儿童卵巢颗粒细胞瘤所致Meigs综合征并糖类抗原125水平升高1例并文献复习

Ovarian granulosa cell tumor with Meigs syndrome and elevated carbohydrate antigen 125 level in children:a case report and literature review

目的探讨儿童卵巢颗粒细胞瘤(OGCT)所致Meigs综合征的诊治。方法选择2023年2月山西省儿童医院诊治的1例OGCT所致Meigs综合征并血清糖类抗原(CA)125水平升高患儿(患儿1)为研究对象。回顾性分析其临床病例资料,并结合近30年国内外数据库中关于OGCT所致Meigs综合征相关研究文献进行文献复习,总结该病临床特点。本研究经山西省儿童医院伦理委员会批准(审批文号:IRB-WZ-2023-024)。患儿1的监护人对诊治知情同意。结果①患儿1病例资料:女性,11岁,因“腹痛、腹胀伴呼吸困难10 d”入本院。入院查体结合腹部超声、胸腹部CT提示,右侧卵巢肿物、大量腹水及双侧胸腔积液。腹腔穿刺结果为血性腹水,血清CA_(125)水平为1047 U/mL(显著高于正常值)。入院后6 h时,对其采取“左侧胸腔积液闭式引流术,右侧输卵管、卵巢及肿物+大网膜切除术”治疗。患儿1术后恢复顺利,术后第7天胸部X射线正位片显示,双侧胸腔积液消失;腹部超声结果亦未见腹水。术后对其切除组织的病理诊断结果为幼年型OGCT。术中证实肿瘤确有破裂,对其OGCT的国际妇产科联盟(FIGO)临床分期为Ⅰc期。对患儿1采取博来霉素、卡铂、依托泊苷化疗后,预后良好。患儿1出院时,被诊断为Meigs综合征(幼年型OGCT,Ⅰc期)。患儿1术后1个月复查血清CA_(125)结果显示恢复正常,术后对其随访5个月结果显示,未发现肿瘤复发及转移。②文献检索结果:按照本研究设定的文献检索策略,6例(患者2~7)OGCT所致Meigs综合征患者,加上患儿1,共计7例该病患者被纳入文献复习。这7例OGCT所致Meigs综合征患者中,3例为儿童,4例为成年人;对其OGCT病理学分型结果显示,5例(包括1例儿童)为成人型,2例为幼年型;2例血清CA_(125)水平正常,5例明显升高;7例均伴胸腔积液及腹水,OGCT最大直径均≥10 cm,均采取手术切除肿瘤治疗,对Ⅰc期者还进行术后化疗。结论儿童OGCT所致Meigs综合征临床罕见。虽然巨大卵巢肿物合并胸腔积液、腹水,并且血清CA_(125)水平升高,是卵巢恶性肿瘤的高危预测因素,但是临床应警惕Meigs综合征相关良性或低恶性卵巢肿瘤可能,避免扩大手术范围及过度治疗。

Objective To investigate the diagnosis and treatment of ovarian granulosa cell tumor(OGCT)with Meigs syndrome in children.Methods A child with OGCT-induced Meigs syndrome with elevated serum carbohydrate antigen(CA)125 level(child 1)diagnosed and treated in Children′s Hospital of Shanxi in February 2023 was selected as the study object.The clinical data of child 1 were retrospectively analyzed,and the literature related to OGCT-induced Meigs syndrome in domestic and foreign databases in the past 30 years was reviewed,and the clinical characteristics of the disease were summarized.This study was approved by the Ethics Committee of Children′s Hospital of Shanxi(Approval No.IRB-WZ-2023-024).The guardians of child 1 gave informed consent to the diagnosis and treatment.Results①Case data of child 1:a female,11 years old,was admitted to hospital with"abdominal pain,distension with dyspnea for 10 days".The admission physical examination combined with abdominal ultrasound and chest and abdominal CT showed right ovarian mass,massive ascites and bilateral pleural effusion.Abdominal puncture extracted bloody ascites and serum CA_(125)level was 1047 U/mL(significantly higher than normal).Six hours after admission,"left pleural effusion closed drainage,resection of right fallopian tube,ovary and tumor+omentectomy"was performed.child 1 recovered smoothly after surgery.The chest X-ray showed that bilateral pleural effusion disappeared,and abdominal ultrasound showed no ascites at the 7th day after surgery.The pathological diagnosis of resected tissue after operation was juvenile OGCT.It was found that the tumor did rupture during operation,and according to the Federation International of Gynecology and Obstetrics(FIGO),the clinical stage of OGCT wasⅠc.After chemotherapy with bleomycin,carboplatin and etoposide,the prognosis of child 1 were good.Child 1 was diagnosed as Meigs syndrome(juvenile OGCTⅠc)at discharge.Serum CA_(125)level of child 1 decreased to normal 1 month after operation and no tumor recurrence or metastasis was found during the 5-month follow-up after operation.②Literature retrieval results:6 cases(patients 2-7)of OGCT-induced Meigs syndrome were found according to the literature retrieval strategy set in this study,and a total of 7 cases were included in literature review plus child 1.Of the 7 OGCT-induced Meigs syndrome patients,3 were children and 4 were adults.According to pathological classification of tumor,5 cases(including 1 child)were adult type and 2 cases were juvenile type.Serum CA_(125)levels were normal in 2 cases and significantly elevated in 5 cases.All 7 cases were accompanied by pleural effusion and ascites,and the maximum diameter of OGCT were all≥10 cm.All the 7 cases underwent surgical resection of the tumor,and the patients with stageⅠc also received chemotherapy.Conclusions Pediatric OGCT-induced Meigs syndrome is rare in clinic.Although large ovarian masses combined with pleural effusion and ascites,and elevated serum CA_(125)levels are high risk predictors of ovarian malignancy,however,we should be alert to the possibility of benign or low malignant ovarian tumors related to Meigs syndrome and to avoid expanding the scope of surgery and overtreatment.