11例正常大小卵巢癌综合征的临床及病理分析

Normal sized ovary carcinoma syndrome Clinical and pathological analysis of 11 cases

目的探讨正常大小卵巢癌综合征的临床特点、诊断标准、有效的治疗方法及其预后。方法回顾性分析１９８４～１９９３年在我院治疗及病理复核证实的１１例正常大小卵巢癌综合征，其中７例为性腺外苗勒氏管肿瘤，也称卵巢外腹膜浆液性乳头状腺癌（ＥＰＳＰＣ），２例卵巢浆液性腺癌，１例恶性间皮瘤，１例原发灶不明的转移性腺癌。上述无论何种类型，均宜最大限度切除肿瘤，术后残存肿瘤＜２ｃｍ，并辅以有效多程综合化疗。结果１例恶性间皮瘤存活９０个月，随访至１９９７年６月仍健在。１例ＥＰＳＰＣ已存活８７个月，随访至１９９７年１１月仍健在。９例已死亡患者平均生存１７．８个月，比文献报道存活时间长。结论对此综合征患者必须高度重视鉴别诊断，首选手术治疗，尽量切除癌灶（残存肿瘤＜２ｃｍ），术后辅以有效化疗或放疗，以获得最佳生存和预后。

Objective To evaluate clinical pathologic features, diagnostic criteria, effective treatment and prognosis of a clinically rare “normal sized ovary carcinoma syndrome”. Methods Histologic slides of 11 cases from 1984 to 1993 of normal sized ovary carcinoma syndrome were retrospectively reviewed. Seven of the 11 cases were extraovarian peritoneal serous papillary carcinoma(EPSPC). The remaining 4 cases were serous adenocarcinoma of the ovary (2 cases), malignant mesothelioma (1 case) and metastatic poorly differentiated adenocarcinoma of unknown origin (1 case). All patients received relatively thorough surgical resection combined with multiple courses of adjuvant chemotherapy. Results The patient with malignant mesothelioma survived for 90 months, one case with EPSPC survived for 87 months up to Dec 1997. The average survival time of 9 patients who died of their diseases was 17.8 months, being longer than that reported in the literature. Conclusion Following accurate diagnosis of normal sized ovary carcinoma syndrome, radical tumor resection is the first treatment of choice. For residual tumor, postoperative chemotherapy or radiotherapy is essential to obtain better prognosis.