Diffuse intrinsic pontine glioma—current status and future strategies

Abstract

A pivotal approach in improving the prognosis of these tumors should include the initiation of biopsy and encouraging families to consider autopsy to study the molecular biology. This will help in redefining this tumor by its molecular signature and profiling targeted therapy. Continued advances should be pursued in neuroimaging technology including identifying surrogate markers of early disease progression. Defining strategies to enhance local delivery of drugs into tumors with the help of newer surgical techniques are important. Exhaustive research in all these aspects as a multidisciplinary approach could provide hope to children with these fatal tumors.

Pre-radiation chemotherapy improves survival in pediatric diffuse intrinsic pontine gliomas

“…It is generally assumed that both adjuvant chemotherapy (CT) before RT and radiosensitizers have no effect on prognosis [1][2][3][4][5][6][7][8][9]. However, one trial conducted by our team showed an improvement in median overall survival (OS) compared to standard RT protocol [10].…”

“…Diagnosis is based on clinical and radiological examination. If clinico-radiological presentation is typical, histological confirmation through surgical biopsy is not compulsory before starting treatment [1][2][3][4].…”

Microbeam radiation therapy: Clinical perspectives

“…As an example, diffuse intrinsic pontine gliomas, which constitute 15% of all childhood brain tumours (600 new cases/year in Europe), are inoperable. Their response to radiation and chemotherapy is only transient, with patients having a median overall survival of 10 months [1,2].…”

“…As an example, diffuse intrinsic pontine gliomas, which constitute 15% of all childhood brain tumours (600 new cases/year in Europe), are inoperable. Their response to radiation and chemotherapy is only transient, with patients having a median overall survival of 10 months [1,2].Microbeam radiation therapy (MRT), a novel radiotherapy method invented by Slatkin and coworkers in 1992 [3], is based on a spatial fractionation of synchrotron-generated X-ray beams. Spatial fractionation and the underlying laws of radiation physics were discovered by Alban Koehler at the beginning of the 20th century.…”

Malignant brainstem tumors in children, excluding diffuse intrinsic pontine gliomas

“…Many chemotherapeutic agents have been tested, but none to date have made a clear improvement in prognosis. 11,19,21,22 Conversely, children with low-grade brainstem tumorsaBBreViatiOnS AA = anaplastic astrocytoma; ADC = apparent diffusion coefficient; ATRT = atypical teratoid rhabdoid tumor; DIPG = diffuse intrinsic pontine glioma; DWI = diffusion-weighted imaging; ETANTR = embryonal tumor with abundant neuropil and true rosettes; GBM = glioblastoma multiforme; GTR = gross-total resection; HGG = high-grade glioma; NTR = near-total resection; OS = overall survival; PNET = primitive neuroectodermal tumor; STR = subtotal resection. OBJectiVe Malignant tumors of the brainstem, excluding classic diffuse intrinsic pontine gliomas (DIPGs), are a very rare, heterogeneous group of neoplasms that have been infrequently described in the literature.…”