Status in Merkel Cell Carcinoma. Dermatol Surg. 2002; 28 (2) 113-117
- 23 Koljonen V, Suominen S. Sentinel node biopsy in local anaesthesia in patients with head and neck Merkel cell carcinoma. European Journal of Plastic Surgery. 2008; 30 (5) 205-210
- 24 Koljonen V, Tukiainen E, Haglund C. et al . Proliferative activity detected by Ki67 correlates with poor outcome in Merkel cell carcinoma. Histopathology. 2006; 49 (5) 551-553
- 25 Poulsen M, Round C, Keller J. et al . Factors Influencing Relapse-Free Survival in Merkel Cell Carcinoma of the Lower Limb – A Review of 60 Cases. Int J Radiat Oncol Biol Phys. 2009;
- 26 Guihard S, Noel G. [Merkel cell carcinoma, role of radiotherapy and literature review]. Cancer Radiother. 2009; 13 (1) 47-54
- 27 Meier G, Waldherr C, Herrmann R. et al . Successful targeted radiotherapy with 90Y-DOTATOC in a patient with Merkel cell carcinoma. A Case Report. Oncology. 2004; 66 (2) 160-163
- 28 Sihto H, Kukko H, Koljonen V. et al . Clinical factors associated with Merkel cell polyomavirus infection in Merkel cell carcinoma. J Natl Cancer Inst. 2009; 101 (13) 938-945
- 29 Helmbold P, Lahtz C, Herpel E. et al . Frequent hypermethylation of RASSF1A tumour suppressor gene promoter and presence of Merkel cell polyomavirus in small cell lung cancer. Eur J Cancer. 2009;
- 30 Loyo M, Guerrero-Preston R, Brait M. et al . Quantitative detection of Merkel cell virus in human tissues and possible mode of transmission. Int J Cancer. 2009;
- 31 Tolstov YL, Pastrana DV, Feng H. et al . Human Merkel cell polyomavirus infection II. MCV is a common human infection that can be detected by conformational capsid epitope immunoassays. Int J Cancer. 2009;
- 32 Becker JC, Houben R, Ugurel S. et al . MC polyomavirus is frequently present in Merkel cell carcinoma of European patients. J Invest Dermatol. 2009; 129 (1) 248-250
- 33 Fakhry C, Westra WH, Li S. et al . Improved survival of patients with human papillomavirus-positive head and neck squamous cell carcinoma in a prospective clinical trial. J Natl Cancer Inst. 2008; 100 (4) 261-269
Korrespondenzadresse
Prof. Dr. med. LarsSteinstraesser
BG Kliniken Bergmannsheil
Ruhr Universität Bochum
Klinik für Plastische Chirurgie
und Schwerbrandverletzte
Bürkle-de-la-Camp Platz 1
44789 Bochum
Email: lars.steinstraesser@ruhr-uni-bochum.de