Departments of Neurosurgery.
Pediatric Hematology/Oncology.
Appl Immunohistochem Mol Morphol. 2020 Aug;28(7):e58-e62. doi: 10.1097/PAI.0000000000000620.
Atypical teratoid/rhabdoid tumors (AT/RT) are highly aggressive malignant primitive neoplasms that commonly occur in children younger than 2 years of age. The prognosis is generally dismal with a median survival time of <1 year. The majority of AT/RT occur in the posterior fossa and less frequently the supratentorium. Primary pediatric spinal AT/RT are exceedingly rare and only 15 cases have been reported to date. Here we report a very unusual case of primary spinal AT/RT extensively involving the spinal cord from T11 down to the cauda equina. In this patient, the tumor was highly aggressive and resulted in extensive dissemination into the nerve roots and paraspinal soft tissue rapidly resulting in the patient's death 1 month after diagnosis. to the best of our knowledge, this degree of involvement of the spine by a primary AT/RT has not been described before.
非典型畸胎样/横纹肌样肿瘤（AT/RT）是一种高度侵袭性的恶性原始肿瘤，常见于 2 岁以下的儿童。预后通常较差，中位生存时间<1 年。大多数 AT/RT 发生在后颅窝，较少发生在幕上。原发性儿童脊髓 AT/RT 极为罕见，迄今为止仅报告了 15 例病例。在这里，我们报告了一例非常罕见的原发性脊髓 AT/RT 广泛累及脊髓从 T11 到马尾的病例。在该患者中，肿瘤侵袭性强，迅速向神经根和脊柱旁软组织广泛扩散，导致患者在诊断后 1 个月死亡。据我们所知，以前尚未描述过原发性 AT/RT 对脊柱如此程度的累及。
