Merkel Cell Carcinoma, Version 1.2018, NCCN Clinical Practice Guidelines in Oncology

Bichakjian, Christopher K.; Olencki, Thomas; Aasi, Sumaira Z.; Alam, Murad; Andersen, James S.; Blitzblau, Rachel; Bowen, Glen M.; Contreras, Carlo M.; Daniels, Gregory A.; Decker, Roy H.; Farma, Jeffrey M.; Fisher, Kris; Gastman, Brian; Ghosh, Karthik; Grekin, Roy C.; Grossman, Kenneth; Ho, Alan L.; Lewis, Karl D.; Loss, Manisha J.; Lydiatt, Daniel D.; Messina, Jane L.; Nehal, Kishwer S.; Nghiem, Paul; Puzanov, Igor; Schmults, Chrysalyne D.; Shaha, Ashok R.; Thomas, Valencia D.; Xu, Yaohui; Zic, John A.; Hoffmann, K; Engh, Anita M.

doi:10.6004/jnccn.2018.0055

Cited by 215 publications

(346 citation statements)

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“…Prior to development of distant metastatic disease, initial therapy of the patients in this study cohort typically followed National Comprehensive Cancer Network guidelines . One exception was that patients treated at our facility who had risk factors associated with local and regional MCC recurrence were more likely to receive adjuvant radiation than is typical, according to data from the National Cancer Database .…”

Section: Discussionmentioning

confidence: 99%

“…However, data regarding the timing and pattern of distant MCC metastases are scarce limited to case reports and small series . Therefore, existing surveillance guidelines for metastatic MCC are not evidence based, which leads to vague and inconsistent management recommendations across institutions . Evidence‐based standardization of surveillance practices could facilitate efficient use of resources and earlier detection of metastases.…”

Section: Introductionmentioning

confidence: 99%

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Patterns of distant metastases in 215 Merkel cell carcinoma patients: Implications for prognosis and surveillance

et al. 2019

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Approximately one‐third of Merkel cell carcinoma (MCC) patients eventually develop distant metastatic disease. Little is known about whether the location of the primary lesion is predictive of initial distant metastatic site, or if survival likelihood differs depending on the metastatic site. Such data could inform imaging/surveillance practices and improve prognostic accuracy. Multivariate and competing‐risk analyses were performed on a cohort of 215 MCC patients with distant metastases, 31% of whom had two or more initial sites of distant metastasis. At time of initial distant metastasis in the 215 patients, metastatic sites (n = 305) included non‐regional lymph nodes (present in 41% of patients), skin/body wall (25%), liver (23%), bone (21%), pancreas (8%), lung (7%), and brain (5%). Among the 194 patients who presented with MCC limited to local or regional sites (stage I‐III) but who ultimately developed distant metastases, distant progression occurred in 49% by 1 year and in 80% by 2 years following initial diagnosis. Primary MCC locations differed in how likely they were to metastasize to specific organs/sites (P < .001). For example, liver metastases were far more likely from a head/neck primary (43% of 58 patients) versus a lower limb primary (5% of 39 patients; P < .0001). Skin‐only distant metastasis was associated with lower MCC‐specific mortality as compared to metastases in multiple organs/sites (HR 2.7; P = .003), in the liver (HR 2.1; P = .05), or in distant lymph nodes (HR 2.0; P = .045). These data reflect outcomes before PD1‐pathway inhibitor availability, which may positively impact survival. In conclusion, primary MCC location is associated with a pattern of distant spread, which may assist in optimizing surveillance. Because it is linked to survival, the site of initial distant metastasis should be considered when assessing prognosis.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Patterns of distant metastases in 215 Merkel cell carcinoma patients: Implications for prognosis and surveillance

et al. 2019

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“…For the initial management of MCC, the National Comprehensive Cancer Network (NCCN) guidelines recommend wide local excision of the primary tumor and sentinel lymph node biopsy . On the basis of the histopathologic findings and resulting staging, lymphadenectomy and adjuvant radiation therapy may follow.…”

Section: Discussionmentioning

confidence: 99%

“…INCMGA00012 (MGA012), an antibody which structurally and functionally resembles pembrolizumab and nivolumab, is also undergoing a phase II clinical trial for MCC (NCT03599713). The success of PD‐1 axis checkpoint inhibitors has led to the recommendation of avelumab, pembrolizumab, and nivolumab for metastatic MCC in the 2018 NCCN guidelines …”

