Merkel Cell Carcinoma: New Trends

Abstract

Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor of the skin mainly seen in the elderly. Its incidence is rising due to ageing of the population, increased sun exposure, and the use of immunosuppressive medication. Additionally, with the availability of specific immunohistochemical markers, MCC is easier to recognize. Typically, these tumors are rapidly progressive and behave aggressively, emphasizing the need for early detection and prompt diagnostic work-up and start of treatment. In this review, the tumor biology and immunology, current diagnostic and treatment modalities, as well as new and combined therapies for MCC, are discussed. MCC is a very immunogenic tumor which offers good prospects for immunotherapy. Given its rarity, the aggressiveness, and the frail patient population it concerns, MCC should be managed in close collaboration with an experienced multidisciplinary team.

Merkel Cell Carcinoma

“…MCC is more common in males and elderly fair-skinned individuals subjected to chronic sun exposure. Moreover, the risk for developing MCC is increased in immunocompromised patients (e.g., chronic lymphocytic leukemia, HIV/AIDS, medical treatment for autoimmune diseases, solid organ transplantation and other types of cancers) [4][5][6].…”

“…MCC typically presents as a rapidly growing, erythematous lesion in the dermal layer of the skin [4][5][6]. The acronym AEIOU accounts for 90% of all MCC presentation: asymptomatic, expanding rapidly, immune suppression, older than 50 years, and ultraviolet (UV) radiation-exposed [7].…”

“…Besides rapid local growth, systemic tumor progression is also fast. In fact, MCC frequently metastasizes to the lymph nodes and distal organs, including liver, bone, pancreas, lung, and brain [4][5][6]. As patients are often elderly and the metastatic potential of the tumor is high, MCC prognosis is poor [4][5][6].…”

“…Because of MCC's aggressiveness, a multidisciplinary evaluation of patients is critical in outlining the therapeutic choice. In fact, appropriate management typically involves a combination of surgery, radiation, and chemotherapy [4][5][6]. Recently, advanced MCC has been treated with immune checkpoint inhibitors (ICI), demonstrating their remarkable efficacy [4][5][6]9,10].…”

“…In fact, appropriate management typically involves a combination of surgery, radiation, and chemotherapy [4][5][6]. Recently, advanced MCC has been treated with immune checkpoint inhibitors (ICI), demonstrating their remarkable efficacy [4][5][6]9,10].…”