Hydrocephalus in primary intradural spinal cord tumors: a systematic review of the literature in the pediatric populatio
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SHORT REVIEW
Hydrocephalus in primary intradural spinal cord tumors: a systematic review of the literature in the pediatric population Marios Lampros 1 & Spyridon Voulgaris 1 & George A. Alexiou 1,2 Received: 31 May 2020 / Revised: 28 July 2020 / Accepted: 3 September 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Hydrocephalus in children with primary intradural spinal cord tumors is exceedingly rare. Herewith, we performed a systematic literature review to address epidemiology, suggested pathophysiological mechanisms, prognostic factors, and treatment of such cases. We performed a systematic review with the best available evidence on cases of pediatric primary intradural tumors of the spinal cord presented with hydrocephalus. The patients were subjected to quantitative analysis on a basis of epidemiological features (age, sex, tumor type and location, clinical presentation, survival, dissemination). The possible pathophysiological theories are discussed in detail. Forty-four studies with a total of 121 patients were included in the study. Astrocytomas were the most frequent tumor (64.5%) type, while most tumors were located in cervical (31.4%) or cervicothoracic region (25.6%). About half of the cases concerned children under 6 years of age. The block of subarachnoid CSF (cerebrospinal fluid) pathways from disseminated tumor cells and the neoplastic inflammation caused by tumor elements advocated to be the major pathogenetic mechanisms. Surgical excision of the tumor and hydrocephalus treatment is usually performed. Primary intradural spinal cord tumors should be considered in children with communicative hydrocephalus of unknown etiology. Onset of hydrocephalus after tumor removal is related to higher mortality. Keywords Hydrocephalus . Spinal cord tumor . Children . Epidemiology . Pathophysiology . Prognosis . Treatment
Introduction Primary spinal cord tumors (PSCT) comprise 4–8% of central nervous system neoplasms, and contrary to adults, the most frequent are intramedullary [1].In pediatric population, astrocytomas and ependymomas are the most frequent histological types, consisting of 60% and 30%, respectively, of all primary spinal cord tumors. It is estimated that more than half of pediatric spinal tumors are malignant [2]. Intradural tumors make up the one-third of PSCT, and most of them are located intramedullary [3]. Primary intradural spinal cord tumors (PISCT) presented with hydrocephalus (HC) are rare. Kyrieleis et al. in 1931 was the first to describe a case of HC in a patient with spinal cord * George A. Alexiou [email protected]; [email protected] 1
Department of Neurosurgery, School of Medicine, University of Ioannina, Ioannina, Greece
2
Department of Neurosurgery, University Hospital of Ioannina, PO BOX 103, Neohoropoulo, 45500 Ioannina, Greece
tumor [4]. Up to date, the pathophysiology mechanisms and other clinical features have been poorly studied. Herewith, a systematic review is performed on epidemiology, possible pathophysiological mechanisms, risk fact
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