INTRODUCTION: @entity14 ( @entity14 ) is the second most common @entity5 in Germany, however, it is extremely rare in @entity1 and adolescents. In these @entity1 , previous literature describes aggressive behavior and diagnosis at advanced stage. METHOD: Thirty-one @entity1 with @entity14 age  <= 18 years and treated between 1990 and 2012 have been identified through the structures and registries of the German Society for Pediatric Oncology and Hematology. RESULTS: The age range was 9-18 years (median 13.5  years); the median follow-up time was 43.9 months (range 1-124 months). Twenty-six @entity1 (84%) were tested for a @entity74 ( @entity74 ); of these, 11 @entity1 (35% of all @entity1 ) tested positive (eight cases of @entity4309 , one @entity1 with @entity1412 , two @entity1 with constitutional mismatch @entity14183 ). An unfavorable histology was reported in 55% of the records (n = 17), a poor differentiation (grade III) in 68% of @entity189 (n = 21). @entity1349 ( @entity1349 ) and event-free survival at 5  years was 52.0% and 65.6%, respectively. Five-year survival according to stage was 100% in stage II (n = 2), 100% in stage III (n = 13), and 12.9% in stage IV (n = 15; P < 0.001). Five-year @entity1349 in @entity1 with and without a defined @entity74 was 100% and 36.5% (P = 0.019), respectively. CONCLUSION: @entity1 and adolescents with @entity14 are frequently diagnosed in advanced stages and have an unfavorable prognosis. In this study, a high percentage of pediatric @entity14 @entity1 presented with a @entity5 and showed an especially favorable @entity1349 .

Pediatric XXXX is Associated With Excellent Outcome in the Context of Cancer Predisposition Syndromes.

@entity1 :: ('9606', 'Species') :: ['children', 'patients', 'Children', 'patient'] @entity4309 :: ('MESH:D003123', 'Disease') :: ['Lynch syndrome'] @entity5 :: ('MESH:D009369', 'Disease') :: ['adult cancer', 'tumor predisposition syndrome'] @entity189 :: ('MESH:D002277', 'Disease') :: ['carcinoma'] @entity14183 :: ('MESH:D049914', 'Disease') :: ['repair deficiency'] @entity74 :: ('MESH:D030342', 'Disease') :: ['genetic tumor syndrome', 'GTS'] @entity14 :: ('MESH:D015179', 'Disease') :: ['Colorectal carcinoma', 'CRC'] @entity1412 :: ('MESH:D011125', 'Disease') :: ['familial adenomatous polyposis'] @entity1349 :: ('MESH:D011475', 'Disease') :: ['OS', 'Overall survival']
@entity14 :: (MESH:D015179,Disease) :: ['Colorectal Carcinoma']
