Department of Neurosurgery, Cliniques universitaires Saint-Luc, Université catholique de Louvain, Brussels, Belgium.
Laboratory of Neural Differentiation (NEDI), Animal Molecular and Cellular Biology Group, Louvain Institute of Biomolecular Science and Technology, Université catholique de Louvain, Louvain-la-Neuve, Belgium.
Childs Nerv Syst. 2024 Apr;40(4):979-986. doi: 10.1007/s00381-023-06224-4. Epub 2023 Nov 23.
Extradural malignant rhabdoid tumors of the spine are highly malignant and invasive tumors (WHO grade IV) with poor prognosis, most frequently occurring in young children before 2 years of age. Pain and motor deficit are the most common presenting signs.
We report a case of a 2-year-old girl presenting with axial ataxia and paraparesis related to an extradural malignant rhabdoid tumor causing posterior thoracic spinal cord compression (D3-D6). She underwent two near-total removal of the tumor, adjuvant chemotherapy according to the Eu-Rhab protocol and proton beam therapy. She then developed multiple cranial nerve paresis (meningeal carcinomatosis) after 4 cycles of chemotherapy and died at 4.32 months of follow-up.
The role of the PET scan was essential to guide us to remove a residue, while two concomitant spinal MRIs were considered negative. We reviewed the 16 cases reported in the literature. Multiple surgeries and radiotherapy seem to be correlated with longer survival. No child younger than 2 years old had a documented survival higher than 4.32 months.
脊柱硬膜外恶性横纹肌样瘤是一种高度恶性和侵袭性肿瘤（WHO 分级 IV），预后不良，最常发生在 2 岁以下的儿童。疼痛和运动功能障碍是最常见的表现。
我们报告了一例 2 岁女孩的病例，表现为与硬膜外恶性横纹肌样瘤相关的轴向共济失调和截瘫，导致后胸段脊髓受压（D3-D6）。她接受了两次接近全切除肿瘤，根据 Eu-Rhab 方案进行辅助化疗和质子束治疗。在完成 4 个周期的化疗后，她出现了多种颅神经麻痹（脑膜癌病），并在随访 4.32 个月后死亡。
PET 扫描对指导我们切除残留肿瘤至关重要，而两次同时进行的脊髓 MRI 被认为是阴性的。我们回顾了文献中报道的 16 例病例。多次手术和放疗似乎与更长的生存时间相关。没有 2 岁以下的儿童的生存时间超过 4.32 个月。
