卵巢环状小管性索瘤临床及病理分析

Clinical and pathological analysis of patients with ovarian sex cord tumor with annular tubules

目的:探讨卵巢环状小管性索瘤(sex cord tumor with annular tubules,SCTAT)的临床表现、病理特征、临床诊治方法及预后。方法:报道4例卵巢环状小管性索瘤的临床诊治过程、病理特征及随诊情况。结果:4例SCTAT患者中,1例合并黏膜黑斑-息肉综合征(Peutz-Jeghers syndrome,PJS)。平均发病年龄28岁,典型临床表现为月经紊乱,盆腔包块。病理特征为肿瘤细胞形成单一或复合的闭合环状小管,其核心为嗜酸性透明物质。治疗以手术为主。2例术后随访良好,2例出现复发,其中1例多次复发,且发生远处转移,死亡。复发以原肿瘤部位同侧的盆、腹膜后淋巴结转移多见。5年总生存率为100%。结论:患侧附件切除+患侧盆腹腔淋巴结清扫术是治疗SCTAT的有效方法,复发病例建议完全切除肿瘤,长期随访。尽管复发风险高,但SCTAT预后相对较好。

Objective:To investigate the clinical manifestations,pathological features,clinical diagnosis and treatment and prognosis of ovarian sex cord tumor with annular tubules(SCTAT).Methods:4 cases of SCTAT were reported,and the clinical diagnosis and treatment,pathological features and follow-up were described.Results:Among the 4 patients with SCTAT,1 patient was combined with Peutz-Jeghers syndrome(PJS).The average age of onset was 28 years old.The pathological features showed that the tumor cells formed single or compound closed annular tubules with eosinophilic hyaline substance at the core.The treatment was mainly surgery.Postoperative follow-up was good in 2 patients,and recurrence occurred in 2 patients,including 1 patient with multiple recurrence,distant metastasis and death.Recurrence was more common in the ipsilateral pelvic and retroperitoneal lymph node metastasis.The 5-year overall survival rate was 100%.Conclusion:Adnexectomy and pelvic lymph node dissection is an effective method for the treatment of SCTAT.Complete tumor resection is recommended for recurrent cases and long-term follow-up is recommended.Despite the high risk of recurrence,SCTAT has a relatively good prognosis.