Epidemiology, Characteristics and Prognostic Factors of Primary Atypical Teratoid/Rhabdoid Tumours in the Spinal Canal: A Systematic Review

• Published in: Neurospine Vol. 21; no. 1; pp. 182 - 203
• Main Authors: Li, Zhibin, Wang, Yubo, Zhao, Liyan, Li, Yunqian
• Format: Journal Article
• Language: English
• Published: Korea (South) Korean Spinal Neurosurgery Society 01.03.2024; 대한척추신경외과학회
• Subjects: prognosis; Review; rhabdoid tumor; spinal canal; therapeutics; 신경외과학; rhabdoid tumor; spinal canal; therapeutics; prognosis
• ISSN: 2586-6583; 2586-6591
• DOI: 10.14245/ns.2347096.548

Primary atypical teratoid/rhabdoid tumours (AT/RTs) in the spinal canal are rare central nervous system (CNS) neoplasms that are challenging to diagnose and treat. To date, there has been no standard treatment regimen for these challenging malignant tumours. Thus, we conducted this research to explore potential prognostic factors and feasible treatment modalities for improving the prognosis of these tumours. Articles were retrieved from the PubMed, Medline and EMBASE databases, using the keywords "atypical teratoid/rhabdoid tumor," "rhabdoid tumor," "spine," "spinal," "spinal neoplasm" and "spinal cord neoplasm." All eligible cases demonstrated SMARCB1-deficient expression validated by pathological examination. We collected and analysed data related to clinical presentation, radiological features, pathological characteristics, treatment modalities and prognosis via Kaplan-Meier and Cox regression analyses. Thirty-six articles comprising 58 spinal AT/RT patients were included in the study. The median progression-free survival (PFS) and overall survival (OS) were 18 and 22 months, respectively. Kaplan-Meier analysis demonstrated significant survival improvements for OS in the nonmetastasis, male, radiotherapy and intrathecal chemotherapy groups as well as for PFS in the chemotherapy and radiotherapy groups. Multivariate analysis revealed that chemotherapy and radiotherapy were prognostic factors for improved PFS, and that intrathecal chemotherapy reduced the risk of mortality. Spinal AT/RTs are uncommon malignant entities with a dismal survival rate. Although our review is limited by variability between cases, there is some evidence revealing potential risk factors and the importance of systematic chemotherapy, intrathecal chemotherapy and radiotherapy in spinal AT/RT treatment modalities.