Updated Review of the Pathogenesis and Management of Merkel Cell Carcinoma

Background:Merkel cell carcinoma is a rare, aggressive, malignant cutaneous tumor of the elderly or immunosuppressed individuals that usually appears on sun-exposed areas of the body. Its pathogenesis is still debated, and, currently, no standardized treatment exists.Objective:To provide a current updated review of the most relevant data concerning the pathogenesis and management of Merkel cell carcinoma.Methods:Using relevant MeSH terms, we performed a review of the literature on these subjects from 1980 to June 2009.Results and Conclusion:The current management of Merkel cell carcinoma is based on surgical excision as the majority of patients present with localized disease, whereas up to 30% have regional lymph node metastases. In these cases, the best outcome is achieved with multidisciplinary management that includes radiotherapy. Chemotherapy is part of the treatment in advanced cases and is mandatory for distant metastatis. Given that a recent work showed the presence of a previously unknown polyomavirus, which the authors called Merkel cell polyomavirus, the therapeutical approach to Merkel cell carcinoma could be reconsidered in the future.

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Merkel Cell Carcinoma - A Rare Form of Skin Cancer with New Therapeutic Options

Revista de Chimie ◽

10.37358/rc.19.9.7527 ◽

2019 ◽

Vol 70 (9) ◽

pp. 3245-3249

Author(s):

Dana Lucia Stanculeanu ◽

Oana Toma ◽

Cristian Vasile ◽

Cornelia Nitipir ◽

Alina Bodilcu ◽

...

Keyword(s):

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Regional Lymph Node ◽

Performance Status ◽

Lymph Node Involvement ◽

The Body ◽

Therapeutic Options ◽

Merkel Cell ◽

Node Involvement ◽

And Performance

Merkel cell carcinoma (MCC) is a rare primary neuroendocrine carcinoma of the skin, with very aggressive behavior. Etiological factors contributing to MCC development, include exposure to UV radiation, infection with Merkel cell polyomavirus (MCPyV) and chronic immunosuppression. It is commonly found in regions of the body that are exposed to sun -55% of cases are located in the head and neck. The 5 years survival rate for metastatic disease is approximately 25%. The estimated mortality rate for MCC is between 33% and 46%. Therapeutic options for MCC depends on disease characteristics (stage of disease at presentation, location of the tumor, regional lymph node involvement, comorbidities and performance status of the patient) and it includes multimodal treatments like surgery, chemotherapy and emerging immunotherapeutic treatments with early promising results. Our clinic experience is very limited with this type of malignancy, only three cases were diagnosed and treated in our department, but with encouraging results regarding the newest immunotherapeutic options, with good quality of life and results similar with the data from the literature.

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Merkel Cell Carcinoma of the Eyelid: A Case Report

European Journal of Ophthalmology ◽

10.1177/112067210001000214 ◽

2000 ◽

Vol 10 (2) ◽

pp. 173-176 ◽

Cited By ~ 4

Author(s):

L. Collaço ◽

J.P. Silva ◽

M. Gonçalves ◽

P. Abrantes

Keyword(s):

Case Report ◽

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Neuroendocrine Tumour ◽

The Elderly ◽

Primary Site ◽

Wide Resection ◽

Merkel Cell ◽

Unique Case ◽

Upper Eyelid

A 22-year-old woman who presented with a tumour of the upper eyelid with clinical, histological, immunohistochemical and ultrastructural features of a Merkel cell carcinoma is reported. This is a unique case of Merkel cell carcinoma in such a young patient. This neuroendocrine tumour is typically found in the elderly. It can grow rapidly, has the potential for local recurrence and early metastatic spread. The authors recommend wide resection of the primary site, which proved effective in this case.

