%0 Journal Article
%T Merkel Cell Carcinoma: Interdisciplinary Management of a Rare Disease
%A Sven Schneider
%A Dietmar Thurnher
%A Boban M. Erovic
%J Journal of Skin Cancer
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/189342
%X Background. The goal of this paper is to review contemporary multidisciplinary treatment with reference to Merkel cell carcinoma. Management of this rare but highly aggressive skin cancer is a complex undertaking that necessitates an understanding of its etiology, epidemiology, clinical presentation, and the coordinated work of several clinical specializations. Recent Findings. The contemporary literature employs a multidisciplinary approach to achieve the best patient's treatment. Conclusion. This paper presents an algorithm for contemporary management for the rare and aggressive Merkel cell carcinoma. Multidisciplinary approach in a tumor center provides high-quality care for patients with Merkel cell carcinoma. 1. Introduction Merkel cell carcinoma (MCC) is a rare neuroendocrine skin tumor, with a high risk of local and distant spread. The incidence of MCC is 0.32 per 100.000 [1], showing an increasing incidence with advanced age and in male Caucasians [2]. Progression in incidence might be linked to the constantly increasing exposure to ultraviolet B radiation considering the fact that MCCs are localized frequently in sun-exposed areas of the body. Other known risk factors are immunosuppression in transplant recipients [3], HIV [4], and in particular Merkel cell carcinoma polyomavirus infection [5]. The head and neck area is the most frequently affected site (29每40.6%), followed by extremities (21每38%), trunk (7每23%), and unknown primary sites (3.4每12%) [6每8]. Unfortunately, clinical appearance of MCC is heterogeneous. It frequently presents as an asymptomatic, reddish, bluish, or purple tumor of the skin. Size at the time of first consultation is usually smaller than 2ˋcm, although MCC is characterized by rapid growth [9]. Due to the long list of, mostly, benign skin tumors, diagnosis based on clinical parameters is challenging. A recently performed study showed that in 56% of patients with MCC a benign tumor was initially presumed, mirroring the problems in clinical examination and challenges in clinical diagnosis [8]. However, diagnosis is finally achieved by histopathological analysis of small biopsies or samples of totally excised tumor. Additionally, people＊s awareness of this disease is very low compared to malignant melanoma. This suggestion might be supported by the observation that most patients are seen with an advanced stage of disease. The 5-year survival rate ranges from 30 to 64% [6, 10], although survival is strongly dependent on the presence of regional and distant metastasis with a far worse outcome in advanced stages of disease.
%U http://www.hindawi.com/journals/jsc/2013/189342/