Retrospective analysis of the clinical and radiological features of 94 consecutive DIPGs patients to investigate the fac

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ORIGINAL ARTICLE

Retrospective analysis of the clinical and radiological features of 94 consecutive DIPGs patients to investigate the factors determining the development of hydrocephalus and its impact on clinical status and survival Carlo Giussani 1 & Lelio Guida 1 & Veronica Biassoni 2 & Elisabetta Schiavello 2 & Giorgio Carrabba 3 & Andrea Trezza 1 & Erik Sganzerla 1 & Maura Massimino 2 Received: 30 December 2019 / Accepted: 23 March 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020

Abstract Purpose There is no consensus in the literature about the impact of hydrocephalus on clinical course and overall survival of diffuse intrinsic pontine gliomas (DIPG) patients as well as about its specific treatment. Authors reviewed a series of DIPG patients to investigate factors related to the onset of hydrocephalus, its treatment, and its impact on clinical course and prognosis. Methods A retrospective observational study was performed enrolling pediatric patients affected by DIPG from 2008 to 2018. Clinical and radiological charts were reviewed to find patients’ demographic, pathologic and radiologic features in hydrocephalic and non-hydrocephalic patients. In the hydrocephalus cohort, treatment strategy and its effectiveness and complications were analyzed. Results Ninety-four pediatric patients were enrolled in the study. Patients who developed hydrocephalus showed significantly lesser maximum axial tumor areas than patients without hydrocephalus (respectively 6.5 cm2 vs 16.45 cm2, p < 0.005). Hydrocephalus developed in 33 patients (35%) with an onset interval of 5.24 ± 1.21 months (range 3.2–7.3). The majority of hydrocephalic patients (28 cases, 90%) were treated by ventriculoperitoneal shunt, the remaining 3 patients being treated by endoscopic third ventriculostomy. Mean overall survival was 16.6 months ± 20 months without significative difference between the groups. Conclusion The onset of hydrocephalus occurs in the first moths of the disease story and found a negative correlation with tumor maximal axial diameter. Early treatment of hydrocephalus presents a very low complications rate with satisfying clinical outcome, as it allows the patients to continue the neurooncological therapies being a part of the treatment armamentarium instead of a palliative solution. Keywords Diffuse intrinsic pontine glioma . Hydrocephalus . Endoscopic third ventriculostomy . Ventriculo peritoneal shunt

Introduction Diffuse intrinsic pontine glioma (DIPG) is a devastating disease accounting for 6–9% of brain tumors in children [1]. The

* Carlo Giussani [email protected] 1

Neurosurgery, School of Medicine, Ospedale San Gerardo, Università degli Studi di Milano Bicocca, Monza, Italy

2

Pediatric Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

3

Neurosurgery, School of Medicine, Fondazione IRCCS Ca’ Granda, Università degli studi di Milano, Milan, Italy

current treatment strategy includes at least focal radiotherapy, but different other medical treatments have been proposed during

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