Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, EPIP Area, Whitefield, Bangalore, Karnataka, 560066, India.
Department of Pathology, Sri Sathya Sai Institute of Higher Medical Sciences, EPIP Area, Whitefield, Bangalore, Karnataka, 560066, India.
Spinal Cord Ser Cases. 2020 Oct 2;6(1):93. doi: 10.1038/s41394-020-00343-9.
Pregnancy-related spinal tumors (PRSTs) are unusual tumors that present during pregnancy or within a year after delivery. We describe a fatal holocord recurrence of a spinal ependymoma, which, to the best of our knowledge, is one of the most extensive PRSTs reported thus far.
A 21-year-old primigravida presented at 6 months of gestation with urinary incontinence for 2 months and spastic paraparesis for 1 month. MRI showed a conus intramedullary lesion from T10 to 12. Near-total resection of the lesion was performed. The histopathological diagnosis was that of a cellular ependymoma (WHO grade II). The patient presented 6 months later with progressive quadriparesis and breathing difficulty. MRI demonstrated holocord recurrence of the tumor with edema extending to the pontomedullary junction. The patient succumbed to respiratory failure before decompression of the tumor could be performed.
This case highlights an unusual clinical course of a pregnancy-related, low-grade spinal ependymoma. The possible hormonal and genetic mechanisms underlying the aggressive involvement of the entire spinal cord by the recurrent tumor are discussed in the light of a literature review. Future studies may shed light on the possibility of utilizing these mechanisms as therapeutic targets to alter the clinical course of aggressive spinal ependymomas.
妊娠相关脊柱肿瘤（PRSTs）是一种罕见的肿瘤，在妊娠期间或分娩后一年内出现。我们描述了一例脊髓髓内室管膜瘤全脊髓复发的致死性病例，据我们所知，这是迄今为止报道的最为广泛的 PRST 之一。
一名 21 岁初产妇，妊娠 6 个月，出现 2 个月的尿失禁和 1 个月的痉挛性截瘫。MRI 显示 T10 至 12 的圆锥内髓内病变。行病变近全切除术。组织病理学诊断为细胞性室管膜瘤（WHO 分级 II）。患者 6 个月后出现进行性四肢瘫痪和呼吸困难。MRI 显示肿瘤全脊髓复发，水肿延伸至桥脑延髓交界处。在对肿瘤进行减压之前，患者因呼吸衰竭而死亡。
本例突出了妊娠相关低度脊髓室管膜瘤的不寻常临床病程。根据文献复习，讨论了复发性肿瘤全脊髓侵袭的可能激素和遗传机制。未来的研究可能揭示利用这些机制作为治疗靶点改变侵袭性脊髓室管膜瘤临床病程的可能性。
