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

Giussani, Carlo; Guida, Lelio; Biassoni, Veronica; Schiavello, Elisabetta; Carrabba, Giorgio; Trezza, Andrea; Sganzerla, Erik P.; Massimino, Maura

doi:10.1007/s00381-020-04589-4

Cited by 5 publications

(11 citation statements)

References 16 publications

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“…Review of the literature yielded 18 articles describing 383 patients with glioma of the thalamus or brainstem, citing rates of HCP from 22 to 100% (overall 55.6%, n = 213/383), and intervention in 22-63% (overall 53.3%, n = 204/383) ( Table 3) (18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35). Of these cases, there were 132 ventricular shunts (including 2 Torkildsen shunts) (22), 70 ETVs, and two EVDs that were ultimately weaned (31).…”

Section: Literature Reviewmentioning

confidence: 99%

“…Of these cases, there were 132 ventricular shunts (including 2 Torkildsen shunts) (22), 70 ETVs, and two EVDs that were ultimately weaned (31). Nine patients with evidence of HCP were untreated, with reports citing anatomic distortion from thalamic gliomas (n = 2 ETV deferred) (23), reasons unspecified, (36), patient refusal, or poor overall clinical condition (n = 6) (18,22). Infectious complications were reported in association with placement of one Torkildsen shunt (0.01% of all shunts placed) (22).…”

Section: Literature Reviewmentioning

confidence: 99%

“…Infectious complications were reported in association with placement of one Torkildsen shunt (0.01% of all shunts placed) (22). Non-infectious complications secondary to shunt placement included shunt malfunction [n = 4, one Torkildsen shunt converted to a VPS (22), one confirmed VPS malfunction requiring revision (18) and two suspected VPS malfunctions (25)], and contralateral subdural hematoma that resolved after placement of temporary subduroperitoneal shunts (n = 3) (31). Of note, there were four reported cases of extraneural metastases via CSF seeding through shunt systems (19,28,32,34).…”

Section: Literature Reviewmentioning

confidence: 99%

“…These occurred most often after other evidence of tumor recurrence or progression, particularly with leptomeningeal dissemination. Reported ETV complications (n = 6, 8.5%) consisted of failure to effectively treat HCP requiring conversion to VPS (18,(23)(24)(25).…”

Section: Literature Reviewmentioning

confidence: 99%

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Ventricular Cerebrospinal Fluid Sampling in Pediatric Diffuse Midline Glioma Patients: Institutional Experience and Review of the Literature

Stellpflug

Romanski

et al. 2020

Front. Pediatr.

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Increasing evidence suggests that circulating biomarkers may serve diagnostic and longitudinal monitoring purposes in pediatric neuro-oncology. Mutant tumor DNA is detectable in the cerebrospinal fluid (CSF) of pediatric diffuse midline glioma (DMG) patients and quantity can reflect disease burden. CSF sampling ("liquid biopsy") via a CSF access device could therefore play a role in DMG management. Therefore, we set to evaluate the incidence of hydrocephalus (HCP) in DMG patients, and to characterize ventricular reservoir placement and access practices. Methods: A single institution retrospective review of DMG patients ≤21-years-old was performed (1984-2019). MEDLINE searches for reports of ventricular reservoir or shunt placement in DMG, and reservoir access for intraventricular chemotherapy (IVC) were performed. Results: At our institution, 62.6% of DMG patients (67/108) required intervention for HCP: 19.4% provided transient CSF access (ETV alone n = 3, EVD n = 8, unspecified n = 2), and 80.6% permanent CSF access (ETV + reservoir n = 13, shunt n = 41). Further, 22/34 patients with initially transient CSF devices required conversion to a permanent device. Five devices were revised for malfunction, one for infection. Seventeen articles cited HCP in 22 to 100% of DMG patients. IVC administration was described in 632 patients (seven articles), with 42 infectious and 63 non-infectious complications. Conclusions: Management of HCP is often necessary in children with DMG. Given the low rate of clinical risk associated with VAD placement and access, and the potential utility of longitudinal disease monitoring via CSF analysis, VAD placement could be considered in future clinical trials to guide DMG treatment.

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Section: Literature Reviewmentioning

confidence: 99%

Section: Literature Reviewmentioning

confidence: 99%

Section: Literature Reviewmentioning

confidence: 99%

Section: Literature Reviewmentioning

confidence: 99%

See 2 more Smart Citations

Ventricular Cerebrospinal Fluid Sampling in Pediatric Diffuse Midline Glioma Patients: Institutional Experience and Review of the Literature

Stellpflug

Romanski

et al. 2020

Front. Pediatr.

