Coburn M C, Pricolo V E, DeLuca F G, Bland K I
Department of Surgery, Brown University School of Medicine, Providence, Rhode Island 02902, USA.
Ann Surg Oncol. 1995 Sep;2(5):386-91. doi: 10.1007/BF02306370.
Unlike familial polyposis coli, where the premalignant nature of adenomatous polyps is well established, the cancer risk in juvenile polyposis has generally been considered not increased.
This study reviews all cases of juvenile polyposis reported in the English language to date to assess the occurrence and prognosis of carcinoma in the gastrointestinal tract.
A total of 218 patients 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 chronic anemia, followed by acute gastrointestinal bleeding, rectal prolapse of polyp, protein-losing enteropathy, and intussusception. A family history of juvenile polyposis could be established in approximately 50% of patients, and associated congenital malformations were detected in 15%. Ninety-nine patients underwent 138 gastrointestinal operations: 121 colorectal, 12 gastric, and 5 small intestinal procedures. The development of a gastrointestinal carcinoma was reported in 36 cases (17%). Mean age at diagnosis of carcinoma was 35.5 years (range: 4-60 years). Most malignancies were located in the distal colon and rectum, with only one case of gastric and one case of duodenal carcinoma. Tumor stage at diagnosis was usually advanced, with poor survival figures.
This study shows that juvenile polyposis syndromes carry a more significant risk of carcinoma than generally appreciated. Therefore, more intense endoscopic surveillance may be warranted, and definitive surgical options should often be considered in these syndromes.
与家族性腺瘤性息肉病不同，后者腺瘤性息肉的癌前性质已得到充分证实，而幼年性息肉病的癌症风险通常被认为并未增加。
本研究回顾了迄今为止以英文报道的所有幼年性息肉病病例，以评估胃肠道癌的发生情况和预后。
共有218例患者符合纳入标准。诊断时的平均年龄为18.5岁（范围：9个月至67岁）。未发现性别偏好。最常见的首发症状是慢性贫血，其次是急性胃肠道出血、息肉性直肠脱垂、蛋白丢失性肠病和肠套叠。约50%的患者有幼年性息肉病家族史，15%的患者检测到相关先天性畸形。99例患者接受了138次胃肠道手术：121次结直肠手术、12次胃部手术和5次小肠手术。报告了36例（17%）胃肠道癌的发生。诊断癌症时的平均年龄为35.5岁（范围：4至60岁）。大多数恶性肿瘤位于结肠远端和直肠，仅1例胃癌和1例十二指肠癌。诊断时的肿瘤分期通常较晚，生存率较差。
本研究表明，幼年性息肉病综合征的癌症风险比一般认为的要高得多。因此，可能需要更密集的内镜监测，并且在这些综合征中通常应考虑确定性的手术选择。
