nach oben

Wiener Medizinische Wochenschrift

Erschienen in:

01.08.2013 | main topic

Merkelzellkarzinom: eine Überprüfung und Aktualisierung über die Pathogenese, Diagnose und Therapieansätze

verfasst von: José Carlos Cardoso, MD, Vera Teixeira, MD, Assoc. Prof. Georgi Tchernev, Uwe Wollina, PhD

Erschienen in: Wiener Medizinische Wochenschrift | Ausgabe 15-16/2013

Einloggen, um Zugang zu erhalten

Zusammenfassung

Das Merkelzellkarzinom (MCC) oder primäres kutanes neuroendokrines Karzinom ist ein relativ seltener Hautkrebs. Es handelt sich um eine aggressive Neubildung mit einer Neigung zu lokalen Rezidiven, Lymphknoten- und Fernmetastasen. Seine Inzidenz ist in den letzten zwei Jahrzehnten angestiegen.

Die Entdeckung eines neuen Polyomavirus (MCPyV) bei zahlreichen MCC hat unser Pathogeneseverständnis deutlich beeinflusst.

Es herrscht Konsens darüber, dass ein entsprechendes Staging einschließlich der Sentinel-Lymphknotenbiopsie für eine adäquate Therapieplanung unbedingte Vorraussetzung ist. In der Behandlung kommen die aggressive Tumorchirurgie des primären MCC und der regionalen Lymphknotenstation (bei positivem Sentinel) in Kombination mit einer adjuvanten Strahlentherapie zum Einsatz. Die Chemotherapie ist für gewöhnlich dem Stadium der Fernmetastasierung vorbehalten und rein palliativer Natur.

In der vorliegenden Arbeit geben wir eine Übersicht zur Kenntnis des MCC mit Betonung auf die neueren Befunde zur Pathogenese sowie zu aktuellen therapeutischen Empfehlungen.

Vorheriger Artikel Malignant melanoma: epidemiologic aspects, diagnostic and therapeutic approach

Nächster Artikel Subungual acrolentiginous amelanotic melanoma treated with amputation of the distal and middle phalanges 

Toker C. Trabecular carcinoma of the skin. Arch Dermatol. 1972;105:107.PubMedCrossRef

Muñoz IP, Masferrer JP, Vegas JO, Montalvo MSM, Dàaz RJ, Casa AMP. Merkel cell carcinoma from 2008 to 2012: reaching a new level of understanding. Cancer Treat Rev. 2013;39:421–9.

Feng H, Shuda M, Chang Y, Moore PS. Clonal integration of a polyomavirus in human Merkel cell carcinoma. Science. 2008;319:1096–100.PubMedCrossRef

Nicolaidou E, Mikrova A, Antoniou C, Katsambas AD. Advances in Merkel cell carcinoma pathogenesis and management: a recently discovered virus, a new international consensus staging system and new diagnostic codes. Br J Dermatol. 2012;166:16–21.PubMedCrossRef

Miller RW, Rabkin CS. Merkel cell carcinoma and melanoma: etiological similarities and differences. Cancer Epidemiol Biomarkers Prev. 1999;8:153–8.PubMed

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;58:375–81.PubMedCrossRef

Calonje E, Brenn T, Lazar A, McKee PH. Tumours of the surface epithelium. In: Calonje E, Brenn T, Lazar A, McKee PH, Eds. McKee’s Pathology of the Skin with Clinical Correlations. 4th Ed. Toronto: Elsevier; 2012. pp. 1076–149.CrossRef

Engels EA, Frisch M, Goedert JJ, et al. Merkel cell carcinoma and HIV infection. Lancet. 2002;359:497–8.PubMedCrossRef

Penn I, First MR. Merkel’s cell carcinoma in organ recipients: report of 41 cases. Transplantation. 1999;68:1717–21.PubMedCrossRef

10.

Varga E, Kiss M, Szabo K, et al. Detection of Merkel cell polyomavirus DNA in Merkel cell carcinomas. Br J Dermatol. 2009;161:930–2.PubMedCrossRef

11.

Becker JC, Houben R, Ugurel S, et al. MC polyomavirus is frequently present in Merkel cell carcinoma of European patients. J Invest Dermatol. 2009;129:248–50.PubMedCrossRef

12.

