Incidence and Management of Rectal Cuff and Anal Transitional Zone Neoplasia in Patients With Familial Adenomatous Polyposis

Abstract

BACKGROUND: Rectal cuff and anal transitional zone neoplasia is an increasing challenge in patients with familial adenomatous polyposis who have undergone restorative proctocolectomy. Its real incidence, range of severity, and treatment efficacy are poorly documented. OBJECTIVE: We sought to document the evolution of rectal cuff and anal transitional zone neoplasia and describe its management. DESIGN: This is a retrospective cohort study collecting prospectively recorded data. SETTINGS: This study involved a hereditary colorectal cancer group in a large academic medical center. PATIENTS: All patients undergoing primary restorative proctocolectomy at this institution were included INTERVENTIONS: Surveillance pouchoscopy and treatment of rectal cuff/anal transitional zone neoplasia were performed. MAIN OUTCOME MEASURES: The primary outcomes measured were the presence and the severity of rectal cuff/anal transitional zone neoplasia. Excision by cautery, snare, mucosectomy, or redo pouch was evaluated. RESULTS: A total of 165 patients were included: 52% were male (86/165) with a median age at restorative proctocolectomy of 31.0 years (SD 12.8). In 117 of 165, the proctocolectomy was their first operation and, in 48 of 165, it followed a colectomy. Of the patients, 83% (137/165) had stapled anastomosis; 17% had mucosectomy with handsewn anastomosis; and 14% (23/165) were treated with sulindac at some point during their surveillance. Median follow-up was 10.1 years (interquartile range, 4.5–17.2) and the median number of pouchoscopies per patient was 4 (interquartile range, 2–8). Seventy-eight of 165 (47.3%) developed rectal cuff/anal transitional zone adenomas, more in the stapled group (52.3%; 72/137) than in the handsewn group (21.4%; 6/28; p < 0.005). Median time to adenoma was 4.5 years (interquartile range, 2.4–8.9). Three patients developed cancer under surveillance, and, in 3 other patients, cancers developed when screenings lapsed. Five patients developed symptomatic anal stenosis secondary to repeated surgeries (median, 9 procedures; range, 2–10). LIMITATIONS: There was no quality-of-life measurement in patients who developed rectal cuff/anal transitional zone neoplasia. CONCLUSIONS: Rectal cuff/anal transitional zone adenomas are more common than previously reported. Mild polyposis can be controlled endoscopically, but repeated procedures in a higher stage are associated with risk of anal stenosis. Compliance with surveillance is essential to avoid cancer. See Video Abstract at http://links.lww.com/DCR/B594. INCIDENCIA Y TRATAMIENTO DE LA NEOPLASIA DEL REMANENTE RECTAL Y DE LA ZONA DE TRANSICIóN ANAL EN PACIENTES CON POLIPOSIS ADENOMATOSA FAMILIAR ANTECEDENTES: La neoplasia del remanente rectal y de la zona de transición anal presenta un desafío mayor en pacientes con poliposis adenomatosa familiar tratados con una proctocolectomía restaurativa. Su incidencia real, el espectro de la gravedad y la eficacia del tratamiento están mal documentados. OBJETIVO: Buscamos documentar la evolución de la neoplasia del remanente rectal y de la zona de transición anal y describir su tratamiento. DISEÑO: Estudio de cohorte retrospectivo que recabó datos registrados prospectivamente. AMBITO: Un grupo con cáncer colorrectal hereditario de un importante centro médico académico. PACIENTES: Todos los pacientes operados por primera vez de proctocolectomía restaurativa en nuestra institución. INTERVENCIONES: Endoscopía del pouch para vigilancia y tratamiento de la neoplasia del remanente rectal / zona de transición anal. PRINCIPALES VARIABLES ANALIZADAS: La presencia y la gravedad de la neoplasia del remanente rectal / zona de transición anal. Resección con cauterio, asa, mucosectomía o rehacer el pouch. RESULTADOS: Se incluyeron un total de 165 pacientes: 52% eran hombres (86/165) con una mediana de edad al momento de la proctocolectomía restaurativa de 31,0 años (DE 12,8). En 117/165 la proctocolectomía fue su primera cirugía y en 48/165 fue posterior a una colectomía. En 83% (137/165) tenía una anastomosis con engrapadora, 17% tenía mucosectomía y anastomosis con sutura manual (HS). El 14% de los pacientes (23/165) fueron tratados con sulindac en algún momento durante su vigilancia. La mediana de seguimiento fue de 10,1 años (IQR: 4,5, 17,2) y la mediana del número de endoscopías del pouch por paciente fue de 4. (IQR: 2, 8) 78/165 (47,3%) desarrollaron adenomas en la zona de transición anal /remanente rectal, mayor en el grupo con engrapadaora (52,3%; 72/137) comparado con el grupo con sutura manual (21,4%; 6/28) (p <0,005). La mediana del tiempo hasta el adenoma fue de 4,5 años (IQR: 2,4, 8,9). Tres pacientes que se encontraban en vigilancia desarrollaron cáncer y en 3, otros cánceres se desarrollaron transcurrida la vigilancia. 5 pacientes desarrollaron estenosis anal sintomática secundaria a múltiples cirugías (mediana de 9 procedimientos; rango 2-10). LIMITACIONES: Falta de medición de la calidad de vida en pacientes que desarrollaron neoplasia del remanente rectal / zona de transición anal. CONCLUSIONES: Los adenomas de la zona de transición anal / remanente rectal son más comunes de lo reportado anteriormente. La poliposis leve se puede tratar por endoscopía, pero procedimientos repetidos en estadíos mayores se asocian con el riesgo de estenosis anal. El apego a la vigilancia es fundamental para evitar el cáncer. Consulte Video Resumen en http://links.lww.com/DCR/B594. (Traducción—Dr. Lisbeth Alarcon-Bernes)

