目的 探讨遗传性卵巢上皮性癌(卵巢癌)综合征(HOCS)的家族性遗传特点、临床病理特征、治疗及预后.方法 2000年1月至2007年1月间共收治卵巢癌患者580例,其中符合HOCS诊断者42例(遗传组),回顾性分析其临床病理资料及随访资料,并详细调查其家族伴发的恶性肿瘤情况.随机选取同期收治的散发性卵巢癌患者100例(散发组)作为对照.结果 HOCS的发生率为7.2%(42/580).42例HOCS患者来自39个家系,其中,8个家系中相关肿瘤分布于先证者同代,无法根据系谱判断其源自父系或母系;31个家系中相关肿瘤至少累及连续两代,其中27个(87%,27/31)家系来自母系、4个(13%,4/31)家系来自父系.遗传组患者平均发病年龄(49±10)岁,散发组为(55±10)岁,两组比较,差异有统计学意义(P<0.05);遗传组和散发组均以卵巢浆液性腺癌为主(其发生率分别为90%和84%,P>0.05),而散发组卵巢黏液性腺癌发生率(11%)明显高于遗传组(为0,P<0.05).遗传组主要临床表现为腹胀、食欲差,占64%,与散发组的70%比较,差异无统计学意义(P>0.05).遗传组和散发组分期均以Ⅲ期为主,分别占62%和63%,两组比较,差异无统计学意义(P>0.05).遗传组初治患者占33%(14/42),散发组占40%(40/100),两组比较,差异无统计学意义(P>0.05).手术方式以肿瘤细胞减灭术为主,遗传组接受了2次手术的患者占36%(15/42)、接受了3次或以上手术者也占36%(15/42),散发组分别占50%(50/100)、27%(27/100),两组间分别比较,差异均无统计学意义(P>0.05);遗传组平均化疗13.3个疗程,散发组平均11.8个疗程,两组比较,差异无统计学意义(P>0.05).遗传组3、5年累积生存率分别为73.6%、54.9%,散发组分别为47.4%、21.2%,两组间分别比较,差异均有统计学意义(P<0.05).结论 HOCS来自母系的发生率较高,发病年龄早,病理类型以卵巢浆液性腺癌为主,就诊时多数为晚期(Ⅲ期),主要治疗手段为肿瘤细胞减灭术+多疗程化疗的综合治疗,预后较散发性卵巢癌好.
目的 探討遺傳性卵巢上皮性癌(卵巢癌)綜閤徵(HOCS)的傢族性遺傳特點、臨床病理特徵、治療及預後.方法 2000年1月至2007年1月間共收治卵巢癌患者580例,其中符閤HOCS診斷者42例(遺傳組),迴顧性分析其臨床病理資料及隨訪資料,併詳細調查其傢族伴髮的噁性腫瘤情況.隨機選取同期收治的散髮性卵巢癌患者100例(散髮組)作為對照.結果 HOCS的髮生率為7.2%(42/580).42例HOCS患者來自39箇傢繫,其中,8箇傢繫中相關腫瘤分佈于先證者同代,無法根據繫譜判斷其源自父繫或母繫;31箇傢繫中相關腫瘤至少纍及連續兩代,其中27箇(87%,27/31)傢繫來自母繫、4箇(13%,4/31)傢繫來自父繫.遺傳組患者平均髮病年齡(49±10)歲,散髮組為(55±10)歲,兩組比較,差異有統計學意義(P<0.05);遺傳組和散髮組均以卵巢漿液性腺癌為主(其髮生率分彆為90%和84%,P>0.05),而散髮組卵巢黏液性腺癌髮生率(11%)明顯高于遺傳組(為0,P<0.05).遺傳組主要臨床錶現為腹脹、食欲差,佔64%,與散髮組的70%比較,差異無統計學意義(P>0.05).遺傳組和散髮組分期均以Ⅲ期為主,分彆佔62%和63%,兩組比較,差異無統計學意義(P>0.05).遺傳組初治患者佔33%(14/42),散髮組佔40%(40/100),兩組比較,差異無統計學意義(P>0.05).手術方式以腫瘤細胞減滅術為主,遺傳組接受瞭2次手術的患者佔36%(15/42)、接受瞭3次或以上手術者也佔36%(15/42),散髮組分彆佔50%(50/100)、27%(27/100),兩組間分彆比較,差異均無統計學意義(P>0.05);遺傳組平均化療13.3箇療程,散髮組平均11.8箇療程,兩組比較,差異無統計學意義(P>0.05).遺傳組3、5年纍積生存率分彆為73.6%、54.9%,散髮組分彆為47.4%、21.2%,兩組間分彆比較,差異均有統計學意義(P<0.05).結論 HOCS來自母繫的髮生率較高,髮病年齡早,病理類型以卵巢漿液性腺癌為主,就診時多數為晚期(Ⅲ期),主要治療手段為腫瘤細胞減滅術+多療程化療的綜閤治療,預後較散髮性卵巢癌好.
