title: Merkel cell carcinoma: emerging biology, current approaches, and future directions.
abstract: Merkel cell carcinoma (MCC) is an aggressive neuroendocrine cutaneous cancer that predominantly occurs in patients who are older, and is associated with a high rate of distant failure and mortality. Current management strategies that incorporate surgery and radiotherapy achieve high rates of locoregional control, but distant failure rates remain problematic, highlighting the need for new effective systemic therapies. Chemotherapy can achieve high response rates of limited duration in the metastatic setting, but its role in definitive management remains unproven. Recent developments in our knowledge about the biology of MCC have led to the identification of new potential therapeutic targets and treatments. A key finding has been the discovery that a human polyomavirus may be a causative agent. However, emerging data suggests that MCC may actually be two distinct entities, viral-associated and viral-negative MCC, which is likely to have implications for the management of MCC in the future and for the development of new treatments. In this review, we discuss recent discoveries about the biology of MCC, current approaches to management, and new therapeutic strategies that are being investigated.
authors.affiliation: From the Division of Research, Peter MacCallum Cancer Centre, and the Sir Peter MacCallum Department of Oncology, University of Melbourne, Melbourne, Australia; Department of Radiation Oncology and Skin and Melanoma Tumour Stream, Peter MacCallum Cancer Centre, and the Department of Pathology, University of Melbourne, Melbourne, Australia; Division of Cancer Medicine, and Head and Neck Tumour Stream, Peter MacCallum Cancer Centre, the Sir Peter MacCallum Department of Oncology and Department of Medicine, University of Melbourne, Melbourne, Australia.
authors.forename: Richard
authors.initials: R
authors.lastname: Tothill
authors.affiliation: From the Division of Research, Peter MacCallum Cancer Centre, and the Sir Peter MacCallum Department of Oncology, University of Melbourne, Melbourne, Australia; Department of Radiation Oncology and Skin and Melanoma Tumour Stream, Peter MacCallum Cancer Centre, and the Department of Pathology, University of Melbourne, Melbourne, Australia; Division of Cancer Medicine, and Head and Neck Tumour Stream, Peter MacCallum Cancer Centre, the Sir Peter MacCallum Department of Oncology and Department of Medicine, University of Melbourne, Melbourne, Australia.
authors.forename: Vanessa
authors.initials: V
authors.lastname: Estall
authors.affiliation: From the Division of Research, Peter MacCallum Cancer Centre, and the Sir Peter MacCallum Department of Oncology, University of Melbourne, Melbourne, Australia; Department of Radiation Oncology and Skin and Melanoma Tumour Stream, Peter MacCallum Cancer Centre, and the Department of Pathology, University of Melbourne, Melbourne, Australia; Division of Cancer Medicine, and Head and Neck Tumour Stream, Peter MacCallum Cancer Centre, the Sir Peter MacCallum Department of Oncology and Department of Medicine, University of Melbourne, Melbourne, Australia.
authors.forename: Danny
authors.initials: D
authors.lastname: Rischin
mesh_terms: D000368:Aged; D015266:Carcinoma, Merkel Cell / Q000473:pathology / Q000628:therapy*; D003131:Combined Modality Therapy / Q000379:methods / Q000639:trends; D005260:Female; D005544:Forecasting; D006801:Humans; D007167:Immunotherapy / Q000379:methods / Q000639:trends; D008207:Lymphatic Metastasis; D008297:Male; D058990:Molecular Targeted Therapy / Q000379:methods / Q000639:trends; D009362:Neoplasm Metastasis; D009367:Neoplasm Staging; D027601:Polyomavirus Infections / Q000150:complications; D049268:Positron-Emission Tomography / Q000379:methods; D018714:Radiotherapy, Adjuvant / Q000379:methods / Q000639:trends; D021701:Sentinel Lymph Node Biopsy; D012878:Skin Neoplasms / Q000473:pathology / Q000628:therapy*; D035703:Therapies, Investigational / Q000379:methods / Q000639:trends; D014412:Tumor Virus Infections / Q000150:complications