Song Dengpan, Xu Dingkang, Gao Qiang, Hu Peizhu, Guo Fuyou
Department of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Front Oncol. 2020 Feb 11;10:99. doi: 10.3389/fonc.2020.00099. eCollection 2020.
Primary spinal cord glioblastoma multiforme (scGBM) is an uncommon entity in pediatrics, and intracranial metastasis originating in spinal cord gliomas is very rare. A 7-year-old female presented with weakness in the limbs, paralysis of the lower limbs and incontinence. The initial MRI of the spinal cord revealed expansion and abnormal signals from T2 to T5. She was initially diagnosed with Neuromyelitis optica spectrum disorders and treated with high-dose glucocorticoid and gamma globulin. Four months later, her symptoms worsened and follow-up imaging showed multiple intracranial mass lesions. We performed a subtotal resection of the right thalamic basal ganglia tumor and gross total resection of the right frontal lobe tumor under microscopic examination. Histopathology revealed scGBM with intracranial metastasis and the molecular pathology diagnosis suggested H3K27M mutant diffuse midline glioma WHO grade IV, which had previously been misdiagnosed as a Neuromyelitis optica spectrum disorders. We review the literature of intracranial metastases originating from pediatric primary spinal cord glioblastoma multiforme and summarize possible methods of differentiation, including changes in muscle strength or tone, intramedullary heterogeneously enhancing solitary mass lesions and cord expansion in MRI. Finally, we emphasize that in unexpected radiological changes or disadvantageous response to the treatment, a biopsy to achieve a pathological diagnosis is necessary to discard other diseases, especially neoplasms.
原发性脊髓多形性胶质母细胞瘤(scGBM)在儿科中并不常见,起源于脊髓胶质瘤的颅内转移非常罕见。一名7岁女性出现肢体无力、下肢瘫痪和尿失禁。脊髓的初始MRI显示T2至T5节段脊髓增粗和信号异常。她最初被诊断为视神经脊髓炎谱系障碍,并接受了大剂量糖皮质激素和γ球蛋白治疗。四个月后,她的症状恶化,后续影像学检查显示颅内有多个占位性病变。我们在显微镜下对右侧丘脑基底节肿瘤进行了次全切除,并对右侧额叶肿瘤进行了全切除。组织病理学显示为scGBM伴颅内转移,分子病理学诊断提示为H3K27M突变型弥漫性中线胶质瘤WHO四级,此前曾被误诊为视神经脊髓炎谱系障碍。我们回顾了源自儿科原发性脊髓多形性胶质母细胞瘤的颅内转移的文献,并总结了可能的鉴别方法,包括肌力或肌张力变化、MRI上髓内不均匀强化的孤立性占位性病变和脊髓增粗。最后,我们强调,在出现意外的影像学变化或对治疗的不利反应时,进行活检以获得病理诊断对于排除其他疾病,尤其是肿瘤是必要的。