Merkel cell carcinoma: Epidemiology, disease presentation, and current clinical practice outcomes

Yaghi, Marita; Benedetto, Paul X; Greskovich, J.F.; Haber, Richard M.; Dominguez, Barbara; Liang, Hong; Nahleh, Zeina; Arteta-Bulos, Rafael

doi:10.1016/j.jdin.2022.06.004

Cited by 14 publications

(29 citation statements)

References 18 publications

(17 reference statements)

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“…The head and neck are affected most frequently (43-47%), followed by the distal limbs (upper limbs: 12-31%, lower limbs: 12-20%) and the torso (5-11%). 10,18,22,31,32,34,35 In accordance with other UVassociated skin tumors, dominance of the left side has been reported -which may potentially be explained by asymmetrical UV exposure. [67][68][69] Extracutaneous manifestations of primary MCC such as the oral, nasopharyngeal, genital, or gastrointestinal mucous membranes are rare with about 0.5% of cases.…”

Section: Clinical Presentationsupporting

confidence: 70%

“…In accordance with other UV‐associated skin tumors, dominance of the left side has been reported ‐ which may potentially be explained by asymmetrical UV exposure 67–69 . Extracutaneous manifestations of primary MCC such as the oral, nasopharyngeal, genital, or gastrointestinal mucous membranes are rare with about 0.5% of cases 35,36 . In about 2% of cases, no cutaneous primary tumor can be identified and the patients first present with metastases 34 .…”

Section: Pathogenesismentioning

confidence: 66%

“…39,40 About 10% of MCC cases are associated with immunosuppression. 34,35,41 Patients with chronic lymphocytic leukemia as well as those with AIDS carry an especially high risk of MCC.…”

Section: Epidemiologymentioning

confidence: 99%

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Modern diagnostics and treatment of Merkel cell carcinoma

Weilandt,

Peitsch

2023

J Deutsche Derma Gesell

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SummaryMerkel cell carcinoma (MCC) is a rare, aggressive skin cancer with epithelial and neuroendocrine differentiation, the incidence of which has increased substantially during the most recent decades. Risk factors include advanced age, fair skin type, UV exposure, and immunosuppression. Pathogenetically, a type caused by the Merkel cell polyomavirus is distinguished from a UV‐induced type with a high tumor mutational burden.Clinically, MCC presents as a mostly painless, rapidly growing, reddish‐violet tumor with a shiny surface, which is preferentially localized in the head‐neck region and at the distal extremities. A reliable diagnosis can only be made based on histological and immunohistochemical features. At initial diagnosis, 20–26% of patients show locoregional metastases and 8–14% distant metastases, making staging examinations indispensable. If there is no clinical evidence of metastases, a sentinel lymph node biopsy is recommended.Essential columns of therapy are surgery, adjuvant or palliative radiotherapy and, in advanced inoperable stages, medicamentous tumor therapy. The introduction of immune checkpoint inhibitors has led to a paradigm shift, as they provide a considerably longer duration of response and better survival rates than chemotherapy. The PD‐L1 inhibitor avelumab is approved for treatment of metastatic MCC in Germany, but the PD‐1 antibodies pembrolizumab and nivolumab are also used with success. Adjuvant and neoadjuvant treatment concepts, immune combination therapies and targeted therapies as monotherapy or in combination with immune checkpoint inhibitors are in the clinical trial phase.

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Section: Clinical Presentationsupporting

confidence: 70%

Section: Pathogenesismentioning

confidence: 66%

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Modern diagnostics and treatment of Merkel cell carcinoma

Weilandt,

Peitsch

2023

J Deutsche Derma Gesell

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“…The current study suggests that MMS may provide a survival advantage compared with WLE for surgical management of the MCC primary tumor. However, we controlled for use of adjuvant radiotherapy in this analysis given previous work demonstrating its efficacy. It may be hypothesized that the complete histopathologic clearance of the primary tumor that can be achieved with MMS may preclude the need for radiotherapy to the primary site as has been previously suggested; however, this issue cannot be directly addressed with the data from the current study, and further investigation evaluating outcomes after MMS with and without radiotherapy is required to answer this question …”

