scholarly journals New Insights into the Biological and Clinical Aspects of Merkel Cell Carcinoma

Cancers ◽  
2021 ◽  
Vol 13 (9) ◽  
pp. 2259
Author(s):  
Virve Koljonen ◽  
Weng-Onn Lui ◽  
Jürgen C. Becker

The Special Issue in Cancers, “The Biological and Clinical Aspects of Merkel Cell Carcinoma”, walks the avid reader through the interesting and sometimes even mysterious facets of Merkel cell carcinoma (MCC), starting at its carcinogenesis to also cover innovative treatment options [...]

2019 ◽  
Vol 72 (5) ◽  
pp. 337-340 ◽  
Author(s):  
Celestine M Trinidad ◽  
Carlos A Torres-Cabala ◽  
Victor G Prieto ◽  
Phyu P Aung

Merkel cell carcinoma (MCC) is a rare primary cutaneous neuroendocrine carcinoma. The annual incidence of MCC is increasing in the USA. Timely diagnosis and proper staging of this tumour are crucial as MCC has high rates of regional recurrence and lymph node and distant metastasis. In this review, we outline the key differences between the tumor node metastasis (TNM) staging criteria for MCC in the seventh and eighth editions of the AJCC Cancer Staging Manual. We also discuss histopathological parameters that are not included in the eighth edition of the manual but have been shown in other studies to predict a worse prognosis in patients with MCC. Correct assessment and reporting of these clinically relevant histopathological parameters is of utmost importance for practising pathologists as management differs according to the stage of the tumour. This review aims to increase awareness of all these parameters, and proper recognition would guide the treating clinicians towards the most appropriate treatment options that can be given to patients.


2020 ◽  
Vol 21 (15) ◽  
pp. 5394
Author(s):  
Mauro Cives ◽  
Francesco Mannavola ◽  
Lucia Lospalluti ◽  
Maria Chiara Sergi ◽  
Gerardo Cazzato ◽  
...  

Non-melanoma skin cancers (NMSCs) include basal cell carcinoma (BCC), squamous cell carcinoma (SCC) and Merkel cell carcinoma (MCC). These neoplasms are highly diverse in their clinical presentation, as well as in their biological evolution. While the deregulation of the Hedgehog pathway is commonly observed in BCC, SCC and MCC are characterized by a strikingly elevated mutational and neoantigen burden. As result of our improved understanding of the biology of non-melanoma skin cancers, innovative treatment options including inhibitors of the Hedgehog pathway and immunotherapeutic agents have been recently investigated against these malignancies, leading to their approval by regulatory authorities. Herein, we review the most relevant biological and clinical features of NMSC, focusing on innovative treatment approaches.


2019 ◽  
Vol 10 ◽  
pp. 172
Author(s):  
Ricardo Lourenço Caramanti ◽  
Feres Eduardo Chaddad Neto ◽  
Lucas Crociati Meguins ◽  
Carlos Eduardo Rocha ◽  
Dionei Freitas de Moraes ◽  
...  

Background: Merkel cell carcinoma (MCC) is a rare neuroendocrine skin tumor. In our knowledge, only 30 cases of brain metastasis were reported in literature. The authors report a case of 57-year-old male with elevated intracranial pressure signs, which a frontal mass with pathological diagnosis of MCC. Case Description: A 57-year-old male was admitted with a 3-month history of progressive headache, associated with nausea and dizziness. The magnetic resonance imaging showed a left frontal lobe, parasagittal, and nodular lesion with perilesional edema. The patient underwent complete surgical resection with success. The adjuvant treatment was radiotherapy and chemotherapy. Conclusion: In our knowledge, there is a little number of cases of MCC reported in literature. Surgical management is considered in cases with intracranial hypertension or focal signs. The adjuvant treatment options are immunotherapy and radiotherapy.


2021 ◽  
Author(s):  
Loren E. Hernandez ◽  
Noreen Mohsin ◽  
Marita Yaghi ◽  
Fabio Frech ◽  
Isabella Dreyfuss ◽  
...  

Author(s):  
Valerie Glutsch ◽  
Hermann Kneitz ◽  
Anja Gesierich ◽  
Matthias Goebeler ◽  
Sebastian Haferkamp ◽  
...  

Abstract Background Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine cutaneous malignancy with poor prognosis. In Europe, approved systemic therapies are limited to the PD-L1 inhibitor avelumab. For avelumab-refractory patients, efficient and safe treatment options are lacking. Methods At three different sites in Germany, clinical and molecular data of patients with metastatic MCC being refractory to the PD-L1 inhibitor avelumab and who were later on treated with combined IPI/NIVO were retrospectively collected and evaluated. Results Five patients treated at three different academic sites in Germany were enrolled. Three out of five patients investigated for this report responded to combined IPI/NIVO according to RECIST 1.1. Combined immunotherapy was well tolerated without any grade II or III immune-related adverse events. Two out of three responders to IPI/NIVO received platinum-based chemotherapy in between avelumab and combined immunotherapy. Conclusion In this small retrospective study, we observed a high response rate and durable responses to subsequent combined immunotherapy with IPI/NIVO in avelumab-refractory metastatic MCC patients. In conclusion, our data suggest a promising activity of second- or third-line PD-1- plus CTLA-4-blockade in patients with anti-PD-L1-refractory MCC.


2019 ◽  
Vol 9 (1) ◽  
pp. 53-58
Author(s):  
K. V. Orlova ◽  
V. V. Nazarova ◽  
N. N. Petenko ◽  
L. V. Demidov

Merkel cell carcinoma (MCC) is a rare and aggressive skin cancer with limited treatment options in later stages, when the mortality rate due to the disease is as high as 46 %. It has been demonstrated earlier that MCC is an immunogenic tumor, therefore the emergence of immune checkpoint inhibitors has changed the treatment principles for patients with advanced MCC. In this article, we present the initial results of the use of avelumab, an anti‑PD–L1 antibody, in the treatment of patients with metastatic and/or locally advanced MCC as part of the early access program in Russia.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e21073-e21073
Author(s):  
Siva Narayanan ◽  
Smeet Gala

e21073 Background: 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. It has no approved drug therapies and limited treatment options, especially to manage the metastatic disease. Our objective was to conduct a comprehensive review of MCC disease burden. Methods: We conducted a targeted literature review of studies published from 2010 to 2016 to assess epidemiology, patient burden, and unmet needs associated with MCC. An article was retrieved for full review if the abstract met each of the following criteria: reported incidence, natural morbidity or mortality of MCC; derived from a peer-reviewed journal; and reported in English-language published since January 2010. Results: The incidence of MCC (per 100,000 persons per year) is reported highest in Australia (0.82-1.60), followed by the US (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). Inadequate data available from countries like Canada, UK, Germany and most part of Asia-Pacific. Typically, stage I-II MCC is observed in approximately 60% - 75% patients, stage III in 10% - 30% patients, and stage IV (metastatic) in 2% - 16% patients. The 5-year and 10-year survival is 0% - 68%, and 21% - 65% respectively, based on factors such as disease stage and gender. Although less than 20% patients progress to advanced metastatic stage IV MCC, they experience the highest unmet need due to lack of effective therapeutic options. Data on economic burden and humanistic burden (e.g., impact on patient quality of life) is not available. Conclusions: Epidemiological review indicates an increasing incidence of MCC; however, available estimates are at least 5 years old. Epidemiological data as well as data on clinical, economic and humanistic burden of MCC is lacking from several parts of the world. Further 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 cost-effective modalities of disease management.


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