Materiały źródłowe

• #1 SciELO Brazil – Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease

  https://www.scielo.br/j/abd/a/QYKxwGcZTZtB5BFFtL39Kps/

  Merkel cell carcinoma is a rare skin cancer with neuroendocrine differentiation. The risk factors include sun exposure, advanced age, immunosuppression (such as transplant recipients, patients with lymphoproliferative neoplasms, or patients with HIV), and Merkel cell polyomavirus infection. […] The incidence rate of MCC varies in different regions of the world. In the European Union, between 1995 and 2002, its annual incidence rate was 0.13 per 100,000 inhabitants, but higher in groups aged 65 years or older. In the United States, the incidence rate was 0.79 per 100,000 inhabitants in 2011. […] A higher incidence of MCC was observed in an Australian study, with a rate of 1.6 per 100,000 inhabitants in the state of Queensland between 2006 and 2010, more frequent in males (2.5 per 100,000) than in females (0.9 per 100,000) and with an average life expectancy of 75.5 years for men and 78 years for women at the diagnosis.

• #2 Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease | Anais Brasileiros de Dermatologia

  https://www.anaisdedermatologia.org.br/en-merkel-cell-carcinoma-epidemiology-clinical-articulo-S0365059623000211

  Merkel cell carcinoma is a rare skin cancer with neuroendocrine differentiation. The risk factors include sun exposure, advanced age, immunosuppression (such as transplant recipients, patients with lymphoproliferative neoplasms, or patients with HIV), and Merkel cell polyomavirus infection. […] The incidence rate of MCC varies in different regions of the world. In the European Union, between 1995 and 2002, its annual incidence rate was 0.13 per 100,000 inhabitants, but higher in groups aged 65 years or older. […] In 2018, Paulson et al. reported that the number of cases increased 95% between 2000 and 2013 in the US, compared with an increase of 57% for melanoma and 15% for all other solid tumors. […] Regarding the epidemiology of MCC in Brazil, Melo et al. published a study with data collected from Population-Based (20002015) and Hospital-Based (20002017) Cancer Registries. A total of 881 patients with the disease were analyzed, most of which were female (51.2%), aged over 60 years (82.2%), white (67.6%), and diagnosed predominantly in stages III or IV (50.5%).

• #2 Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease | Anais Brasileiros de Dermatologia

  https://clinics.elsevier.es/en-merkel-cell-carcinoma-epidemiology-clinical-articulo-S0365059623000211

  Merkel cell carcinoma is a rare skin cancer with neuroendocrine differentiation. The risk factors include sun exposure, advanced age, immunosuppression (such as transplant recipients, patients with lymphoproliferative neoplasms, or patients with HIV), and Merkel cell polyomavirus infection. […] The incidence rate of MCC varies in different regions of the world. In the European Union, between 1995 and 2002, its annual incidence rate was 0.13 per 100,000 inhabitants, but higher in groups aged 65 years or older. In the United States, the incidence rate was 0.79 per 100,000 inhabitants in 2011. […] A higher incidence of MCC was observed in an Australian study, with a rate of 1.6 per 100,000 inhabitants in the state of Queensland between 2006 and 2010, more frequent in males (2.5 per 100,000) than in females (0.9 per 100,000) and with an average life expectancy of 75.5 years for men and 78 years for women at the diagnosis.

• #3 Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease | Anais Brasileiros de Dermatologia

  https://clinics.elsevier.es/en-merkel-cell-carcinoma-epidemiology-clinical-articulo-S0365059623000211

  Merkel cell carcinoma is a rare skin cancer with neuroendocrine differentiation. The risk factors include sun exposure, advanced age, immunosuppression (such as transplant recipients, patients with lymphoproliferative neoplasms, or patients with HIV), and Merkel cell polyomavirus infection. […] The incidence rate of MCC varies in different regions of the world. In the European Union, between 1995 and 2002, its annual incidence rate was 0.13 per 100,000 inhabitants, but higher in groups aged 65 years or older. In the United States, the incidence rate was 0.79 per 100,000 inhabitants in 2011. […] A higher incidence of MCC was observed in an Australian study, with a rate of 1.6 per 100,000 inhabitants in the state of Queensland between 2006 and 2010, more frequent in males (2.5 per 100,000) than in females (0.9 per 100,000) and with an average life expectancy of 75.5 years for men and 78 years for women at the diagnosis.

• #4 Key Statistics for Merkel Cell Carcinoma | American Cancer Society

  https://www.cancer.org/cancer/types/merkel-cell-skin-cancer/about/key-statistics.html

  Skin cancer is by far the most common type of cancer in the United States. Merkel cell carcinoma (MCC) is a type of skin cancer, but it’s not common. About 3,000 people are diagnosed with Merkel cell carcinoma in the United States each year. […] The number of people diagnosed with Merkel cell carcinoma each year has been rising quickly over the past few decades. This is in part because of an increased awareness of this cancer, but it might also be due to an increase in risk factors. For instance, people are living longer, and more people are living with weakened immune systems (from previous treatment for cancer or other medical conditions). […] Most Americans diagnosed with Merkel cell carcinoma are older than age 70. Men are more likely to have MCC than women. More than 9 out of 10 Americans diagnosed with Merkel cell carcinoma are White people.

• #5 Merkel Cell Carcinoma—Update on Diagnosis, Management and Future Perspectives

  https://www.mdpi.com/2072-6694/15/1/103

  Merkel cell carcinoma (MCC) is a rare but highly aggressive skin cancer. The identification of the driving role of Merkel cell polyomavirus (MCPyV) and ultraviolet-induced DNA damage in the oncogenesis of MCC allowed a better understanding of its biological behavior. […] MCC usually affects the elderly, with a median age of diagnosis between 75 and 79 years, but cases of younger, mainly immunosuppressed patients, have been reported. […] According to the SEER-18 Database of the National Cancer Institute in the United States, including 6600 MCC cases from 2000 to 2013, there is a 0.7 cases/100,000 person-year incidence and a 95% increase in reported MCC cases in the last years. […] This increase can be explained by the greater diagnostic awareness of physicians, the improved immunohistochemistry diagnostic techniques (CK20 antibody use), and the aging and subsequent immune-senescence of a sun-exposed population.

• #6 SciELO Brazil – Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease

  https://www.scielo.br/j/abd/a/QYKxwGcZTZtB5BFFtL39Kps/

  Although rare, the incidence of MCC has increased dramatically. In 2018, Paulson et al. reported that the number of cases increased 95% between 2000 and 2013 in the US, compared with an increase of 57% for melanoma and 15% for all other solid tumors. […] Regarding the epidemiology of MCC in Brazil, Melo et al. published a study with data collected from Population-Based (2000-2015) and Hospital-Based (2000-2017) Cancer Registries. A total of 881 patients with the disease were analyzed, most of which were female (51.2%), aged over 60 years (82.2%), white (67.6%), and diagnosed predominantly in stages III or IV (50.5%). […] Moreover, age-standardized mean incidence rates were found to increase significantly in men between the years 2000 (0.31/1,000,000) and 2015 (1.21/1,000,000), with an annual percentage variation of 9.4 (95% CI: 4.7-14.4; p 0.001). […] MCC is often underdiagnosed and part of its increased incidence is thought to be due to better-trained pathologists and also to the development of biomarkers that have improved disease detection.

