Neoplasia in adult exstrophy patients
Neoplasia in adult exstrophy patients
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DOI：
10.1046/j.1464-410x.2001.02136.x
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发表时间：
2001-05-01
影响因子：
4.5
通讯作者：
Woodhouse, CRJ
中科院分区：
文献类型：
--
作者：
Smeulders, N;Woodhouse, CRJ
目的回顾性分析103例出生于1964年以前的典型外旋畸形患者的临床资料，探讨其肿瘤发生率。这些患者被分为两组：42名被认为是发展成肿瘤的高危人群，因为他们（在某些时候）有混合的尿液和粪便在结肠直肠水库，而61从来没有接触过这样的混合物，并被认为是有低风险的neoplasina.Results在最低的35年的后续行动，完整的数据可用于61例：42例失访，其中14例为高风险肿瘤，28例为低风险肿瘤。在高风险组中，有3人患有结肠癌（其中2人在1980年之前出现并死亡），1人患有结肠原位癌，10人患有良性结肠肿瘤，3人患有膀胱癌（其中2人死亡）。在低风险人群中。有一名膀胱癌患者（死亡）和一名肾透明细胞癌患者。4例膀胱癌患者中有3例在5岁以前接受过膀胱切除术。假设所有丢失的患者均存活且无瘤形成，则先天性外翻的成人中瘤形成的风险为17.5%。主要风险是那些暴露于结直肠水库中尿液和粪便混合的人（38%）。即使在低风险患者的恶性肿瘤的风险是3.3%，中位数（范围）年龄为42（40-44）岁，这是27倍，高于年龄匹配的一般population.Conclusions结肠镜检查的患者被认为是在大肠癌的高风险似乎是一个有效的屏幕，通过确定癌前阶段，因为在这之后没有人死亡。尽管在出生后的最初几年内进行了膀胱闭合或改道手术，但外翻患者的膀胱癌发病率几乎是年龄匹配的普通人群的700倍。早期的Cybergene没有保护作用。
Objective To document the incidence of neoplasia in a cohort of 103 patients born with classical exstrophy.Patients and methods The notes of patients born before 1964 with exstrophy were reviewed retrospectively. The patients were divided into two groups: 42 were thought to be at high risk of developing neoplasia because they had (at some time) had mixing of urine and faeces in a colorectal reservoir, whereas 61 had never been exposed to such a mixture and were thought to have a low risk of neoplasia.Results At a minimum of 35 years of follow-up, complete data were available for 61 patients: 42 were lost to follow-up, of whom 14 were at high risk and 28 at low risk of neoplasia. In the high-risk group, there were three with colonic carcinoma (two of whom presented before 1980 and died), one with carcinoma in situ of the colon, 10 with benign colonic neoplasms and three with bladder cancer (two of whom died). In the low-risk group. there was one patient with bladder cancer ( who died) and one with a clear cell carcinoma of the kidney. Three of the four patients with bladder cancer had undergone cystectomy before 5 years of age. Assuming that all the lost patients are alive and free of neoplasia, the risk of neoplasia in adults born with exstrophy is 17.5%. The main risk is in those who have been exposed to mixing of urine and faeces in a colorectal reservoir (38%). Even in low-risk patients the risk of malignant neoplasia is 3.3% at a median (range) age of 42 (40-44) years, which is 27 times higher than that of the age-matched general population.Conclusions Annual colonoscopy of patients deemed at high risk of colorectal neoplasia appears to be an effective screen for colorectal carcinoma, by identifying a premalignant stage, as there were no deaths after this was introduced. Despite bladder closure or diversion surgery within the first few years of life, patients with exstrophy have an almost 700-fold greater incidence of carcinoma of the bladder than the age-matched general population. Early cystectomy is not protective.