卵巢粒层细胞瘤的研究进展

Latest Research Progress of Ovary Granulosa Cell Tumors

卵巢粒层细胞瘤(GCT)是一种少见的卵巢功能性肿瘤,占卵巢肿瘤的2%～5%。卵巢GCT按照组织病理学类型分为成年型GCT(AGCT)及幼年型GCT(JGCT),其病因迄今尚未阐明。其临床上最常见临床症状为雌激素刺激症状。手术是卵巢GCT的首选治疗方法。卵巢GCT具有远期复发特点,目前认为其临床分期是影响患者预后最重要的因素,抑制素(inhibin)水平、抗苗勒管激素(AMH)等可作为诊断卵巢GCT的血清学标志物,为其复发提供一定的临床病理学诊断参考。研究发现,在AGCT中,翼状螺旋/叉头转录因子(FOXL)2基因中存在一个单一、复发的体细胞突变(402C→G),而在JGCT患者中却少有该突变。FOXL2及其突变体对卵巢GCT发展的病理生理作用,迄今尚不清楚。总之,卵巢GCT患者的总体预后较好,而复发患者的预后相对较差,因其具有远期复发特点,所以应对该病患者坚持长期随访。

Ovarian granulosa cell tumor （GCT）,a group of low grade and potential recurrence functional tumor,with the adult GCT （AGCT）and juvenile GCT（JGCT）.Ovarian GCT is one of the ovarial sex cord stromal tumors,accounting for 2%-5% of ovarial tumors.Etiology of GCT is still unclear.The most common symptom of GCT patients was menstrual disorder or abnormal vaginal bleeding.Surgery is the mainstay of initial management for patients with a suspected GCT.Ovarian GCT are characterized with recurrence.Stages of GCT are always indicative to be prognosis factors.Inhibin and anti-Mullerian hormone （AMH）were reported to have a certain value for diagnosis and follow-up of GCT.A single recurrent somatic mutation of forkhead transcription factor （FOXL）2 gene c.402C→G in whole transcriptome sequencing of FOXL2 gene in AGCT.While the mutation were absent in JGCT patients.To date,its biological role of this mutation is yet unclear.In summary,patients with early stage of GCT have an excellent prognosis.Recurrence of GCT is associated with a poor prognosis.For its long-term recurrence, long-term follow-up is needed.