卵巢生长性畸胎瘤综合征并文献复习

Ovarygrowing teratoma syndrome of report and literature review

目的探讨卵巢生长性畸胎瘤综合征(growing teratoma syndrome,GTS)的临床病理特征、治疗及预后。方法回顾性分析1例卵巢GTS的临床和病理资料,总结其特殊的病程特点和病理学变化。结果超声检查见右侧卵巢包块大小93 mm×72mm,肿瘤大体呈囊实性,实性区以菜花样组织为主,质软,囊内见少量毛发及油脂;镜下肿瘤实性区见多灶未成熟原始神经管;免疫表型:NSE呈阳性,Syn、S-100、CD99、CD56呈局灶阳性;术前肿瘤标志物AFP 48.7 ng/mL、CA125 84.2 U/mL,手术切除右侧卵巢包块、子宫、双侧附件、大网膜及阑尾,术后化疗2次。患者术后17个月复发盆腔包块,彩超示大小47 mm×35 mm×24mm,肿瘤肉眼观察呈实性,质软,部分为骨性组织,镜下可见少量不成熟软骨及间叶组织,肿瘤标志物在正常范围,行盆腔内包块切除,左、右盆腔行淋巴结清扫。术后1个月复查CA125轻度升高,给予连续5次化疗。随访19个月,患者无复发。结论GTS的诊断需结合全部病程,术后复发肿瘤病理诊断为成熟性畸胎瘤是鉴别GTS的重要提示,故应定期检测肿瘤标志物和行腹盆腔超声检查,依据患者个体情况进行合理治疗,避免不必要的化疗。保留年轻患者生育能力和防治术后并发症,是保证良好预后的关键。

Purpose To study the clinical characteristics of disease development, pathology, treatment and prognosis in ovarian growing teratoma syndrome （GTS）. Methods The clinical and pathological data of 1 eases of ovarian GTS were analyzed retrospectively, and the special course of disease and path-ological changes were summarized. Results Ultrasound examination revealed a mass measuring 93 mm×72 mm in the right ovary. Grossly, the tumor was cystic and solid , and solid area was mainly cauliflower like, with soft texture and small amount of hair and grease. Histopathologieal examination showed there were multifocal immature primitive neural tube in the solid area. Immunohistochemical staining showed the tumor cell were posi- tive for NSE and focal positive for Syn, S-100, CD99, and CD56. In the preoperation, AFP and CA125 were 48.7 ng/mL and 84. 2 U/mL. The right ovary mass and uterus, bilateral appendages, omentum and appendix were resected by radical operation, and two courses of chemotherapy postoperative were given. After 17 months, a solid peritoneal mass mesauring 47ram×35mm×24mm, was found in the original position by gynecological ultrasound. Grossly, it was solid and soft with some bone tissue. Histopathological examination showed a few immature carti- lage and mesenchymal tissue. Tumor markers were within the normal range, the peritoneal mass and bilateral pelvic lymph nodes were excised thoroughly. A slight elevated in CA125 one month after surgery. After successive five courses of chemothera- py, no tumor recurred for 19 months follow up. Conclusion The diagnosis of GTS requires a combination of the overall course of the disease. The pathologic diagnosis of mature teratoma on postoperative recurred tumor are important indications for the differential diagnosis of GTS. Therefore, the tumor markers and abdominal and pelvic ultrasonography should be checked regularly, and reasonable treatment should be carried out according to the individual condition of the patient to avoid unnec-essary chemotherapy. Keeping the fertility of young patients and preventing postoperative complications is the key to ensure a good prognosis.