Diagnosis and treatment of Merkel cell carcinoma: European consensus-based interdisciplinary guideline – Update 2022

Gauci, Marie-Léa; Aristei, Cynthia; Becker, Jürgen C.; Blom, Astrid; Bataille, Véronique; Dréno, Brigitte; Marmol, Véronique Del; Forsea, Ana Maria; Fargnoli, Maria Concetta; Grob, Jean‐Jacques; Gomes, Fábio; Hauschild, Axel; Höeller, Christoph; Harwood, Catherine A.; Kelleners‐Smeets, Nicole W.J.; Kaufmann, Roland; Lallas, Aimilios; Malvehy, Josep; Moreno‐Ramírez, D.; Peris, Ketty; Pellacani, Giovanni; Saïag, Philippe; Stratigos, Alexander J.; Vieira, Ricardo; Zalaudek, Iris; Akkooi, A.C.J. van; Lorigan, Paul; Garbe, Claus; Lebbe, Célèste

doi:10.1016/j.ejca.2022.03.043

Cited by 91 publications

(164 citation statements)

References 186 publications

(243 reference statements)

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“…Chemotherapeutic agents such as platinum‐based drugs, etoposide, doxorubicin, taxanes, and anthracyclins, either alone or in combination, are used for tumor management, despite improvements in terms of OS of MCC patients being limited 31 . The most effective chemotherapeutic regimen for MCC management is the combination of etoposide and platinum 32 . As the metastatic stage occurs in 40%−50% of patients, chemotherapy is advised in perioperative case settings or in inoperable and metastatic MCC 33,34 .…”

Section: Causes Therapy Diagnosis and Prognosismentioning

confidence: 99%

“…Sentinel lymph node biopsy (SLNB) is recommended as a routine staging approach in patients with primary clinical stage I−II, as an improved overall survival (OS) is observable in patients undergoing this approach. 32 Until the advent of immunomodulatory therapies, radiotherapy (RT) represented the main approach for MCC patient management. Indeed, upon the surgical removal of the lesion, adjuvant RT can improve disease-, local/ regional relapse-and distant metastasis-free survivals as well as OS compared to surgery alone without adjuvant RT.…”

Section: Causes Therapy Diagnosis and Prognosismentioning

confidence: 99%

“…However, evidence indicates that MCCP-affected patients are characterized by a more favorable prognosis compared to patients suffering from MCCN. 32…”

Section: Causes Therapy Diagnosis and Prognosismentioning

confidence: 99%

“…Moreover, MCCs express the neuroendocrine marker neuron‐specific enolase (HGNC:3350), despite being expressed by other neuroendocrine tumors. MCC‐negative markers which are typically expressed by other malignancies comprise (i) premelanosome protein (PMEL or HMB‐45) (HGNC:10880) and proteins from the S‐100 family, which are melanoma‐specific markers; (ii) thyroid transcription factor‐1 (TTF‐1 or NK2 homeobox 1 [NKX2‐1]) (HGNC:11825), expressed in up to 90% of small cell lung carcinomas; (iii) various lymphomas‐specific lymphocyte markers; (iv) carcinoembryonic antigen, which comprise several highly related glycoproteins, and keratin 7 (HGNC:6445), expressed by sweat gland carcinomas 32 . An exhaustive MCC diagnosis provides the tumor histological assessment accompanied by a positive keratin 20 and negative TTF‐1 immunostaining.…”

Section: Causes Therapy Diagnosis and Prognosismentioning

confidence: 99%

“…The extracutaneous extension of the primary tumor is another important prognostic predictor, since an increased tumor thickness has been related to a poor disease‐free survival 37,38 . A poor outcome has been related to lymphovascular invasion 32 . An important independent prognostic predictor is the lymph node status as the nodal involvement occurs in ∼30% of MCC patients.…”

Section: Causes Therapy Diagnosis and Prognosismentioning

confidence: 99%

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MicroRNA dysregulations in Merkel cell carcinoma: Molecular mechanisms and clinical applications

Mazziotta

Cervellera

Lanzillotti

et al. 2022

Journal of Medical Virology

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Merkel cell carcinoma (MCC) is an aggressive skin malignancy with two distinct etiologies. The first, which accounts for the highest proportion, is caused by Merkel cell polyomavirus (MCPyV), a DNA tumor virus. A second, UV‐induced, MCC form has also been identified. Few MCC diagnostic, prognostic, and therapeutic options are available. MicroRNAs (miRNAs) are small noncoding RNA molecules, which play a key role in regulating various physiologic cellular functions including cell cycling, proliferation, differentiation, and apoptosis. Numerous miRNAs are dysregulated in cancer, by acting as either tumor suppressors or oncomiRs. The aim of this review is to collect, summarize, and discuss recent findings on miRNAs whose dysregulation has been assumed to play a role in MCC. The potential clinical application of miRNAs as diagnostic and prognostic biomarkers in MCC is also described. In the future, miRNAs will potentially gain clinical significance for the improvement of MCC diagnostic, prognostic, and therapeutic options.

