Fistula-Related Cancer in Crohn’s Disease: A Systematic Review

SIMPLE SUMMARY: Cancer arising at the site of a chronic perianal fistula is rare in patients with Crohn’s disease. The relationship between perianal fistula in CD (Chron’s disease) and SCC (squamous cell carcinoma) development is not clear but chronic inflammation of ano-rectal mucosa, delayed wound healing and cell turnover may play important roles. The aim of this systematic review was to determine the clinical characteristics of patients with squamous cell carcinoma arising from perianal fistula in CD, the surgery and oncological treatment, the role of HPV infection, immunosuppression and the survival of these patients. Fistula-related carcinoma in CD can be very difficult to diagnose. An early diagnosis has the potential to improve the outcome of disease. ABSTRACT: 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.