The intersect of neurosurgery with diffuse intrinsic pontine glioma

Kuzan-Fischer, Claudia M.; Souweidane, Mark M.

doi:10.3171/2019.5.peds18376

Cited by 13 publications

(9 citation statements)

References 74 publications

(92 reference statements)

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“…The typical imaging feature of DIPG is high intensity on T2WI and low to iso-intensity on T1WI, with nondelineated tumor margins and engulfment of the basilar artery [13]. On FLAIR images, DIPGs usually appear homogeneously hyperintense [14].…”

Section: Discussionmentioning

confidence: 99%

T2-FLAIR Mismatch Sign and Response to Radiotherapy in Diffuse Intrinsic Pontine Glioma

et al. 2021

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Purpose: The T2-fluid-attenuated inversion recovery (FLAIR) mismatch sign was previously reported as a diagnostic indicator of diffuse astrocytoma, isocitrate dehydrogenase-mutant, and 1p/19q noncodeletion. Subsequently, it was reported that the same findings were observed in diffuse intrinsic pontine glioma (DIPG). We investigated the clinical significance of T2-FLAIR mismatch sign in DIPG. Methods: Twenty-one patients with DIPG (Male: Female = 12:9) were treated at our institute between 2004 and 2019. All patients were treated with local radiotherapy of 54 Gy/30 fractions. The positive T2-FLAIR mismatch sign was defined if it fulfilled the following criteria: (1) T2-FLAIR mismatch volume was >50% of T2 high volume at nonenhanced area, (2) the FLAIR low lesion is not associated with gadolinium enhancement (inside of enhancement or just outside of enhancement defined as edema), and (3) signal-intensity of FLAIR lowest lesion at tumor is lower than the normal cerebellar cortex. Results: In our patient series, T2-FLAIR mismatch sign was found in 5 out of 21 patients. Objective response rate of radiotherapy was 100% in patients positive for T2-FLAIR mismatch, while it was 25.0% in patients negative for T2-FLAIR mismatch, and this difference was statistically significant (p < 0.01, Fisher’s exact test). In patients under the age of 18-years, T2-FLAIR mismatch positive had a slightly better prognosis (p < 0.05, Wilcoxon test). Conclusion: T2-FLAIR mismatch sign in DIPG may be an indicator for better response to radiotherapy and a better prognostic factor.

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Section: Discussionmentioning

confidence: 99%

T2-FLAIR Mismatch Sign and Response to Radiotherapy in Diffuse Intrinsic Pontine Glioma

et al. 2021

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“…Clinical and radiological characteristics are critical for diagnosing DIPGs because the hystological sampling is not always possible. DIPGs appear hyperintense on T2-weighted images and hypointense on T1W1 [19]. T2-FLAIR mismatch sign has been described as an imaging marker for predicting non-enhancing IDH-mutant, 1p/19q-intact lower-grade glioma [20].…”

Section: Discussionmentioning

confidence: 99%

T2-FLAIR Mismatch Sign and Nimotuzumab Therapy in Pediatric Diffuse Intrinsic Pontine Gliomas

Vural

Yazol

Türkcan

et al. 2022

Preprint

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Purpose In this study, we retrospectively investigated the clinical and radiological features, the importance of the T2-FLAIR mismatch marker and the response to treatment of patients diagnosed with diffuse intrinsic pontine gliomas (DIPG) in our center. Methods Eighteen patients treated with a diagnosis of DIPG between January 2008 and January 2021 in Gazi University Medical Faculty, Department of Pediatric Oncology were retrospectively evaluated. The evaluation was made as T2-FLAIR mismatch sign positive or negative. After a tumor board review, the diagnosis of DIPG was made clinically and radiologically and all patients received local radiotherapy. Nimotuzumab was given as monotherapy or in combination with other medications Results Twelve patients had the characteristics T2-FLAIR mismatch sign and median OS for patients with T2-FLAIR mismatch positive and negative were 12.5 months and 9.2 months respectively (p = 0.77). Median PFS for patients with T2-FLAIR mismatch sign positive and negative were 10.6 months and 4.8 months respectively (p = 0.84). After nimotuzumab therapy, there was 4 cases with PR (44.4%), and 1 patient with SD (11.1%). Median OS for patients who were treated with and without nimotuzumab were 16.5 and 6.2 months respectively (p < 0.05). Median PFS for patients who were treated with and without nimotuzumab were 13.3 and 3.7 months respectively (p < 0.05). Conclusion In conclusion, DIPGs have poor prognosis, T2-FLAIR mismatch sign may be an imaging marker for prognosis that patients with T2-FLAIR mismatch sign had a somewhat better prognosis in our study. Nimotuzumab therapy may be a promising treatment option for DIPG.

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“…Given the paucity of tissue, and the overall low disease incidence, the rise of collaborative efforts aimed at changing disease prognosis has been one of the few positive notes of recent decades. Starting in the 2000s, not-for-profit foundations have been sponsoring DIPG research, resulting in the foundation of the DIPG Collaborative and its affiliated DIPG Registry—a comprehensive database of clinical, radiological, pathologic, and molecular data [ 39 , 40 ]. These efforts were sponsored not only by governmental funding agencies, but also—and to a great extent—by private donors.…”

Section: Diffuse Midline Gliomas: the Landscapementioning

confidence: 99%

“…Scheme showing the supratentorial (transfrontal) approach for catheter insertion in DIPG in the pons. Adapted with permission from [ 39 ].…”

Section: Figurementioning

confidence: 99%

Convection Enhanced Delivery for Diffuse Intrinsic Pontine Glioma: Review of a Single Institution Experience

Tosi

Souweidane

2020

Pharmaceutics

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Diffuse intrinsic pontine gliomas (DIPGs) are a pontine subtype of diffuse midline gliomas (DMGs), primary central nervous system (CNS) tumors of childhood that carry a terrible prognosis. Because of the highly infiltrative growth pattern and the anatomical position, cytoreductive surgery is not an option. An initial response to radiation therapy is invariably followed by recurrence; mortality occurs approximately 11 months after diagnosis. The development of novel therapeutics with great preclinical promise has been hindered by the tightly regulated blood–brain barrier (BBB), which segregates the tumor comportment from the systemic circulation. One possible solution to this obstacle is the use of convection enhanced delivery (CED), a local delivery strategy that bypasses the BBB by direct infusion into the tumor through a small caliber cannula. We have recently shown CED to be safe in children with DIPG (NCT01502917). In this review, we discuss our experience with CED, its advantages, and technical advancements that are occurring in the field. We also highlight hurdles that will likely need to be overcome in demonstrating clinical benefit with this therapeutic strategy.

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The intersect of neurosurgery with diffuse intrinsic pontine glioma

Cited by 13 publications

References 74 publications

T2-FLAIR Mismatch Sign and Response to Radiotherapy in Diffuse Intrinsic Pontine Glioma

T2-FLAIR Mismatch Sign and Response to Radiotherapy in Diffuse Intrinsic Pontine Glioma

T2-FLAIR Mismatch Sign and Nimotuzumab Therapy in Pediatric Diffuse Intrinsic Pontine Gliomas

Convection Enhanced Delivery for Diffuse Intrinsic Pontine Glioma: Review of a Single Institution Experience

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