Bashti Malek, Di Long, Daftari Manav, Jaman Emade, Cardinal Tyler, Robinson Michael W, Boddu James V, Abla Adib
Neurological Surgery, University of Miami Miller School of Medicine, Miami, USA.
Cureus. 2024 Aug 27;16(8):e67937. doi: 10.7759/cureus.67937. eCollection 2024 Aug.
We present a rare case of an intraparenchymal chordoma in the brain stem of a 69-year-old male with a history of multiple chordoma recurrences. Chordomas are uncommon tumors that originate from notochordal remnants, with intraparenchymal presentations in the brain stem being particularly rare. A 69-year-old male with a history of clival chordoma three years after primary endoscopic resection and adjuvant proton-beam radiotherapy and a recurrence one year postoperatively for which he underwent a second surgery, presented with severe headaches, weakness, diaphoresis, and difficulty ambulating. Head CT in the ER revealed a 2.7 x 3.5 cm hyperdense lesion in the pons, indicating acute hemorrhage. Magnetic resonance imaging (MRI) suggested a hemorrhagic radiation-induced cavernoma. A right retrosigmoid craniotomy was performed, and the lesion was resected without major complications. Final pathology reported an intraparenchymal hemorrhagic chordoma. To our knowledge, this is the first case of intra-axial chordoma, particularly in the brain stem. It highlights the importance of considering intraparenchymal chordoma on the differential when evaluating for recurrence versus other treatment-induced pathologies and changes. This may prompt the neurosurgeon to reconsider treatment options and weigh the risks of watchful waiting versus biopsy or even aggressive surgical management.
我们报告了一例罕见的脑实质内脊索瘤病例，患者为一名69岁男性，有多次脊索瘤复发史。脊索瘤是一种罕见的肿瘤，起源于脊索残余组织，脑实质内表现尤其罕见。一名69岁男性，曾接受过初次内镜下切除术及辅助质子束放疗，三年前患有斜坡脊索瘤，术后一年复发并接受了二次手术，此次因严重头痛、乏力、多汗及行走困难前来就诊。急诊头部CT显示脑桥有一个2.7×3.5厘米的高密度病变，提示急性出血。磁共振成像（MRI）提示为放射性诱导的出血性海绵状血管瘤。遂行右侧乙状窦后开颅手术，病变切除顺利，无重大并发症。最终病理报告为脑实质内出血性脊索瘤。据我们所知，这是首例脑内脊索瘤病例，尤其是位于脑干的。这凸显了在评估复发与其他治疗引起的病变及变化时，将脑实质内脊索瘤纳入鉴别诊断的重要性。这可能促使神经外科医生重新考虑治疗方案，并权衡密切观察等待与活检甚至积极手术治疗的风险。
