Abstract
INTRODUCTION: Long-term Familial Adenomatous Polyposis (FAP) outcomes depend on timely diagnosis and treatment. PURPOSE: To analyse the experience in FAP management over a 40-year period. MATERIAL AND METHODS: Charts from FAP patients (1982‒2023 were reviewed. Demographic data and surgical outcomes were compared between an early (1982 to 2006) and a recent period (2007 to 2023). RESULTS: 176 FAP patients were identified. Mean age at treatment (35.6 vs. 29.7), at diagnosis (35.6 vs. 29.7), and younger than 30-years (30.6 % vs. 50 %) showed that most patients were diagnosed earlier in the recent period. The authors detected a reduction of asymptomatic patients (93.5 % vs. 59.5 %, p = 0.0001) and symptoms duration (21.5 vs. 10.6 months, p = 0.0001). Simultaneously, both polyposis diagnoses were motivated by family clustering (26.1 % to 57.1 %, p = 0.02) and not associated with CRC (40.2 % to 67.8 %, p = 0.003) increased. Global CRC incidence decreased from 59.7 % to 32.1 % (p = 0.003). Gradually, pouch surgery (69 %) and the laparoscopic approach (92.7 %) turned out to be the most frequent surgical choices. Long-term mortality also diminished (19.5 % to 9.5 %) mainly due to fewer deaths resulting from CRC (11.9 % to 4.8 %). Diagnosis of desmoid disease (9.8 % to 22.6 %) and duodenal cancer (8.7 % to 2.4 %) changed differently. CONCLUSIONS: Comparison of outcomes during a 40-year period revealed a clear improvement in FAP management. This scenario resulted from 1) Continuous orientation to family members to advise an earlier diagnosis; 2) Prophylactic surgical treatment with reduced CRC association, and 3) Increased use of laparoscopic techniques with less morbidity and better outcomes.