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松果体成熟畸胎瘤全切术后3年发生的转移性β人绒毛膜促性腺激素分泌性生殖细胞肿瘤的成功治疗。

Successful treatment of metastatic βHCG-secreting germ cell tumor occurring 3 years after total resection of a pineal mature teratoma.

作者信息

Cardellicchio Stefania, Farina Silvia, Buccoliero Anna Maria, Agresti Benedetta, Genitori Lorenzo, de Martino Maurizio, Fangusaro Jason, Sardi Iacopo

机构信息

Neuro-Oncology Unit, Department of Paediatric Medicine, Meyer Children's University Hospital, viale G. Pieraccini 24, 50139, Florence, Italy.

出版信息

Eur J Pediatr. 2014 Aug;173(8):1011-5. doi: 10.1007/s00431-014-2282-x. Epub 2014 Feb 21.

Abstract

UNLABELLED

Patients diagnosed with intracranial teratoma are at risk for developing a recurrent malignant germ cell tumor. We describe a 14-year-old boy initially diagnosed with a mature teratoma in the pineal region that recurred as a metastatic beta-human chorionic gonadotropin (βHCG)-secreting germ cell tumor 3 years after gross total resection. A surveillance brain MRI scan during follow-up demonstrated multiple lesions within the ventricular and subependymal area infiltrating the brain parenchyma along with concomitant elevated levels of βHCG in both the serum and cerebrospinal fluid. The patient underwent chemotherapy with PEI (cis-platinum, etoposide, ifosfamide) followed by radiation therapy according to the SIOP CNS GCT protocol. The patient is currently alive without evidence of disease 35 months after starting therapy.

CONCLUSIONS

A careful and long-term follow-up including scheduled tumor markers as well as surveillance MRI scans is required for patients with intracranial teratoma in an effort to detect and diagnose recurrent malignant disease, especially since multimodal therapy provides the potential for long-term cure.

摘要

未标注

被诊断为颅内畸胎瘤的患者有发生复发性恶性生殖细胞瘤的风险。我们描述了一名14岁男孩,最初被诊断为松果体区成熟畸胎瘤,在全切除术后3年复发为转移性分泌β-人绒毛膜促性腺激素(βHCG)的生殖细胞瘤。随访期间的脑部MRI监测扫描显示,脑室和室管膜下区域有多个病变,浸润脑实质,同时血清和脑脊液中的βHCG水平均升高。根据SIOP CNS GCT方案,该患者接受了PEI(顺铂、依托泊苷、异环磷酰胺)化疗,随后进行了放射治疗。开始治疗35个月后,患者目前存活,无疾病证据。

结论

对于颅内畸胎瘤患者,需要进行仔细的长期随访,包括定期检测肿瘤标志物以及监测MRI扫描,以便发现和诊断复发性恶性疾病,特别是因为多模式治疗有可能实现长期治愈。

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