双侧基底神经节生殖细胞瘤1例报告及文献复习

F. Yavuz, Y. Samancı, I. Kulac, S. Peker
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引用次数: 0

摘要

中枢神经系统生殖细胞瘤是最常见的生殖细胞肿瘤,主要影响青少年和年轻人。它们通常为中线肿瘤,主要位于松果体和鞍上区域;然而,基底神经节生殖细胞瘤(BGGs)是罕见的,通常是单侧的,迄今为止只有16例组织病理学证实的双侧BGGs报道。在本文中,我们报告一个罕见的病例双侧BGG在一个14岁的男孩。本文介绍了双侧BGGs的神经放射学表现,并与先前报道的病例一起讨论了其管理策略。一位14岁的患者因右肩和手臂不自主的抽搐而被转介到我科。MRI扫描显示双侧基底节区弥漫性T2/FLAIR高信号,MR波谱提示恶性疾病。进行了立体定向活检,标本的组织学检查显示为生殖细胞瘤。开始了新辅助化疗,然后是全心室放射治疗,以促进肿瘤。bgg大多是单方面的,但也很少看到双边实体。尽管生存率很高,但症状性结果可能不是很好。因此,识别最初的MRI表现和早期诊断这些肿瘤是至关重要的,以获得更好的结果和最小的残疾。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Bilateral Germinoma of the Basal Ganglia: A Case Report and Literature Review
Central nervous system germinomas are the most frequent germ cell tumors, predominantly affecting adolescents and young adults. They are generally midline tumors primarily located in the pineal gland and suprasellar regions; however, basal ganglia germinomas (BGGs) are rare and generally unilateral, with only 16 histopathologically-confirmed bilateral BGGs reported to date. In this paper, we are presenting a rare case of bilateral BGG in a 14-year-old boy. The neuroradiological findings of bilateral BGGs are presented, and the strategy for their management is discussed along with previously reported cases. A 14-year-old suffering from involuntary jerky movements of the right shoulder and arm was referred to our department. An MRI scan revealed diffuse T2/FLAIR hyperintensity in the bilateral basal ganglia, and MR spectroscopy suggested a malignant disease. A stereotactic biopsy was conducted, and the histologic examination of the specimen revealed germinoma. Neoadjuvant chemotherapy followed by whole ventricular radiation therapy with a boost to the tumor was initiated. BGGs are mostly unilateral, but rarely bilateral entities may also be seen. Despite excellent survival rates, symptomatic outcomes may not be very favorable. Therefore, it is crucial to recognize the initial MRI findings and diagnose these tumors early for better outcomes and minimal disabilities.
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