第四脑室卵黄囊瘤1例报告并文献复习

Yolk sac tumor in fourth ventricle(report of one case and review of literature)

目的探讨颅内卵黄囊瘤的临床特点、影像学特征及治疗方法,提高对颅内卵黄囊瘤(YST)的认识。方法回顾性分析中国医科大学附属盛京医院神经外科手术治疗的1例经病理证实的第四脑室卵黄囊瘤患儿的临床资料;并对相关文献进行复习。结果患儿行开颅显微镜下手术全切除肿瘤,术中见肿瘤基底位于小脑蚓部,充满第四脑室,约3 cm×3 cm×4 cm大小,色暗红,供血丰富,质地较软;仔细分离肿瘤周边,逐渐深入,分块完整切除肿瘤。病理检查为卵黄囊瘤。术后患儿恢复良好,随访1个月未见复发,目前仍在随访中。结论颅内第四脑室卵黄囊瘤临床非常罕见;其病因尚不明确,临床表现与肿瘤大小和部位有关,影像学检查有一定诊断价值,血清及脑脊液甲胎蛋白(alpha fetoprotein,AFP)浓度升高。目前治疗大多采用手术完整切除肿瘤,术后联合放化疗。因第四脑室卵黄囊瘤的恶性程度较高,故预后差。

Objective To explore the clinical features,imaging features and treatment of intracranial yolk sac tumor,and to improve the understanding of intracranial yolk sac tumor.Methods The clinical data of a child with yolk sac tumor(YST)in the fourth ventricle confirmed by pathology was analyzed retrospectively,and related literatures were reviewed.Results The patient underwent microsurgical resection of the lesion,and the tumor base was located in the cerebellum,filled with the fourth ventricle,about 3 cm×3 cm×4 cm in size,dark red,rich in blood supply,soft texture,carefully separated around the tumor,gradually deepening,gradually divided complete removal of the tumor.Postoperative pathological examination confirmed YST.Postoperatively,the child recovered well.No recurrence was observed during the follow-up period and It was still in follow-up.Conclusions The intracranial fourth ventricle YST is very rare,and its etiology is still unclear.The clinical manifestations are related to its size and location.The imaging examination has certain diagnostic value,and alpha fetoprotein(AFP)concentration in serum and cerebrospinal fluid is increased.At present,surgical resection is often used,combined with radiotherapy and chemotherapy because of high degree of malignancy and poor prognosis.