Perianal Fistulizing Crohn's Disease-Associated Anorectal and Fistula Cancers: Systematic Review and Expert Consensus.

Sergey Khaitov, Parakkal Deepak, David T Rubin, Anders Dige, Serre-Yu Wong, Cathy Rowan, Elvira Diaz Brockmans, Cindy C Y Law, Elisabeth Giselbrecht, Bram Verstockt, David Sachar, Leon Shin-Han Winata, Benjamin D McDonald, Phillip Lung, Luke Hanna, Carla Hamarth, Corina Behrenbruch, David H Ballard, Stefan D Holubar, Gabriele Bislenghi, Benjamin L Cohen, Michele Carvello, Jeroen Geldof, Lilli Lundby, Cristiana Bonifacio, Alexandros D Polydorides, Phil Tozer, Emre Altinmakas, Dermot P B McGovern, Jean-Frederic Colombel, Ailsa Hart, Shaji Sebastian, Antonino Spinelli, Amy L Lightner, Celina Ang

Journal: Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association 2025;23(6):927-945.e2

PMID: 38871152

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Abstract

BACKGROUND & AIMS

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.

METHODS

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.

RESULTS

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.

CONCLUSIONS

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.

Address: Department of Gastroenterology, Icahn School of Medicine at Mount Sinai, New York, New York. Electronic address: [email protected].; Department of Gastroenterology, Beaumont Hospital, Dublin, Ireland.; Department of Medicine, Universidad Iberoamericana, Santo Domingo, Dominican Republic.; Department of Gastroenterology, Icahn School of Medicine at Mount Sinai, New York, New York.; Department of Oncology, Icahn School of Medicine at Mount Sinai, New York, New York.; Department of Surgery, Icahn School of Medicine at Mount Sinai, New York, New York.; Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, New York, New York.; Medical Imaging, St. Vincent's Hospital, Melbourne, Australia.; Department of Gastroenterology, University Hospitals of Leuven, Leuven, Belgium.; Department of Biomedical Sciences, Humanitas University, Milan, Italy; IRCCS Humanitas Research Hospital, Milan, Italy.; University of Chicago Medicine Inflammatory Bowel Disease Center, Chicago, Illinois.; Department of Gastroenterology, Washington University School of Medicine in St. Louis, St. Louis, Missouri.; The F. Widjaja Foundation Inflammatory Bowel Disease Institute, Cedars-Sinai Medical Center, Los Angeles, California.; Radiology Department, St. Mark's Hospital and Academic Institute, London, United Kingdom.; Department of Surgery, Pelvic Floor Unit, Aarhus University Hospital, Aarhus, Denmark.; Department of Colorectal Surgery, Scripps Clinic, San Diego, California.; Department of Colorectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio.; IBD Unit, St. Mark's Hospital and Academic Institute, London, United Kingdom; Imperial College London, London, United Kingdom.; Department of Radiology, University of Chicago Medicine, Chicago, Illinois.; Department of Gastroenterology and Hepatology, University Hospital Ghent, Ghent, Belgium.; Department of Gastroenterology, Aarhus University Hospital, Aarhus, Denmark.; Department of Gastroenterology, Hepatology, and Nutrition, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio.; Radiology Department, IRCCS Humanitas Research Hospital, Milan, Italy.; Department of Abdominal Surgery, University Hospitals Leuven, Leuven, Belgium.; Department of Colorectal Surgery, St. Vincent's Hospital, Melbourne, Australia.; Mallinckrodt Institute of Radiology, Washington University School of Medicine in St. Louis, St. Louis, Missouri.; Department of Radiology, Icahn School of Medicine at Mount Sinai, New York, New York.; IBD Unit, Hull University Teaching Hospitals NHS Trust, Hull, United Kingdom.; Imperial College London, London, United Kingdom; Department of Colorectal Surgery, St. Mark's Hospital and Academic Institute, London, United Kingdom; Robin Phillips Fistula Research Unit, St. Mark's Hospital and Academic Institute, London, United Kingdom.

DOI: 10.1016/j.cgh.2024.05.029

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MeSH Terms: Humans, Crohn Disease, Rectal Fistula, Anus Neoplasms, Consensus, Rectal Neoplasms, Carcinoma, Squamous Cell