Hallenberger Tim J, von Seth Emma, Roethlisberger Michel, Guzman Raphael, Soleman Jehuda
Department of Neurosurgery, University Hospital Basel, Basel, Switzerland.
Faculty of Medicine, University of Basel, Basel, Switzerland.
J Neurosurg Case Lessons. 2024 Jul 8;8(2). doi: 10.3171/CASE2443.
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.
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.
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.
异时性颅内生殖细胞肿瘤(iGCT)——在同一患者不同时间点出现的不相关、组织学上不同的iGCT——仍然很罕见。在此,作者报告了这样一例病例,并讨论了导致这种现象的文献及潜在病理生理机制。
一名9岁男孩出现新发平衡障碍、头痛、恶心、视觉障碍和左侧面部轻瘫。磁共振成像(MRI)扫描显示疑似起源于松果体的松果体区畸胎瘤并伴有连续性梗阻性脑积水。诊断为成熟畸胎瘤并进行了切除。术后恢复良好,患者能够恢复正常日常活动。然而,在最初的松果体区畸胎瘤切除术后3.5年的随访MRI检查中,发现蝶鞍区有一个新的、缓慢进展的病变,漏斗柄增粗。活检显示为新发生的纯生殖细胞瘤。该患者接受了放疗加化疗,在最后一次随访时无复发。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发生了异时性生殖细胞瘤。不同的理论试图阐释这一现象,但均无法完全解释清楚。
尽管罕见,但异时性iGCT是神经外科医生应了解的一种现象。对于接受iGCT治疗的患者,建议进行密切的长期临床、影像学和实验室随访。https://thejns.org/doi/10.3171/CASE2443