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肛周外生殖器部位原发性实性肿瘤389例临床和病理诊断分析

Clinical and pathological diagnoses of 389 patients with primary solid tumors localized in the perianal and external genitalia regions

摘要目的:分析肛周外生殖器部位实性肿瘤患者的临床及组织病理诊断情况,为临床医生对该部位良恶性肿瘤的正确识别提供帮助。方法:回顾性分析2016年1月1日至2023年5月1日于北京大学第三医院皮肤科确诊的389肛周外生殖器部位实性肿瘤患者的病例资料,分析患者的临床表现和皮损组织病理、皮肤镜特征及诊断、治疗情况。结果:389例肛周外生殖器部位实性肿瘤患者中,男123例,女266例,年龄8 ~ 89(41.86 ± 16.62)岁;依据皮肤肿瘤组织学分类,其中黑色素细胞肿瘤最常见,占33.16%(129/389),其次是其他肿瘤(21.59%,84/389)、角质形成细胞肿瘤(21.08%,82/389)、软组织与神经肿瘤(13.62%,53/389)以及附属器肿瘤(10.54%,41/389);良性肿瘤占83.03%(323/389),恶性肿瘤占16.97%(66/389),最常见的良性肿瘤是黑色素痣,其中女性主要累及大阴唇,男性最常累及阴阜。发生于该部位的恶性肿瘤中女性相对常见的是鳞状细胞癌(40%,10/25);男性相对常见的恶性肿瘤是乳房外Paget病(39.02%,16/41)。其中,鲍恩样丘疹病临床初诊的误诊率最高(11/11),其次是鳞状细胞癌(78.26%,18/23)和基底细胞癌(4/10),不同性别及不同年龄组间误诊率差异不具有统计学意义(均 P>0.05)。367例患者接受局麻手术切除治疗,预后良好,另有22例恶性肿瘤患者接受光动力、扩大切除术及皮瓣转移术等治疗,随访3个月至3年余均未复发。 结论:肛周外生殖器部位的实性肿瘤皮损形态多样,其中黑色素细胞、角质形成细胞及其他类肿瘤占比高,恶性肿瘤相对少见,部分疾病易被误诊。发生于该部位的肿瘤整体预后较好,多数皮损手术切除后无复发。

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abstractsObjective:To analyze clinical and histopathological diagnoses of patients with solid tumors localized on the perianal and external genitalia regions, aiming to help clinicians correctly identify benign and malignant tumors.Methods:A retrospective analysis was conducted on clinical, histopathological, and dermoscopic features, diagnosis and treatment of 389 patients diagnosed with solid tumors localized in the perianal and external genitalia regions in the Department of Dermatology, Peking University Third Hospital from January 2016 to May 2023.Results:Among the 389 patients with solid tumors in perianal and external genitalia regions, 123 were males and 266 were females, with the age being 8 - 89 (41.86 ± 16.62) years. Based on the histological classification of skin tumors, melanocytic tumors were the most common type (33.16% [129/389]), followed by other tumors (21.59%, 84/389), keratinocytic tumors (21.08%, 82/389), soft tissue and neural tumors (13.62%, 53/389), and appendageal tumors (10.54%, 41/389). There were 83.03% (323/389) of benign tumors and 16.97% (66/389) of malignant tumors. Melanocytic nevus was the most common benign tumor, mostly involving the labia majora of females and mons pubis of males. Among the malignant tumors, squamous cell carcinoma was relatively common in females (40%, 10/25), while extramammary Paget′s disease was relatively common in males (39.02%, 16/41). The initial misdiagnosis rate of Bowenoid papulosis was the highest (11/11), followed by squamous cell carcinoma (78.26%, 18/23) and basal cell carcinoma (40%, 4/10) ; there was no significant difference in the initial misdiagnosis rate between different gender groups or different age groups (both P>0.5). A total of 367 patients underwent surgical excision under local anesthesia and all showed a good prognosis; the remaining 22 with malignant tumors were treated with photodynamic therapy, extended resection, free flap transfer, etc., and experienced no recurrence during the follow-up of 3 months to more than 3 years. Conclusions:Solid tumors in the perianal and external genitalia regions were polymorphous, melanocytic tumors, keratinocytic tumors and other tumors accounted for a large proportion, while malignant tumors were relatively rare, and some were easily misdiagnosed. Most of the tumors in the regions had a good prognosis, without recurrence after the surgical resection.

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