• #7 Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease | Anais Brasileiros de Dermatologia

  https://www.anaisdedermatologia.org.br/en-merkel-cell-carcinoma-epidemiology-clinical-articulo-S0365059623000211

  Merkel cell carcinoma is a rare skin cancer with neuroendocrine differentiation. The risk factors include sun exposure, advanced age, immunosuppression (such as transplant recipients, patients with lymphoproliferative neoplasms, or patients with HIV), and Merkel cell polyomavirus infection. […] The incidence rate of MCC varies in different regions of the world. In the European Union, between 1995 and 2002, its annual incidence rate was 0.13 per 100,000 inhabitants, but higher in groups aged 65 years or older. […] In 2018, Paulson et al. reported that the number of cases increased 95% between 2000 and 2013 in the US, compared with an increase of 57% for melanoma and 15% for all other solid tumors. […] Regarding the epidemiology of MCC in Brazil, Melo et al. published a study with data collected from Population-Based (20002015) and Hospital-Based (20002017) Cancer Registries. A total of 881 patients with the disease were analyzed, most of which were female (51.2%), aged over 60 years (82.2%), white (67.6%), and diagnosed predominantly in stages III or IV (50.5%).

• #8 Clinical and Epidemiological Characteristics of Merkel Cell Carcinoma in a Series of 38 Patients | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-clinical-epidemiological-characteristics-merkel-cell-articulo-S1578219019301246

  Merkel cell carcinoma is a rare yet aggressive cutaneous tumor with a poor prognosis. […] Data from recent studies indicate that MCC is on the rise, with some series reporting a triple-fold increase in the number of new cases, which is attributable to an increase in known risk factors (advanced age and immunosuppression) and probably also to a greater awareness of the disease among dermatologists and pathologists. […] In this series, we observed that Merkel cell carcinoma has become more common in recent years and is now diagnosed at an older age and found in new anatomic locations. […] On comparing the 2 time periods, we observed a 116% increase in MCC cases diagnosed between 2002-2009 (n=12) and 2010-2017 (n=26). […] The increase in incidence observed in the second period (2010-2017) is noteworthy and supports reports from recent years. […] Our analysis of 38 patients with MCC shows a rise in incidence in recent years and a trend towards an older age at diagnosis and occurrence of tumors at nontypical sites.

• #9 The biology and treatment of Merkel cell carcinoma: current understanding and research priorities | Nature Reviews Clinical Oncology

  https://www.nature.com/articles/s41571-018-0103-2

  As of 2013, the annual incidence of MCC in the USA was 0.7 cases per 100,000 people. The incidence of MCC in the USA almost doubled between 2000 and 2013 and is expected to exceed 3,000 cases per year by 2025, with similar increases in Australia and many but not all European countries. The basis for this increasing incidence is unclear but might be related to an ageing population and improvements in diagnostic recognition. The frequency of MCC is higher closer to the equator and much lower among those of non-white ethnicities, suggesting an association with sensitivity to ultraviolet radiation. Australia currently has the highest reported incidence of MCC (up to 1.6 cases per 100,000 person-years), notably with a higher percentage of VN-MCCs, probably reflecting a higher risk of environmental ultraviolet exposure. MCC also has a higher incidence among immunosuppressed populations. Apart from immunosuppressed individuals, MCC arises almost exclusively in those of an advanced age. Incidence estimates fall below zero in patients under age 40 years of age, and 90% of patients are 50 years of age. Unlike many other cancers, in which disease incidence peaks and then declines with increasing age, the incidence of MCC continues to increase even as patients reach 80 or even 90 years of age, possibly owing to immune senescence. Thus, MCC affects populations that frequently have substantial comorbidities that could complicate patient management.

• #10 Merkel Cell Carcinoma (MCC) > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/merkel-cell-carcinoma-mcc

  Merkel cell carcinoma is a rare, aggressive form of skin cancer. The condition is most common among fair-skinned people of European ancestry, but can also affect individuals with a darker complexion. It is frequently diagnosed in adults, more commonly aged 65 and older. […] Merkel cell carcinoma is a rare form of cancer. In the U.S., approximately 2,500 people are diagnosed with the condition each year. However, the number of cases is rising, and is predicted to reach nearly 3,300 in 2025. The increasing incidence is likely due to an aging population in which the proportion of people over age 65 is rising, though improvements in the detection of the condition may also play a role. […] Although Merkel cell carcinoma is one of the most dangerous forms of skin cancer, the majority (75%) of people survive five years or more after diagnosis when the cancer has not spread. But those numbers worsen if the cancer spreads regionally or to distant locations in the body, underlining the importance of early detection and treatment. […] Because Merkel cell carcinoma can recur, patients may undergo screening every three to six months for the first two years after treatment, then every six to 12 months onward.

• #11 Merkel Cell Carcinoma—Update on Diagnosis, Management and Future Perspectives

  https://www.mdpi.com/2072-6694/15/1/103

  Merkel cell carcinoma (MCC) is a rare but highly aggressive skin cancer. The identification of the driving role of Merkel cell polyomavirus (MCPyV) and ultraviolet-induced DNA damage in the oncogenesis of MCC allowed a better understanding of its biological behavior. […] MCC usually affects the elderly, with a median age of diagnosis between 75 and 79 years, but cases of younger, mainly immunosuppressed patients, have been reported. […] According to the SEER-18 Database of the National Cancer Institute in the United States, including 6600 MCC cases from 2000 to 2013, there is a 0.7 cases/100,000 person-year incidence and a 95% increase in reported MCC cases in the last years. […] This increase can be explained by the greater diagnostic awareness of physicians, the improved immunohistochemistry diagnostic techniques (CK20 antibody use), and the aging and subsequent immune-senescence of a sun-exposed population.

• #12 The biology and treatment of Merkel cell carcinoma: current understanding and research priorities | Nature Reviews Clinical Oncology

  https://www.nature.com/articles/s41571-018-0103-2

  As of 2013, the annual incidence of MCC in the USA was 0.7 cases per 100,000 people. The incidence of MCC in the USA almost doubled between 2000 and 2013 and is expected to exceed 3,000 cases per year by 2025, with similar increases in Australia and many but not all European countries. The basis for this increasing incidence is unclear but might be related to an ageing population and improvements in diagnostic recognition. The frequency of MCC is higher closer to the equator and much lower among those of non-white ethnicities, suggesting an association with sensitivity to ultraviolet radiation. Australia currently has the highest reported incidence of MCC (up to 1.6 cases per 100,000 person-years), notably with a higher percentage of VN-MCCs, probably reflecting a higher risk of environmental ultraviolet exposure. MCC also has a higher incidence among immunosuppressed populations. Apart from immunosuppressed individuals, MCC arises almost exclusively in those of an advanced age. Incidence estimates fall below zero in patients under age 40 years of age, and 90% of patients are 50 years of age. Unlike many other cancers, in which disease incidence peaks and then declines with increasing age, the incidence of MCC continues to increase even as patients reach 80 or even 90 years of age, possibly owing to immune senescence. Thus, MCC affects populations that frequently have substantial comorbidities that could complicate patient management.

• #13 Key Statistics for Merkel Cell Carcinoma | American Cancer Society

  https://www.cancer.org/cancer/types/merkel-cell-skin-cancer/about/key-statistics.html

  Skin cancer is by far the most common type of cancer in the United States. Merkel cell carcinoma (MCC) is a type of skin cancer, but it’s not common. About 3,000 people are diagnosed with Merkel cell carcinoma in the United States each year. […] The number of people diagnosed with Merkel cell carcinoma each year has been rising quickly over the past few decades. This is in part because of an increased awareness of this cancer, but it might also be due to an increase in risk factors. For instance, people are living longer, and more people are living with weakened immune systems (from previous treatment for cancer or other medical conditions). […] Most Americans diagnosed with Merkel cell carcinoma are older than age 70. Men are more likely to have MCC than women. More than 9 out of 10 Americans diagnosed with Merkel cell carcinoma are White people.

