[Colorectal cancer in familial adenomatous polyposis: Are there clinical predictive factors?]. / El cáncer colorrectal en la poliposis adenomatosa familiar: ¿existen factores clínicos de predicción?

BACKGROUND: Familial Adenomatous Polyposis (FAP) is a hereditary disorder with multiple colorectal polyps that exhibit an almost inevitable risk of colorectal cancer (CRC) in untreated patients. GOALS: To evaluate clinical features related to CRC risk at diagnosis. MATERIAL AND METHODS: Charts from 88 patients were reviewed to collect information regarding age, family history, symptoms, polyposis severity and association with CRC. RESULTS: 41 men (46.6%) and 47 women (53.4%) were assisted. CRC was detected in 53 patients (60.2%), with a frequency of 9.1% under 20 years, 58% between 21-40 and 85% over 41 years of age. Average age of patients without CRC was lower at treatment (29.5 vs. 40.0 years; p=0.001). Family history was reported by 58 patients (65.9%), whose average age did not differ from those who didn't report it (33.4 vs. 34.4; p=0.17). Asymptomatic patients comprised 10.2% of the total; in this group, CRC incidence was much lower when compared to those presenting symptoms (1.1% vs. 65.8%; p=0.001). Patients without CRC presented a shorter length of symptoms (15.2 vs. 26.4 months; p=0.03) and less frequent weight loss (11.4% vs. 33.9%; p=0.01). At colonoscopy, polyposis was classified as attenuated in 12 patients (14.3%), who presented greater average age (48.2 vs. 33.3 years; p=0.02) and equal CRC incidence (58.3% vs. 58.3%; p=0.6) when compared to those with classic polyposis. CONCLUSIONS: The risk of CRC in FAP patients 1) increases significantly after the second decade; 2) is associated with higher age, weight loss, presence and duration of simptomatology; 3) is similar in patients with attenuated or classic phenotype.

Evaluating causes of death in familial adenomatous polyposis.

BACKGROUND: Familial adenomatous polyposis is a genetic syndrome associated with an increased risk of colorectal cancer (CRC) and different extracolonic manifestations. GOALS: The goal of this study is to evaluate the frequency of death causes. MATERIAL AND METHODS: Charts from 97 patients treated from 1977 to 2008 were reviewed. Retrieved data and family information allowed us to classify causes of death in those related to CCR to other malignancies or other causes. RESULTS: There were analyzed data from 46 men (47.4%) and 51 women (52.6%) with an average age of 35.1 years (14 to 82). At diagnosis, 57 patients (58.7%) already had CRC-associated polyposis. There were performed 93 colectomies, one internal diversion, and one partial resection. Two patients were not operated on. Results from 19 deceased patients (19.5%) were analyzed. CRC, other tumors (desmoid tumors, lymphoma, and gastric cancer), and other causes (complication of duodenal cancer surgery, complication after ileorectal anastomosis (IRA), and coronary disease) were responsible for 12 (63.1%), four (21.1%), and three (15.8%) of all deaths, respectively. Death from CRC occurred in the context of either systemic, rectal, or pouch recurrence. Desmoid disease was the second cause of death (10.5% of all causes), leading to a fatal outcome 22% of all patients who developed DT during the study period. Upper digestive carcinomas were responsible for other two death cases. CONCLUSIONS: (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.

Incidence and management of perineal hernia after laparoscopic proctectomy.

Perineal hernia (PH) is formed by the protrusion of intra-abdominal viscera through a defect in the pelvic floor. This is a rare complication after conventional abdominoperineal resection, pelvic exanteration, proctectomy, and other pelvic procedures. The purpose of the present paper is to report 4 cases of PH after laparoscopic abdominoperineal resection for rectal cancer and to review literature data about the incidence, predisposing factors, and treatment of this challenging problem. When added to other 3 cases previously reported in the Brazilian series of laparoscopic surgery, this group of 7 cases comprises a PH incidence of 3.5% after rectal resection procedures. Surgical treatment is indicated only in symptomatic patients with no signs of cancer recurrence. Proposed methods of surgical repair include abdominal, perineal, or combined approaches to the hernia in association with the use of autologous tissues or prosthetic meshes. Preventive measures are represented by closure of the pelvic peritoneum whenever possible, primary perineal suture and wound care to avoid infection.