@entity420 in XXXX .

@entity1 @entity5 @entity30 @entity2802 @entity1744 @entity204 @entity420
BACKGROUND: An association between @entity420 and @entity30 ( @entity30 ) is well-established. We present our recent experience with this entity in order to further elucidate its clinicopathological features and update our series from 1991. METHODS: A retrospective review was undertaken of all surgical @entity1 with @entity420 and @entity30 seen at our institution between 1993 and 2009. Follow-up was assessed until time of @entity204 or by interview with survivors. Survival was calculated based on @entity2802 ( @entity5 extent, lymph node status, metastases staging) staging and comparing between our current and previous series. RESULTS: Twenty-nine @entity1 (ten females and 19 males) were identified and followed for a median of 2 years. The median age at onset of @entity30 symptoms was 25, and the median age at @entity5 diagnosis was 55.4, for a mean interval of 25.3 years. Twenty-two @entity5 were ileal and five were jejunal. There were no @entity5 in excluded intestinal loops. Significant differences in 2-year survival were determined for: node-negative (79.3%, 95%CI 58.3-100%) versus node-positive @entity5 (49% %, 95%CI 20.0-78.0%), and for localized (92.3%, 95%CI 77.8-100%) versus metastatic disease (33.3%, 95%CI 6.6-60%). Overall 36-month survival was 69.3% (95%CI 51.5-87.1%) compared to 40% among those without excluded loops in our series from 1991. Sixteen @entity1 had long periods of quiescent disease before diagnosis (7-45 years), and 16 required surgery for bowel obstruction that was refractory to medical management. Adequate information was not retrievable for three @entity1 . CONCLUSIONS: A comparison to our previous series reveals similar clinical characteristics and a high rate of node-positive @entity5 at diagnosis. Our findings also confirm two important clinical indicators of @entity5 : recrudescent symptoms after long periods of relative quiescence and @entity1744 that is refractory to medical therapy.
