原发直肠肛管小细胞癌1例报告并文献复习
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詹玮, Email: 16799507@qq.com

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国家自然科学基金资助项目(82060523);贵州省卫计委科学技术基金资助项目(gzwjkj2018-1-035)。


Primary anorectal small-cell carcinoma: a case report and literature review
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    摘要:

    背景与目的:原发直肠肛管小细胞癌(SCC)是直肠肛管肿瘤罕见的类型,占比不超过1%。与常见的直肠肛管腺癌相比,直肠肛管SCC的临床症状以及影像学表现不具有特异性,诊断较为困难,术后极易发生复发和远处转移,预后差,其总体病死率极高。由于直肠肛管SCC的罕见性和特殊性,目前国内外文献报道较少,缺乏相关的研究数据和治疗经验,对其认识不够深入,尚无统一的最佳治疗方案,这给临床对其诊治带来了一定的困扰。笔者通过收治1例原发直肠肛管SCC患者,分析诊疗经过,探讨直肠肛管SCC的诊断和治疗方法,以期为该类疾病诊断和治疗提供相应的临床经验和新的思路。
    方法:回顾性分析1例原发直肠肛管SCC患者的临床资料,对患者的病史特点、一般情况、影像学检查、病理组织形态和免疫标记物特征进行分析诊断,根据其特点制定相应的治疗策略,并结合相关文献复习。
    结果:患者表现出便血的临床特点,影像学检查考虑为直肠肛管恶性肿瘤,经过2次肠镜下组织活检病理提示为SCC,遂行腹腔镜辅助Miles手术,术后病理诊断为直肠肛管SCC。患者术后通过辅以盆腔放疗和“顺铂+依托泊苷”EP化疗方案的综合治疗,定期复查胸部CT、全腹CT、盆腔MRI、结肠镜检查、血清肿瘤标志物等均未发生肿瘤复发和远处转移,已无瘤生存13个月,目前继续随访观察中。
    结论:原发直肠肛管SCC是一种罕见的疾病,总体生存率低。直肠肛管SCC的临床症状、影像学表现与常见的直肠肛管腺癌相似,不具有特异性,肠镜下的组织活检取材较为困难,确诊需要结合病理组织形态和多种免疫组化标记物。手术切除是治疗局部进展期直肠肛管SCC最为主要且有效的方法,应尽早的进行手术治疗,术后辅以盆腔放疗和“顺铂+依托泊苷”EP化疗方案的综合治疗,能改善患者的预后,延长生存期。

    Abstract:

    Background and Aims: Primary anorectal small-cell carcinoma (SCC) is a rare type of cancer, accounting for less than 1% of all anorectal tumors. Compared with common anorectal adenocarcinoma, the clinical symptoms and imaging manifestations of anorectal SCC are not specific, so the diagnosis is difficult, postoperative recurrence and distant metastasis are easy to occur, the prognosis is poor, and the overall mortality is extremely high. Due to the rarity and particularity of anorectal SCC, there are so far few domestic and foreign literature reports available, lack of relevant research data and treatment experience as well as in-depth understanding of its characteristics, and no unified optimal treatment plan, which bring certain troubles to clinical diagnosis and treatment. Here, the authors discuss the diagnosis and treatment modality of anorectal SCC through analyzing the diagnosis and treatment process in a treated case of primary anorectal SCC, so as to provide corresponding clinical experience and new ideas for the diagnosis and treatment of this condition.  
    Methods: The clinical data of one patient with primary anorectal SCC were retrospectively analyzed. The patient's medical history, general condition, imaging examination, pathological tissue morphology and characteristics of immunomarker were analyzed and diagnosed, and corresponding treatment strategies were developed according to its characteristics, combined with review of relevant literature.
    Results: The clinical feature of the patient presented was bloody stool, which was considered as anorectal malignant tumor after imaging examination, and SCC was pathologically suggested after twice colonoscopic biopsies. Then, laparoscopic-assisted Miles surgery was performed, and postoperative pathologic diagnosis was SCC of the anorectal canal. After operation, additional comprehensive treatment compromising pelvic radiotherapy and "cisplatin plus etoposide" EP chemotherapy regimen was applied. Regular reexaminations such as chest CT, total abdominal CT, pelvic MRI, colonoscopy, serum tumor markers showed no tumor recurrence or distant metastasis. The patient has survived with tumor-free status for 13 months by now, and was still in follow-up observation.
    Conclusion: Primary anorectal SCC is a rare disease with low overall survival. The clinical symptoms and imaging manifestations of anorectal SCC are similar to those of common anorectal adenocarcinoma without specificity. Biopsy material is difficult to obtain under colonoscopy, and the diagnosis requires combining the histopathological findings and a variety of immunohistochemical markers. Surgical resection is the most important and effective method for the treatment of locally advanced anorectal SCC. Surgical treatment should be carried out as soon as possible. Postoperative combined treatment with pelvic radiotherapy and "cisplatin plus etoposide" EP chemotherapy regimen can improve the prognosis of patients and prolong their survival time.

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陈忠胜, 李梁和, 张建栋, 姜洞彬, 廖欣, 李珀, 詹玮.原发直肠肛管小细胞癌1例报告并文献复习[J].中国普通外科杂志,2020,29(10):1172-1177.
DOI:10.7659/j. issn.1005-6947.2020.10.003

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  • 收稿日期:2020-08-13
  • 最后修改日期:2020-09-20
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  • 在线发布日期: 2020-10-25