Management of hydrocephalus related to diffuse leptomeningeal glioneuronal tumour: a multifaceted condition
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LETTER TO THE EDITOR
Management of hydrocephalus related to diffuse leptomeningeal glioneuronal tumour: a multifaceted condition Federica Garibotto 1
&
Marco Pavanello 2 & Claudia Milanaccio 1 & Gabriele Gaggero 3 & Pietro Fiaschi 4,5
Received: 7 August 2020 / Accepted: 17 August 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Dear Editor: We read with keen interest the paper of Chen et al. [1]. In the case proposed by Chen, communicating hydrocephalus was the first symptom of diffuse leptomeningeal glioneuronal tumour (DLGNT). DLGNT is generally considered an indolent and slowly progressive neoplasm and, often, neurological symptoms appear in a later stage of the disease, when the thickening of the leptomeninges produces changes in bloodbrain barrier (BBB), cerebrospinal (CSF) flow obstruction and cranial and spinal nerve compression. Nevertheless, cases of DLGNT with a considerable morbidity and an aggressive behaviour have also been described, with symptoms related to compression or infiltration of eloquent brain areas [2]. To date, no treatment has demonstrated to significantly modify the disease course and a homogeneous and standard treatment for DLGNT has not yet been defined. Craniospinal irradiation must be considered at disease progression but is not recommended as a first-line therapy. Most patients are treated as low-grade gliomas, with carboplatin/vincristine for 18 months, or temozolomide (also as upfront treatment). Other antineoplastic or biological drugs that can be employed include etoposide, vinca alkaloids, bevacizumab and mTOR inhibitors. In such a clinical scenario, most symptoms of DLGNTs
* Federica Garibotto [email protected] 1
Neuro-oncology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy
2
Division of Neurosurgery, IRCCS Istituto Giannina Gaslini, Genoa, Italy
3
Pathology Unit, Ospedale Policlinico San Martino, IRCCS for Oncology and Neuroscience, Genoa, Italy
4
Department of Neurosurgery, Ospedale Policlinico San Martino, IRCCS for Oncology and Neuroscience, Genoa, Italy
5
Dipartimento di Neuroscienze, riabilitazione, oftalmologia, genetica e scienze materno-infantili (DINOGMI), Università di Genova, Genoa, Italy
are not-specific and clinicians need to be particularly aware when a paediatric patient presents hydrocephalus and diffuse leptomeningeal magnetic resonance imaging (MRI) contrast enhancing lesions. In fact, DLGNT-associated hydrocephalus is frequently reported. This assumed, we think that if on one hand the build-up of experience regarding oncological therapies is mandatory; on the other hand, the management of hydrocephalus in DLGNT needs an accumulation of evidences regarding CSF alterations, thus evaluating alternatives to ventriculo-peritoneal shunt (VPS) or Ommaya placing. The choice of Ommaya reservoir and VPS is related to the need of further therapy (such as intrathecal chemotherapy or CSF diversion) but they entail a considerable risk of infection; their correct functioning is related to CSF features, and complications are
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