Kotsafti Andromachi, Scarpa Melania, Angriman Imerio, Castagliuolo Ignazio, Caruso Antonino
Laboratory of Advanced Translational Research, Veneto Institute of Oncology IOV-IRCCS, 35128 Padua, Italy.
First Surgical Clinic Section, Department of Surgical, Oncological and Gastroenterological Sciences, University of Padua, 35128 Padua, Italy.
Cancers (Basel). 2021 Mar 22;13(6):1445. doi: 10.3390/cancers13061445.
Perianal fistulizing Crohn's disease is a very disabling condition with poor quality of life. Patients with perianal fistulizing Crohn's disease are also at risk of perianal fistula-related squamous cell carcinoma (SCC). Cancer arising at the site of a chronic perianal fistula is rare in patients with Crohn's disease and there is a paucity of data regarding its incidence, diagnosis and management. A systematic review of the literature was undertaken using Medline, Embase, Pubmed, Cochrane and Web of Science. Several small series have described sporadic cases with perianal cancer in Crohn's disease. The incidence rate of SCC related to perianal fistula was very low (<1%). Prognosis was poor. Colorectal disease, chronic perianal disease and HPV infection were possible risk factors. Fistula-related carcinoma in CD (Chron's disease) can be very difficult to diagnose. Examination may be limited by pain, strictures and induration of the perianal tissues. HPV is an important risk factor with a particular carcinogenesis mechanism. MRI can help clinicians in diagnosis. Examination under anesthesia is highly recommended when findings, a change in symptoms, or simply long-standing disease in the perineum are present. Future studies are needed to understand the role of HPV vaccination in preventing fistula-related cancer.
肛周瘘管性克罗恩病是一种严重影响生活质量的致残性疾病。肛周瘘管性克罗恩病患者也有发生肛周瘘管相关鳞状细胞癌（SCC）的风险。在克罗恩病患者中，慢性肛周瘘管部位发生的癌症较为罕见，关于其发病率、诊断和治疗的数据也很匮乏。我们使用Medline、Embase、Pubmed、Cochrane和Web of Science对文献进行了系统综述。几个小系列报道了克罗恩病患者发生肛周癌的散发病例。与肛周瘘管相关的SCC发病率非常低（<1%）。预后较差。结直肠疾病、慢性肛周疾病和人乳头瘤病毒（HPV）感染可能是危险因素。克罗恩病（CD）中与瘘管相关的癌很难诊断。检查可能会受到疼痛、肛周组织狭窄和硬结的限制。HPV是一个具有特殊致癌机制的重要危险因素。磁共振成像（MRI）有助于临床医生进行诊断。当出现相关发现、症状改变或会阴部位存在长期疾病时，强烈建议在麻醉下进行检查。未来需要开展研究以了解HPV疫苗接种在预防瘘管相关癌症中的作用。