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Section: Causes Therapy Diagnosis and Prognosismentioning

confidence: 99%

Section: Causes Therapy Diagnosis and Prognosismentioning

confidence: 99%

“…However, evidence indicates that MCCP-affected patients are characterized by a more favorable prognosis compared to patients suffering from MCCN. 32…”

Section: Causes Therapy Diagnosis and Prognosismentioning

confidence: 99%

Section: Causes Therapy Diagnosis and Prognosismentioning

confidence: 99%

Section: Causes Therapy Diagnosis and Prognosismentioning

confidence: 99%

See 3 more Smart Citations

MicroRNA dysregulations in Merkel cell carcinoma: Molecular mechanisms and clinical applications

Mazziotta

Cervellera

Lanzillotti

et al. 2022

Journal of Medical Virology

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Avelumab treatment for patients with metastatic Merkel cell carcinoma can be safely stopped after 1 year and a PET/CT–confirmed complete response

Zijlker,

Levy,

Wolters

et al. 2023

Cancer

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BackgroundImmune checkpoint inhibitor treatment of patients with metastatic Merkel cell carcinoma (mMCC) has shown high response rates, ranging from 33% to 73%. The ideal duration of treatment, however, is currently unknown. The aim of this study was to evaluate if avelumab treatment for mMCC can be safely stopped after 1 year of treatment and a complete response (CR) confirmed by fluorodeoxyglucose positron emission tomography/computed tomography (FDG‐PET/CT) imaging.MethodsPatients who received more than one dose of avelumab treatment for mMCC between November 2017 and February 2022 were included in this study. Treatment was discontinued in case of a FDG‐PET/CT confirmed CR after 1 year (26 cycles) of avelumab or a CR and unacceptable toxicity earlier. The primary end point was recurrence‐free survival (RFS).ResultsSixty‐five patients were included: 25 (38%) had a FDG‐PET/CT–confirmed CR at discontinuation of avelumab. In those 25 patients, reasons for discontinuation of treatment were completion of 1 year of treatment in 13 (52%), toxicity in five (20%), and patient preference in seven (28%). Median duration of treatment in this group was 11 months (interquartile range, 6.1–11.7). Median follow‐up was 27 months (interquartile range, 15.8–33.8). The 12‐month RFS was 88% (95% CI, 0.74–1) and median RFS was not reached. Two patients (8%) had a recurrence at 4 and 7 months after discontinuation of treatment.ConclusionsPatients with mMCC who acquire a CR on PET/CT imaging appear to have durable responses after discontinuation of treatment after 1 year.

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Brazilian Society of Surgical Oncology recommendations on Merkel cell carcinoma surgical treatment

Lobo,

Wainstein,

Barros

et al. 2024

Journal of Surgical Oncology

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BackgroundMerkel cell carcinoma (MCC) is a rare neuroendocrine skin cancer with poor 5‐year survival rates. Surgery and radiation are the current first‐line treatments for local and nodal disease.ObjectivesThe Brazilian Society of Surgical Oncology developed this document aiming to guide the surgical oncology role in multimodal MCC management.MethodsThe consensus was established in three rounds of online discussion, achieving consensus on specific topics including diagnosis, staging, treatment, and follow‐up.ResultsPatients suspected of having MCC should undergo immunohistochemical examination and preferably undergo pathology review by a dermatopathologist. Initial staging should be performed with dermatologic and nodal physical examination, combined with complementary imaging. Whole‐body imaging, preferably with positron emission tomography (PET) or computed tomography (CT) scans, are recommended. Due to the need for multidisciplinary approaches, we recommend that all cases should be discussed in tumor boards and referred to other specialties as soon as possible, reducing potential treatment delays. We recommend that all patients with clinical stage I or II may undergo local excision associated with sentinel lymph node biopsy. The decision on margin size should consider time to recovery, patient's comorbidities, and risk factors. Patients with positive sentinel lymph nodes or the presence of risk factors should undergo postoperative radiation therapy at the primary site. Exclusive radiation is a viable option for patients with low performance. Patients with positive sentinel lymph node biopsy should undergo nodal radiation therapy or lymphadenectomy. In patients with nodal clinical disease, in addition to primary tumor treatment, nodal radiation therapy and/or lymphadenectomy are recommended. Patients with advanced disease should preferably be enrolled in clinical trials and discussed in multidisciplinary meetings. The role of surgery and radiation therapy in the metastatic/advanced setting should be discussed individually and always in tumor boards.ConclusionThis document aims to standardize a protocol for initial assessment and treatment for Merkel cell carcinoma, optimizing oncologic outcomes in middle‐income countries such as Brazil.

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Diagnosis and treatment of Merkel cell carcinoma: European consensus-based interdisciplinary guideline – Update 2022

Cited by 91 publications

References 186 publications

MicroRNA dysregulations in Merkel cell carcinoma: Molecular mechanisms and clinical applications

MicroRNA dysregulations in Merkel cell carcinoma: Molecular mechanisms and clinical applications

Avelumab treatment for patients with metastatic Merkel cell carcinoma can be safely stopped after 1 year and a PET/CT–confirmed complete response

Brazilian Society of Surgical Oncology recommendations on Merkel cell carcinoma surgical treatment

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