Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine tumor of the skin. Risk factors include extensive sun damage, infection with Merkel cell polyomavirus, and an immunocompromised state. PRAME, also known as preferentially expressed antigen in melanoma, is a cancer-testis antigen recently found to be a useful diagnostic tool in the workup of melanocytic neoplasms. However, the expression pattern of PRAME in Merkel cell carcinoma is unknown. In this study, we examine PRAME expression in Merkel cell carcinoma and explore its prognostic implications. The institutional archives at the University of Virginia were used to search for tumors classified as Merkel cell carcinoma from 2004 to 2022. All potential cases were reviewed to confirm the diagnosis, and electronic medical records were searched for clinical and demographic data. Tumors were subsequently immunostained for PRAME and Merkel cell polyomavirus. Cox proportional hazards regression models were used to estimate relative (all-cause) survival of PRAME positivity and MCPyV positivity in our study as well as MCC-specific survival of PRAME positivity. Univariate and multivariable models were created for each outcome related to all-cause survival. A total of 39 cases were included in the study. Twenty-eight percent (11 cases) demonstrated strong PRAME expression, and 27% of cases were positive for Merkel cell polyomavirus. There was no statistically significant correlation between PRAME expression and virus positivity. With respect to PRAME, the adjusted all-cause mortality hazard ratio was 11.4 (95% CI: 1.8, 70.8). The unadjusted MCC-specific hazard ratio was 4.6 (95% CI: 0.8, 27.5). The adjusted hazard ratio pertaining to Merkel cell polyomavirus infection was 0.25 (95% CI: 0.02, 2.96). In this limited cohort, PRAME expression appears to correlate with worse outcomes in Merkel cell carcinoma.

中文翻译：

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默克尔细胞癌中的 PRAME 表达。


默克尔细胞癌（MCC）是一种罕见的侵袭性皮肤神经内分泌肿瘤。危险因素包括广泛的阳光损伤、默克尔细胞多瘤病毒感染和免疫功能低下状态。 PRAME，也称为黑色素瘤中优先表达的抗原，是一种癌症睾丸抗原，最近被发现是黑色素细胞肿瘤检查中有用的诊断工具。然而，PRAME 在 Merkel 细胞癌中的表达模式尚不清楚。在本研究中，我们检查了 Merkel 细胞癌中的 PRAME 表达并探讨了其预后意义。弗吉尼亚大学的机构档案用于搜索 2004 年至 2022 年被归类为默克尔细胞癌的肿瘤。对所有潜在病例进行审查以确认诊断，并搜索电子病历以获取临床和人口统计数据。随后对肿瘤进行 PRAME 和 Merkel 细胞多瘤病毒免疫染色。在我们的研究中，使用 Cox 比例风险回归模型来估计 PRAME 阳性和 MCPyV 阳性的相对（全因）生存率以及 PRAME 阳性的 MCC 特异性生存率。为与全因生存相关的每个结果创建了单变量和多变量模型。该研究共纳入 39 例病例。 28%（11 例）表现出强烈的 PRAME 表达，27% 的病例默克尔细胞多瘤病毒呈阳性。 PRAME 表达与病毒阳性之间没有统计学上显着的相关性。对于 PRAME，调整后的全因死亡风险比为 11.4（95% CI：1.8，70.8）。未经调整的 MCC 特定风险比为 4.6（95% CI：0.8，27.5）。与默克尔细胞多瘤病毒感染相关的调整后风险比为 0.25（95% CI：0.02，2.96）。 在这个有限的队列中，PRAME 表达似乎与默克尔细胞癌的较差结果相关。