scholarly journals Merkel Cell Carcinoma from Molecular Pathology to Novel Therapies

2021 ◽  
Vol 22 (12) ◽  
pp. 6305
Author(s):  
Karolina Stachyra ◽  
Monika Dudzisz-Śledź ◽  
Elżbieta Bylina ◽  
Anna Szumera-Ciećkiewicz ◽  
Mateusz J. Spałek ◽  
...  

Merkel cell carcinoma (MCC) is an uncommon and highly aggressive skin cancer. It develops mostly within chronically sun-exposed areas of the skin. MCPyV is detected in 60–80% of MCC cases as integrated within the genome and is considered a major risk factor for MCC. Viral negative MCCs have a high mutation burden with a UV damage signature. Aberrations occur in RB1, TP53, and NOTCH genes as well as in the PI3K-AKT-mTOR pathway. MCC is highly immunogenic, but MCC cells are known to evade the host’s immune response. Despite the characteristic immunohistological profile of MCC, the diagnosis is challenging, and it should be confirmed by an experienced pathologist. Sentinel lymph node biopsy is considered the most reliable staging tool to identify subclinical nodal disease. Subclinical node metastases are present in about 30–50% of patients with primary MCC. The basis of MCC treatment is surgical excision. MCC is highly radiosensitive. It becomes chemoresistant within a few months. MCC is prone to recurrence. The outcomes in patients with metastatic disease are poor, with a historical 5-year survival of 13.5%. The median progression-free survival is 3–5 months, and the median overall survival is ten months. Currently, immunotherapy has become a standard of care first-line therapy for advanced MCC.

2020 ◽  
Vol 231 (4) ◽  
pp. S286
Author(s):  
Michael J. Minarich ◽  
Elizabeth A. Handorf ◽  
Leah G. Stockton ◽  
Maitham A. Moslim ◽  
Stephanie H. Greco ◽  
...  

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Aldo V. Londino III ◽  
Brett A. Miles

Merkel cell carcinoma (MCC) is an uncommon neuroendocrine malignancy with a propensity for the head and neck. It typically presents asymptomatically in elderly Caucasians and is characterized by early local and regional spread. There is currently limited data on the appropriate algorithm for treatment of MCC. However, multimodal therapy with wide surgical excision with or without radiation therapy has become standard of care. The location of the primary tumor and intensive adjuvant therapy is often required, provides a challenge to the reconstructive head and neck surgeon. Occasionally, free tissue transfer reconstructive techniques are employed in the reconstruction of MCC defects. This paper will discuss the role of free tissue transfer as a reconstructive option after surgery for advanced head and neck MCC.


Author(s):  
Deborah Yihler ◽  
Kathrin Vollmer ◽  
Antonio Cozzio

ABSTRACT Merkel cell carcinoma (MCC) is a rare and difficult-to-treat cutaneous malignancy with a poor prognosis. Treatment protocols for localized MCC are well established. Until recently, metastatic MCC has generally been treated with chemotherapy, which was often associated with poor clinical responses and significant toxicity. In this report, the case of a patient with metastatic MCC who received avelumab, an immune checkpoint inhibitor, after an inadequate response to first-line radiotherapy and chemotherapy, is presented. Nine months after the initiation of the treatment with avelumab, the patient achieved a partial remission with no treatment-related adverse events. After a follow-up of 17 months, a systematically ongoing partial response was reported. In conclusion, this case study offers a clinical insight into the patient’s case and highlights the importance of immunotherapy as a first-line therapy for metastatic MCC.


2014 ◽  
Vol 104 (4) ◽  
pp. 422-425 ◽  
Author(s):  
Dominic J. Roda ◽  
Brian Albano ◽  
Bharti Rathore ◽  
Linda Zhou

Merkel cell carcinoma is a rare, often fatal, neuroendocrine cutaneous malignancy with a highly variable clinical presentation. Due to the rapid progression of the disease, early detection and treatment is vital to survival. Here, we report the case of a 45-year-old woman whose podiatrist noticed an unusual lesion on her lower left leg and referred her to a dermatologist for work-up. A diagnostic excisional biopsy confirmed the diagnosis and was followed by treatment that included wide local excision of the primary lesion with subsequent chemotherapy and radiation. At the time of diagnosis, sentinel lymph node biopsy was positive. Due to the increasing prevalence of Merkel cell carcinoma in the past decade and its propensity to present on the lower extremity, podiatric physicians need to be aware of the clinical presentation and treatment guidelines for this elusive disease.


2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Eric P. Arruda ◽  
Kevin M. Higgins

Merkel cell carcinoma (MCC) is a rare and typically aggressive form of skin cancer. It most commonly affects the elderly and has a predilection for the sun-exposed skin of the head and neck region. Other etiological factors include immune suppression, organ transplantation, and polyoma virus infection. MCC has a propensity to spread to regional lymphatics with a high locoregional recurrence rate. Since its discovery in 1972, treatment paradigms have shifted, with no consensus on optimal management strategies. Currently, standard of care includes surgical intervention to the primary and locoregional site with adjuvant radiotherapy for high-risk disease. In this paper, we discuss the history, pathology, and epidemiology of this rare disease with a focus on the evidentiary basis of treatment protocols. The use of sentinel lymph node biopsy as a management option will be the focus of this paper.


2015 ◽  
Vol 33 (29) ◽  
pp. 3338-3345 ◽  
Author(s):  
Sandro V. Porceddu ◽  
Michael J. Veness ◽  
Alexander Guminski

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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