Göbel U, Schneider D T, Teske C, Schönberger S, Calaminus G
ESPED-Geschäftsstelle, Koordinierungsstelle für Klinische Studien, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany.
Klin Padiatr. 2010 May;222(3):140-4. doi: 10.1055/s-0030-1249661. Epub 2010 May 31.
We analyzed 15 children and adolescents with extracranial germ cell tumor (GCT) and brain metastases reported to the MAHO/MAKEI registry.
Between 1982 and 2009, 2 077 patients were prospectively enrolled onto the MAHO/MAKEI studies (overall survival: 0.88+/-0.03). All patients with advanced malignant GCTs received cisplatin-based chemotherapy (overall survival: 0.81+/-0.04 (734/823).
15 patients with brain metastases were reported; in 6 of them at diagnosis and 9 respectively during follow-up (6 weeks-28 months after end of therapy, mean=10 months). Most patients were male (13/15) and adolescent (10/15). 8 patients suffered from mediastinal GCTs. Pure Choriocarcinoma (CC) or CC in combination with other histologies was diagnosed in 12 patients. Clinical symptoms were reported in most patients. In all patients with secondary brain metastases the previously normalised tumor markers AFP and/ or HCG increased again prior to the onset of neurological symptoms. Only 1 of the patients with primary brain metastases survived, whereas 4 of 9 with secondary metastases are in remission after additional treatment.
The risk for intracranial metastases increases with age, male gender and mediastinal or testicular primary site and choriocarcinoma histology. Development of neurological symptoms at initial diagnosis or during follow-up should lead to rapid clinical re-evaluation including CNS imaging and assessment of tumor markers. Treatment of brain metastases includes intensified chemotherapy and surgical resection, irradiation has to be considered in special clinical situations.
我们分析了向MAHO/MAKEI登记处报告的15例患有颅外生殖细胞肿瘤(GCT)并伴有脑转移的儿童和青少年。
1982年至2009年期间,2077例患者前瞻性纳入MAHO/MAKEI研究(总生存率:0.88±0.03)。所有晚期恶性GCT患者均接受以顺铂为基础的化疗(总生存率:0.81±0.04(734/823))。
报告了15例脑转移患者;其中6例在诊断时出现,9例分别在随访期间出现(治疗结束后6周 - 28个月,平均 = 10个月)。大多数患者为男性(13/15)且为青少年(10/15)。8例患者患有纵隔GCT。12例患者被诊断为纯绒毛膜癌(CC)或CC合并其他组织学类型。大多数患者有临床症状报告。在所有继发性脑转移患者中,先前已正常化的肿瘤标志物甲胎蛋白(AFP)和/或人绒毛膜促性腺激素(HCG)在神经症状出现前再次升高。原发性脑转移患者中只有1例存活,而继发性转移的9例患者中有4例在接受额外治疗后病情缓解。
颅内转移的风险随着年龄、男性性别、纵隔或睾丸原发部位以及绒毛膜癌组织学类型而增加。在初始诊断或随访期间出现神经症状应导致快速的临床重新评估,包括中枢神经系统成像和肿瘤标志物评估。脑转移的治疗包括强化化疗和手术切除,在特殊临床情况下必须考虑放疗。