Perianal fistulizing Crohn’s disease (PFCD)-associated anorectal and fistula cancers are rare but often devastating diagnoses. However, given the low incidence and consequent lack of data and clinical trials in the field, there is little to no guidance on screening and management of these cancers. To inform clinical practice, we developed consensus guidelines on PFCD-associated anorectal and fistula cancers by multidisciplinary experts from the international TOpClass consortium. We conducted a systematic review by standard methodology, using the Newcastle-Ottawa Scale quality assessment tool. We subsequently developed consensus statements using a Delphi consensus approach. Of 561 articles identified, 110 were eligible, and 76 articles were included. The overall quality of evidence was low. The TOpClass consortium reached consensus on 6 structured statements addressing screening, risk assessment, and management of PFCD-associated anorectal and fistula cancers. Patients with long-standing (>10 years) PFCD should be considered at small but increased risk of developing perianal cancer, including squamous cell carcinoma of the anus and anorectal carcinoma. Risk factors for squamous cell carcinoma of the anus, notably human papilloma virus, should be considered. New, refractory, or progressive perianal symptoms should prompt evaluation for fistula cancer. There was no consensus on timing or frequency of screening in patients with asymptomatic perianal fistula. Multiple modalities may be required for diagnosis, including an examination under anesthesia with biopsy. Multidisciplinary team efforts were deemed central to the management of fistula cancers. Inflammatory bowel disease clinicians should be aware of the risk of PFCD-associated anorectal and fistula cancers in all patients with PFCD. The TOpClass consortium consensus statements outlined herein offer guidance in managing this challenging scenario.

中文翻译：

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肛周瘘管性克罗恩病相关肛门直肠癌和瘘管癌：系统评价和专家共识


肛周瘘管克罗恩病 (PFCD) 相关的肛门直肠癌和瘘管癌很少见，但诊断结果往往是毁灭性的。然而，由于发病率低以及因此缺乏该领域的数据和临床试验，因此对这些癌症的筛查和管理几乎没有指导。为了指导临床实践，我们由国际 TOpClass 联盟的多学科专家制定了有关 PFCD 相关肛门直肠癌和瘘管癌的共识指南。我们使用纽卡斯尔-渥太华量表质量评估工具，通过标准方法进行了系统审查。我们随后使用德尔菲共识方法制定了共识声明。在确定的 561 篇文章中，110 篇符合条件，76 篇文章被纳入。证据的总体质量较低。 TOpClass 联盟就 6 项结构化声明达成共识，涉及 PFCD 相关的肛门直肠癌和瘘管癌的筛查、风险评估和管理。患有长期（> 10 年）PFCD 的患者应考虑患肛周癌的风险虽小但增加，包括肛门鳞状细胞癌和肛门直肠癌。应考虑肛门鳞状细胞癌的危险因素，特别是人乳头瘤病毒。新出现的、难治性的或进行性的肛周症状应促使评估瘘管癌。对于无症状肛周瘘患者的筛查时间或频率尚未达成共识。诊断可能需要多种方式，包括麻醉下进行活检。多学科团队的努力被认为是瘘管癌治疗的核心。 炎症性肠病临床医生应了解所有 PFCD 患者患 PFCD 相关肛门直肠癌和瘘管癌的风险。本文概述的 TOpClass 联盟共识声明为管理这一具有挑战性的场景提供了指导。