• #14 JNCCN 360 – Advanced Skin Cancers – Merkel Cell Carcinoma and Melanoma: Trends and Survival in the United States

  https://jnccn360.org/advanced-skin-cancers/news/merkel-cell-carcinoma-and-melanoma-trends-and-survival-in-the-united-states/

  During 2000 to 2021, 19,444 patients with Merkel cell carcinomas and 646,619 patients with melanoma were diagnosed. Of them, 90% of Merkel cell carcinomas and 95% of melanomas were found in non-Hispanic White individuals. Over 70% of Merkel cell carcinomas occurred in people aged 70 or older vs 37% of melanomas. Additionally, 46% of Merkel cell carcinomas and 22% of melanomas were diagnosed in the head-and-neck region. […] The 5- and 10- year cancer-free survival rates for Merkel cell carcinoma were 69% and 66%, respectively, and for melanoma, they were 90% and 86%, respectively. Cancer-specific mortality was higher among patients with Merkel cell carcinoma than in those with melanoma, but it improved in both groups after 2011, when BRAF and checkpoint inhibitors were introduced. […] Among 14,560 people with Merkel cell carcinoma and 484,117 with melanoma during 2004 to 2021, over half (55%) of Merkel cell carcinoma tumors were localized; in contrast, a much higher proportion of melanomas were localized (78%). During follow-up, 3,686 (25%) individuals with Merkel cell carcinoma and 50,691 (10%) with melanoma died of the disease.

• #15 JNCCN 360 – Advanced Skin Cancers – Merkel Cell Carcinoma and Melanoma: Trends and Survival in the United States

  https://jnccn360.org/advanced-skin-cancers/news/merkel-cell-carcinoma-and-melanoma-trends-and-survival-in-the-united-states/

  During 2000 to 2021, 19,444 patients with Merkel cell carcinomas and 646,619 patients with melanoma were diagnosed. Of them, 90% of Merkel cell carcinomas and 95% of melanomas were found in non-Hispanic White individuals. Over 70% of Merkel cell carcinomas occurred in people aged 70 or older vs 37% of melanomas. Additionally, 46% of Merkel cell carcinomas and 22% of melanomas were diagnosed in the head-and-neck region. […] The 5- and 10- year cancer-free survival rates for Merkel cell carcinoma were 69% and 66%, respectively, and for melanoma, they were 90% and 86%, respectively. Cancer-specific mortality was higher among patients with Merkel cell carcinoma than in those with melanoma, but it improved in both groups after 2011, when BRAF and checkpoint inhibitors were introduced. […] Among 14,560 people with Merkel cell carcinoma and 484,117 with melanoma during 2004 to 2021, over half (55%) of Merkel cell carcinoma tumors were localized; in contrast, a much higher proportion of melanomas were localized (78%). During follow-up, 3,686 (25%) individuals with Merkel cell carcinoma and 50,691 (10%) with melanoma died of the disease.

• #16 SciELO Brazil – Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease

  https://www.scielo.br/j/abd/a/QYKxwGcZTZtB5BFFtL39Kps/

  Merkel cell carcinoma is a rare skin cancer with neuroendocrine differentiation. The risk factors include sun exposure, advanced age, immunosuppression (such as transplant recipients, patients with lymphoproliferative neoplasms, or patients with HIV), and Merkel cell polyomavirus infection. […] The incidence rate of MCC varies in different regions of the world. In the European Union, between 1995 and 2002, its annual incidence rate was 0.13 per 100,000 inhabitants, but higher in groups aged 65 years or older. In the United States, the incidence rate was 0.79 per 100,000 inhabitants in 2011. […] A higher incidence of MCC was observed in an Australian study, with a rate of 1.6 per 100,000 inhabitants in the state of Queensland between 2006 and 2010, more frequent in males (2.5 per 100,000) than in females (0.9 per 100,000) and with an average life expectancy of 75.5 years for men and 78 years for women at the diagnosis.

• #17 Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease – PubMed

  https://pubmed.ncbi.nlm.nih.gov/36870886/

  Merkel cell carcinoma is a rare skin cancer with neuroendocrine differentiation. The risk factors include sun exposure, advanced age, immunosuppression (such as transplant recipients, patients with lymphoproliferative neoplasms, or patients with HIV), and Merkel cell polyomavirus infection. […] This article describes the current state of knowledge of the epidemiology, diagnosis, and staging of Merkel cell carcinoma, as well as new strategies for its systemic treatment. […] The presence of occult metastasis in a lymph node is frequent and a sentinel lymph node biopsy should be performed.

• #18 SciELO Brazil – Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease

  https://www.scielo.br/j/abd/a/QYKxwGcZTZtB5BFFtL39Kps/

  Although rare, the incidence of MCC has increased dramatically. In 2018, Paulson et al. reported that the number of cases increased 95% between 2000 and 2013 in the US, compared with an increase of 57% for melanoma and 15% for all other solid tumors. […] Regarding the epidemiology of MCC in Brazil, Melo et al. published a study with data collected from Population-Based (2000-2015) and Hospital-Based (2000-2017) Cancer Registries. A total of 881 patients with the disease were analyzed, most of which were female (51.2%), aged over 60 years (82.2%), white (67.6%), and diagnosed predominantly in stages III or IV (50.5%). […] Moreover, age-standardized mean incidence rates were found to increase significantly in men between the years 2000 (0.31/1,000,000) and 2015 (1.21/1,000,000), with an annual percentage variation of 9.4 (95% CI: 4.7-14.4; p 0.001). […] MCC is often underdiagnosed and part of its increased incidence is thought to be due to better-trained pathologists and also to the development of biomarkers that have improved disease detection.

• #19 The biology and treatment of Merkel cell carcinoma: current understanding and research priorities | Nature Reviews Clinical Oncology

  https://www.nature.com/articles/s41571-018-0103-2

  As of 2013, the annual incidence of MCC in the USA was 0.7 cases per 100,000 people. The incidence of MCC in the USA almost doubled between 2000 and 2013 and is expected to exceed 3,000 cases per year by 2025, with similar increases in Australia and many but not all European countries. The basis for this increasing incidence is unclear but might be related to an ageing population and improvements in diagnostic recognition. The frequency of MCC is higher closer to the equator and much lower among those of non-white ethnicities, suggesting an association with sensitivity to ultraviolet radiation. Australia currently has the highest reported incidence of MCC (up to 1.6 cases per 100,000 person-years), notably with a higher percentage of VN-MCCs, probably reflecting a higher risk of environmental ultraviolet exposure. MCC also has a higher incidence among immunosuppressed populations. Apart from immunosuppressed individuals, MCC arises almost exclusively in those of an advanced age. Incidence estimates fall below zero in patients under age 40 years of age, and 90% of patients are 50 years of age. Unlike many other cancers, in which disease incidence peaks and then declines with increasing age, the incidence of MCC continues to increase even as patients reach 80 or even 90 years of age, possibly owing to immune senescence. Thus, MCC affects populations that frequently have substantial comorbidities that could complicate patient management.

• #20 Update on Merkel Cell Carcinoma: Epidemiology, Etiopathogenesis, Clinical Features, Diagnosis, and Staging | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-update-on-merkel-cell-carcinoma-articulo-S1578219016303407

  Risk for MCC is 13-fold higher in HIV-infected individuals than in the general population. […] The most important discovery related to pathogenesis came in 2008 with the description of the Merkel cell polyomavirus (MCPyV) in the genome of MCC tumor cells. […] Staging is of great importance for establishing prognosis and guiding proper treatment in MCC. The simple, traditional system described by Yiengpruksawan et al. has 3 stages: stage I, localized skin lesion; stage II, regional lymph node involvement; stage III, metastatic disease. The American Joint Committee on Cancer proposed a new, more complete staging system in 2010 based on the analysis of over 4000 cases. This new system highlights the importance of tumor size, regional lymph node status, and metastasis in relation to survival.