Touz A, Gaitan J, Maruani A, et al. Merkel cell polyomavirus strains in patients with Merkel cell carcinoma. Emerg Infect Dis. 2009;15:960–2.CrossRef

13.

Helmbold P, Lahtz C, Enk A, et al. Frequent occurrence of RASSF1 promoter hypermethylation and Merkel cell polyomavirus in Merkel cell carcinoma. Mol Carcinog. 2009;48:903–9.PubMedCrossRef

14.

Duncavage EJ, Zehnbauer BA, Pfeifer JD. Prevalence of polyomavirus in Merkel cell carcinoma. Mod Pathol. 2009;22:1–6.CrossRef

15.

Andres C, Belloni B, Puchta U, et al. Prevalence of MCPyV in Merkel cell carcinoma and non-MCC tumours. J Cutan Pathol. 2010;37:28–34.PubMedCrossRef

16.

Sastre-Garau X, Peter M, Avril MF, et al. Merkel cell carcinoma of the skin: pathological and molecular evidence for a causative role of MCV in oncogenesis. J Pathol. 2009;218:48–56.PubMedCrossRef

17.

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;101:938–45.PubMedCrossRef

18.

Busam KJ, Jungbluth AA, Rekthman N, et al. Merkel cell polyomavirus expression in Merkel cell carcinomas and its absence in combined tumours and pulmonary neuroendocrine carcinomas. Am J Surg Pathol. 2009;33:1378–85.PubMedCrossRef

19.

Paik JY, Hall G, Clarkson A, et al. Immunohistochemistry for Merkel cell polyomavirus is highly specific but not sensitive for the diagnosis of Merkel cell carcinoma in the Australian population. Hum Pathol. 2011;42:1385–90.PubMedCrossRef

20.

Kuwamoto S, Higaki H, Kanai K, et al. Association of Merkel cell polyomavirus infection with morphologic differences in Merkel cell carcinoma. Hum Pathol. 2011;42:632–40.PubMedCrossRef

21.

Martin B, Poblet E, Rios JJ, et al. Merkel cell carcinoma with divergent differentiation: histopathological and immunohistochemical study with PCR analysis for Merkel cell polyomavirus. Histopathol. 2013;62:711–22.CrossRef

22.

Schrama D, Peitsch WK, Zapatka M, et al. Merkel cell polyomavirus status is not associated with clinical course of Merkel cell carcinoma. J Invest Dermatol. 2011;131:1631–8.PubMedCrossRef

23.

Handschel J, Muller D, Depprich RA, et al. The new polyomavirus (MCPyV) does not affect the clinical course in Mccs. J Oral Maxillofac Surg. 2010;39:1086–90.CrossRef

24.

Hodgson NC. Merkel cell carcinoma: changing incidence trends. J Surg Oncol. 2005;89:1–4.PubMedCrossRef

25.

Agelli M, Clegg LX. Epidemiology of primary Merkel cell carcinoma in the United States. J Am Acad Dermatol. 2003;49:832–41.PubMedCrossRef

26.

Yom SS, Rosenthal DI, Adel K, et al. Merkel cell carcinoma of the tongue and head and neck oral mucosal sites. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006;101:761–8.PubMedCrossRef

27.

Coleman NM, Smith-Zagone MJ, Tanyi J, et al. Primary neuroendocrine carcinoma of the vagina with Merkel cell carcinoma phenotype. Am J Surg Pathol. 2006;30:405–10.PubMed

28.

Goessling W, McKee PH, Mayer RJ. Merkel cell carcinoma. J Clin Oncol. 2002;20:588–98.PubMedCrossRef

29.

Saeb-lima M, Montante-Montes de Oca D, Albores-Saavedra J. Merkel cell carcinoma with eccrine differentiation: a clinicopathologic study of 7 cases. Ann Diagn Pathol. 2008;12:410–4.PubMedCrossRef

30.

Iacocca MV, Abernethy JL, Stefanato CM, et al. Mixed Merkel cell carcinoma and squamous cell carcinoma of the skin. J Am Acad Dermatol. 1998;39:582–7.CrossRef

31.

