正常大小卵巢癌综合征的临床病理特征研究

Clinicopathological characteristics of normal sized ovarian carcinoma syndrome

目的观察正常大小卵巢癌综合征（normal sized ovarian carcinoma syndrome，NOCS）患者的临床病理特点，探讨其治疗方法和预后相关因素。方法回顾性分析2001-01-01—2012-06—30山东省肿瘤医院妇瘤科收治的39例NCICS患者，从同期治疗的1768例初治卵巢浆液性乳头状腺癌患者中随机选取100例作为对照。结果NOCS组发病年龄晚，中位年龄55岁。体征多表现为腹水和腹腔包块，分别为74．4％（29／35）和69．2％（27／35），Ⅲ期患者为88．6％（31／35）。手术中肉眼观察双侧卵巢正常大小或萎缩，表面可见菜花样结节或乳头状突起；大网膜形成饼状团块为73％；病理为原发性卵巢浆液性腺癌（primarily serous adenocarcinoma of ovary carcinoma，PSOC）32例，卵巢外腹膜浆液性乳头状腺癌（extraovarian peritoneal serous papillary carcinoma，EPSPC）5例，腹膜恶性间皮瘤2例，NOCS血清CA125水平明显升高，246．9～5013U／mL，中位数为2761．8U／mL。术后行辅助化疗取得较长的生存期。残余灶直径≤1cm的生存期明显长于直径〉1cm者。NOCS组患者中位生存期为23．4个月。NOCS组和卵巢浆液性腺癌组1年生存率分别为73．7％（28／38）和92．0％（91／99），差异有统计学意义，P=0．001；3年生存率分别为39．1％（9／23）和74．0％（57／77），差异有统计学意义，P〈O．001；5年生存率分别为18．2％（2／11）和35．8％（24／67），差异有统计学意义，P=0．014。结论NOCS发病隐匿，早期诊断困难，需剖腹探查或腹腔镜检查后明确诊断。治疗采取手术＋化疗的综合治疗模式。该疾病对化疗较敏感。残存肿瘤的大小是影响患者生存期的一个重要因素。

OBJECTIVE To explore the clinical features, therapeutic methods and prognostic factors of normal sized ovarian carcinoma syndrome （NOCS）. METHODS Thirty-nine cases with normal-sized ovarian carcinoma syn- drome admitted between Jan 2001 and Jun 2012 were analyzed retrospectively. Totally 100 cases ovarian serous adenocar- cinoma were selected randomly as control group. RESULTS The median age of the patients with normal sized ovary car- cinoma syndrome was 55 years old. Abdominal mass and anorexia were the most common symptoms,ascites and abdomi- nal mass were main clinical presentations. The majority of NOCS（88.6 %） was diagnosed in stage III. Diffuse metastatic diseases of the peritoneal cavity were often observed but the ovaries were in normal size, with or without a fine granularity on their external surface. The most common findings were extensive abdomino-pelvic cavity involvement and omental cake （73 %）; all cases were confirmed as serous adenocarcinoma by pathology. The serum CA-125 level was high obviously. The median survival time was 23.4 months. The 1-year,3-year and 5-year survival rates for NOCS were 73.7%, 39.1% and 18.2%, respectively, which were lower than 92.0%, 74. 0% and 35.8% for ovarian serous adenocarcinoma（P〈 0.05）. CONCLUSIONS NOCS were more often diagnosed in elder women, with the most common presence of ascites and omental cake. Computed tomography（CT） and magnetic resonance（MR） imaging revealed massive ascites, and nodu- lar/irregular thickening of the mesentery and peritoneum, but the diameter of the ovaries was normal. Combined therapies including cytoreductive surgery and postoperative chemotherapy were essential to obtain better effect and prognosis was worse compared with ovarian serous adenocarcinoma.