• #21 Nationwide multidisciplinary consensus on the clinical management of Merkel cell carcinoma: a Delphi panel | Journal for ImmunoTherapy of Cancer

  https://jitc.bmj.com/content/10/6/e004742

  Among the immunosuppressed patients: the risk of developing MCC is approximately 13 times greater in those with HIV infection, 25 times greater in those with organ transplants, and 40 times greater in those with chronic lymphocytic leukemia. Furthermore, immunosuppressed patients have significantly less survival rate compared with immunocompetent patients. […] Several studies have established the presence of MCPyV in the majority (80%) of MCC and antibodies that recognize MCPyV oncoproteins are found in approximately 50% of patients with MCC. […] Imaging played a crucial role in diagnosis and initial staging, planning for surgery or radiation therapy, assessment of treatment response and surveillance of recurrence and metastases. […] The panelists unanimously agreed that the information concerning avelumab provided by the JAVELIN Merkel 200 study is adequate and reliable and that the expanded access program data could have concrete clinical implications.

• #22 Nationwide multidisciplinary consensus on the clinical management of Merkel cell carcinoma: a Delphi panel | Journal for ImmunoTherapy of Cancer

  https://jitc.bmj.com/content/10/6/e004742

  Among the immunosuppressed patients: the risk of developing MCC is approximately 13 times greater in those with HIV infection, 25 times greater in those with organ transplants, and 40 times greater in those with chronic lymphocytic leukemia. Furthermore, immunosuppressed patients have significantly less survival rate compared with immunocompetent patients. […] Several studies have established the presence of MCPyV in the majority (80%) of MCC and antibodies that recognize MCPyV oncoproteins are found in approximately 50% of patients with MCC. […] Imaging played a crucial role in diagnosis and initial staging, planning for surgery or radiation therapy, assessment of treatment response and surveillance of recurrence and metastases. […] The panelists unanimously agreed that the information concerning avelumab provided by the JAVELIN Merkel 200 study is adequate and reliable and that the expanded access program data could have concrete clinical implications.

• #23 Nationwide multidisciplinary consensus on the clinical management of Merkel cell carcinoma: a Delphi panel | Journal for ImmunoTherapy of Cancer

  https://jitc.bmj.com/content/10/6/e004742

  Among the immunosuppressed patients: the risk of developing MCC is approximately 13 times greater in those with HIV infection, 25 times greater in those with organ transplants, and 40 times greater in those with chronic lymphocytic leukemia. Furthermore, immunosuppressed patients have significantly less survival rate compared with immunocompetent patients. […] Several studies have established the presence of MCPyV in the majority (80%) of MCC and antibodies that recognize MCPyV oncoproteins are found in approximately 50% of patients with MCC. […] Imaging played a crucial role in diagnosis and initial staging, planning for surgery or radiation therapy, assessment of treatment response and surveillance of recurrence and metastases. […] The panelists unanimously agreed that the information concerning avelumab provided by the JAVELIN Merkel 200 study is adequate and reliable and that the expanded access program data could have concrete clinical implications.

• #24 Nationwide multidisciplinary consensus on the clinical management of Merkel cell carcinoma: a Delphi panel | Journal for ImmunoTherapy of Cancer

  https://jitc.bmj.com/content/10/6/e004742

  Among the immunosuppressed patients: the risk of developing MCC is approximately 13 times greater in those with HIV infection, 25 times greater in those with organ transplants, and 40 times greater in those with chronic lymphocytic leukemia. Furthermore, immunosuppressed patients have significantly less survival rate compared with immunocompetent patients. […] Several studies have established the presence of MCPyV in the majority (80%) of MCC and antibodies that recognize MCPyV oncoproteins are found in approximately 50% of patients with MCC. […] Imaging played a crucial role in diagnosis and initial staging, planning for surgery or radiation therapy, assessment of treatment response and surveillance of recurrence and metastases. […] The panelists unanimously agreed that the information concerning avelumab provided by the JAVELIN Merkel 200 study is adequate and reliable and that the expanded access program data could have concrete clinical implications.

• #25 Merkel Cell Carcinoma—Update on Diagnosis, Management and Future Perspectives

  https://www.mdpi.com/2072-6694/15/1/103

  MCC is the second deadliest skin malignancy after melanoma, with a case-by-case fatality rate worse than stage-matched melanomas. […] Although its incidence is low, MCC has a strong propensity to recur locally, spread regionally to the lymph node (LN) basin, and disseminate. […] In most cases, patients are diagnosed with a locoregionally advanced disease with an estimated five-year disease-specific survival of around 64%. […] Serological assessment of MCPyV-oncoprotein antibodies (against the viral capsid protein VP1) can be considered as part of the baseline workup. […] Seropositivity can be present in almost half of MCC patients. […] Seronegative patients are at almost 40% higher risk of recurrence and could benefit from more intensive surveillance. […] The stage of the disease at the time of diagnosis is the most critical prognostic factor of survival.

• #26 Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease | Anais Brasileiros de Dermatologia

  https://www.anaisdedermatologia.org.br/en-merkel-cell-carcinoma-epidemiology-clinical-articulo-S0365059623000211

  Merkel cell carcinoma is a rare skin cancer with neuroendocrine differentiation. The risk factors include sun exposure, advanced age, immunosuppression (such as transplant recipients, patients with lymphoproliferative neoplasms, or patients with HIV), and Merkel cell polyomavirus infection. […] The incidence rate of MCC varies in different regions of the world. In the European Union, between 1995 and 2002, its annual incidence rate was 0.13 per 100,000 inhabitants, but higher in groups aged 65 years or older. […] In 2018, Paulson et al. reported that the number of cases increased 95% between 2000 and 2013 in the US, compared with an increase of 57% for melanoma and 15% for all other solid tumors. […] Regarding the epidemiology of MCC in Brazil, Melo et al. published a study with data collected from Population-Based (20002015) and Hospital-Based (20002017) Cancer Registries. A total of 881 patients with the disease were analyzed, most of which were female (51.2%), aged over 60 years (82.2%), white (67.6%), and diagnosed predominantly in stages III or IV (50.5%).

• #27 SciELO Brazil – Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease

  https://www.scielo.br/j/abd/a/QYKxwGcZTZtB5BFFtL39Kps/

  Although rare, the incidence of MCC has increased dramatically. In 2018, Paulson et al. reported that the number of cases increased 95% between 2000 and 2013 in the US, compared with an increase of 57% for melanoma and 15% for all other solid tumors. […] Regarding the epidemiology of MCC in Brazil, Melo et al. published a study with data collected from Population-Based (2000-2015) and Hospital-Based (2000-2017) Cancer Registries. A total of 881 patients with the disease were analyzed, most of which were female (51.2%), aged over 60 years (82.2%), white (67.6%), and diagnosed predominantly in stages III or IV (50.5%). […] Moreover, age-standardized mean incidence rates were found to increase significantly in men between the years 2000 (0.31/1,000,000) and 2015 (1.21/1,000,000), with an annual percentage variation of 9.4 (95% CI: 4.7-14.4; p 0.001). […] MCC is often underdiagnosed and part of its increased incidence is thought to be due to better-trained pathologists and also to the development of biomarkers that have improved disease detection.

• #28

  https://journals.lww.com/eurjcancerprev/fulltext/2023/05000/epidemiology_of_merkel_cell_carcinoma_in_tuscany.13.aspx

  Merkel cell carcinoma (MCC) is a rare, aggressive skin cancer that still has a poor prognosis. MCC incidence has increased in recent years worldwide. […] We reported a dramatic increase in MCC diagnoses in the last 5 years compared with the previous years, with a crude incidence rate of 1,15/100000 inhabitants, almost doubling the last reported data in Italy. […] Considering the generally low incidence of MCC worldwide, larger cohorts would be necessary to validate our data and to obtain a better prognostic stratification.

• #29 Epidemiology of Merkel cell carcinoma. A population-based study from 1985 to 2013, in northeastern of France – Archive ouverte HAL

  https://hal.science/hal-03594850v1

  Merkel Cell Carcinoma (MCC) is an aggressive skin cancer with an increasing incidence. Population-based epidemiologic data about MCC in France are rare. Our study aims to describe the epidemiology of MCC in Bas-Rhin, Northeastern of France, between 1985 and 2013. […] Incidence of MCC in Bas-Rhin quadrupled between 1985 and 2013. The highest incidence rate was observed in people 70 years. Better survival was associated with female sex and younger age. We hypothesize that MCC will still increase and be diagnosed in increasingly younger patients in next generations.

