Merkel Cell Carcinoma (MCC) is a rare, lethal cutaneous skin cancer with no approved drug therapies and limited treatment
options. The incidence of MCC (per 100,000 persons per year) is reported highest in Australia (0.82-1.60), followed
by the United States of America (0.6), Netherlands (0.35), Sweden (0.18-0.33), Finland (0.24-0.25), Spain (0.28), Denmark
(0.22), South East Scotland (0.133), France (0.13). The 5-year and 10-year survival is 0%-68%, and 21%-65% respectively,
based on factors such as disease stage and sex. Although less than 20% of patients progress to advanced metastatic stage
IV MCC, they experience the highest unmet need due to lack of effective therapeutic options. Further research is warranted
to quantify clinical, humanistic, and economic burden of MCC adequately and assess comparative effectiveness of evolving
treatment options to better inform patients, prescribers and payer organizations.
Merkel cell carcinoma (MCC) is a rare, aggressive skin cancer
characterized by high incidence of local recurrences, regional
nodal metastases, distant metastases, and high mortality
rates. Most individuals diagnosed with MCC have a history
of other skin cancers and other malignancies such as multiple
myeloma, chronic lymphocytic leukemia, and Hodgkin and
non-Hodgkin lymphoma. MCC is commonly staged using
the 2010 tumor (T) node (N) metastasis (M) staging system.
Based on TNM staging, stage I involves primary tumor ≤ 2 cm,
maximum tumor dimension, without evidence of regional
lymph node involvement; stage II involves primary tumor >2
cm (T2 or T3) or a primary tumor with invasion into bone,
muscle, fascia, or cartilage (T4), without evidence of lymph
node involvement; stage III involves any primary tumor with
regional lymph node disease and stage IV involves metastasis
beyond the regional lymph nodes, regardless of the status of
the primary tumor and regional nodes.
The treatment landscape has not changed in past 20 years. Depending on the stage of disease and patient’s health status,
the primary treatment of MCC involves surgical intervention,
radiotherapy, chemotherapy, or a combination of two or
more [5]. Currently, MCC treatment in the pipeline includes
targeted therapies and a majority of them are being studied in phase 2/3 clinical trials. However, the optimal treatment remains
uncertain and there are limited options for metastatic
stage IV MCC.
A comprehensive review of global epidemiology of MCC is
lacking. This review summarizes the trends in incidence of
MCC, the variation in estimates based on geography, age and
sex of patients, and disease-related survival. This review also
highlights the broader patient burden and unmet needs associated
with MCC reported in literature.
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