Outcomes of Multidisciplinary Management in Pediatric Low-Grade Gliomas
To evaluate the outcomes in pediatric low-grade gliomas managed in a multidisciplinary setting.
We conducted a single-institution retrospective study of 181 children with Grade I-II gliomas. Log-rank and stepwise Cox proportional hazards models were used to analyze freedom from progression (FFP) and overall survival (OS).
Median follow-up was 6.4 years. Thirty-four (19%) of patients had neurofibromatosis Type 1 (NF1) and because of their favorable prognosis were evaluated separately. In the 147 (81%) of patients without NF1, actuarial 7-year FFP and OS were 67 ± 4% (standard error) and 94 ± 2%, respectively. In this population, tumor location in the optic pathway/hypothalamus was associated with worse FFP (39% vs. 76%, p < 0.0003), but there was no difference in OS. Age ≤5 years was associated with worse FFP (52% vs. 75%, p < 0.02) but improved OS (97% vs. 92%, p < 0.05). In those with tissue diagnosis, gross total resection (GTR) was associated with improved 7-year FFP (81% vs. 56%, p < 0.02) and OS (100% vs. 90%, p < 0.03). In a multivariate model, only location in the optic pathway/hypothalamus predicted worse FFP (p < 0.01). Fifty patients received radiation therapy (RT). For those with less than GTR, adjuvant RT improved FFP (89% vs. 49%, p < 0.003) but not OS. There was no difference in OS between patient groups given RT as adjuvant vs. salvage therapy. In NF1 patients, 94% of tumors were located in the optic pathway/hypothalamus. With a conservative treatment strategy in this population, actuarial 7-year FFP and OS were 73 ± 9% and 100%, respectively.
Low-grade gliomas in children ≤5 years old with tumors in the optic pathway/hypothalamus are more likely to progress, but this does not confer worse OS because of the success of salvage therapy. When GTR is not achieved, adjuvant RT improves FFP but not OS. Routine adjuvant RT can be avoided and instead reserved as salvage.
Available from: Adrianna Ranger
- "As stated earlier, those who have optic-hypothalamic gliomas in the context of NF-1 generally have a much more indolent course than those in whom NF-1 does not exist(Massimi et al., 2007;Oh et al., 2011). This said, the prognosis is quite variable, depending upon the lesion, and may be better than for those with pure optic gliomas (again, in the absence of NF-1). "
Available from: Sridharan Gururangan
- "Extrapolating data from adult phase III trials, it seems appropriate to use focal radiotherapy in doses of 45-54 Gy in 1.8 to 2.0 Gy fractions. Three-D conformal RT, intensity modulated RT, and proton-beam therapy are new radiation delivery techniques that are designed to minimize damage to normal brain but await validation in larger studies (Watson, Kadota et al. 2001). A preliminary report from St. Jude Children's Hospital explored the usefulness of 3-D conformal RT in patients with localized LGG (Merchant, Happersett et al. 1999). "
Available from: Yaacov Richard Lawrence
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ABSTRACT: Optic nerve gliomas (ONG) are rare astrocytic neoplasms. A paucity of literature exists on the epidemiology and outcomes of ONG. Here, we present a series of 445 cases of ONG obtained from the Surveillance, epidemiology and end results (SEER) database. Data on patient and tumor characteristics as well as initial treatment with surgery or radiation were extracted from the SEER Database. Survival rates were calculated using the Kaplan-Meier method. A multivariate analysis was performed to determine independent prognostic factors predicting mortality hazard ratios (HRs) using Cox proportional hazards modeling. The median age range at diagnosis was 5-9 years. Twenty percent of patients were over the age of 20 years. Amongst patients with information available on tumor grade (n = 131), 83% had a low-grade tumors and 17% had a high-grade tumors. Sixteen percent of patients received radiation therapy and 18.4% of patient underwent a sub- or gross total resection. The 5 year overall survival was 96% and 20% for patients with low- and high-grade tumors, respectively. In a multivariate analysis, grade was the only significant predictor of overall survival (HR 29.3, CI: 4.3, 205.4, P < 0.001). Age at diagnosis, receipt of radiation therapy, and extent of surgical resection were not significantly correlated with overall survival. In conclusion, ONG are rare tumors seen predominantly in children. The overall prognosis of high-grade tumors remains poor in all age groups despite multi-modality treatment.
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