22例卵巢正常大小卵巢癌综合征的临床诊断及病理分析

Normal-sized ovary carcinoma syndrome-Clinical and pathological analysis of 22 cases

目的探讨正常大小卵巢综合征(normal-sized ovary carcinoma syndrome,NOCS)的临床特点、诊断标准及预后。方法回顾性分析1991-2000年在我院治疗及病理复核证实的22例正常大小卵巢癌综合征,均为我院首诊患者,其中9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(The extraovarian peritoneal serouspapillary carcinoma,EPSPC),1例原发灶不明的转移性腺癌(疑为EPSPC),7例卵巢浆液性囊腺癌3例子宫内膜样腺癌,2例透明细胞癌。上述无论何种类型,均最大限度手术切除肿瘤病灶,术后残存肿瘤尽可能<2 cm。结果1例EPSPC存活90个月,随访至2004年10月仍健在。1例双卵巢浆液性囊腺癌已存活92个月,随访至2004年11月仍健在,另1例为子宫内膜样腺癌为76个月,目前仍健在。8例已死亡EPSPC患者平均生存12.8个月,比文献报道存活时间略长。结论对此综合征患者尤其EPSPC患者,必须高度重视鉴别诊断,尽量切除癌灶(残存肿瘤<2 cm),术后辅以有效化疗或放疗,以期获得最佳生存和预后。

Objeetive To evaluate elinical-patholngic features,diagnostic criteria,effetctive treatmetna and prognosis of a clinically rare＂normal-sized ovary carcinoma syndrome＂ .Methods Histologic slides of 22 cases from 1991 to 21300 of normal-sized ovary carcinoma syndrome were retrospectively revlewed.Nine of the 22 cases were extraovaria peritoneal serous paplillary carcinoma（ EP- B SPC）. The remaining 13 cases were serous adenocarcinoma of the ovary （7cases）, endometrium like adenocarcinoma（3cases）, clear cell carcinoma（2cases）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 One of the patient with EPSPC surfives for 90 months up to Oct 2004, ome case serous adenocarcinoma survives for 92 months up to Dec 2004, and ome case endometritum like adenocareinoma surviver for 76 months up to now. The average survival time of 8 patients who died of their diseases（EPSPC） was 12.8 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 is essential to obtain better prognosis.