• #30 Merkel Cell Carcinoma—Update on Diagnosis, Management and Future Perspectives

  https://www.mdpi.com/2072-6694/15/1/103

  Merkel cell carcinoma (MCC) is a rare but highly aggressive skin cancer. The identification of the driving role of Merkel cell polyomavirus (MCPyV) and ultraviolet-induced DNA damage in the oncogenesis of MCC allowed a better understanding of its biological behavior. […] MCC usually affects the elderly, with a median age of diagnosis between 75 and 79 years, but cases of younger, mainly immunosuppressed patients, have been reported. […] According to the SEER-18 Database of the National Cancer Institute in the United States, including 6600 MCC cases from 2000 to 2013, there is a 0.7 cases/100,000 person-year incidence and a 95% increase in reported MCC cases in the last years. […] This increase can be explained by the greater diagnostic awareness of physicians, the improved immunohistochemistry diagnostic techniques (CK20 antibody use), and the aging and subsequent immune-senescence of a sun-exposed population.

• #31 Clinical and Epidemiological Characteristics of Merkel Cell Carcinoma in a Series of 38 Patients | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-clinical-epidemiological-characteristics-merkel-cell-articulo-S1578219019301246

  Merkel cell carcinoma is a rare yet aggressive cutaneous tumor with a poor prognosis. […] Data from recent studies indicate that MCC is on the rise, with some series reporting a triple-fold increase in the number of new cases, which is attributable to an increase in known risk factors (advanced age and immunosuppression) and probably also to a greater awareness of the disease among dermatologists and pathologists. […] In this series, we observed that Merkel cell carcinoma has become more common in recent years and is now diagnosed at an older age and found in new anatomic locations. […] On comparing the 2 time periods, we observed a 116% increase in MCC cases diagnosed between 2002-2009 (n=12) and 2010-2017 (n=26). […] The increase in incidence observed in the second period (2010-2017) is noteworthy and supports reports from recent years. […] Our analysis of 38 patients with MCC shows a rise in incidence in recent years and a trend towards an older age at diagnosis and occurrence of tumors at nontypical sites.

• #32 Key Statistics for Merkel Cell Carcinoma | American Cancer Society

  https://www.cancer.org/cancer/types/merkel-cell-skin-cancer/about/key-statistics.html

  Skin cancer is by far the most common type of cancer in the United States. Merkel cell carcinoma (MCC) is a type of skin cancer, but it’s not common. About 3,000 people are diagnosed with Merkel cell carcinoma in the United States each year. […] The number of people diagnosed with Merkel cell carcinoma each year has been rising quickly over the past few decades. This is in part because of an increased awareness of this cancer, but it might also be due to an increase in risk factors. For instance, people are living longer, and more people are living with weakened immune systems (from previous treatment for cancer or other medical conditions). […] Most Americans diagnosed with Merkel cell carcinoma are older than age 70. Men are more likely to have MCC than women. More than 9 out of 10 Americans diagnosed with Merkel cell carcinoma are White people.

• #33 The biology and treatment of Merkel cell carcinoma: current understanding and research priorities | Nature Reviews Clinical Oncology

  https://www.nature.com/articles/s41571-018-0103-2

  As of 2013, the annual incidence of MCC in the USA was 0.7 cases per 100,000 people. The incidence of MCC in the USA almost doubled between 2000 and 2013 and is expected to exceed 3,000 cases per year by 2025, with similar increases in Australia and many but not all European countries. The basis for this increasing incidence is unclear but might be related to an ageing population and improvements in diagnostic recognition. The frequency of MCC is higher closer to the equator and much lower among those of non-white ethnicities, suggesting an association with sensitivity to ultraviolet radiation. Australia currently has the highest reported incidence of MCC (up to 1.6 cases per 100,000 person-years), notably with a higher percentage of VN-MCCs, probably reflecting a higher risk of environmental ultraviolet exposure. MCC also has a higher incidence among immunosuppressed populations. Apart from immunosuppressed individuals, MCC arises almost exclusively in those of an advanced age. Incidence estimates fall below zero in patients under age 40 years of age, and 90% of patients are 50 years of age. Unlike many other cancers, in which disease incidence peaks and then declines with increasing age, the incidence of MCC continues to increase even as patients reach 80 or even 90 years of age, possibly owing to immune senescence. Thus, MCC affects populations that frequently have substantial comorbidities that could complicate patient management.

• #34 Clinical and Epidemiological Characteristics of Merkel Cell Carcinoma in a Series of 38 Patients | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-clinical-epidemiological-characteristics-merkel-cell-articulo-S1578219019301246

  Merkel cell carcinoma is a rare yet aggressive cutaneous tumor with a poor prognosis. […] Data from recent studies indicate that MCC is on the rise, with some series reporting a triple-fold increase in the number of new cases, which is attributable to an increase in known risk factors (advanced age and immunosuppression) and probably also to a greater awareness of the disease among dermatologists and pathologists. […] In this series, we observed that Merkel cell carcinoma has become more common in recent years and is now diagnosed at an older age and found in new anatomic locations. […] On comparing the 2 time periods, we observed a 116% increase in MCC cases diagnosed between 2002-2009 (n=12) and 2010-2017 (n=26). […] The increase in incidence observed in the second period (2010-2017) is noteworthy and supports reports from recent years. […] Our analysis of 38 patients with MCC shows a rise in incidence in recent years and a trend towards an older age at diagnosis and occurrence of tumors at nontypical sites.

• #35 Update on Merkel Cell Carcinoma: Epidemiology, Etiopathogenesis, Clinical Features, Diagnosis, and Staging | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-update-on-merkel-cell-carcinoma-articulo-S1578219016303407

  Merkel cell carcinoma (MCC) is a rare, highly aggressive tumor, and local or regional disease recurrence is common, as is metastasis. MCC usually develops in sun-exposed skin in patients of advanced age. Its incidence has risen 4-fold in recent decades as the population has aged and immunohistochemical techniques have led to more diagnoses. […] The incidence of MCC in Spain is unknown at this time. However, it has risen 3-fold in recent decades in the United States, from 0.15 per 100000 population in 1981 to 1.44 per 100000 population in 2011. The increase is attributable to greater awareness of the disease among dermatologists and pathologists; greater ease of diagnosis thanks to immunohistochemical techniques; and increased population risk due to UV light exposure, aging, and immunosuppression.

• #36 Merkel Cell Carcinoma—Update on Diagnosis, Management and Future Perspectives

  https://www.mdpi.com/2072-6694/15/1/103

  Merkel cell carcinoma (MCC) is a rare but highly aggressive skin cancer. The identification of the driving role of Merkel cell polyomavirus (MCPyV) and ultraviolet-induced DNA damage in the oncogenesis of MCC allowed a better understanding of its biological behavior. […] MCC usually affects the elderly, with a median age of diagnosis between 75 and 79 years, but cases of younger, mainly immunosuppressed patients, have been reported. […] According to the SEER-18 Database of the National Cancer Institute in the United States, including 6600 MCC cases from 2000 to 2013, there is a 0.7 cases/100,000 person-year incidence and a 95% increase in reported MCC cases in the last years. […] This increase can be explained by the greater diagnostic awareness of physicians, the improved immunohistochemistry diagnostic techniques (CK20 antibody use), and the aging and subsequent immune-senescence of a sun-exposed population.

