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Low-Grade Glioma

  • David M. Routman
  • Paul D. BrownEmail author
Chapter

Abstract

Approximately 3000 new cases of low-grade glioma (LGG) will be diagnosed annually in the United States. LGG includes grade I and grade II glioma and represents a heterogeneous group of tumors. The pathologic criteria for diagnosis of LGG have been updated per the World Health Organization 2016 classification system. Pilocytic grade I astrocytoma is the most common grade I lesion, and grade II lesions are mainly diffuse astrocytoma and oligodendroglioma, which comprise the majority of adult LGG and are the focus of this chapter.

Diffuse LGG (grade II lesions) are distinguished by genetic characteristics on molecular testing, with IDH1 mutations found in the majority of all grade II LGG. 1p/19q codeletions define the diagnosis of oligodendrogliomas and are mutually exclusive from TP53 mutations. TP53 mutations along with ATRX mutations are found in the majority of grade II astrocytomas but are not required for diagnosis. Astrocytomas, IDH wild type, are most likely to be a higher-grade lesion but can represent a grade II lesion if confirmed histologically.

Seizure is the most common presenting symptom, with occasional lesions discovered incidentally or with patients potentially presenting with focal neurologic deficits. Diffuse grade II gliomas are most commonly found in patients in their late 30s to early 40s. Grade II lesions are poorly circumscribed on magnetic resonance imaging (MRI) and have minimal enhancement, with significant signal abnormality best seen on T2-weighted or fluid-attenuated inversion recovery (FLAIR) sequencing.

Primary therapy includes maximal safe resection versus observation in select cases. Adjuvant options include radiation therapy (RT) versus observation after resection with RT at the time of progression. RT can improve symptomatology and improve progression-free survival (PFS). Dosing is generally 50.4–54 Gy delivered in 28–30, 1.8 Gy daily fractions. For high-risk patients, defined as age older than 40 or age under 40 with a subtotal resection (STR), the addition of procarbazine, CCNU, and vincristine (PCV) to RT has been shown to improve overall survival (OS). Temozolomide (TMZ) in comparison to PCV is currently under investigation. Further prospective work is needed to clarify optimal timing and intensity of therapy, weighing the side effects of early intervention with the potential survival benefit.

Keywords

Low-grade glioma Pilocytic astrocytoma Grade II astrocytoma Grade II oligodendroglioma 

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© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Radiation OncologyMayo ClinicRochesterUSA

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