Posterior Fossa Tumors in Infants: 13-year single center retrospective review.

Natália Dassi, Carolina Silva De Aguiar, Patricia Dastoli, Nasjla S Silva, Sergio Cavalheiro, Andrea M Cappellano
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 Methods: Single center retrospective study of infants diagnosed with posterior fossa tumors between 2010 and 2023.
 Results: Nineteen patients were included. Mean age 8.8 months; eight males. The most common presenting symptom was torticollis (n-6, 31.6%), followed by vomiting (n-3, 15.8%) and increased head circumference (n-3, 15.8%). Atypical Teratoid Rhabdoid Tumor (AT/RT) was the most common subtype (n-11, 57.9%), followed by Medulloblastoma (MB). Metastatic disease at diagnosis was evident in five cases. Seventeen patients initially underwent correction of hydrocephalus. Regarding surgical resection, one (5.3%) patient underwent biopsy, 13 (68.4%) complete resection, four (21%) partial resection. Only one patient, diagnosed with ependymoma, did not receive chemotherapy after surgery, 14/19 received high-dose chemotherapy, eight of these followed by autologous bone marrow transplantation (ABMT). Focal radiotherapy (RT) was performed in four patients with AT/RT and in one ependymoma, following ABMT and complete resection, respectively. Event-free survival at 2 and 5 years was 64.9% and overall survival 63.2% and 54.2% respectively.
 Conclusion: Despite aggressive histological subtypes, neurosurgical challenges, and limitation of adjuvant therapy, we have shown acceptable survival rates in this challenging group of patients showing the importance of appropriate supportive care, improvement in surgical techniques and a multi-professional approach.","PeriodicalId":491452,"journal":{"name":"Archives of Pediatric Neurosurgery","volume":"37 1","pages":"0"},"PeriodicalIF":0.0000,"publicationDate":"2023-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Archives of Pediatric Neurosurgery","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.46900/apn.v5i3.206","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
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Abstract

Introduction/Background: Central nervous system (CNS) tumors in infants are rare, ranging between 1 and 10% of all pediatric CNS tumors. The infratentorial location is a challenge to neuro-oncologist and -surgeons due their subtle clinical presentation, different histopathological types, biological behaviors, and anesthetic-surgery difficulties. Methods: Single center retrospective study of infants diagnosed with posterior fossa tumors between 2010 and 2023. Results: Nineteen patients were included. Mean age 8.8 months; eight males. The most common presenting symptom was torticollis (n-6, 31.6%), followed by vomiting (n-3, 15.8%) and increased head circumference (n-3, 15.8%). Atypical Teratoid Rhabdoid Tumor (AT/RT) was the most common subtype (n-11, 57.9%), followed by Medulloblastoma (MB). Metastatic disease at diagnosis was evident in five cases. Seventeen patients initially underwent correction of hydrocephalus. Regarding surgical resection, one (5.3%) patient underwent biopsy, 13 (68.4%) complete resection, four (21%) partial resection. Only one patient, diagnosed with ependymoma, did not receive chemotherapy after surgery, 14/19 received high-dose chemotherapy, eight of these followed by autologous bone marrow transplantation (ABMT). Focal radiotherapy (RT) was performed in four patients with AT/RT and in one ependymoma, following ABMT and complete resection, respectively. Event-free survival at 2 and 5 years was 64.9% and overall survival 63.2% and 54.2% respectively. Conclusion: Despite aggressive histological subtypes, neurosurgical challenges, and limitation of adjuvant therapy, we have shown acceptable survival rates in this challenging group of patients showing the importance of appropriate supportive care, improvement in surgical techniques and a multi-professional approach.
婴儿后窝肿瘤:13年单中心回顾性分析。
简介/背景:婴儿中枢神经系统(CNS)肿瘤是罕见的,约占所有小儿中枢神经系统肿瘤的1 - 10%。幕下的位置对神经肿瘤学家和外科医生来说是一个挑战,因为它们的临床表现微妙,不同的组织病理类型,生物学行为和麻醉手术困难。 方法:对2010 - 2023年诊断为后窝肿瘤的婴儿进行单中心回顾性研究。结果:共纳入19例患者。平均年龄8.8个月;八个男性。最常见的症状是斜颈(n-6, 31.6%),其次是呕吐(n-3, 15.8%)和头围增大(n-3, 15.8%)。非典型畸胎体横纹肌样瘤(AT/RT)是最常见的亚型(n-11, 57.9%),其次是髓母细胞瘤(MB)。5例确诊时有明显的转移性疾病。17例患者最初接受了脑积水矫正。手术切除方面,1例(5.3%)患者行活检,13例(68.4%)患者行完全切除,4例(21%)患者行部分切除。只有1例诊断为室管膜瘤的患者术后未接受化疗,14/19接受了大剂量化疗,其中8例随后进行了自体骨髓移植(ABMT)。在ABMT和完全切除后,分别对4例AT/RT和1例室管膜瘤进行局灶放疗(RT)。2年和5年无事件生存率分别为64.9%,总生存率为63.2%和54.2%。 结论:尽管具有侵袭性的组织学亚型、神经外科挑战和辅助治疗的局限性,我们在这一具有挑战性的患者群体中显示出可接受的生存率,这表明适当的支持治疗、手术技术的改进和多专业方法的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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