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Nonmelanoma Cutaneous Head and Neck Cancer and Merkel Cell Carcinoma: Current Concepts, Advances, and Controversies

Journal of Clinical Oncology ◽

10.1200/jco.2014.60.7333 ◽

2015 ◽

Vol 33 (29) ◽

pp. 3338-3345 ◽

Cited By ~ 29

Author(s):

Sandro V. Porceddu ◽

Michael J. Veness ◽

Alexander Guminski

Keyword(s):

Cell Carcinoma ◽

Head And Neck ◽

Merkel Cell Carcinoma ◽

Surgical Excision ◽

Current Evidence ◽

Ageing Population ◽

Merkel Cell ◽

Tumor Boards ◽

Solar Radiation Exposure ◽

Cure Rates

Nonmelanoma skin cancer (NMSC) is the most common cancer worldwide and the most frequently observed malignancy in whites. Approximately 75% to 80% are basal cell carcinomas and 20% to 25% are squamous cell carcinomas. Incidence is increasing, partly reflecting an ageing population, and NMSC is more commonly seen in men. The predominant causative agent is ultraviolet solar radiation exposure, with the majority of cases occurring on the head and neck. Surgical excision is typically the treatment of choice, providing histopathologic information, high cure rates, and acceptable cosmetic and functional outcomes. Radiation therapy is reserved for cases where surgery is not the preferred choice or for high-risk cases where adjuvant therapy is recommended. Although overall mortality rates are low, patients with complex cases such as those with immunosuppression should be considered for management within multidisciplinary tumor boards. In contrast, Merkel cell carcinoma is a rare and aggressive malignancy, frequently arising on the head and neck in older whites, with a poorer prognosis. This article focuses on the current evidence guiding practice, recent advances, and areas of controversy in NMSC and Merkel cell carcinoma of the head and neck.

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Faculty Opinions recommendation of Narrow excision margins are appropriate for Merkel cell carcinoma when combined with adjuvant radiation: Analysis of 188 cases of localized disease and proposed management algorithm.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.738394298.793586199 ◽

2021 ◽

Author(s):

Desiree Ratner

Keyword(s):

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Adjuvant Radiation ◽

Merkel Cell ◽

Management Algorithm ◽

Localized Disease ◽

Excision Margins

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Merkel Cell Carcinoma

Archives of Pathology & Laboratory Medicine ◽

10.5858/2003-127-0367-mcc ◽

2003 ◽

Vol 127 (3) ◽

pp. 367-369 ◽

Cited By ~ 3

Author(s):

Michael O. Idowu ◽

Melissa Contos ◽

Satinder Gill ◽

Celeste Powers

Keyword(s):

Gastrointestinal Bleeding ◽

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Upper Gastrointestinal Bleeding ◽

Neuroendocrine Differentiation ◽

Neck Region ◽

The Elderly ◽

Merkel Cell ◽

First Case ◽

Upper Gastrointestinal

Abstract Merkel cell carcinoma (MCC) is an uncommon, highly aggressive cutaneous neoplasm of neuroendocrine differentiation with a poor prognosis. MCC most often presents as a painless, firm, raised lesion in sun-exposed sites of the head and neck region of the elderly. We report a case of a metastatic MCC to the stomach presenting as upper gastrointestinal bleeding. To our knowledge, this is the second reported case of MCC presenting as upper gastrointestinal bleeding and the first case confirmed by the newer immunohistochemical techniques. The literature is reviewed.

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Unusual Ulcerated Merkel Cell Carcinoma of the Skin: Report of a Case

Tumori Journal ◽

10.1177/030089160609200618 ◽

2006 ◽

Vol 92 (6) ◽

pp. 555-558 ◽

Cited By ~ 2

Author(s):

Giorgio Lucandri ◽

Paolo Mazzocchi ◽

Bruno Bascone ◽

Marco Giordano ◽

Massimo Castaldi ◽

...

Keyword(s):

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Postoperative Radiotherapy ◽

Subcutaneous Fat ◽

Surgical Excision ◽

Prognostic Indicators ◽

Merkel Cell ◽

Biological Factors ◽

Axillary Lymphadenectomy ◽

Therapeutic Possibilities

Merkel cell carcinoma is an aggressive skin cancer, usually related to a severe prognosis. Treatment consists of wide surgical excision, adjuvant radiotherapy and/or polychemotherapy, but local-regional and distant relapses are common. Detection of histopathological and biological factors may select subgroups of patients suitable for different treatments. Herein we report the case of a patient treated for a wide bleeding and ulcerated Merkel cell carcinoma. Ulceration represents an uncommon feature because the tumor usually spreads from the dermis into subcutaneous fat, so it may be added to poor prognostic indicators. After surgical excision and postoperative radiotherapy, the patient recurred at regional nodes. Axillary lymphadenectomy was followed by courses of polychemotherapy. We obtained a satisfactory survival (31 months) by timing these different therapeutic possibilities.

