Merkel cell carcinoma overlapping Bowen's disease: two cases report and literature review

作者全名：Chen, Xueqin; Song, Xiao; Huang, Hui; Zhang, Lian; Song, Zhiqiang; Yang, Xichuan; Lei, Shanchuan; Zhai, Zhifang

作者地址：[Chen, Xueqin; Song, Xiao; Huang, Hui; Zhang, Lian; Song, Zhiqiang; Yang, Xichuan; Zhai, Zhifang] Army Med Univ, Southwest Hosp, Dept Dermatol, 30 Gaotanyan St, Chongqing 400038, Peoples R China; [Lei, Shanchuan] Chongqing Med Univ, Yongchuan Hosp, Dept Dermatol, 439 Xuanhua Rd, Chongqing 402160, Peoples R China

通信作者：Zhai, ZF (通讯作者)，Army Med Univ, Southwest Hosp, Dept Dermatol, 30 Gaotanyan St, Chongqing 400038, Peoples R China.; Lei, SC (通讯作者)，Chongqing Med Univ, Yongchuan Hosp, Dept Dermatol, 439 Xuanhua Rd, Chongqing 402160, Peoples R China.

来源：JOURNAL OF CANCER RESEARCH AND CLINICAL ONCOLOGY

ESI学科分类：CLINICAL MEDICINE

WOS号：WOS:001210710900002

JCR分区：Q3

影响因子：2.7

年份：2024

卷号：150

期号：4

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结束页：　

文献类型：Article

关键词：Merkel cell carcinoma; Bowen's disease; Squamous cell carcinoma in situ; Histopathology; Therapy

摘要：Purpose Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor of the skin, which mainly occurs in the sun exposed sites of white patients over 65 years, with a higher recurrence and metastasis rate. Clinically, MCC overlapping Bowen's disease (BD) is a very rare subtype of MCC. Few cases in the literature have been described and the management is not well defined. We summarize and update the epidemiology, clinical and histopathological features, metastasis characteristics, local recurrence rate and management of it by presenting two cases of MCC overlapping BD and reviewing the literature over the last 11 years.Design We consulted databases from PubMed, ResearchGate and Google Scholar by MeSh "Merkel cell carcinoma" and "Bowen's disease", "Bowen disease" or "squamous cell carcinoma in situ", from January 2013 to December 2023 and reviewed the literatures. We reported two additional cases.Results Total 13 cases of MCC overlapping BD were retrospectively analyzed, in whom mainly in elderly women over 70 years, the skin lesions were primarily located on the faces, followed by the extremities and trunk. Most of them were asymptomatic, firm, dark red nodules arising on rapidly growing red or dark brown patches, or presenting as isolated nodules. Dermoscopy evaluation was rarely performed in the pre-operative diagnostic setting. All cases were confirmed by histopathology and immunohistochemistry. The most definitive treatment was extended local excision, but local recurrences were common. Of the 13 cases, 4 cases experienced local or distant metastasis. One suffered from an in-transit recurrence of MCC on the ipsilateral leg after local excision and lymph node dissection, whose metastasis completely subsided after avelumab treatment and without recurrence or metastasis during 6 months of follow-up.Conclusions MCC overlapping BD is a very rare skin tumor mainly predisposed on the faces, with high misdiagnosis rate and recurrence rate. Advanced disease at diagnosis is a poor prognostic factor, suggesting that earlier detection may improve outcome. The acronym, AEIOUN, has been proposed to aid in clinical identification. Our reports and the literature review can provide a better awareness and management of it.

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