Kwon Ji-Eyon, Ji So Young, Hwang Kihwan, Lee Kyu Sang, Choe Gheeyoung, Kim Chae-Yong, Han Jung Ho
Departments of Neurosurgery and.
Department of Neurosurgery, Seoul National University College of Medicine, Seoul, South Korea.
J Neurosurg Case Lessons. 2021 Jun 14;1(24):CASE21110. doi: 10.3171/CASE21110.
Chordomas, which are rare malignant neoplasms arising from notochordal remnants, often cause gradually progressive clinical symptoms. Intradural cranial chordomas (ICCs) are extremely rare and generally have a favorable prognosis. However, the authors reported the case of a primary ICC originating in the pineal gland presenting with recurrent thalamic hemorrhage and displaying an aggressive postoperative clinical course.
A 41-year-old man arrived at the emergency department with morning headaches and recurrent syncope that had lasted several months. Computed tomography and magnetic resonance imaging (MRI) revealed a pineal gland mass causing obstructive hydrocephalus and a subacute hematoma in the right thalamus. Three weeks after an endoscopic third ventriculostomy was performed, recurrent hemorrhage was observed in the right thalamus. The tumor was promptly removed surgically. The yellowish-white tumor did not exhibit abundant bleeding. No evidence of intratumoral hemorrhage around the hematoma pocket was found. Histopathological examination revealed the characteristics of a chordoma with minimal vascularity. MRI performed 10 weeks postoperatively for worsening headaches revealed abnormal enhancement of multiple cranial nerves, suggesting leptomeningeal seeding (LMS) of the tumor.
Despite radiotherapy and intrathecal chemotherapy, the patient's neurological status worsened; he died 2 years postoperatively. A pineal ICC may cause recurrent thalamic hemorrhage and potentially fatal LMS, even in the early postoperative period.
脊索瘤是一种起源于脊索残余组织的罕见恶性肿瘤，常导致渐进性临床症状。硬脊膜内颅脊索瘤（ICCs）极为罕见，通常预后良好。然而，作者报告了一例起源于松果体的原发性ICCs病例，该病例表现为复发性丘脑出血，并在术后呈现侵袭性临床病程。
一名41岁男性因晨起头痛和复发性晕厥持续数月而就诊于急诊科。计算机断层扫描和磁共振成像（MRI）显示松果体区肿块导致梗阻性脑积水以及右侧丘脑亚急性血肿。在进行内镜下第三脑室造瘘术后三周，右侧丘脑出现复发性出血。肿瘤随即通过手术切除。黄白色肿瘤未见大量出血。在血肿腔周围未发现肿瘤内出血迹象。组织病理学检查显示为血管较少的脊索瘤特征。术后10周因头痛加重行MRI检查发现多条颅神经异常强化，提示肿瘤软脑膜播散（LMS）。
尽管进行了放疗和鞘内化疗，患者神经功能仍恶化；术后2年死亡。即使在术后早期，松果体ICCs也可能导致复发性丘脑出血和潜在致命的LMS。
