Abstract
Merkel cell carcinoma (MCC) is a rare and aggressive cutaneous malignancy. The infectivity of Merkel cell polyomavirus (MCPyV), an apparent agent in MCC development, may be exacerbated with impaired immune responses. This paper reviews relevant data regarding the role of immunosuppression in the development of MCC and describes modes of immunodeficient states. Because of the inherently low incidence rate of MCC, several case studies and series are also briefly mentioned to provide a more comprehensive summary of MCC in the setting of immunosuppression. We describe immunosuppressed patients who have experienced excessive UV radiation, organ transplantation, human immunodeficiency virus infection/AIDS, autoimmune diseases, and lymphoproliferative disorders. Iatrogenic forms of immunosuppression are also highlighted. Studies that quantify risks consistently report that individuals with a history of solid organ transplantation, autoimmune diseases, AIDS, and/or lymphoproliferative diseases have a significantly elevated risk of developing MCC. Overall, immunocompromised patients also appear to have an early onset and more aggressive course of MCC, with poorer outcomes. Recommendations for multidisciplinary approaches are proposed to effectively prevent and manage MCC in these patients.
Highlights
IntroductionMerkel cell carcinoma (MCC) is a rare and highly aggressive neuroendocrine cancer of the skin
Merkel cell carcinoma (MCC) is a rare and highly aggressive neuroendocrine cancer of the skin.MCC is thought to originate from the nerve-associated Merkel cell touch receptors, which are in the layer of basal cells at the deepest portion of the epidermis [1]
Some have suggested modifying or decreasing immunosuppressive regimens as a way to improve the clinical course of post-transplant skin cancer; information on this strategy is very limited with regard to MCC, and the potential risk of skin cancer must be balanced against the possible risk of organ rejection
Summary
Merkel cell carcinoma (MCC) is a rare and highly aggressive neuroendocrine cancer of the skin. With a 33% mortality rate, MCC is deadlier than other more common forms of skin cancer [2]. Most reports of MCC in the setting of immunosuppression are relatively brief compared with well-described skin cancer counterparts such as malignant melanoma. Both MCC and malignant melanoma share risk factors (e.g., UV exposure, immunosuppression), but melanoma has a higher incidence rate and MCC generally has a worse overall prognosis [14,15,22]. A large population-based study found a significant 3-fold increased risk (95% CI, 1.74–4.95) of MCC development in patients with malignant melanoma [22]. The immunosuppressed microenvironment of malignant melanoma may be conducive for development of secondary malignancies, including MCC [23]
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