Ishida Hideyuki, Ishibashi Keiichiro, Iwama Takeo
Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University, 1981 Kamoda, Kawagoe, Saitama, 350-8550, Japan.
Surg Today. 2018 Mar;48(3):253-263. doi: 10.1007/s00595-017-1538-2. Epub 2017 May 26.
The risk of malignant tumors developing in association with juvenile polyposis syndrome (JPS) was evaluated to explore the optimal treatment for this rare disease.
We reviewed the data on JPS cases reported in Japan between January, 1971 and March, 2016.
A total of 171 cases were evaluable. Of these 171 patients, 83 (48.5%) were female and the median age at diagnosis was 28 years (range 1-80 years). The polyps were located in the stomach alone (n = 62; 36.3%), in the stomach and intestine (n = 47; 27.4%), or in the colorectum alone (n = 62; 36.3%). The sites of malignant tumors were the stomach (n = 31), colorectum (n = 29), small intestine (n = 2), breast (n = 1), and thyroid (n = 1). The lifetime risk (at 70 years) of any malignant tumor was 86.2%. The lifetime risk of gastric cancer was 73.0% and that of colorectal cancer was 51.1%. The risk of these cancers developing was dependent on the type of polyp distribution.
Long-term surveillance of the stomach and colorectum based on the phenotype of JPS seems a reasonable approach to monitor these patients for the development of malignant tumors.
评估与幼年息肉病综合征（JPS）相关的恶性肿瘤发生风险，以探索针对这种罕见疾病的最佳治疗方法。
我们回顾了1971年1月至2016年3月间日本报道的JPS病例数据。
共有171例病例可供评估。在这171例患者中，83例（48.5%）为女性，诊断时的中位年龄为28岁（范围1 - 80岁）。息肉仅位于胃部（n = 62；36.3%）、胃部和肠道（n = 47；27.4%）或仅位于结直肠（n = 62；36.3%）。恶性肿瘤的发生部位为胃部（n = 31）、结直肠（n = 29）、小肠（n = 2）、乳腺（n = 1）和甲状腺（n = 1）。任何恶性肿瘤的终身风险（70岁时）为86.2%。胃癌的终身风险为73.0%，结直肠癌的终身风险为51.1%。这些癌症发生的风险取决于息肉分布类型。
基于JPS的表型对胃部和结直肠进行长期监测，似乎是监测这些患者恶性肿瘤发生情况的合理方法。
