Merkel cell carcinoma: The prognostic implications of an occult primary in stage IIIB (nodal) disease

Foote, Matthew; Veness, Michael J.; Zarate, Dannie; Poulsen, Michael

doi:10.1016/j.jaad.2011.09.009

Cited by 70 publications

(48 citation statements)

References 31 publications

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“…Recent publications of MCCs with an unknown primary site found that patients with occult tumors have significantly improved MCC outcomes (recurrence and survival). 18,19 One possible explanation for the improved outcome of occult MCCs is that MCCs are highly immunogenic tumors and may undergo spontaneous T-cell–mediated regression. Alternatively, neuroendocrine tumors may arise in other noncutaneous organs and those MCCs may have improved outcomes compared with those of cutaneously derived MCCs.…”

Section: Discussionmentioning

confidence: 99%

Effect of Host, Tumor, Diagnostic, and Treatment Variables on Outcomes in a Large Cohort With Merkel Cell Carcinoma

et al. 2014

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IMPORTANCE Merkel cell carcinoma (MCC) is a rare, aggressive, neuroendocrine-derived skin cancer with high rates of recurrence and associated mortality. Few published studies have used comprehensive patient data and long-term follow-up to examine factors that predict MCC outcomes. OBJECTIVE To characterize MCC in a large defined-population cohort and analyze predictors of disease recurrence and survival. SETTING, DESIGN, AND PARTICIPANTS Retrospective cohort study of 218 patients with MCC from the cancer registry of Kaiser Permanente Northern California, a large integrated health care delivery system. Patients were diagnosed as having MCC and followed up from January 1, 1995, through December 31, 2009. We examined host (age, sex, race, and immunosuppression), tumor (anatomic site, size, and extent), diagnostic (results of imaging and pathologic nodal evaluation), and treatment (surgery, radiation therapy, and chemotherapy) variables for their association with MCC outcomes. EXPOSURE Host, tumor, diagnostic, and treatment factors. MAIN OUTCOMES AND MEASURES Recurrence (locoregional and distant) of MCC and patient survival (overall and MCC specific). RESULTS We estimated adjusted hazard ratios (AHRs) and 95% CIs for outcomes using Cox proportional hazards regression models. After adjustment for host, tumor, diagnostic, and treatment variables, tumor extent (categorized as local, regional, and distant) remained significantly associated with all outcomes. Immunosuppression was associated with higher MCC-specific mortality (AHR, 4.9 [95% CI, 1.7–14.4]), and an unknown primary site was associated with a lower risk for distant metastasis (0.1 [0.0–0.7]) and improved survival (0.4 [0.2–0.9]). Pathological nodal evaluation was associated with a lower risk for metastasis (AHR, 0.2 [95% CI, 0.0–1.0]) and improved survival. Radiation treatment was associated with a decreased risk for locoregional recurrence (AHR, 0.3 [95% CI, 0.1–0.6]), whereas chemotherapy was not associated with any alteration in outcomes. CONCLUSIONS AND RELEVANCE Tumor site and extent, results of pathologic nodal evaluation, and the presence of radiation treatment were associated with MCC recurrence. Immunosuppression, tumor extent, and results of pathologic nodal evaluation were associated with MCC-specific survival, whereas chemotherapy was not associated with any outcomes. Our findings may help to inform diagnostic and therapeutic management of MCCs.

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Section: Discussionmentioning

confidence: 99%

Effect of Host, Tumor, Diagnostic, and Treatment Variables on Outcomes in a Large Cohort With Merkel Cell Carcinoma

et al. 2014

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“…While rare, spontaneous regression in MCC is much more common (1.3 per 1000 cases) than in other malignancies (1 in 60,000-100,000 cases) [38]. Furthermore, a subset of advanced stage MCC patients present with unknown primary tumors (no primary skin lesions are detectable) likely as the result of immune-mediated clearance of the primary lesion and these patients have markedly improved overall and disease-specific s urvival [39].…”

Section: Immune Response Against MCCmentioning

confidence: 99%

Rationale For Immune-Based Therapies in Merkel Polyomavirus-Positive and -Negative Merkel Cell Carcinomas

Vandeven

Nghiem

2016

Immunotherapy

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Merkel cell carcinoma (MCC) is a rare but often deadly skin cancer that is typically caused by the Merkel cell polyomavirus (MCPyV). Polyomavirus T-antigen oncoproteins are persistently expressed in virus-positive MCCs (∼80% of cases), while remarkably high numbers of tumor-associated neoantigens are detected in virusnegative MCCs, suggesting that both MCC subsets may be immunogenic. Here we review mechanisms by which these immunogenic tumors evade multiple levels of host immunity. Additionally, we summarize the exciting potential of diverse immunebased approaches to treat MCC. In particular, agents blocking the PD-1 axis have yielded strikingly high response rates in MCC as compared with other solid tumors, highlighting the potential for immune-mediated treatment of this disease.

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“…Neuroendocrine Merkel cell carcinoma of unknown primary (MCCUP) represents a manifestation of MCCUP. In large retrospective studies the incidence of UPMCC among MCC population ranged from 19-25% [8][9][10].…”

Section: Introductionmentioning

confidence: 99%

Neuroendocrine Merkel cell nodal carcinoma of unknown primary site: Management and outcomes of a rare entity

Κοττέας

Pavlidis

2015

Critical Reviews in Oncology/Hematology

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Merkel cell carcinoma: The prognostic implications of an occult primary in stage IIIB (nodal) disease

Cited by 70 publications

References 31 publications

Effect of Host, Tumor, Diagnostic, and Treatment Variables on Outcomes in a Large Cohort With Merkel Cell Carcinoma

Effect of Host, Tumor, Diagnostic, and Treatment Variables on Outcomes in a Large Cohort With Merkel Cell Carcinoma

Rationale For Immune-Based Therapies in Merkel Polyomavirus-Positive and -Negative Merkel Cell Carcinomas

Neuroendocrine Merkel cell nodal carcinoma of unknown primary site: Management and outcomes of a rare entity

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