Fernandez-Figueras MT, Puig L, Gilaberte M, et al. Merkel cell (primary neuroendocrine) carcinoma of the skin with nodal metastasis showing rhabdomyosarcomatous differentiation. J Cutan Pathol. 2002;29:619–22.PubMedCrossRef

32.

Adhikari LA, McCalmont TH, Folpe AL. Heterologous rhabdomyoblastic differentiation: the role of immunohistochemistry for Merkel cell polyomavirus large T-antigen in confirmation. J Cutan Pathol. 2012;39:47–51.PubMedCrossRef

33.

Boutilier R, Desormeau L, Cragg F, et al. Merkel cell carcinoma. Squamous and atypical fibroxanthoma-like differentiation in successive local tumour recurrences. Am J Dermatopathol. 2001;23:46–9.PubMedCrossRef

34.

Covello R, Licci S, Ferrari A, et al. Merkel cell carcinoma of the thumb with squamous and leiomyosarcomatous differentiation. Eur J Dermatol. 2010;20:529–30.PubMed

35.

Vanchinathan V, Marinelli EC, Kartha RV, et al. A malignant cutaneous neuroendocrine tumour with features of Merkel cell carcinoma and differentiating neuroblastoma. Am J Dermatopathol. 2009;31:193–6.PubMedCrossRef

36.

Lemos BD, Storer BE, Iyer JG, et al. Pathologic nodal evaluation improves prognostic accuracy in Merkel cell carcinoma: analysis of 5,823 cases as the basis of the first consensus staging system. J Am Acad Dermatol. 2010;63:751–61.PubMedCrossRef

37.

Andea AA, Coit DG, Amin B, Busam KJ. Merkel cell carcinoma: histologic features and prognosis. Cancer. 2008;113:2549–58.PubMedCrossRef

38.

Bobos M, Hytiroglou P, Kostopoulos I, et al. Immunohistochemical distinction between Merkel cell carcinoma and small cell carcinoma of the lung. Am J Dermatopathol. 2006;28:99–104.PubMedCrossRef

39.

40.

Edge SB, Byrd DR, Compton CC, Fritz AG, Greene FL, Trotti A. AJCC Cancer Staging Manual. 7th Ed. New York: Springer; 2010. pp. 299–344.

41.

McCardle TW, Sondak VK, Zager J, Messina JL. Merkel cell carcinoma: pathologic findings and prognostic factors. Curr Probl Cancer. 2010;34:47–64.PubMedCrossRef

42.

Su LD, Lowe L, Bradford CR, et al. Immunostaining for cytokeratin 20 improves detection of micrometastatic Merkel cell carcinoma in sentinel lymph nodes. J Am Acad Dermatol. 2002;46:661–6.PubMedCrossRef

43.

National Comprehensive Cancer Network. Clinical Practice Guidelines in Oncology. Merkel Cell Carcinoma. v.1. Fort Washington, PA: NCCN, 2010.

44.

Concannon R, Larcos GS, Veness M. The impact of (18) F-FDG PET-CT scanning for staging and management of Merkel cell carcinoma: results from Westmead Hospital, Sydney, Australia. J Am Acad Dermatol. 2010;62:76–84.PubMedCrossRef

45.

Anderson S, Beer K, Banic A, et al. MRI of Merkel cell carcinoma: histologic correlation and review of the literature. AJR Am J Roentgenol. 2005;185:1441–8.PubMedCrossRef

46.

Trantola TI, Vallow LA, Halyard MY, et al. Unknown primary Merkel cell carcinoma: 23 new cases and a review. J Am Acad Dermatol. 2013;68:433–40.CrossRef

47.

Medina-Franco H, Urist MM, Fiveash J, et al. Multi-modality treatment of Merkel cell carcinoma: case series and literature review of 1,024 cases. Ann Surg Oncol. 2001;8:204–8.PubMedCrossRef

48.

MJ O, Tanabe KK, Gadd MA, et al. Multi-modality management of Merkel cell carcinoma. Arch Surg. 1999;134:388–92.CrossRef

49.

Poulsen M. Merkel cell carcinoma of skin: diagnosis and management strategies. Drugs Aging. 2005;22:219–29.PubMedCrossRef

50.

