CAJ | 학술논문

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～G2 9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.
목적 탐토Lynch종합정(우칭유전성비식육성결직장암)상관성란소상피성암(란소암)적림상병리특정.방법 대천진의과대학총의원2004년1월지2011년1월수치적란소암환자260례진행종류가족사문권조사화가계도보분석,병의거아모사특단Ⅱ(AmsterdamⅡ)표준진단위Lynch종합정상관성란소암(LS조),분석기발병특점급유전학특정；수궤수자표법선취동기수치적산발성란소암환자100례(산발조)작위대조,비교량조환자적림상병리특정.결과 260례란소암환자중Lynch종합정상관성란소암10례,점3.8％ (10/260)；기가계중녀성성원공115례,환악성종류30례,점26.1％ (30/115),기중Lynch종합정상관성란소암점8.7％( 10/115).LS조환자적기타상관성종류이결직장암다견,기차위자궁내막양암、유선암화위암등,종류재Lynch종합정가계중취집발생차정수직방식전체,부합상염색체현성유전특정.LS조환자적평균발병년령위(46±7)세,명현저우산발조적(56±11)세(P＜0.05)；LS조병리류형이란소장액성선암(8례,8/10)위주,국제부산과련맹(FIGO)수술병리분기Ⅰ기4례(4/10),분별여산발조[분별위64％ (64/100)、14％( 14/100)]비교,차이균무통계학의의(P＞0.05)；LS조병리분급G1～G2 9례(9/10),명현고우산발조[55％ (55/100),P＜0.05]；LS조환자적3、5년루적생존솔분별위87.5％화52.5％,균고우산발조(분별위55.4％화22.7％),차이균유통계학의의(P＜0.05).결론 Lynch종합정상관성란소암부합상염색체현성유전특정,발병년령조、조직분화호、병리류형이란소장액성선암다견,기예후호우산발성란소암.
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 Ⅱ 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 clinicalpathological characteristics of early age of onset,serous adenocarcinoma,lower grade and better prognosis.