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“…Approximately 35% of children with DIPG develop hydrocephalus at an average of 5 months after diagnosis. 6 This may result from tumor dissemination within the CSF pathways as not all cases of hydrocephalus are from intraventricular obstruction. 7 Potential mechanisms underlying the development of hydrocephalus including interaction of tumor cells with the CSF and associated pathways (leptomeninges, ventricular ependyma) have not yet been studied, 8 and as a consequence, the origins and development of DIPG tumor spread and associated hydrocephalus are not clear.…”

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confidence: 99%

Leptomeningeal disease and tumor dissemination in a murine diffuse intrinsic pontine glioma model: implications for the study of the tumor-cerebrospinal fluid-ependymal microenvironment

Pan

Yue

et al. 2022

Neuro-Oncology Advances

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Background Leptomeningeal disease and hydrocephalus are present in up to 30% of patients with diffuse intrinsic pontine glioma (DIPG), however there are no animal models of cerebrospinal fluid (CSF) dissemination. As the tumor-CSF-ependymal microenvironment may play an important role in tumor pathogenesis, we identified characteristics of the Nestin-tumor virus A (Nestin-Tva) genetically engineered mouse model (GEMM) that make it ideal to study the interaction of tumor cells with the CSF and its associated pathways with implications for the development of treatment approaches to address CSF dissemination in DIPG. Methods A Nestin-TVa model of DIPG utilizing the three most common DIPG genetic alterations (H3.3K27M, PDGF-B, p53) was used for this study. All mice underwent MR imaging and a subset underwent histopathologic analysis with H&E and immunostaining. Results Tumor dissemination within the CSF pathways (ventricles, leptomeninges) from the subependyma was present in 76% (25/33) of mice, with invasion of the choroid plexus, disruption of the ciliated ependyma and regional subependymal fluid accumulation. Ventricular enlargement consistent with hydrocephalus was present in 94% (31/33). Ventricle volume correlated with region specific transependymal CSF flow (periventricular T2 signal), localized anterior to the lateral ventricles. Conclusions This is the first study to report CSF pathway tumor dissemination associated with subependymal tumor in an animal model of DIPG and is representative of CSF dissemination seen clinically. Understanding the CSF-tumor-ependymal microenvironment has significant implications for treatment of DIPG through targeting mechanisms of tumor spread within the CSF pathways.

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Diffuse intrinsic pontine gliomas: First registry effort in Mexico

Robles‐Castro,

Niembro‐Zúñiga,

Ortiz‐Azpilcueta

et al. 2023

Pediatric Blood & Cancer

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Introduction Brainstem tumors comprise 10.9% of all brain tumors, and pediatric diffuse intrinsic pontine gliomas (DIPG) have a fatal prognosis. Some countries have developed national and international register databases to characterize their populations to aid clinical and public policy decisions. This study provides information regarding the clinical characteristics of a retrospective cohort of children with DIPG in México from 2001 to 2021, and assesses the proposed prognostic factors previously described for survival outcome. Methods Health institutions from Mexico were invited to contribute to a retrospective electronic registry of patients with DIPG based on the International DIPG Registry. Fisher's exact test was used to compare long‐ and short‐term survivors. Overall survival was estimated using the Kaplan–Meier method. Differences between survival curves were evaluated using the log‐rank test and Cox proportional hazard regression analysis. Results Total 110 patients were included. The median age of the patients at diagnosis was 7 years. Sixty patients (54.5%) presented with symptoms in less than 6 months; the most frequent symptom was ataxia (56.4%). Ninety patients received treatment (81.8%), the overall survival at 4 years was 11.4%, and 16 patients (14.5%) were admitted for palliative end‐of‐life care. We found no significant survival differences for any of the prognostic factors. Conclusion This study highlights the need to develop strategies to standardize healthcare processes and enhance the quality of care to improve clinical diagnosis in Mexico. We also observed a barrier to the acceptance of palliative end‐of‐life care in the family and medical teams.

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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

Cited by 5 publications

References 16 publications

Ventricular Cerebrospinal Fluid Sampling in Pediatric Diffuse Midline Glioma Patients: Institutional Experience and Review of the Literature

Ventricular Cerebrospinal Fluid Sampling in Pediatric Diffuse Midline Glioma Patients: Institutional Experience and Review of the Literature

Leptomeningeal disease and tumor dissemination in a murine diffuse intrinsic pontine glioma model: implications for the study of the tumor-cerebrospinal fluid-ependymal microenvironment

Diffuse intrinsic pontine gliomas: First registry effort in Mexico

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