儿童颅内混合性生殖细胞肿瘤临床及影像学表现

Clinical and imaging features of pediatric intracranial mixed germ cell tumor

目的:探讨儿童颅内混合性生殖细胞肿瘤(Mixed germ cell tumor,m GCTs)的临床及影像学特征,以提高对该病的认识。方法:回顾性分析33例经病理证实的颅内mGCTs患儿的临床及影像学特征。结果:血清肿瘤标志物升高的患儿29例。33例患儿中,5例为多中心发生,共38个病灶。2例位于脑室内。16例位于松果体区,男14例,平均8.8岁,主要症状为颅内高压。11例内见钙化,3例内见脂肪,内见比例不等的囊性成分,增强后实性成分明显强化。多中心发生的松果体区病灶表现为提前钙化的松果体。10例位于鞍区,90%的症状为尿崩症,60%为女性。多中心发生的鞍区病灶4例,共14个鞍区肿瘤,5个内见钙化,3个内见脂肪,13个瘤内伴不同程度的囊变。2例位于基底节区,主要临床表现为偏瘫、多动症,均为男性,1例双侧发病,共3个病灶。3个基底节区肿瘤中2个为实性团块,1个为不规则T2FLAIR高信号影。结论:8~13岁为颅内mGCTs好发年龄,松果体、鞍区为好发部位,也可位于脑室、基底节区,松果体区、基底节区的mGCTs男性占明显优势,不同部位m GCTs的临床及影像学表现各有特点,两者结合可提高对mGCTs的诊断信心。

Objective:To investigate the clinical and imaging features of pediatric intracranial mixed germ cell tumors(mGCTs),and to improve the understanding of this disease.Methods:The clinical and imaging data of 33 children diagnosed with intracranial m GCTs were retrospectively analyzed.Results:Serum tumor marker increased in 29 cases.Five cases showed multifocal location.Sixteen tumors were located in the pineal region,and 14 children were males with an average age of 8.8years old.The main symptom was cranial hypertension.Calcification was observed in 11 cases,fatty tissue was observed in 3 cases,and cystic components of different proportions were observed.Solid components were significantly enhanced after enhancement.The pineal lesions in mutilfocal lesions presented as early calcified pineal glands.Ten tumors were located in the sellar region,90%of children presented with diabetes insipidus and 60%were female.There were 14 sellar tumors(4 lesions in multifocal location).Calcification was observed in 5 cases,and fatty tissue was observed in 3 cases.There was cystic component in 13 cases.Two cases were located in basal ganglia region(one with bilateral lesions),both of them were male,and the main clinical features were hemiplegia and hyperactivity.Two of the 3 basal ganglia tumors were solid masses and 1 manifested as irregular T2FLAIR hyperintensity.Two cases were located in the ventricle and 5 cases were multi-center lesions.Conclusion:Intracranial mGCTs mostly occur in children with the age of 8~13 years old.Pineal,sellar,and basal ganglia regions were the predilection sites.The clinical and imaging manifestations of mGCTs at different sites were different from each other.