Merkel Cell Carcinoma: An updated review of pathogenesis, diagnosis, and treatment options

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.

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Brain metastasis of Merkel cell carcinoma – A rare case report

Surgical Neurology International ◽

10.25259/sni_166_2019 ◽

2019 ◽

Vol 10 ◽

pp. 172

Author(s):

Ricardo Lourenço Caramanti ◽

Feres Eduardo Chaddad Neto ◽

Lucas Crociati Meguins ◽

Carlos Eduardo Rocha ◽

Dionei Freitas de Moraes ◽

...

Keyword(s):

Brain Metastasis ◽

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Adjuvant Treatment ◽

Treatment Options ◽

Skin Tumor ◽

Merkel Cell ◽

Left Frontal Lobe ◽

Perilesional Edema ◽

Rare Case Report

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.

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Merkel Cell Carcinoma Diagnosis and Treatment

Dermatologic Surgery ◽

10.1111/j.1524-4725.1995.tb00269.x ◽

1995 ◽

Vol 21 (8) ◽

pp. 669-683 ◽

Cited By ~ 51

Author(s):

MICHAEL L. HAAG ◽

FRANK L. GLASS ◽

NEIL A. FENSKE

Keyword(s):

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Diagnosis And Treatment ◽

Merkel Cell

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Diagnosis and Treatment of Merkel Cell Carcinoma in Specialized Dermatology Units: A Clinical Practice Guideline of the Spanish Academy of Dermatology and Venereology

Actas Dermo-Sifiliográficas (English Edition) ◽

10.1016/j.adengl.2019.01.017 ◽

2019 ◽

Vol 110 (6) ◽

pp. 460-468

Author(s):

J. Vázquez Doval ◽

B. Llombart Cussac ◽

A. Pérez Bustillo ◽

S. Paradela de la Morena ◽

M.J. Fuente González ◽

...

Keyword(s):

Clinical Practice ◽

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Clinical Practice Guideline ◽

Practice Guideline ◽

Diagnosis And Treatment ◽

Merkel Cell

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Activity of ipilimumab plus nivolumab in avelumab-refractory Merkel cell carcinoma

Cancer Immunology Immunotherapy ◽

10.1007/s00262-020-02832-0 ◽

2021 ◽

Author(s):

Valerie Glutsch ◽

Hermann Kneitz ◽

Anja Gesierich ◽

Matthias Goebeler ◽

Sebastian Haferkamp ◽

...

Keyword(s):

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Treatment Options ◽

Molecular Data ◽

High Response Rate ◽

Merkel Cell ◽

Platinum Based Chemotherapy ◽

Grade Ii ◽

Third Line ◽

Combined Immunotherapy

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.

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Avelumab in the treatment of patients with merkel cell carcinoma: initial results of early access program in Russia

Malignant tumours ◽

10.18027/2224-5057-2019-9-1-53-58 ◽

2019 ◽

Vol 9 (1) ◽

pp. 53-58

Author(s):

K. V. Orlova ◽

V. V. Nazarova ◽

N. N. Petenko ◽

L. V. Demidov

Keyword(s):

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Immune Checkpoint Inhibitors ◽

Checkpoint Inhibitors ◽

Treatment Options ◽

Locally Advanced ◽

Merkel Cell ◽

Early Access ◽

Access Program ◽

Initial Results

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.

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