Rectal and Pouch Recurrences After Surgical Treatment for Familial Adenomatous Polyposis

Campos, Fábio Guilherme; Imperiale, Antônio Rocco; Seid, Víctor Edmond; Perez, Rodrigo Oliva; Sousa, Ângela Maria; Kiss, Desidério Roberto; Habr‐Gama, Angelita; Cecconello, Ivan

doi:10.1007/s11605-008-0606-8

Cited by 25 publications

(16 citation statements)

References 48 publications

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“…This fear is clearly justified from the long-term risk of neoplasia in the rectal stump after IRA, not infrequently leading to secondary proctectomy. 16,18 A less frequent potential for malignization within the ileal pouch after RPC has also been reported. 19 Among our patients, 2/12 (16.6%) CRC related-causes (representing 10.5% causes of death) were due to cancer developing in the remaining rectum after IRA or at the ileal pouch after RPC.…”

Section: Discussionmentioning

confidence: 97%

“…16,18 Data from the St. Marks Hospital had previously shown a three-fold relative risk of death after IRA. 20 Regarding our patients, the decision to perform an IRA or RPC was established at an individual basis, taking into account a less severe colonic and rectal polyposis, although this endoscopic feature does not guarantee that a rectal cancer will not develop in the future, as we attested in two patients.…”

Section: Discussionmentioning

confidence: 99%

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Evaluating Causes of Death in Familial Adenomatous Polyposis

Campos

Perez

Imperiale

et al. 2010

Journal of Gastrointestinal Surgery

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(1) CRC is still the most prevalent cause of death; (2) even after curative resections, CRC can cause death through rectal or pouch malignization; (3) long-term survival was also strongly related to the development of extracolonic neoplasia, especially desmoid tumors and gastroduodenal carcinoma; (4) our results raise the need for local improvement in familiar screening and help us to define follow-up strategies and patient-information standards.

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Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

Evaluating Causes of Death in Familial Adenomatous Polyposis

Campos

Perez

Imperiale

et al. 2010

Journal of Gastrointestinal Surgery

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“…There appears to be a small but real risk for cancer in the ileal pouch (177–179). Advanced dysplasia and cancer may occur at the anal transition zone, either from rectal tissue unexpectedly remaining with ileo-anal anastomosis or from the short segment of rectum often remaining with restorative proctocolectomy (143,150,180).…”

Section: Lynch Syndrome (Ls)mentioning

confidence: 99%

ACG Clinical Guideline: Genetic Testing and Management of Hereditary Gastrointestinal Cancer Syndromes

Syngal

Brand

Church

et al. 2015

American Journal of Gastroenterology

1,353

1,372

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This guideline presents recommendations for the management of patients with hereditary gastrointestinal cancer syndromes. The initial assessment is the collection of a family history of cancers and premalignant gastrointestinal conditions and should provide enough information to develop a preliminary determination of the risk of a familial predisposition to cancer. Age at diagnosis and lineage (maternal and/or paternal) should be documented for all diagnoses, especially in first- and second-degree relatives. When indicated, genetic testing for a germline mutation should be done on the most informative candidate(s) identified through the family history evaluation and/or tumor analysis to confirm a diagnosis and allow for predictive testing of at-risk relatives. Genetic testing should be conducted in the context of pre- and post-test genetic counseling to ensure the patient's informed decision making. Patients who meet clinical criteria for a syndrome as well as those with identified pathogenic germline mutations should receive appropriate surveillance measures in order to minimize their overall risk of developing syndrome-specific cancers. This guideline specifically discusses genetic testing and management of Lynch syndrome, familial adenomatous polyposis (FAP), attenuated familial adenomatous polyposis (AFAP), MUTYH-associated polyposis (MAP), Peutz–Jeghers syndrome, juvenile polyposis syndrome, Cowden syndrome, serrated (hyperplastic) polyposis syndrome, hereditary pancreatic cancer, and hereditary gastric cancer.

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“…This risk was addressed in long-term follow-up studies, suggesting that a more frequent indication of RPC instead of IRA may improve life expectancy by reducing rectal stump cancer rates. 87,93 Data from the St. Marks Hospital had previously shown a three-fold relative risk of death after IRA. 94 …”

Section: Causes Of Deathmentioning

confidence: 99%

Adenocarcinomas after Prophylactic Surgery for Familial Adenomatous Polyposis

Smith¹,

Schaffer²,

Ballard³

et al. 2013

JCT

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The incidence of familial adenomatous polyposis (FAP) is one in 7,000 to 12,000 live births. Virtually, all surgically untreated patients with FAP inevitably develop colorectal-cancer in their lifetime because they carry the adenomatous polyposis coli gene. Thus prophylactic proctocolectomy is indicated. Surgical treatment of FAP is still controversial. There are however, four surgical options: ileorectal anastomosis, restorative proctocolectomy with ileal pouch-anal anastomosis, proctocolectomy with ileostomy, and proctocolectomy with continent-ileostomy. Conventional proctocolectomy options largely lie between colectomy with ileorectal anastomosis or ileal pouch-anal anastomosis. Detractors of ileal pouch-anal anastomosis prefer ileorectal anastomosis because of better functional results and quality of life. The functional outcome of total colectomy with ileorectal anastomosis is undoubtedly far superior to that of the ileoanal pouch; however, the risk for rectal cancer is increased by 30%. Even after mucosectomy, inadvertent small mucosal residual islands remain. These residual islands carry the potential for the development of subsequent malignancy. We reviewed the literature (1975–2012) on the incidence, nature, and possible etiology of subsequent ileal-pouch and anal transit zone adenocarcinoma after prophylactic surgery procedure for FAP. To date there are 24 studies reporting 92 pouch-related cancers; 15 case reports, 4 prospective and 5 retrospective studies. Twenty three of 92 cancers (25%) developed in the pouch mucosa and 69 (75%) in anal transit zone (ATZ). Current recommendation for pouch surveillance and treatment are presented. Data suggest lifetime surveillance of these patients.

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Rectal and Pouch Recurrences After Surgical Treatment for Familial Adenomatous Polyposis

Cited by 25 publications

References 48 publications

Evaluating Causes of Death in Familial Adenomatous Polyposis

Evaluating Causes of Death in Familial Adenomatous Polyposis

ACG Clinical Guideline: Genetic Testing and Management of Hereditary Gastrointestinal Cancer Syndromes

Adenocarcinomas after Prophylactic Surgery for Familial Adenomatous Polyposis

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