DIPG in Children – What Can We Learn from the Past?

Vanan, Magimairajan Issai; Eisenstat, David D.

doi:10.3389/fonc.2015.00237

Cited by 172 publications

(171 citation statements)

References 150 publications

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“…Children with these sporadically occurring BSGs most often present with cerebellar signs, long tract signs, and cranial nerve palsies, and die of their tumor within 12 months. [17][18][19] Since the presence of localizing signs/symptoms was the most frequent reason for treatment, the incidental identification of these tumors infrequently changed clinical management, similar to that reported for NF1-OPG. 20 Fourth, and not surprisingly, children with NF1-BSG who received tumor-directed therapy had shorter PFS than those who did not require treatment for their tumor (whether they received CSF diversion or not), likely reflecting a different biology that drove tumor progression in the first place.…”

Section: Resultsmentioning

confidence: 63%

A multi-institutional study of brainstem gliomas in children with neurofibromatosis type 1

et al. 2017

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Objective: To define the clinical and radiologic features of brainstem gliomas (BSGs) in children with neurofibromatosis type 1 (NF1).Methods: We performed a retrospective cross-sectional study of 133 children with NF1 and concurrent BSGs cared for at 4 NF1 referral centers. BSG was determined using radiographic criteria. Age at diagnosis, tumor location and appearance, clinical symptoms, treatment, and presence of a concurrent optic pathway glioma were assessed. Results:The average age at BSG diagnosis was 7.2 years, and tumors occurred most often in the midbrain and medulla (66%). The majority of children with NF1-BSGs were asymptomatic (54%) and were not treated (88%). Only 9 of the 72 asymptomatic children received treatment because of progressive tumor enlargement. In contrast, 61 children presented with clinical signs/symptoms attributable to their BSG; these individuals were older and more often had focal lesions. Thirty-one patients underwent treatment for their tumor, and 14 received CSF diversion only. Progression-free survival was ;3 years shorter for children receiving tumor-directed therapy relative to those who had either no treatment or CSF diversion only. Overall survival was 85% for the tumor-directed therapy group, whereas no deaths were reported in the untreated or CSF diversion groups.Conclusions: Unlike children with sporadically occurring BSGs, most children with NF1-BSGs were asymptomatic, and few individuals died from complications of their tumor. Those requiring tumor-directed treatment tended to be older children with focal lesions, and had clinically more aggressive disease relative to those who were not treated or underwent CSF diversion only. Neurofibromatosis type 1 (NF1) is a common cancer predisposition syndrome in which affected children and adults develop benign and malignant nervous system tumors.1 WHO grade I gliomas (pilocytic astrocytomas [PAs]), including those involving the optic pathway and brainstem, predominate in children with NF1. Optic pathway gliomas (OPGs), the most frequently encountered of these low-grade gliomas, are seen in 15%-20% of children with NF1. These tumors typically come to medical attention in young preschool children (mean age, 4.5 years) who present with reduced visual acuity or precocious puberty, or who are incidentally discovered on screening neuroimaging. 2,3While OPGs represent 66%-75% of all CNS tumors in children with NF1, 4 the second most common brain tumor is the brainstem glioma (BSG), representing approximately 18% of NF1-associated brain neoplasms.4,5 Unlike NF1-OPG, NF1-BSGs tend to arise at a slightly older ageFrom the

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Section: Resultsmentioning

confidence: 63%

A multi-institutional study of brainstem gliomas in children with neurofibromatosis type 1

et al. 2017

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Section: Discussionmentioning

confidence: 99%

“…Early surgical intervention to determine the diagnosis is recommended [54–56]. The standard treatment of DIPG is external beam radiation therapy administered in fractions over approximately 6 weeks at a total dose of 60 Gy [54]. Diffuse intrinsic pontine glioma is almost invariably fatal with a mean patient overall survival of 9–12 months from the time of diagnosis [57].…”

Section: Discussionmentioning

confidence: 99%

Progressive multifocal exophytic pontine glioblastoma: a case report with literature review

Chen

Weng

et al. 2017

Chin J Cancer

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Multifocal pontine glioblastoma exhibiting an exophytic growth pattern in the cerebello-pontine angle (CPA) is rare. We present a case of a 5-year-old girl with consecutive neurological imaging and other clinical findings indicating progressive multifocal exophytic pontine glioblastoma. Three lesions were reported, of which two were initially presented, and one was developed 2 months later. One lesion demonstrated a progressing exophytic extension in the cistern of the left side of the CPA. The other two lesions were located and confined within the pons. Initial magnetic resonance imaging and positron emission tomography–computed tomography indicated low-grade glioma or inflammatory disease. However, 2 and 3 months later, subsequent magnetic resonance spectroscopy (MRS) displayed elevated choline and depressed N-acetyl aspartate peaks compared with the peaks on the initial MRS, indicating a high-grade glioma. Subtotal resection was performed for the CPA lesion. Histopathologic examination showed discrepant features of different parts of the CPA lesion. The patient received no further chemotherapy or radiotherapy and died 2 months after surgery. The multifocal and exophytic features of this case and the heterogeneous manifestations on neurological images were rare and confusing for both diagnosis and surgical decision-making. Our case report may contribute knowledge and helpful guidance for other medical doctors.

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“…DIPG account for 80% of pediatric brainstem tumors and primarily affect young children with a median survival of less than one year [17,18]. …”

Section: Pediatric Brain Tumors and Clinical Featuresmentioning

confidence: 99%

Modeling and Targeting MYC Genes in Childhood Brain Tumors

Hutter

Bolin

Weishaupt

et al. 2017

Genes

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Brain tumors are the second most common group of childhood cancers, accounting for about 20%–25% of all pediatric tumors. Deregulated expression of the MYC family of transcription factors, particularly c-MYC and MYCN genes, has been found in many of these neoplasms, and their expression levels are often correlated with poor prognosis. Elevated c-MYC/MYCN initiates and drives tumorigenesis in many in vivo model systems of pediatric brain tumors. Therefore, inhibition of their oncogenic function is an attractive therapeutic target. In this review, we explore the roles of MYC oncoproteins and their molecular targets during the formation, maintenance, and recurrence of childhood brain tumors. We also briefly summarize recent progress in the development of therapeutic approaches for pharmacological inhibition of MYC activity in these tumors.

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DIPG in Children – What Can We Learn from the Past?

Cited by 172 publications

References 150 publications

A multi-institutional study of brainstem gliomas in children with neurofibromatosis type 1

A multi-institutional study of brainstem gliomas in children with neurofibromatosis type 1

Progressive multifocal exophytic pontine glioblastoma: a case report with literature review

Modeling and Targeting MYC Genes in Childhood Brain Tumors

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