Kokoska ER, Kokoska MS, Collins BT, Stapleton DR, Wade TP. Early aggressive treatment for Merkel cell carcinoma improves outcome. Am J Surg. 1997;174:688–93.PubMedCrossRef

51.

Haag ML, Glass LF, Fenske NA. Merkel cell carcinoma. Diagnosis and treatment. Dermatol Surg. 1995;21:669–83.PubMed

52.

Balkissoon J, Rasgon BM, Schweitzer L. Lymphatic mapping for staging of head and neck cancer. Semin Oncol. 2004;31:382–93.PubMedCrossRef

53.

Schmalbach CE, Lowe L, Teknos TN, et al. Reliability of sentinel lymph node biopsy for regional staging of head and neck Merkel cell carcinoma. Arch Otolaryngol Head Neck Surg. 2005;131:610–4.PubMedCrossRef

54.

Lewis KG, Weinstock MA, Weaver AL, Otloey CC. Adjuvant local irradiation for Merkel cell carcinoma. Arch Dermatol. 2006;142:693–700.PubMedCrossRef

55.

Jabbour J, Cumming R, Scolyer RA, et al. Merkel cell carcinoma: assessing the effect of wide local excision, lymph node dissection, and radiotherapy on recurrence and survival in early-stage disease—results from a review of 82 consecutive cases diagnosed between 1992 and 2004. Ann Surg Oncol. 2007;14:1943–52.PubMedCrossRef

56.

Mojica P, Smith D, Ellenhorn JDI. Adjuvant radiation therapy is associated with improved survival in Merkel cell carcinoma of the skin. J Clin Oncol. 2007;25:1043–7.PubMedCrossRef

57.

Guler-Nizam E, Leiter U, Metzier G, et al. Clinical course and prognostic factors of Merkel cell carcinoma of the skin. Br J Dermatol. 2009;161:90–4.PubMedCrossRef

58.

Veness MJ, Morgan GJ, Gebski V. Adjuvant locoregional radiotherapy as best practice in patients with Merkel cell carcinoma of the head and neck. Head Neck. 2005;27:208–16.PubMedCrossRef

59.

Garneski KM, Nghiem P. Merkel cell carcinoma adjuvant therapy: current data support radiation but not chemotherapy. J Am Acad Dermatol. 2007;57:166–9.PubMedCrossRef

60.

Voog E, Biron P, Martin JP, Blay JY. Chemotherapy for patients with locally advanced or metastatic Merkel cell carcinoma. Cancer. 1999;85:2589–95.PubMedCrossRef

61.

Tai PT, Yu E, Winquist E, et al. Chemotherapy in neuroendocrine/Merkel cell carcinoma of the skin: case series and review of 204 cases. J Clin Oncol. 2000;18:2493–9.PubMed

62.

Poulsen MG, Rischin D, Porter I, et al. Does chemotherapy improve survival in high-risk stage I and II Merkel cell carcinoma of the skin? Int J Radiat Oncol Biol Phys. 2006;64:114–9.PubMedCrossRef

Titel: Merkelzellkarzinom: eine Überprüfung und Aktualisierung über die Pathogenese, Diagnose und Therapieansätze
verfasst von: José Carlos Cardoso, MD
Vera Teixeira, MD
Assoc. Prof. Georgi Tchernev
Uwe Wollina, PhD
Publikationsdatum: 01.08.2013
Verlag: Springer Vienna
Erschienen in: Wiener Medizinische Wochenschrift / Ausgabe 15-16/2013
Print ISSN: 0043-5341
Elektronische ISSN: 1563-258X
DOI: https://doi.org/10.1007/s10354-013-0218-0

Springer Medizin Österreich

Zusammenfassung

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 15-16/2013

Malignant melanoma: epidemiologic aspects, diagnostic and therapeutic approach

Advanced basal cell carcinoma

Cutaneous side effects of vemurafenib: a case report and discussion

Cetuximab for treatment of advanced squamous cell carcinoma in solid organ transplant recipients

Atypical fibroxanthoma—a diagnosis of exclusion!

Nachruf Prof. Dr. Walter H. Hörl 11.7.1945 – 25.6.2013