摘要
目的探讨Lynch综合征（又称遗传性非息肉性结直肠癌）相关性卵巢上皮性癌（卵巢癌）的临床病理特征。方法对天津医科大学总医院2004年1月至2011年1月收治的卵巢癌患者260例进行肿瘤家族史问卷调查和家系图谱分析，并依据阿姆斯特丹Ⅱ（AmsterdamⅡ）标准诊断为Lynch综合征相关性卵巢癌（Ls组），分析其发病特点及遗传学特征；随机数字表法选取同期收治的散发性卵巢癌患者100例（散发组）作为对照，比较两组患者的临床病理特征。结果260例卵巢癌患者中Lynch综合征相关性卵巢癌10例，占3．8％（10／260）；其家系中女性成员共115例，患恶性肿瘤30例，占26．1％（30／115），其中Lynch综合征相关性卵巢癌占8．7％（10／115）。LS组患者的其他相关性肿瘤以结直肠癌多见，其次为子宫内膜样癌、乳腺癌和胃癌等，肿瘤在Lynch综合征家系中聚集发生且呈垂直方式传递，符合常染色体显性遗传特征。Ls组患者的平均发病年龄为（46±7）岁，明显低于散发组的（56±11）岁（P〈0．05）；LS组病理类型以卵巢浆液性腺癌（8例，8／10）为主，国际妇产科联盟（FIGO）手术病理分期I期4例（4／10），分别与散发组[分别为64％（64／100）、14％（14／100）]比较，差异均无统计学意义（P〉0．05）；LS组病理分级G1—G29例（9／10），明显高于散发组[55％（55／100），P〈0．05]；LS组患者的3、5年累积生存率分别为87．5％和52．5％，均高于散发组（分别为55．4％和22．7％），差异均有统计学意义（P〈0．05）。结论Lynch综合征相关性卵巢癌符合常染色体显性遗传特征，发病年龄早、组织分化好、病理类型以卵巢浆液性腺癌多见，其预后好于散发性卵巢癌。
Objective To explore the clinicalpathological characteristics of Lynch syndrome associated ovarian cancer. Methods Totally 260 cases ovarian cancer patients were admitted to Tianjin Medical University General Hospital during Jan. 2004 and Jan. 2011, among which 10 patients （LS group） belonged to Lynch syndrome associated ovarian cancer according to Amsterdam 11 criteria. One hundred ovarian cancer patients without any family cancer history were enrolled randomizely as control group （sporadic group）. Results Lynch syndrome associated ovarian cancer accounted for 3.8% （ 10/260）, the incidence rate of ovarian cancer for female family members of Lynch syndrome was 8. 7% （ 10/115 ）. Mean age at time of diagnosis in LS group was （46 ±7） years, significantly earlier than that in sporadic group [ （56± 11 ） years,P 〈 0. 05 ]. There was no statistical difference between two groups in histological type or International Federation of Gynecology and Obstetrics （ FIGO ） stage （ P 〉 0. 05 ） . Most of the tissue differentiation in LS group were well or moderate differentiated, there was statistical difference between the two groups（9/10 vs. 55% ,P 〈0. 05）. The 3-year and 5-year survival rate in LS group were 87. 5% and 52. 5% respectively, compared with 55.4% and 22. 7% in sporadic group （all P 〈 0. 05）. Conclusion Compared with sporadic ovarian cancer, Lynch syndrome associated ovarian cancer is more likely present as the cliniealpathological characteristics of early age of onset,serous adenocarcinoma,lower grade and better prognosis.
出处
《中华妇产科杂志》
CAS
CSCD
北大核心
2012年第3期201-204,共4页
Chinese Journal of Obstetrics and Gynecology
关键词
结直肠肿瘤
遗传性非息肉性
卵巢肿瘤
囊腺癌
浆液
发病年龄
预后
Colorectal neoplasms, hereditary nonpolyposis
Ovarian neoplasms
Cystadenocarcinoma, serous
Age of onset
Prognosis
