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肛周瘘管黏液腺癌的手术治疗:病例系列

Surgical management of mucinous adenocarcinoma arising in perianal fistula: A case series.

作者信息

Derakhshani Saeed, Ghadim Milad Karimian, Salari Abolfazl, Ghahari Mohammadreza

机构信息

Department of General Surgery, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Kasra General Hospital, Tehran, Iran.

出版信息

Surg Open Sci. 2025 Apr 17;26:1-5. doi: 10.1016/j.sopen.2025.04.005. eCollection 2025 Jun.

Abstract

Perianal mucinous adenocarcinoma (MAC) is a rare malignancy arising in the anal canal, often associated with chronic inflammatory conditions such as perianal fistulas. This case series aims to elucidate the clinical features and surgical management of patients with perianal MAC arising from perianal fistulas. Four cases of perianal MAC are presented, highlighting the diverse clinical presentations, diagnostic pathways, and therapeutic interventions. Each case demonstrates unique aspects of disease progression, treatment response, and long-term outcomes. Key features include the challenges of diagnosing MAC amidst benign conditions, the role of neoadjuvant chemotherapy in improving resectability, and the importance of individualized surgical approaches. All patients were male and presented with a variety of symptoms ranging from changes in bowel habits to perianal abscesses. The diagnosis was challenging due to the resemblance of MAC to benign conditions and delayed presentation. All patients underwent abdominoperineal resection (APR) and colostomy insertion (perineal or abdominal). Two cases required reconstructive procedures where a V-Y flap and an omental flap were employed. A multidisciplinary approach ensures that patients receive appropriate neoadjuvant and adjuvant treatment. With the median follow-up of 3 years, all patients were alive. Our multidisciplinary approach effectively managed perianal MAC by integrating surgical techniques, including APR and reconstructive methods, along with neoadjuvant and adjuvant treatment. Performing appropriate surgical techniques leads to tumor-free margins, in addition to systemic therapy, and improves both patient survival and quality of life.

摘要

肛周黏液腺癌(MAC)是一种发生于肛管的罕见恶性肿瘤,常与肛周瘘等慢性炎症性疾病相关。本病例系列旨在阐明由肛周瘘引起的肛周MAC患者的临床特征及手术治疗方法。本文报告了4例肛周MAC病例,突出了其多样的临床表现、诊断途径及治疗干预措施。每个病例都展示了疾病进展、治疗反应及长期预后的独特方面。关键特征包括在良性疾病中诊断MAC的挑战、新辅助化疗在提高可切除性方面的作用以及个体化手术方法的重要性。所有患者均为男性,表现出从排便习惯改变到肛周脓肿等多种症状。由于MAC与良性疾病相似且就诊延迟,诊断颇具挑战性。所有患者均接受了腹会阴联合切除术(APR)及结肠造口术(会阴或腹部)。2例患者需要采用V-Y皮瓣和网膜瓣进行重建手术。多学科方法可确保患者接受适当的新辅助和辅助治疗。中位随访3年,所有患者均存活。我们的多学科方法通过整合包括APR和重建方法在内的手术技术以及新辅助和辅助治疗,有效地管理了肛周MAC。除全身治疗外,采用适当的手术技术可实现切缘无肿瘤,从而提高患者生存率和生活质量。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d97c/12049996/aaceeaca3697/ga1.jpg

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