Section: Discussionmentioning

confidence: 99%

Molecular and immune targets for Merkel cell carcinoma therapy and prevention

Cohen

Tsai

2019

Molecular Carcinogenesis

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Merkel cell carcinoma (MCC) is a rare neuroendocrine carcinoma of the skin, for which the exact mechanisms of carcinogenesis remain unknown. Therapeutic options for this highly aggressive malignancy have historically been limited in both their initial response and response durability. Recent improvements in our understanding of MCC tumor biology have expanded therapeutic options for these patients, namely through the use of immunotherapies such as immune checkpoint inhibitors. Further elucidation of the tumor mutational landscape has identified molecular targets for therapies, which have demonstrated success in other cancer types. In this review, we discuss both current and investigational immune and molecular targets of therapy for MCC.

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“…Wide excision is recommended, aiming to achieve 1–2‐cm clear margins when feasible . However, surgical margins should be balanced with morbidity of surgery, and undue delay in proceeding to radiation therapy should be avoided . Mohs micrographic surgery can be useful to ensure clear margins, particularly for in‐depth excision or when cosmetic results are important .…”

Section: Treatment Of Patients With Early‐stage MCC (N0 M0)mentioning

confidence: 99%

Recent Therapeutic Advances and Change in Treatment Paradigm of Patients with Merkel Cell Carcinoma

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Merkel cell carcinoma (MCC) is a rare, aggressive, primary cutaneous neuroendocrine tumor that typically presents as an indurated nodule on sun‐exposed areas of the head and neck in the white population. Major risk factors include immunosuppression, UV light exposure, and advanced age. Up to 80% of MCC are associated with Merkel cell polyomavirus. About 50% of patients present with localized disease, and surgical resection with or without adjuvant radiotherapy is generally indicated in this context. However, recurrence rates are high and overall prognosis rather poor, with mortality rates of 33%–46%. MCC is a chemosensitive disease, but responses in the advanced setting are seldom durable and not clearly associated with improved survival. Several recent trials with checkpoint inhibitors (pembrolizumab, avelumab, nivolumab) have shown very promising results with a favorable safety profile, in both chemonaïve and pretreated patients. In 2017, avelumab was approved by several regulatory agencies for the treatment of metastatic MCC, the first drug to be approved for this orphan disease. More recently, pembrolizumab has also been approved by the U.S. Food and Drug Administration in this setting. Immunotherapy has therefore become the new standard of care in advanced MCC. This article reviews current evidence and recommendations for the diagnosis and treatment of MCC and discusses recent therapeutic advances and their implications for care in patients with advanced disease. This consensus statement is the result of a collaboration between the Spanish Cooperative Group for Neuroendocrine Tumors, the Spanish Group of Treatment on Head and Neck Tumors, and the Spanish Melanoma Group. Implications for Practice Merkel cell carcinoma (MCC) is an uncommon aggressive skin cancer associated with advanced age, UV light exposure, and immunosuppression. Up to 80% are associated with Merkel cell polyomavirus. MCC is a chemosensitive disease, but tumor responses in the advanced setting are short‐lived with no long‐term survivors. Recent clinical trials with immune checkpoint inhibitors (i.e., pembrolizumab, avelumab, nivolumab) have shown promising results, with avelumab becoming the first drug to receive regulatory approval for this orphan indication. Further follow‐up is needed, however, to define more adequately the long‐term benefits of these drugs, and continued research is warranted to optimize immunotherapeutic strategies in this setting.

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Merkel Cell Carcinoma, Version 1.2018, NCCN Clinical Practice Guidelines in Oncology

Cited by 215 publications

References 210 publications

Patterns of distant metastases in 215 Merkel cell carcinoma patients: Implications for prognosis and surveillance

Patterns of distant metastases in 215 Merkel cell carcinoma patients: Implications for prognosis and surveillance

Molecular and immune targets for Merkel cell carcinoma therapy and prevention

Recent Therapeutic Advances and Change in Treatment Paradigm of Patients with Merkel Cell Carcinoma

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