• #37 SciELO Brazil – Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease

  https://www.scielo.br/j/abd/a/QYKxwGcZTZtB5BFFtL39Kps/

  Although rare, the incidence of MCC has increased dramatically. In 2018, Paulson et al. reported that the number of cases increased 95% between 2000 and 2013 in the US, compared with an increase of 57% for melanoma and 15% for all other solid tumors. […] Regarding the epidemiology of MCC in Brazil, Melo et al. published a study with data collected from Population-Based (2000-2015) and Hospital-Based (2000-2017) Cancer Registries. A total of 881 patients with the disease were analyzed, most of which were female (51.2%), aged over 60 years (82.2%), white (67.6%), and diagnosed predominantly in stages III or IV (50.5%). […] Moreover, age-standardized mean incidence rates were found to increase significantly in men between the years 2000 (0.31/1,000,000) and 2015 (1.21/1,000,000), with an annual percentage variation of 9.4 (95% CI: 4.7-14.4; p 0.001). […] MCC is often underdiagnosed and part of its increased incidence is thought to be due to better-trained pathologists and also to the development of biomarkers that have improved disease detection.

• #38 Key Statistics for Merkel Cell Carcinoma | American Cancer Society

  https://www.cancer.org/cancer/types/merkel-cell-skin-cancer/about/key-statistics.html

  Skin cancer is by far the most common type of cancer in the United States. Merkel cell carcinoma (MCC) is a type of skin cancer, but it’s not common. About 3,000 people are diagnosed with Merkel cell carcinoma in the United States each year. […] The number of people diagnosed with Merkel cell carcinoma each year has been rising quickly over the past few decades. This is in part because of an increased awareness of this cancer, but it might also be due to an increase in risk factors. For instance, people are living longer, and more people are living with weakened immune systems (from previous treatment for cancer or other medical conditions). […] Most Americans diagnosed with Merkel cell carcinoma are older than age 70. Men are more likely to have MCC than women. More than 9 out of 10 Americans diagnosed with Merkel cell carcinoma are White people.

• #39 SciELO Brazil – A review of the epidemiology and treatment of Merkel cell carcinoma A review of the epidemiology and treatment of Merkel cell carcinoma

  https://www.scielo.br/j/clin/a/fs9BcMwfN5GMydXLfzYFT8F/?lang=en

  Merkel cell carcinoma is a very rare and aggressive neoplasm. The incidence of Merkel cell carcinoma is increasing due to the advancing age of the population, higher rates of sun exposure and an increasing number of immunocompromised individuals. […] The estimated annual incidence of MCC is 0.23 per 100,000 individuals for Caucasians, whereas the incidence in those of African descent is 0.01 per 100,000 and appears to be even lower in Polynesians. Merkel cell carcinoma occurs predominantly in elderly persons, with a mean of 69 years-old at diagnosis. […] It is well accepted that Merkel carcinoma commonly occurs in immunocompromised patients, and in this population, changing the immunosuppression regimen from calcineurin inhibitors (azathioprine and cyclosporine) to serolimus results in better cure rates and regression of tumors.

• #40 SciELO Brazil – A review of the epidemiology and treatment of Merkel cell carcinoma A review of the epidemiology and treatment of Merkel cell carcinoma

  https://www.scielo.br/j/clin/a/fs9BcMwfN5GMydXLfzYFT8F/?lang=en

  The incidence of MCC is increasing due to the advancing age of the population, a higher incidence of damaging sun exposure, and an increase in the number of immunocompromised individuals. Regarding its etiology of MCC, the recently described Merkel cell polyomavirus must be considered. While local or regional surgical treatment remains the standard of care, adjuvant radiotherapy or radiotherapy alone have been shown to be reasonable therapeutic options.

• #41 Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease | Anais Brasileiros de Dermatologia

  https://www.anaisdedermatologia.org.br/en-merkel-cell-carcinoma-epidemiology-clinical-articulo-S0365059623000211

  MCC is characteristically an aggressive, locally invasive tumor, with a high incidence of local recurrence and regional lymph node involvement. […] The SLNB is an important staging tool recommended for all patients with a clinically negative node, to perform pathological node staging whenever possible. […] According to the 8th edition of the AJCC staging system, the five-year OS estimates for the clinical and pathological staging of local disease were 45.0% and 62.8% for stage I; 30.9% and 54.6% for stage IIA and 27.3% and 34.8% for stage IIB, respectively.

• #42 Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease | Anais Brasileiros de Dermatologia

  https://clinics.elsevier.es/en-merkel-cell-carcinoma-epidemiology-clinical-articulo-S0365059623000211

  Although rare, the incidence of MCC has increased dramatically. In 2018, Paulson et al. reported that the number of cases increased 95% between 2000 and 2013 in the US, compared with an increase of 57% for melanoma and 15% for all other solid tumors. […] Regarding the epidemiology of MCC in Brazil, Melo et al. published a study with data collected from Population-Based (2000-2015) and Hospital-Based (2000-2017) Cancer Registries. A total of 881 patients with the disease were analyzed, most of which were female (51.2%), aged over 60 years (82.2%), white (67.6%), and diagnosed predominantly in stages III or IV (50.5%). […] MCC is characteristically an aggressive, locally invasive tumor, with a high incidence of local recurrence and regional lymph node involvement. The SLNB is an important staging tool recommended for all patients with a clinically negative node, to perform pathological node staging whenever possible.

• #43 Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease | Anais Brasileiros de Dermatologia

  https://www.anaisdedermatologia.org.br/en-merkel-cell-carcinoma-epidemiology-clinical-articulo-S0365059623000211

  MCC is characteristically an aggressive, locally invasive tumor, with a high incidence of local recurrence and regional lymph node involvement. […] The SLNB is an important staging tool recommended for all patients with a clinically negative node, to perform pathological node staging whenever possible. […] According to the 8th edition of the AJCC staging system, the five-year OS estimates for the clinical and pathological staging of local disease were 45.0% and 62.8% for stage I; 30.9% and 54.6% for stage IIA and 27.3% and 34.8% for stage IIB, respectively.

• #44 Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease | Anais Brasileiros de Dermatologia

  https://clinics.elsevier.es/en-merkel-cell-carcinoma-epidemiology-clinical-articulo-S0365059623000211

  According to the 8th edition of the AJCC staging system, the five-year OS estimates for the clinical and pathological staging of local disease were 45.0% and 62.8% for stage I; 30.9% and 54.6% for stage IIA and 27.3% and 34.8% for stage IIB, respectively. The five-year OS for revised stage IIIA was 40.3%, while it was 26.8% for stage IIIB. In stage IV disease, the two-year survival rate is only 26%.

• #45 Patients With Merkel Cell Carcinoma Face 40% 5-Year Recurrence Rate, According to Recent Study – The ASCO Post

  https://ascopost.com/news/march-2022/patients-with-merkel-cell-carcinoma-face-40-5-year-recurrence-rate-according-to-recent-study/

  Patients treated for Merkel cell carcinoma face a 5-year recurrence rate of 40% markedly higher than the recurrence rates for melanoma and other skin cancers, according to research published by McEvoy et al in JAMA Dermatology. […] Additionally, in the study cohort of more than 600 patients, 95% of Merkel cell carcinoma recurrences happened in the first 3 years, suggesting that surveillance efforts should be focused on that time span, according to the study authors. […] The authors sought to characterize posttreatment recurrence risk of Merkel cell carcinoma diagnosed at pathologic and clinical stages. […] The investigators found four factors associated with a higher recurrence risk: advanced age, male sex, immunosuppression, and a known primary lesion amid clinically detectable nodal disease.