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Frequency and prognosis of metastasis to liver, lung, bone and brain from Merkel cell carcinoma

Future Oncology ◽

10.2217/fon-2020-0064 ◽

2020 ◽

Vol 16 (16) ◽

pp. 1101-1113 ◽

Cited By ~ 1

Author(s):

Yi-Jun Xia ◽

Dong-Sheng Cao ◽

Jun Zhao ◽

Bang-Zhong Zhu ◽

Juan Xie

Keyword(s):

Lymph Node ◽

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Distant Metastasis ◽

Regional Lymph Node ◽

Distant Metastases ◽

Lymph Node Biopsy ◽

Merkel Cell ◽

Factors Affecting ◽

Node Removal

Aim: To describe the factors affecting distant metastasis of Merkel cell carcinoma (MCC) and the prognosis of metastatic MCC. Materials & methods: The MCC patient information was downloaded from the SEER database. Logistic regression and Cox proportional hazard models were conducted to screen for significant factors. Results: A total of 3449 patients were enrolled. Surgery and chemotherapy were significantly correlated with the occurrence of distant metastasis. In the cause-specific survival rate of MCC, regional lymph node removal, sentinel lymph node biopsy, radiation and chemotherapy can significantly reduce the prognostic risk of patients with distant metastases. Conclusion: Our study screened out the factors affecting the distant metastasis and prognosis of MCC and more prospective studies are needed to verify our findings.

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A Case of Merkel Cell Carcinoma Metastasis to the Head and the Body of the Pancreas

The Internet Journal of Gastroenterology ◽

10.5580/2148 ◽

2009 ◽

Vol 8 (1) ◽

Keyword(s):

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

The Body ◽

Merkel Cell ◽

Carcinoma Metastasis

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Merkel Cell Carcinoma: Evaluation of the Clinico-Pathological Characteristics, Treatment Strategies and Prognostic Factors in a Monocentric Retrospective Series (n=143)

Frontiers in Oncology ◽

10.3389/fonc.2021.737842 ◽

2021 ◽

Vol 11 ◽

Author(s):

Marco Rastrelli ◽

Paolo Del Fiore ◽

Irene Russo ◽

Jacopo Tartaglia ◽

Alessandro Dal Monico ◽

...

Keyword(s):

Prognostic Factors ◽

Prognostic Factor ◽

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Regional Lymph Node ◽

Treatment Strategies ◽

University Hospital ◽

Merkel Cell ◽

Pathological Characteristics ◽

Negative Prognostic Factor

BackgroundMerkel cell carcinoma (MCC) is a rare neuroendocrine tumor of the skin. The incidence of the disease has undergone a significant increase in recent years, which is caused by an increase in the average age of the population and in the use of immunosuppressive therapies. MCC is an aggressive pathology, which metastasizes early to the lymph nodes. These characteristics impose an accurate diagnostic analysis of the regional lymph node district with radiography, clinical examination and sentinel node biopsy. In recent years, there has been a breakthrough in the treatment of the advanced pathology thanks to the introduction of monoclonal antibodies acting on the PD-1/PD-L1 axis. This study aimed to describe the clinico-pathological characteristics, treatment strategies and prognostic factors of MCC.MethodsA retrospective cohort study was conducted involving 143 consecutive patients who were diagnosed and/or treated for MCC. These patients were referred to the Veneto Institute of Oncology IOV-IRCCS and to the University Hospital of Padua (a third-level center) in the period between December 1991 and January 2020. In the majority of cases, diagnosis took place at the IOV. However, some patients were diagnosed elsewhere and subsequently referred to the IOV for a review of the diagnosis or to begin specific therapeutic regimens.Results143 patients, with an average age of 71 years, were affected mainly with autoimmune and neoplastic comorbidities. Our analysis has shown that age, autoimmune comorbidities and the use of therapy with immunomodulating drugs (which include corticosteroids, statins and beta-blockers) are associated with a negative prognosis. In this sense, male sex is also a negative prognostic factor.ConclusionsAutoimmune and neoplastic comorbidities were frequent in the studied population. The use of drugs with immunomodulatory effects was also found to be a common feature of the population under examination. The use of this type of medication is considered a negative prognostic factor. The relevance of a multidisciplinary approach to the patient with MCC is confirmed, with the aim of assessing the risks and benefits related to the use of immunomodulating therapy in the individual patient.

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