Evaluating Causes of Death in Familial Adenomatous Polyposis

Campos, Fábio Guilherme; Perez, Rodrigo Oliva; Imperiale, Antônio Rocco; Seid, Víctor Edmond; Nahas, Sérgio Carlos; Cecconello, Ivan

doi:10.1007/s11605-010-1288-6

Cited by 55 publications

(44 citation statements)

References 38 publications

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“…Although extra-colonic neoplasms are observed often in patients with FAP, desmoids and upper gastrointestinal malignancies are considered important as causes of death (Tulchinsky et al 2005;de Campos et al 2010). Gastric fundic gland polyps (FGPs), gastric cancer, duodenal adenoma, and ampullary adenoma complicate in patients with FAP (Kashiwagi and Spiegelman 2000).…”

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Clinical Characteristics of Gastric Cancer in Patients with Familial Adenomatous Polyposis

Shibata

Ogawa

Miura

et al. 2013

Tohoku J. Exp. Med.

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Familial adenomatous polyposis is an autosomal dominant hereditary disease leading to the development of numerous colorectal polyps with malignant potential. Extra-colonic neoplasms are observed often in patients with familial adenomatous polyposis, but clinical characteristics of gastric cancer associated with familial adenomatous polyposis are not well understood. We studied the clinical characteristics of five Japanese patients who developed gastric cancer after undergoing colectomy for familial adenomatous polyposis. Gastric cancer was found on gastroduodenal endoscopy performed during postoperative follow-up in all five patients. Mean ages at the time of colectomy and at the time of diagnosis of gastric cancer were 39.2 and 58 years, respectively. Importantly, gastric fundic gland polyps were undetectable in these five patients. The mean duration between colectomy and diagnosis of gastric cancer was more than 20 years in three of five patients (mean: 19 years and 1 month). Cancers were multiple in three of five patients. Two patients developed metachronous gastric cancer in the gastric remnant. All five patients presented with well to moderately differentiated adenocarcinoma; four of the five patients were stage IA. Characteristics of the gastric cancer with familial adenomatous polyposis include a long duration between occurrence of gastric cancer and colectomy, metachronous cancers, multicentric lesions, and a high rate of well to moderately differentiated adenocarcinoma. Long-term and periodic gastroduodenal surveillance endoscopy is recommended for patients with FAP who underwent colectomy.

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confidence: 99%

Clinical Characteristics of Gastric Cancer in Patients with Familial Adenomatous Polyposis

Shibata

Ogawa

Miura

et al. 2013

Tohoku J. Exp. Med.

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“…En el muñón rectal, el riesgo de CCR es de 25% a los 70 años de edad, con una tasa de proctectomía posterior de hasta el 60% a los 70 años 9,10 . En esta serie se logró identificar pólipos del recto remanente en el 45% de los pacientes a quienes se les había practicado una colectomía más IRA, y la mayoría de ellos fue tratado endoscópicamente.…”

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“…La decisión de cuál técnica utilizar depende de varios factores como el compromiso rectal con adenomas, la posibilidad de seguimiento endoscó-pico periódico y el deseo de fertilidad, ya que la extirpación del recto se acompaña de una reducción de la fertilidad 8,9 . Independiente de cuál sea el procedimiento quirúrgico elegido, estos pacientes requieren evaluaciones periódicas del recto remanente, por una incidencia de cáncer en el muñón rectal de 25% a los 70 años de edad, con una tasa de proctectomía posterior de hasta el 60% a los 70 años 9,10 . Por otra parte, en el reservorio ileal la posibilidad de desarrollar adenomas se incrementa con la edad del reservorio, y a los 15 años se estima que es de hasta el 75%, aunque el desarrollo de cáncer es infrecuente 11,12 .…”

