关键词: germ cell tumor germinoma mature teratoma pediatric neurosurgery pineal gland pituitary

来  源:   DOI:10.3171/CASE2443   PDF(Pubmed)

Abstract:
BACKGROUND: Metachronous intracranial germ cell tumors (iGCTs)-unrelated, histologically different iGCTs occurring at different time points-occurring within the same patient remain a rarity. Herein, the authors report such a case and discuss the literature and potential pathophysiological mechanisms leading to this phenomenon.
METHODS: A 9-year-old boy presented with new-onset impaired balance, headaches, nausea, visual disturbances, and left facial paresis. Magnetic resonance imaging (MRI) scans revealed a suspected pineal region teratoma originating from the pineal gland with consecutive obstructive hydrocephalus. A mature teratoma was diagnosed and resected. Postoperative recovery was good, and the patient could return to his normal daily activities. However, a new, slowly progressive lesion in the sellar region with an enlarged infundibular stalk was detected on follow-up MRI 3.5 years after initial pineal region teratoma resection. Biopsy revealed a newly developed pure germinoma. The patient was treated with radiotherapy plus chemotherapy and remained relapse free at the last follow-up. Sixteen other cases have reported a surgically resected primary mature teratoma, wherein patients developed metachronous germinomas during follow-up. Different theories try to elaborate this phenomenon, yet none can completely account for it.
CONCLUSIONS: Although rare, metachronous iGCT is a phenomenon neurosurgeons should be aware of. In patients treated for iGCT, close long-term clinical, imaging, and laboratory follow-up is recommended. https://thejns.org/doi/10.3171/CASE2443.
摘要:
背景:异时性颅内生殖细胞肿瘤(iGCT)-无关,在同一患者的不同时间点发生的组织学上不同的iGCT仍然很少见。在这里,作者报告了这种情况,并讨论了导致这种现象的文献和潜在的病理生理机制。
方法:一名9岁男孩出现新的平衡障碍,头痛,恶心,视觉障碍,左侧面部麻痹.磁共振成像(MRI)扫描显示,疑似松果体区畸胎瘤起源于松果体,并伴有连续的阻塞性脑积水。诊断并切除了成熟的畸胎瘤。术后恢复良好,患者可以恢复正常的日常活动。然而,一个新的,在最初的松果体区畸胎瘤切除3.5年后,在MRI随访中发现鞍区缓慢进展的病变,漏斗状茎扩大。活检显示新发展的纯生殖细胞瘤。该患者接受放疗加化疗治疗,在最后一次随访时仍无复发。其他16例报告了手术切除的原发性成熟畸胎瘤,其中患者在随访期间出现异时生殖细胞瘤。不同的理论试图阐述这种现象,然而,没有人能完全解释它。
结论:虽然罕见,异时性iGCT是神经外科医生应该注意的现象。在接受iGCT治疗的患者中,密切长期临床,成像,建议进行实验室随访。https://thejns.org/doi/10.3171/CASE2443.
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