硬脊膜内腰骶部恶性肾外横纹肌样瘤：一例报告
Intradural lumbosacral malignant extrarenal rhabdoid tumor: a case report.
作者信息
Garling Richard Justin, Singh Rasanjeet, Harris Carolyn, Haridas Abilash
机构信息
Department of Neurosurgery, Wayne State University, 4201 St. Antoine blvd, Suite 6E, Neurosurgery, Detroit, MI, 48201, USA.
Wayne State University School of Medicine, Detroit, MI, USA.
出版信息
Childs Nerv Syst. 2018 Jan;34(1):165-167. doi: 10.1007/s00381-017-3571-2. Epub 2017 Aug 16.
BACKGROUND
Rhabdoid tumors are highly malignant tumors predominantly affecting the pediatric population. When these tumors occur outside of the kidneys, they are referred to as malignant extrarenal rhabdoid tumors (MERT), a rare highly aggressive subtype. Less commonly, these tumors involve the neuro-axis.
OBJECTIVE
Here we present a case of a 15-year-old girl with intradural MERT of the lumbosacral spine who presented with back pain, sudden worsening of lower extremity strength, and complete loss of bowel and bladder control.
RESULTS
The patient's tumor showed loss of INI-1 and negative staining for cytokeratin AE1AE3, CD99, and SOX10.
CONCLUSIONS
To our knowledge, there are no previous case reports of MERT with intradural lumbosacral spinal involvement.
背景
横纹肌样肿瘤是高度恶性肿瘤，主要影响儿童群体。当这些肿瘤发生在肾脏以外时，被称为恶性肾外横纹肌样肿瘤（MERT），这是一种罕见的高度侵袭性亚型。较少见的是，这些肿瘤累及神经轴。
目的
在此，我们报告一例15岁女孩，患有腰骶部硬脊膜内MERT，表现为背痛、下肢力量突然恶化以及大小便完全失禁。
结果
患者的肿瘤显示INI-1缺失，细胞角蛋白AE1AE3、CD99和SOX10染色阴性。
结论
据我们所知，此前尚无腰骶部硬脊膜内受累的MERT病例报告。
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