Section: Discussionmentioning

confidence: 99%

Overall Survival After Mohs Surgery for Early-Stage Merkel Cell Carcinoma

Cheraghlou,

Doudican,

Criscito

et al. 2023

JAMA Dermatol

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ImportanceMerkel cell carcinoma (MCC) is a rare cutaneous malignant neoplasm with increasing incidence and high mortality. Although it is accepted that the optimal treatment for localized tumors is surgical, the data surrounding the optimal surgical approach are mixed, and current National Comprehensive Cancer Network guidelines state that Mohs micrographic surgery (MMS) and wide local excision (WLE) can both be used. The current National Comprehensive Cancer Network guidelines do not advocate a preference for MMS or WLE and suggest that they can be used interchangeably.ObjectiveTo evaluate the association of surgical approach with overall survival after excision of localized T1/T2 MCC.Design, Setting, and ParticipantsThis retrospective cohort study used the National Cancer Database to assess adults with T1/T2 MCC who were diagnosed between January 1, 2004, and December 31, 2018, with pathologically confirmed, negative regional lymph nodes and treated with surgery. The National Cancer Database includes all reportable cases from Commission on Cancer–accredited facilities. Data analysis was performed from October 2022 to May 2023.ExposureSurgical approach.Main Outcomes and MeasuresOverall survival.ResultsA total of 2313 patients (mean [SD] age, 71 [10.6] years; 1340 [57.9%] male) were included in the study. Excision with MMS had the best unadjusted survival, with mean (SE) survival rates of 87.4% (3.4%) at 3 years, 84.5% (3.9%) at 5 years, and 81.8% (4.6%) at 10 years vs 86.1% (0.9%) at 3 years, 76.9% (1.2%) at 5 years, and 60.9% (2.0%) at 10 years for patients treated with WLE. Patients treated with narrow-margin excision had similar survival as those treated with WLE, with mean (SE) survival rates of 84.8% (1.4%) at 3 years, 78.3% (1.7%) at 5 years, and 60.8% (3.6%) at 10 years. On multivariable survival analysis, excision with MMS was associated with significantly improved survival compared with WLE (hazard ratio, 0.59; 95% CI, 0.36-0.97; P = .04). High-volume MCC centers were significantly more likely to use MMS over WLE compared with other centers (odds ratio, 1.99; 95% CI, 1.63-2.44; P &lt; .001).Conclusions and RelevanceIn this cohort study, the use of MMS (compared with WLE) was associated with significantly improved survival for patients with localized MCC with pathologically confirmed negative lymph nodes treated with surgery. These data suggest that Mohs surgery may provide a more effective treatment for MCC primary tumors than conventional WLE, although the lack of randomization and potential for selection bias in this study highlight the need for future prospective work evaluating this issue.

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“…La cirugía sigue siendo la modalidad terapéutica principal, según lo recomendado por las pautas actuales de la Red Nacional Integral del Cáncer (NCCN). Aunque la quimioterapia, y más recientemente la inmunoterapia, se usan en la enfermedad metastásica, la radioterapia (RT) adyuvante y definitiva se puede usar en pacientes con CCM no metastásico (15). Dado que las opciones de tratamiento para la forma locorregional ya se han estandarizado, no existen agentes terapéuticos específicamente aprobados para el tratamiento de la forma avanzada, y la elección del a menudo se basa en los datos disponibles de series retrospectivas y ensayos controlados aleatorios prospectivos.…”

Section: Figura 2 Manifestación Cutánea Nodular De Carcinoma De Célul...unclassified

Carcinoma de células de Merkel en la piel

Ramírez

Acuña²

2023

Rev.méd.sinerg.

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El carcinoma de células de Merkel (MCC) es un cáncer de piel raro pero muy agresivo con características neuroendocrinas. La patogenia del MCC se asocia con la presencia del poliomavirus de células de Merkel o la exposición crónica a la luz ultravioleta (UV). La incidencia está aumentando debido al envejecimiento de la población, la mayor exposición solar y el uso de medicamentos que inhiben el sistema inmunológico. A diferencia de la mayoría de los otros cánceres de piel, el MCC crece rápidamente y forma metástasis con facilidad. Desde hace unos años, se sabe muy poco sobre esta enfermedad y la quimioterapia ha sido el tratamiento estándar. En la actualidad, los nuevos descubrimientos sobre la fisiopatología de esta neoplasia y la introducción de la inmunoterapia permitieron reescribir por completo la historia de estos pacientes. En esta revisión, se brinda un resumen de los cambios más importantes en el manejo del carcinoma de células de Merkel, con un enfoque en la inmunoterapia y un panorama de futuras estrategias de tratamiento.

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Merkel cell carcinoma: Epidemiology, disease presentation, and current clinical practice outcomes

Cited by 14 publications

References 18 publications

Modern diagnostics and treatment of Merkel cell carcinoma

Modern diagnostics and treatment of Merkel cell carcinoma

Overall Survival After Mohs Surgery for Early-Stage Merkel Cell Carcinoma

Carcinoma de células de Merkel en la piel

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