• #46 Tumor-Informed ctDNA Assay May Aid Surveillance Strategies in Merkel Cell Carcinoma

  https://www.onclive.com/view/tumor-informed-ctdna-assay-may-aid-surveillance-strategies-in-merkel-cell-carcinoma

  A tumor-informed ctDNA assay showed high sensitivity and specificity as well as potential for ctDNA to be used as a prognostic biomarker during surveillance in Merkel cell carcinoma. […] Use of circulating tumor DNA (ctDNA) testing demonstrated high baseline sensitivity and specificity prior to treatment, and the presence of ctDNA showed an association with risk of recurrence post-treatment in patients with Merkel cell carcinoma (MCC). […] ctDNA testing exhibited high prognostic accuracy in detecting MCC recurrence, suggesting its potential to reduce frequent surveillance imaging. […] MCC is an aggressive neuroendocrine skin cancer that has a high mortality rate and 5-year recurrence rate of 40%. […] The risk of recurrence was significantly higher in patients who were ctDNA positive at any point during surveillance vs those who were ctDNA negative throughout in both the discovery and validation cohorts.

• #47 Patients With Merkel Cell Carcinoma Face 40% 5-Year Recurrence Rate, According to Recent Study – The ASCO Post

  https://ascopost.com/news/march-2022/patients-with-merkel-cell-carcinoma-face-40-5-year-recurrence-rate-according-to-recent-study/

  Patients treated for Merkel cell carcinoma face a 5-year recurrence rate of 40% markedly higher than the recurrence rates for melanoma and other skin cancers, according to research published by McEvoy et al in JAMA Dermatology. […] Additionally, in the study cohort of more than 600 patients, 95% of Merkel cell carcinoma recurrences happened in the first 3 years, suggesting that surveillance efforts should be focused on that time span, according to the study authors. […] The authors sought to characterize posttreatment recurrence risk of Merkel cell carcinoma diagnosed at pathologic and clinical stages. […] The investigators found four factors associated with a higher recurrence risk: advanced age, male sex, immunosuppression, and a known primary lesion amid clinically detectable nodal disease.

• #48 18F-FDG PET/CT for Posttreatment Surveillance Imaging of Patients with Stage III Merkel Cell Carcinoma | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/63/6/906

  The purpose of this study was to investigate the diagnostic and prognostic value of 18F-FDG PET/CT for surveillance imaging in patients treated for stage III Merkel cell carcinoma (MCC). […] After definitive therapy, recurrences occur in approximately 25% 50% of patients, with a median time to recurrence of about 8 mo, and 90% recurrences occur within 2 y. Follow-up is therefore imperative in MCC, especially in higher stage disease. National Comprehensive Cancer Network (NCCN) guidelines recommend routine follow-up visits every 36 mo for 3 y and every 612 mo thereafter. […] The overall sensitivity and specificity of 18F-FDG PET/CT for detecting primary or metastatic MCC ranges from 86% to 100% and 89% to 100%, respectively, and impact on management has been noted in up to 45% of patients.

• #49 Merkel cell carcinoma | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/types/merkel-cell-skin-cancer

  Each year around 360 people are diagnosed with MCC in England. This is based on the average number of new cases diagnosed in the years 2019, 2021, and 2022. […] Age is a risk factor for most types of cancer, including MCC. The median age of diagnosis of MCC is around 76 years old. […] You have regular appointments with your specialist doctor or nurse after treatment for MCC. You usually have them every 3 months for the first 3 years. And then every 6 months for up to 5 years.

• #50 Merkel Cell Carcinoma—Update on Diagnosis, Management and Future Perspectives

  https://www.mdpi.com/2072-6694/15/1/103

  The 8th edition AJCC staging system for MCC was published in 2017, based on an analysis of over 9000 patients included in the National Cancer Database, from 1998 to 2012. […] Other parameters can have prognostic significance as well, though they are not included in the AJCC staging system. […] Patient-related factors associated with the worst prognosis include male sex, younger age, immunosuppression, and MCPyV seronegativity. […] Approximately 40% of MCC recur, and 80% of recurrences occur within 2 years of the initial diagnosis. […] The median time-to-recurrence is 8 to 9 months. […] A more intense surveillance schedule is recommended for the first three years that gradually decreases afterward, reflecting the diminishing risk of recurrence after three years.

• #51 Staging, treatment, and surveillance of locoregional Merkel cell carcinoma – UpToDate

  https://www.uptodate.com/contents/staging-treatment-and-surveillance-of-locoregional-merkel-cell-carcinoma

  Merkel cell carcinoma (MCC) is a rare, aggressive, cutaneous malignancy that predominantly affects older adults with light skin types and has a high propensity to metastasize. […] The staging work-up, management, and surveillance of locoregional MCC are discussed here. […] POSTTREATMENT SURVEILLANCE includes frequency of follow-up, imaging studies, Merkel cell polyomavirus serology, and the role for ctDNA testing.

• #52 18F-FDG PET/CT for Posttreatment Surveillance Imaging of Patients with Stage III Merkel Cell Carcinoma | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/63/6/906

  In this study, 18F-FDG PET/CT showed high sensitivity and specificity of 92% and 93%, respectively, to detect recurrent MCC lesions in local as well as distant sites. […] 18F-FDG PET/CT results contributed to implementation of treatments in 33% (20/61) patients. […] On the basis of our results, early inclusion of 18F-FDG PET/CT within the first 6 mo after definitive treatment is suitable to initiate imaging surveillance in stage III MCC patients. […] The OS of patients with recurrence detected on 18F-FDG PET/CT was significantly reduced compared with patients with negative 18F-FDG PET/CT scan results. […] Our data indicate that initiating surveillance 18F-FDG PET/CT within 6 mo after completion of definitive treatment in stage III MCC may be useful in early detection of recurrence. Further scans spaced at intervals of 69 mo for at least 2 y may be beneficial.

• #53 Merkel Cell Carcinoma—Update on Diagnosis, Management and Future Perspectives

  https://www.mdpi.com/2072-6694/15/1/103

  MCC is the second deadliest skin malignancy after melanoma, with a case-by-case fatality rate worse than stage-matched melanomas. […] Although its incidence is low, MCC has a strong propensity to recur locally, spread regionally to the lymph node (LN) basin, and disseminate. […] In most cases, patients are diagnosed with a locoregionally advanced disease with an estimated five-year disease-specific survival of around 64%. […] Serological assessment of MCPyV-oncoprotein antibodies (against the viral capsid protein VP1) can be considered as part of the baseline workup. […] Seropositivity can be present in almost half of MCC patients. […] Seronegative patients are at almost 40% higher risk of recurrence and could benefit from more intensive surveillance. […] The stage of the disease at the time of diagnosis is the most critical prognostic factor of survival.

• #54 Tumor-Informed ctDNA Assay May Aid Surveillance Strategies in Merkel Cell Carcinoma

  https://www.onclive.com/view/tumor-informed-ctdna-assay-may-aid-surveillance-strategies-in-merkel-cell-carcinoma

  A tumor-informed ctDNA assay showed high sensitivity and specificity as well as potential for ctDNA to be used as a prognostic biomarker during surveillance in Merkel cell carcinoma. […] Use of circulating tumor DNA (ctDNA) testing demonstrated high baseline sensitivity and specificity prior to treatment, and the presence of ctDNA showed an association with risk of recurrence post-treatment in patients with Merkel cell carcinoma (MCC). […] ctDNA testing exhibited high prognostic accuracy in detecting MCC recurrence, suggesting its potential to reduce frequent surveillance imaging. […] MCC is an aggressive neuroendocrine skin cancer that has a high mortality rate and 5-year recurrence rate of 40%. […] The risk of recurrence was significantly higher in patients who were ctDNA positive at any point during surveillance vs those who were ctDNA negative throughout in both the discovery and validation cohorts.

• #55 Tumor-Informed ctDNA Assay May Aid Surveillance Strategies in Merkel Cell Carcinoma

  https://www.onclive.com/view/tumor-informed-ctdna-assay-may-aid-surveillance-strategies-in-merkel-cell-carcinoma

  The positive predictive value at 1 year in the discovery and validation cohorts was 69% and 94%, respectively. […] The negative predictive value was high 135 days after any negative ctDNA test, reaching 94% and 93% in the discovery and validation cohorts, respectively. […] In this multicenter, prospective, observational study of patients with stage I to IV MCC, we formally validated the utility of a tumor-informed ctDNA assay, the authors concluded. This assay may be particularly impactful in the surveillance of patients with this highly lethal malignancy characterized by a high recurrence rate of 40% within 5 years.

