Diagnosis and treatment of Merkel Cell Carcinoma. European consensus-based interdisciplinary guideline

Lebbe, Célèste; Becker, Jürgen C.; Grob, Jean-Jacques; Malvehy, Josep; Marmol, Véronique Del; Pehamberger, Hubert; Peris, Ketty; Saïag, Philippe; Middleton, Mark R.; Bastholt, Lars; Testori, Alessandro; Stratigos, Alexander J.; Garbe, Claus

doi:10.1016/j.ejca.2015.06.131

Cited by 353 publications

(289 citation statements)

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“…In addition, the patient population in the Iyer et al study had received no more than two lines of chemotherapy for metastatic disease [12], whereas the current study included patients who may have received more than two lines during the course of their treatment. Thus, the results of the current study add to the weight of evidence that although MCC is generally considered to be a chemosensitive tumor, the DOR is often short [9,[11][12][13][14]17,29] and associated with poor survival outcomes [12,15]. This highlights the need for new treatment options that improve the prognosis for patients with this aggressive tumor type.…”

Section: Discussionmentioning

confidence: 72%

“…The National Comprehensive Cancer Network treatment guidelines for mMCC [9] are based on those used for small cell lung cancer, as both are aggressive and poorly differentiated cancers [10]. Treatments typically include platinum agents, such as carboplatin or cisplatin with or without etoposide or topotecan, and are associated with high toxicity [9,11]. Although MCC is generally considered a chemosensitive tumor, responses to chemotherapy in metastatic disease are often not durable: response rates in the secondline (2L) setting range from 23% in patients with known distant metastasis to 45% in patients with unclear (nodal and/or distant) sites of metastasis [12,13].…”

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confidence: 99%

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Real-world treatment outcomes in patients with metastatic Merkel cell carcinoma treated with chemotherapy in the USA

et al. 2017

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aim: This retrospective study of patients in the USA with metastatic Merkel cell carcinoma (mMCC) aimed to assess patient responses to second-line and later (2L+) and first-line (1L) chemotherapy. Patients & methods: Out of 686 patients with MCC identified in The US Oncology Network, 20 and 67 patients with mMCC qualified for the 2L+ and 1L study, respectively; the primary analysis population was restricted to immunocompetent patients. Results: In the 2L+ primary analysis population, objective response rate (ORR) was 28.6%, median duration of response (DOR) was 1.7 months and median progression-free survival was 2.2 months. In the 1L primary analysis population, ORR was 29.4%, median DOR was 6.7 months and median progression-free survival was 4.6 months. conclusion: The low ORR and brief DOR underscore the need for novel therapies. Merkel cell carcinoma (MCC) is a rare, aggressive skin cancer that occurs most frequently in elderly and immunocompromised patients [1][2][3]. There are approximately 1500 cases of MCC per year in the USA, and the incidence has dramatically increased over the last 20 years [4]. MCC typically presents as painless growths that are clinically unremarkable in appearance and are usually found on sun-exposed areas, such as the head and neck [2,3]. These tumors grow rapidly and tend to metastasize early and frequently to local regions of the body, leading to a relatively poor prognosis with this aggressive disease [2,3,5]. Among patients diagnosed with local or regional disease, the reported rates of recurrence range from 43 to 48% [6,7]. The 5-year overall survival (OS) rate is 40% [1] and the mortality rate with MCC is greater than that with other skin cancers, including melanoma [4].Recently, avelumab, a human IgG1 anti-programmed death-ligand 1 (PD-L1) monoclonal antibody, was approved by the US FDA as the first and only approved treatment for patients with metastatic MCC (mMCC) [8]. Before this approval, there was no evidence-based standard therapeutic regimen for mMCC. The National Comprehensive Cancer Network treatment guidelines for mMCC [9] are based on those used for small cell lung cancer, as both are aggressive and poorly differentiated cancers [10]. Treatments typically include platinum agents, such as carboplatin or cisplatin with or without etoposide or topotecan, and are associated with high toxicity [9,11]. Although MCC is generally considered a chemosensitive tumor, responses to chemotherapy in metastatic disease are For reprint orders, please contact: reprints@futuremedicine.com

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Section: Discussionmentioning

confidence: 72%

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confidence: 99%

Real-world treatment outcomes in patients with metastatic Merkel cell carcinoma treated with chemotherapy in the USA

et al. 2017

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“…The preferred treatment for in-transit metastasis is surgical excision if feasible, and the usual treatment for metastatic MCC is chemotherapy. While no standard systemic therapy regimen exists for MCC, there are several chemotherapy regimens that are also active against small cell lung cancer, such as combinations of platinum drugs and etoposide or single-agent topotecan 73 74…”

Section: Current Treatment Recommendationsmentioning

confidence: 99%

Molecular characteristics and potential therapeutic targets in Merkel cell carcinoma

et al. 2016

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Merkel cell carcinoma (MCC) is an aggressive neuroendocrine skin tumour occurring preferentially in elderly and immunosuppressed individuals. Multiple studies have provided insight into the molecular alterations of MCC, leading to the design of several ongoing clinical trials testing chemotherapy, targeted therapy and immunotherapy in patients with recurrent or metastatic disease. The results of some of these studies are available, whereas others are eagerly awaited and will likely shed light on the understanding of MCC biology and potentially improve the clinical outcomes of patients with this rare disease.

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“…Although is not common that these tumors are cured, we should perform follow-up post-treatment with physical examination and locoregional ultrasound test every 4 months for the first 3 years and every 6 months to reach 5 years, with CT or PET-CT annually during these first 5 years accordingly to European interdisciplinary consensus-based guidelines [18]. National Cancer Comprehensive Network guidelines suggest similar recommendations for the follow-up post-treatment although they don´t mention ultrasonography role neither establish any specific frequency for image studies [19].…”

Section: Discussionmentioning

confidence: 99%

Retroperitoneal Merkel Cell Carcinoma without Skin Involvement: Literature Review and One Case Experience

Sota¹,

Vila²,

Giménez³

et al. 2017

Ann Clin Lab Res

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Merkel cell carcinoma (MCC) is an uncommon neuroendocrine tumor that represents a small percentage of skin malignancies, with an aggressive behavior and survival rate at 5 years of 20% to 80%. Among the risk factors, we find Ultraviolet radiation, polyomavirus, other malignancies and immunosuppression.Most of the MCC are diagnosed over 59 years old, being more frequent in men. According to the literature, only 2% to 5% had no skin involvement. Among regions involved, head and neck are the most frequent, followed by limbs and lymph nodes.We described a case of MCC without skin involvement, atypically located in retroperitoneum and given the rarity of both circumstances, we also conducted a review of the reported cases of retroperitoneal MCC without skin tumor.Among retroperitoneal MCC without primary skin involvement, we found 13 reported cases, the average age of these patients was 66 years old, being female only one case. They were all diagnosed by computerized tomography (CT) or Positron emission tomographycomputed tomography (PET-CT). The size of the main mass was variable (15 cm to 3 cm) and selected treatments were chemotherapy, surgery and radiation therapy. Survival rate is unknown in most of them.

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Diagnosis and treatment of Merkel Cell Carcinoma. European consensus-based interdisciplinary guideline

Cited by 353 publications

References 41 publications

Real-world treatment outcomes in patients with metastatic Merkel cell carcinoma treated with chemotherapy in the USA

Real-world treatment outcomes in patients with metastatic Merkel cell carcinoma treated with chemotherapy in the USA

Molecular characteristics and potential therapeutic targets in Merkel cell carcinoma

Retroperitoneal Merkel Cell Carcinoma without Skin Involvement: Literature Review and One Case Experience

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