Merkel cell carcinoma: a population-based study on mortality and the association with other cancers

Ascoli, Valeria; Minelli, Giada; Kanieff, Mark; Frova, Luisa; Conti, Susanna

doi:10.1007/s10552-011-9826-4

Cited by 11 publications

(4 citation statements)

References 23 publications

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“…Most of the MCC patients die with non-localized, i.e. metastatic disease ( 28 ), which accords with the findings in other cancers ( 29 ). Most of the patients present with localized disease ( 4 ).…”

Section: Discussionsupporting

confidence: 88%

Frequency and locations of systemic metastases in Merkel cell carcinoma by imaging

Kouzmina

Koljonen

Leikola

et al. 2017

Acta Radiologica Open

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BackgroundThe primary neuroendocrine skin cancer, Merkel cell carcinoma (MCC), has a well-known predilection to metastasize systemically. However, the experience of systemic metastases in MCC is mainly disseminated through case reports due to the rarity of MCC.PurposeTo elucidate the frequency and locations of systemic metastasis in MCC by reviewing the imaging of patients with metastatic MCC in a national cohort.Material and MethodsPatients with diagnosed metastatic MCC by imaging studies in Finland during 1999–2012 were included in this study. We reviewed their imaging studies to evaluate the most frequent sites for systemic metastasis and determined the latency between the primary tumor diagnosis and systemic metastasis. The material includes 30 MCC patients with complete imaging series and 187 examinations, of which 102 (54%) were CT images.ResultsThe mean latency from the primary tumor diagnosis to systemic metastasis was 2.1 years and the mean latency between the radiologic diagnosis of the metastases and death was 299 days. Metastases were recorded in several organ systems in most of the cases, and at least two separate metastatic sites in 63% of the cases. Metastatic spread was noted in 60% of the cases in distant lymph nodes. Liver and lungs were the most affected solid organs.ConclusionSystemic metastasis in MCC has no predilection site, basically every organ system can be involved. Most of the systemic metastases were recorded during the first two years after the MCC diagnosis.

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Section: Discussionsupporting

confidence: 88%

Frequency and locations of systemic metastases in Merkel cell carcinoma by imaging

Kouzmina

Koljonen

Leikola

et al. 2017

Acta Radiologica Open

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“…Merkel cell carcinoma (MCC) is an aggressive, neuroendocrine, cutaneous cancer generally diagnosed in elderly individuals with sun exposure, and its risk of occurrence is related to an immunocompromised condition and the presence of other cancers [1,2]. Approximately 80% of MCC cases are associated with Merkel cell polyomavirus (MCPyV).…”

Section: Introductionmentioning

confidence: 99%

Expression of the IDO1/TDO2-AhR pathway in tumor cells or the tumor microenvironment is associated with Merkel cell polyomavirus status and prognosis in Merkel cell carcinoma

et al. 2019

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Merkel cell carcinoma (MCC) is a rare, aggressive neuroendocrine skin cancer, with approximately 80% of cases related to Merkel cell polyomavirus (MCPyV). Indoleamine 2,3-dioxygenase 1 (IDO1) and tryptophan 2,3-dioxygenase 2 (TDO2) are the key rate-limiting enzymes of the tryptophan to kynurenine (KYN) metabolic pathway. With aryl hydrocarbon receptor (AhR), an intracellular transcription factor, they play a role in escaping the immunosurveillance process in several cancers. IDO1/TDO2/AhR expression associated with the MCPyV status and prognosis in MCC was investigated. Samples included 24 MCPyV-positive MCCs, 12 MCPyV-negative MCCs with squamous cell carcinoma, and seven MCPyV-negative pure MCCs. They were stained immunohistochemically with IDO1, TDO2, and AhR antibodies and analyzed. Higher IDO1 expression in MCC tumor cells was found in MCPyV-negative than in MCPyV-positive MCC (P < .001). The tumor microenvironment (TME) in MCPyV-negative MCC expressed higher TDO2 than MCPyV-positive MCC (P < .001). Kaplan-Meier and log-rank tests showed that MCC with lower IDO1 expression in tumor cells and with lower TDO2 and AhR expressions in TME had better overall survival than otherwise (P = .043, .008, and .035, respectively); lower TDO2 expression in TME was also associated with longer disease-specific survival (P = .016). This suggests that IDO1, TDO2, and AhR express differentially in tumor cells or TME and play different roles in tumorigenesis between MCPyV-positive and -negative MCC that may affect the MCC biology. Evaluating IDO1/TDO2/AhR expression is important for selecting the most likely patients with MCC for immunotherapies targeting the IDO1/TDO2-AhR pathway.

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“…We believe that our work adds more information to the known epidemiology and clinical characteristics of Merkel Cell Carcinoma in Italy, that until now was limited only to case series or single center studies. Moreover, there is a suggestion for a north-to-south gradient in MCC in Italy [ 22 ], and most of the papers published regarded Northern Italy centers. Our work contributes to expand the knowledge.…”

Section: Discussionmentioning

confidence: 99%

Defining the Profile of Patients with Primary Merkel Cell Carcinoma in Central Italy: A Retrospective Analysis

Gualdi

Ferrara²,

Simonacci³

et al. 2022

Cancers

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Merkel cell carcinoma (MCC) is a rare neuroendocrine skin cancer that usually occurs in elderly people on sun-exposed areas, with a predisposition to local recurrence. Evidence suggests a growing incidence over the past decade; however, robust epidemiologic data are still lacking. We describe the MCC population in clinical practice in a retrospective analysis of demographic, clinical, and tumor characteristics from medical records of primary MCC patients, between 2015 and 2020, at six dermatology clinics in Central Italy. Ninety-four patients were included (57.4% male; mean age 78.2 ± 10.1 years, range 47–99 years). The estimated incidence rate of MCC was 0.93 per 100,000 inhabitants/year. Lower limbs were the most frequently affected site (31.5%), and 54% of patients for whom information was available were immunosuppressed. Lymph node involvement was reported in 42.5% of patients, and distant metastases in almost 20%. Most patients underwent surgery for tumor excision and were mainly referred to specialized dermatology clinics by dermatologists (47.9%) and general surgeons (28.7%). Apart from the relatively balanced prevalence of MCC in men and women, the predominant location on lower limbs, and the higher incidence rate compared with previous reports in Italy, this population is, overall, similar to the populations described in other observational studies. MCC management requires the involvement of several specialties. Increased awareness of MCC and standardization of its management are urgently needed.

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Merkel cell carcinoma: a population-based study on mortality and the association with other cancers

Cited by 11 publications

References 23 publications

Frequency and locations of systemic metastases in Merkel cell carcinoma by imaging

Frequency and locations of systemic metastases in Merkel cell carcinoma by imaging

Expression of the IDO1/TDO2-AhR pathway in tumor cells or the tumor microenvironment is associated with Merkel cell polyomavirus status and prognosis in Merkel cell carcinoma

Defining the Profile of Patients with Primary Merkel Cell Carcinoma in Central Italy: A Retrospective Analysis

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