RESUMO
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.
RESUMO
Patients with inflammatory bowel disease [IBD] may develop, similarly to individuals from general population, rare cases of human papilloma virus [HPV]-related anal canal squamous cell carcinoma [SCC] and intra-epithelial precursor lesions, as well as very rare cases of anal canal adenocarcinoma. Patients with chronic perianal Crohn's disease [CD] are at substantial risk of developing SCC or adenocarcinoma from the fistula-lining epithelium, as well as SCC or adenocarcinoma arising from chronic anorectal ulcerations or strictures. Based on this lesion stratification, we provide in this review tailored incidence estimates and we propose an IBD-specific classification of all types of anal neoplasia that may occur in patients with IBD. After reviewing putative carcinogenesis of all types of neoplasia, we conclude that HPV vaccination could reduce the incidence of HPV-related lesions, although an anal screening programme related to these lesions is not mandatory on the sole basis of IBD. By contrast, we point out that all patients with chronic perianal CD should be explored in depth, including biopsies under anaesthesia and fistula curettage when necessary, in case of any change in anal symptoms âin particular new, increasing, unexplained pain. Finally, we conclude that there is an urgent need for elaborating and evaluating surveillance algorithms in patients with chronic perianal CD, in order to avoid cancers with late diagnosis and poor prognosis.