DFV Neuro, São Paulo, Brazil.
Hospital Alemão Oswaldo Cruz, São Paulo, Brazil.
J Neurol Surg A Cent Eur Neurosurg. 2022 Jul;83(4):388-390. doi: 10.1055/s-0040-1720990. Epub 2021 Feb 22.
Chordoma is a malignant and aggressive tumor originating from remnants of the primitive notochord and usually involving the axial skeleton. Spontaneous regression of clival chordomas was described recently. We present the third case report of spontaneous regression of a clival chordoma and discuss similarities of cases and implications for clinical practice.
We present the case of a previously healthy 21-year-old Caucasian woman who presented with progressive holocranial headache for 3 months, which encouraged image investigation. Magnetic resonance imaging (MRI) revealed an osteolytic clival lesion hyperintense in T2 and hypointense in T1 images. After 2 months of initial evaluation and surgical proposal, she repeated MRI to allow use for intraoperative neuronavigation. Surprisingly, there was tumor regression.
The present reported case is somehow different from previous ones and does not share an underlying inflammatory/immunological recognizable fact, being interpreted by us as a spontaneous partial regression of the tumor. We highlight the need for continuous investigation of chordoma regression to uncover the underlying mechanisms.
脊索瘤是一种起源于原始脊索残余物的恶性和侵袭性肿瘤,通常累及轴性骨骼。最近描述了颅底脊索瘤的自发消退。我们报告了第三例颅底脊索瘤自发消退的病例,并讨论了病例的相似性及其对临床实践的意义。
我们报告了一例先前健康的 21 岁白人女性,她因 3 个月的进行性全颅头痛就诊,这促使进行了影像学检查。磁共振成像(MRI)显示 T2 图像呈高信号,T1 图像呈低信号的溶骨性颅底病变。在最初评估和手术建议后 2 个月,她再次进行 MRI 检查,以便用于术中神经导航。令人惊讶的是,肿瘤出现了消退。
本报告的病例与之前的病例有所不同,没有可识别的炎症/免疫相关因素,我们将其解释为肿瘤的自发部分消退。我们强调需要对脊索瘤的消退进行持续研究,以揭示潜在的机制。