Incidence and Risk Factors of Cancer in the Anal Transitional Zone and Ileal Pouch following Surgery for Ulcerative Colitis and Familial Adenomatous Polyposis

“…However, this result was not significant (p = 0.08) [218]. The main references on epidemiology and risk factor could be as follows: [23,180,194,196,207].…”

“…Severe colic disease (>1000 polyps) was identified as a risk factor in ATZ adenomas in one study [23]. Numerous cases of pouch adenomas following RPC for FAP have been reported [18,23,190,192,[196][197][198][199][200][201][202][203][204][205][206]. This risk increases over time and prevalence has been estimated as 7%, 35% and 75% at 5, 10 and 15 years post-surgery, respectively, [207].…”

Metastatic Rectal Adenocarcinoma in Familial Adenomatous Polyposis: A Case of Socioeconomic Factors Influencing Surveillance Endoscopy

“…If the polyp burden is manageable, colonoscopy can be performed every 1-2 years following the diagnosis [16,17]. Surgery is indicated in patients with >20 rectal polyps, large (>1 cm) polyps, and polyps with advanced histology or by patient preference [15]. Following surgery, surveillance endoscopy is critical for the early detection of polyps and malignancies.…”

“…The cuffless IPAA differentiates itself by creating a direct anastomosis between the ileum and the anal tissue, completely removing the rectal cuff used in traditional IPAA. Postoperative surveillance is still required following all surgical approaches due to the risk for adenoma formation in the ileum, anal transition zone, or residual rectal tissue [6,8,[12][13][14][15]. Pouchoscopy or ileoscopy is recommended at one-to two-year intervals indefinitely in those with a history of IPAA, and flexible sigmoidoscopy is recommended at six-to 12-month intervals indefinitely in those with a history of IRA [16].…”

Nuances of Ileal Pouch-Anal Anastomosis in Familial Adenomatous Polyposis

“…The risk of neoplasia at the IPAA is low in patients with UC but reaches about 70% after 15 years in patients with FAP. 11…”