Malignant potential in XXXX .

@entity1 @entity417 @entity1127 @entity5 @entity189 @entity6518 @entity600 @entity1820 @entity6523 @entity950 @entity1412 @entity360
BACKGROUND: Unlike @entity1412 , where the premalignant nature of @entity6523 is well established, the @entity5 risk in @entity1412 has generally been considered not increased. METHODS: This study reviews all cases of @entity1412 reported in the English language to date to assess the occurrence and prognosis of @entity189 in the gastrointestinal tract. RESULTS: A total of 218 @entity1 met the inclusion criteria. Mean age at diagnosis was 18.5 years (range: 9 months to 67 years). No gender preference was identified. The most common presenting symptom was @entity417 , followed by @entity360 , @entity1127 , @entity6518 , and intussusception. A family history of @entity1412 could be established in approximately 50% of @entity1 , and associated @entity600 were detected in 15%. Ninety-nine @entity1 underwent 138 gastrointestinal operations: 121 colorectal, 12 gastric, and 5 small intestinal procedures. The development of a @entity1820 was reported in 36 cases (17%). Mean age at diagnosis of @entity189 was 35.5 years (range: 4-60 years). Most @entity5 were located in the distal colon and rectum, with only one case of gastric and one case of @entity189 . @entity5 stage at diagnosis was usually advanced, with poor survival figures. CONCLUSIONS: This study shows that @entity950 carry a more significant risk of @entity189 than generally appreciated. Therefore, more intense endoscopic surveillance may be warranted, and definitive surgical options should often be considered in these syndromes.