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Se Justifica El Seguimiento Después De Una Colectomía en Pacientes Con Poloposis Adenomatosa Familiar

et al. 2016

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the follow up after colectomy in patients with familial adenomatous polyposis is justified background: Patients with classic familial adenomatous polyposis syndrome (FAPc) have a germline mutation in the APC gene. The most common phenotypic expression is the presence of multiple colorectal adenomatous polyps (more than 100), with high probability developing colorrectal cancer (CRC) before the fifth decade of life. Prophylactic surgery (total colectomy or restorative proctocolectomy) reduces the risk of developing CRC. However, the risk of developing tumors in other organs remains present. Objetive: Analyze the frequency and type of tumors associated with classic familial adenomatous polyposis syndrome (FAPc) patients undergoing prophylactic colectomy. material and methods: Cohort study. From the registry of hereditary colorrectal cancer (CRC) at our institution, we identified patients with FAPc who underwent total colectomy with ileorrectal anastomosis (TC-IRA) or restorative proctocolectomy (RTPC), from 1999 to 2014. In the follow-up we analyzed related tumors and mortality. results: 27 patients, of whom 18 (66.7%) underwent TC-IRA and 9 (33.3%) underwent RTPC. At the time of surgery, 4 patients had CRC (15%) and 5 had extracolonic tumors (osteomas). In a mean follow-up of 49, 4 months (i: 2 y 178) the following lesions were diagnosed: digestive tract adenomas in 17 (63%) patients, of these 2 required a proctectomy and 3 resection of duodenal adenomas. Eight patients developed desmoid tumors (30%), and 3 of them underwent surgery. One patient had an extradigestive tumor (thyroid cancer) and only 8/27 (29.6%) did not develop other tumors. One patient died due to progression of his CCR. discussion: In this series it is confirmed that most patients will develop neoplasms FAPc after colectomy. conclusion: The removal of the colon and/or rectum is able to prevent the development of CRC. However, two thirds of the patients develop other tumors in which systematic surveillance allowed early detection and treatment.

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“…As a consequence of the widespread indication of prophylactic colectomy, mortality from CRC has decreased substantially over the years (2)(3)(4), making duodenal cancer and desmoid tumors important causes of death in FAP patients (5)(6)(7)(8)(9).…”

Section: Introductionmentioning

confidence: 99%

Advanced duodenal neoplasia and carcinoma in familial adenomatous polyposis: outcomes of surgical management

Campos

Bustamante-Lopez

Kanno

et al. 2017

J. Gastrointest. Oncol.

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Background: In addition to the presence of neoplasia in the colon and rectum, patients with familial adenomatous polyposis (FAP) may develop numerous polyps and carcinoma within the upper gastrointestinal tract. Methods:The aim of the present paper was to review the incidence advanced duodenal polyposis or cancer and their surgical outcomes. A retrospective review of patients' records from our department was performed. respectively. Duodenal carcinomas occurred later (55.8 years) when compared to advanced adenomatosis (45.3 years). Three patients were diagnosed due to symptoms, while the others were detected under endoscopic surveillance. Age interval between FAP treatment and duodenal neoplasia diagnosis was 15.3 years . All but one patient underwent duodenopancreatectomy (DP). Two from the 7 patients undergoing DP died, one from pulmonary embolism 30 days after surgery and the other from recurrent T4N0 duodenal tumor. Thus, major operative morbidity and mortality were 12.5%. Conclusions:In this single-center Brazilian series of FAP patients: (I) advanced duodenal neoplasia or cancer requiring surgery occurred in 5.5% of patients; (II) when reaching the fifth decade of life, patients should be carefully evaluated to diagnose and treat early lesions; (III) in spite of the technical complexity of DP, operative morbidity is acceptable in experienced hands; and (IV) continuous surveillance is necessary during follow-up.

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Evaluating Causes of Death in Familial Adenomatous Polyposis

Cited by 55 publications

References 38 publications

Clinical Characteristics of Gastric Cancer in Patients with Familial Adenomatous Polyposis

Clinical Characteristics of Gastric Cancer in Patients with Familial Adenomatous Polyposis

Se Justifica El Seguimiento Después De Una Colectomía en Pacientes Con Poloposis Adenomatosa Familiar

Advanced duodenal neoplasia and carcinoma in familial adenomatous polyposis: outcomes of surgical management

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