• #56 18F-FDG PET/CT for Posttreatment Surveillance Imaging of Patients with Stage III Merkel Cell Carcinoma | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/63/6/906

  In this study, 18F-FDG PET/CT showed high sensitivity and specificity of 92% and 93%, respectively, to detect recurrent MCC lesions in local as well as distant sites. […] 18F-FDG PET/CT results contributed to implementation of treatments in 33% (20/61) patients. […] On the basis of our results, early inclusion of 18F-FDG PET/CT within the first 6 mo after definitive treatment is suitable to initiate imaging surveillance in stage III MCC patients. […] The OS of patients with recurrence detected on 18F-FDG PET/CT was significantly reduced compared with patients with negative 18F-FDG PET/CT scan results. […] Our data indicate that initiating surveillance 18F-FDG PET/CT within 6 mo after completion of definitive treatment in stage III MCC may be useful in early detection of recurrence. Further scans spaced at intervals of 69 mo for at least 2 y may be beneficial.

• #57 Merkel Cell Carcinoma: An Update and Review

  https://www.mdpi.com/2072-6694/15/5/1534

  Merkel cell carcinoma (MCC) is a rare, very aggressive skin cancer with a high mortality rate and a high tendency of metastatic spread. MCC mainly affects elderly and immunosuppressed individuals. Thus, in a population with a growing life expectancy and consequent age-related immunological impairment, MCC cases are likely to increase in the near future. […] The article series starts with a review by Silling et al. presenting an update on the epidemiology of MCPyV and MCC, as well as the characteristic features and risk factors of MCC. The authors report on the two entities of MCC, UV radiation-induced and MCPyV-induced, and summarize the differences and similarities in pathogenesis as well as prevalence variations in different geographical regions and patient populations. […] As with all rare diseases, rare tumours such as MCC also face the problem of insufficient awareness and are, consequently, afflicted by under- or misdiagnosis and inadequate treatment. In addition, it is less attractive for pharmaceutical industries to develop new therapeutical compounds for diseases of infrequent occurrence. With this Special Issue of Cancers, we hope to disseminate the present knowledge on MCC and improve the awareness of this rare skin cancer. We should, however, keep in mind that with the increasing life expectancy of the population, MCC is also likely to increase and may not be as rare in the future.

• #58 The biology and treatment of Merkel cell carcinoma: current understanding and research priorities | Nature Reviews Clinical Oncology

  https://www.nature.com/articles/s41571-018-0103-2

  As of 2013, the annual incidence of MCC in the USA was 0.7 cases per 100,000 people. The incidence of MCC in the USA almost doubled between 2000 and 2013 and is expected to exceed 3,000 cases per year by 2025, with similar increases in Australia and many but not all European countries. The basis for this increasing incidence is unclear but might be related to an ageing population and improvements in diagnostic recognition. The frequency of MCC is higher closer to the equator and much lower among those of non-white ethnicities, suggesting an association with sensitivity to ultraviolet radiation. Australia currently has the highest reported incidence of MCC (up to 1.6 cases per 100,000 person-years), notably with a higher percentage of VN-MCCs, probably reflecting a higher risk of environmental ultraviolet exposure. MCC also has a higher incidence among immunosuppressed populations. Apart from immunosuppressed individuals, MCC arises almost exclusively in those of an advanced age. Incidence estimates fall below zero in patients under age 40 years of age, and 90% of patients are 50 years of age. Unlike many other cancers, in which disease incidence peaks and then declines with increasing age, the incidence of MCC continues to increase even as patients reach 80 or even 90 years of age, possibly owing to immune senescence. Thus, MCC affects populations that frequently have substantial comorbidities that could complicate patient management.

• #59 Update on Merkel Cell Carcinoma: Epidemiology, Etiopathogenesis, Clinical Features, Diagnosis, and Staging | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-update-on-merkel-cell-carcinoma-articulo-S1578219016303407

  Risk for MCC is 13-fold higher in HIV-infected individuals than in the general population. […] The most important discovery related to pathogenesis came in 2008 with the description of the Merkel cell polyomavirus (MCPyV) in the genome of MCC tumor cells. […] Staging is of great importance for establishing prognosis and guiding proper treatment in MCC. The simple, traditional system described by Yiengpruksawan et al. has 3 stages: stage I, localized skin lesion; stage II, regional lymph node involvement; stage III, metastatic disease. The American Joint Committee on Cancer proposed a new, more complete staging system in 2010 based on the analysis of over 4000 cases. This new system highlights the importance of tumor size, regional lymph node status, and metastasis in relation to survival.

• #60 Immunotherapy for Merkel cell carcinoma: a turning point in patient care | Journal for ImmunoTherapy of Cancer

  https://jitc.bmj.com/content/6/1/23

  Recent results from clinical trials of immune checkpoint inhibitors suggest that these therapies could improve treatment outcomes by unleashing anti-tumor immunity against an immunogenic tumor. […] The success of immune checkpoint inhibitors in several cancer types and the immune susceptibility of MCC has renewed hope for developing more effective treatment options for patients with MCC. […] The totality of these data provided a strong rationale for testing immune checkpoint blockers in patients with advanced MCC. […] The results of the trials described above led to the inclusion of avelumab, pembrolizumab and nivolumab in the January 2018 NCCN guidelines as preferred treatment options for patients with disseminated disease. […] Although data are still preliminary, it appears that rates of MCC regression in treatment-nave patients treated with PD-1/PD-L1-pathway blockers may exceed those of patients who were previously treated. […] Given the role of MCPyV in driving MCC carcinogenesis, a future treatment approach may involve administration of genetically-modified CAR-T cells against MCPyV antigens.

• #61 Immunotherapy for Merkel cell carcinoma: a turning point in patient care | Journal for ImmunoTherapy of Cancer

  https://jitc.bmj.com/content/6/1/23

  Recent results from clinical trials of immune checkpoint inhibitors suggest that these therapies could improve treatment outcomes by unleashing anti-tumor immunity against an immunogenic tumor. […] The success of immune checkpoint inhibitors in several cancer types and the immune susceptibility of MCC has renewed hope for developing more effective treatment options for patients with MCC. […] The totality of these data provided a strong rationale for testing immune checkpoint blockers in patients with advanced MCC. […] The results of the trials described above led to the inclusion of avelumab, pembrolizumab and nivolumab in the January 2018 NCCN guidelines as preferred treatment options for patients with disseminated disease. […] Although data are still preliminary, it appears that rates of MCC regression in treatment-nave patients treated with PD-1/PD-L1-pathway blockers may exceed those of patients who were previously treated. […] Given the role of MCPyV in driving MCC carcinogenesis, a future treatment approach may involve administration of genetically-modified CAR-T cells against MCPyV antigens.

• #62 Burden of Merkel Cell Carcinoma: A Targeted Literature Review

  https://www.mathewsopenaccess.com/full-text/burden-of-merkel-cell-carcinoma-a-targeted-literature-review

  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). […] 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. […] The epidemiological review of MCC shows that its incidence is increasing; however, the epidemiology of MCC in key regions such as Canada, UK, Germany, and most of the Asia-Pacific region is not clearly understood.

• #63 Burden of Merkel Cell Carcinoma: A Targeted Literature Review

  https://www.mathewsopenaccess.com/full-text/burden-of-merkel-cell-carcinoma-a-targeted-literature-review

  The current treatment options usually include a combination of surgery, radiotherapy, and chemotherapy, but there is a significant need of newer, better treatments which can increase the survival. […] Future research is warranted to adequately quantify the burden of illness of MCC and assess comparative effectiveness of evolving treatment options to better inform patients, prescribers and payer organizations concerning optimal modalities of disease management.

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