Anaplastic pilocytic astrocytoma of the cerebellum presenting with conus medullaris drop metastasis

Jared Sweeney, Vaibhav Chumbalkar, Michael D. Staudt, P. Entezami, J. Qian, V. Sukul
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Abstract

Due to its rarity, a complete understanding of the clinical behavior, pathogenesis, and diagnostic definition of anaplastic pilocytic astrocytoma (APA) is currently lacking. The optimal clinical management and use of adjuvant therapies has yet to be defined. We present a 64 year-old-female with progressive headaches, dysarthria, and ataxia, who was found to have right cerebellar mass. A gross total resection was achieved through two staged operations. Pathology demonstrated focal areas of necrosis, tumor infiltration, and increased mitotic activity most consistent with APA. Adjuvant chemotherapy and stereotactic radiosurgery were administered. Approximately two years later, the patient presented with symptoms of cauda equina syndrome, and lumbar spine imaging demonstrated a large intradural mass at the conus medullaris with diffuse leptomeningeal enhancement. A biopsy was performed and was consistent with metastatic APA. APA may rarely progress to metastatic disease, most frequently involving the leptomeninges of the posterior fossa and cervical spine. This report represents the first case of metastases distal to the cervicomedullary junction.
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小脑间变性毛细胞星形细胞瘤表现为髓圆锥滴转移
由于其罕见性,目前对间变性毛细胞星形细胞瘤(APA)的临床行为、发病机制和诊断定义缺乏完整的了解。辅助疗法的最佳临床管理和使用尚未确定。我们报告一位患有进行性头痛、构音障碍和共济失调的64岁女性,她被发现有右侧小脑肿块。病理学显示坏死、肿瘤浸润和有丝分裂活性增加的局灶性区域与APA最为一致。给予辅助化疗和立体定向放射外科治疗。大约两年后,患者出现马尾综合征症状,腰椎影像学显示脊髓圆锥处有一个巨大的硬膜内肿块,并伴有弥漫性软脑膜增强。进行了活检,结果与转移性APA一致。APA可能很少发展为转移性疾病,最常见的是后颅窝和颈椎的软脑膜。本报告代表了第一例颈髓连接处远端转移的病例。
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