NDLI: ED-06BRAINSTEM GLIOMAS IN ADULTS: DO ADULT DIFFUSE INTRINSIC PONTINE GLIOMAS EXIST?

Content Provider	PubMed Central
Author	Garren, Nancy Harris, Sunit Baker, Eva Teri, Kreisl Warren, Katherine
Copyright Year	2014
Abstract	BACKGROUND: The majority of brainstem tumors in children are diffuse intrinsic pontine gliomas (DIPG), and are diagnosed in middle childhood. Recent data suggests DIPG is a disease of development. Relatively little information is available for adult brainstem gliomas (BSG). We reviewed clinical and radiographic information from adults with BSG referred to our institution. METHODS: Patients were enrolled on IRB-approved protocols. Clinical data was obtained from patient charts and electronic records. Radiographic data was reviewed on a Picture Archiving and Communications System (PACS) and assessed for primary tumor location, enhancement, intensity on T1, invasion of surrounding anatomical structures, and presence of CNS disease outside of the brainstem. RESULTS: 18 patients diagnosed with BSG were identified; diagnostic MRI was available for review on 15. M:F was 16:2; median age 29.5 (range 16-71) years at diagnosis. Presenting symptoms included long tract signs (61%), diplopia (50%), other visual changes (22%), imbalance (44%), headache (33%) and nausea/vomiting (33%). Primary tumor location varied: 27% were located in the pons only, 33% in pons with extension to medulla, 13% in pons with extension to midbrain, and 20% in pons with extension to midbrain and medulla. 80% showed contrast enhancement, with the majority (58%) of these enhanced diffusely. Other radiographic findings included encasement of the basilar artery (20%) and evidence of CNS disease outside of the brainstem (20%) at diagnosis (cerebellum and parietal lobe). The majority (88%) received radiation treatment; 56% received concurrent chemotherapy and 44% received adjuvant chemotherapy. Median time to first progression (defined radiographically and clinically) was 22 months (range 3-192 months). CONCLUSIONS: Although adults with brainstem tumors involving the pons have similar presentation as children with DIPG, imaging findings and clinical outcome differ significantly. Investigation into the biology of the adult lesions, including H3.3 mutations, may help define these as more distinct entities.
Related Links	http://dx.doi.org/10.1093/neuonc/nou253.6
Starting Page	67
File Format	PDF
ISSN	15228517
e-ISSN	15235866
Journal	Neuro-Oncology
Issue Number	Suppl 5
Volume Number	16
Language	English
Publisher	Oxford University Press
Publisher Date	2014-11-01
Access Restriction	Open
Rights Holder	Oxford University Press
Subject Keyword	Cancer Research Oncology Clinical Neurology Research in Higher Education
Content Type	Text
Resource Type	Article
Subject	Cancer Research Neurology (clinical) Oncology

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