Meena Rajesh Kumar, Doddamani Ramesh S, Chipde Harshad, Mahajan Swati, Chandra Sarat P, Sawarkar Dattaraj P
Department of Neurosurgery, All India Institute of Medical Sciences (AIIMS), New Delhi, 110049, India.
Department of Pathology, All India Institute of Medical Sciences (AIIMS), New Delhi, 110049, India.
Childs Nerv Syst. 2020 Mar;36(3):655-659. doi: 10.1007/s00381-019-04412-9. Epub 2019 Oct 29.
Atypical teratoid/rhabdoid tumours (AT/RTs) are highly aggressive and uncommon malignant tumours of the central nervous system (CNS) affecting children younger than 3 years of age. Primary spinal cord involvement is an extremely rare presentation. AT/RTs show necrosis and haemorrhages on histopathology frequently. However, spinal atypical teratoid/rhabdoid tumour (AT/RT) with hematomyelia and spinal subarachnoid haemorrhage (SAH), as seen in our case, has never been reported in the literature in the paediatric age group. We report a case of primary spinal AT/RT in a 3-year-old male child presenting acutely with hematomyelia and spinal SAH and try to elucidate its pathophysiological basis.
非典型畸胎样/横纹肌样瘤(AT/RTs)是中枢神经系统(CNS)中极具侵袭性且罕见的恶性肿瘤,影响3岁以下儿童。原发性脊髓受累是一种极其罕见的表现。AT/RTs在组织病理学上常显示坏死和出血。然而,如我们病例中所见的伴有脊髓出血性脊髓炎和脊髓蛛网膜下腔出血(SAH)的脊髓非典型畸胎样/横纹肌样瘤(AT/RT),在儿科年龄组的文献中从未有过报道。我们报告一例3岁男童原发性脊髓AT/RT,急性表现为出血性脊髓炎和脊髓SAH,并试图阐明其病理生理基础。