Central Nervous System Dissemination of Solitary Sporadic Supratentorial Hemangioblastoma: A Case Report and Literature Review.

Seong-Chan Jung, In-Young Kim, Shin Jung, Tae-Young Jung, Kyung-Sub Moon, Yeong-Jin Kim, Sue-Jee Park, Kyung-Hwa Lee
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Abstract

We report a patient with whole neuroaxis dissemination of a sporadic supratentorial hemangioblastoma (HB) for more than 15 years. A 68-year-old female patient presented with severe radiating pain in the right leg. Gadolinium-enhanced lumbar spine MRI showed an intradural mass (2.5 cm in diameter) at the L4 level. The patient had been severely disabled for 22 years after a previous intraventricular brain tumor resection. At that time, the diagnosis was angioblastic meningioma, which was thought to be incorrect. At 14 years after the brain surgery, gamma knife radiosurgery was performed three times for newly developed or recurred supratentorial and infratentorial tumors in the cerebrospinal fluid pathway. The patient underwent lumbar spinal surgery, and a gross total removal of the mass was performed, which confirmed the histopathological diagnosis of HB. We reexamined the old histopathological specimen of the intraventricular tumor from 20 years ago and changed the diagnosis from angioblastic meningioma to supratentorial HB. Six months after spinal surgery, the patient underwent a second spinal surgery and brain surgery, and the histopathological diagnosis was HB following both surgeries, which was the same following the first spinal surgery. Here, we report a sporadic supratentorial HB patient who showed cranial and spinal disseminations for more than two decades along with a literature review.

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孤立性散发性幕上血管母细胞瘤的中枢神经系统扩散:病例报告和文献综述。
我们报告了一名散发性幕上血管母细胞瘤(HB)全神经轴播散的患者,该病已有 15 年之久。一名68岁的女性患者因右腿剧烈放射痛而就诊。钆增强腰椎磁共振成像(MRI)显示,L4 水平处有一个硬膜内肿块(直径 2.5 厘米)。患者曾做过脑室内肿瘤切除手术,22 年来一直严重残疾。当时的诊断是血管母细胞性脑膜瘤,但被认为是不正确的。脑部手术后 14 年,因脑脊液通路中新发或复发的幕上和幕下肿瘤,患者接受了三次伽玛刀放射外科手术。患者接受了腰椎手术,对肿块进行了大体全切除,组织病理学诊断证实为 HB。我们重新检查了 20 年前脑室内肿瘤的旧组织病理学标本,将诊断从血管母细胞脑膜瘤改为幕上 HB。脊柱手术六个月后,患者接受了第二次脊柱手术和脑部手术,两次手术的组织病理学诊断均为HB,与第一次脊柱手术的诊断相同。在此,我们报告了一名散发性幕上 HB 患者,该患者二十多年来一直表现为颅内和脊柱播散,我们还对其进行了文献综述。
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