目的:默克尔细胞癌(MCC)是一种罕见的皮肤神经内分泌肿瘤,主要发生在65岁以上白人患者的阳光照射部位,具有较高的复发率和转移率。临床上,MCC重叠鲍恩氏病(BD)是一种非常罕见的MCC亚型。文献中很少描述案例,管理也没有很好的定义。我们总结和更新流行病学,临床和组织病理学特征,转移特征,通过介绍两例MCC重叠BD并回顾过去11年的文献,介绍了局部复发率和治疗方法。
方法:我们查阅了PubMed的数据库,研究门和谷歌学者由MeSh“默克尔细胞癌”和“鲍恩病”,\"Bowen病\"或\"鳞状细胞原位癌\",2013年1月至2023年12月,对文献进行了综述。我们报告了另外两个病例。
结果:对13例MCC重叠BD进行回顾性分析,其中主要是70岁以上的老年妇女,皮肤损伤主要发生在面部,其次是四肢和躯干。他们大多数无症状,公司,在快速生长的红色或深棕色斑块上出现的暗红色结节,或表现为孤立的结节。术前诊断设置很少进行皮肤镜检查评估。所有病例均经组织病理学和免疫组化证实。最明确的治疗方法是扩大局部切除,但局部复发很常见。在13个案例中,4例出现局部或远处转移。一名患者在局部切除和淋巴结清扫后同侧腿MCC在途复发,其转移在avelumab治疗后完全消退,在6个月的随访中没有复发或转移。
结论:MCC重叠BD是一种非常罕见的皮肤肿瘤,主要倾向于面部,误诊率和复发率高。诊断时的晚期疾病是预后不良的因素,这表明早期检测可能会改善结果。缩写,AEIOUN,已被提议帮助临床鉴定。我们的报告和文献综述可以提供更好的认识和管理。
OBJECTIVE: 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.
METHODS: 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.