목적 탐토유전성란소상피성암(란소암)종합정(HOCS)적가족성유전특점、림상병리특정、치료급예후.방법 2000년1월지2007년1월간공수치란소암환자580례,기중부합HOCS진단자42례(유전조),회고성분석기림상병리자료급수방자료,병상세조사기가족반발적악성종류정황.수궤선취동기수치적산발성란소암환자100례(산발조)작위대조.결과 HOCS적발생솔위7.2%(42/580).42례HOCS환자래자39개가계,기중,8개가계중상관종류분포우선증자동대,무법근거계보판단기원자부계혹모계;31개가계중상관종류지소루급련속량대,기중27개(87%,27/31)가계래자모계、4개(13%,4/31)가계래자부계.유전조환자평균발병년령(49±10)세,산발조위(55±10)세,량조비교,차이유통계학의의(P<0.05);유전조화산발조균이란소장액성선암위주(기발생솔분별위90%화84%,P>0.05),이산발조란소점액성선암발생솔(11%)명현고우유전조(위0,P<0.05).유전조주요림상표현위복창、식욕차,점64%,여산발조적70%비교,차이무통계학의의(P>0.05).유전조화산발조분기균이Ⅲ기위주,분별점62%화63%,량조비교,차이무통계학의의(P>0.05).유전조초치환자점33%(14/42),산발조점40%(40/100),량조비교,차이무통계학의의(P>0.05).수술방식이종류세포감멸술위주,유전조접수료2차수술적환자점36%(15/42)、접수료3차혹이상수술자야점36%(15/42),산발조분별점50%(50/100)、27%(27/100),량조간분별비교,차이균무통계학의의(P>0.05);유전조평균화료13.3개료정,산발조평균11.8개료정,량조비교,차이무통계학의의(P>0.05).유전조3、5년루적생존솔분별위73.6%、54.9%,산발조분별위47.4%、21.2%,량조간분별비교,차이균유통계학의의(P<0.05).결론 HOCS래자모계적발생솔교고,발병년령조,병리류형이란소장액성선암위주,취진시다수위만기(Ⅲ기),주요치료수단위종류세포감멸술+다료정화료적종합치료,예후교산발성란소암호.
Objective To explore the clinicopathological characteristics of hereditary ovarian cancer syndrome(HOCS). Methods From Jan. 2000 to Jan. 2007, among 580 cases of primary ovarian cancer, 42 cases(herediatary group),who had a positive family history of ovarian cancer and met the diagnostic criteria of HOCS, were analyzed retrospectively. One hundred cases without a family history of ovarian cancer were enrolled randomizely as control group (sporadic group). Results The incidence of HOCS was 7.2% (42/580). Forty-two cases associated tumors affected at least 2 successive generations in 31 families and affected 1 generation in 8 families. Eighty-seven percent (27/31)was from maternal lineage, while 13% (4/31)from paternal lineage. Earlier age of onset was significantly difference between two groups[(49±10) years vs. (55±10) years, P<0.05]. There were 90% belong to serous adenocarcinoma in the herediatary group, while 84% in the sporadic group. There was statistical difference in the proportion of mucinous adenocarcinoma (0 vs. 11%, P<0.05). The most common clinical manifestations were abdominal distention and anorexia (64% vs. 70%, P>0.05), International Federational of Gynecology Obstetrics(FIGO)stage Ⅲ (62% vs. 63%, P>0.05) between two groups. Fourteen cases (33%,14/42) were previously untreated in the herediatary group, while 40 cases (40%, 40/100) in the sporadic group. There were 15 cases (36%, 15/42) underwent secondary surgery and 15 cases (36%, 15/42) underwent third surgery or more in berediatary group, while 50 cases (50%, 50/100) and 27 cases(27%, 27/100) in the sporadic group. The mean number of ehemotberapy cycles received in two groups was 13.3 and 11.8 (P>0.05). The 3-year and 5-year survival rate in herediatary group were 73.6% and 54.9% respectively, compared with 47.4% and 21.2% (P<0.05) in sporadic group. Conclusion Hereditary ovarian cancer mostly from maternal lineage are featuring in early age of onset, serous adenocarcinoma, advanced stage (stage Ⅲ), and better prognosis after the comprehensive treated by cytoreductive surgery plus with chemotherapy.
