LGG-23. REPEATED PROGRESSIONS IN PEDIATRIC CHIASMATIC-HYPOTHALAMIC GLIOMAS (CHG): CAN WE IDENTIFY SUCCESSFUL TREATMENT STRATEGIES?. Issue 2 (22nd June 2018)

• Record Type: Journal Article
• Authors: Kandels, Daniela; Schmidt, René; Warmuth-Metz, Monika; Bison, Brigitte; Kortmann, Rolf-Dieter; Timmermann, Beate; Pietsch, Torsten; Gnekow, Astrid K
• Is Part Of: Neuro-oncology. Volume 20:Issue 2(2018)supplement 2
• Page Start: i109
• Page End: i109
• Publication Date: 2018-06-22
• Subjects: Brain Neoplasms -- Periodicals; Brain -- Tumors -- Periodicals; Brain -- Cancer -- Periodicals; Nervous system -- Cancer -- Periodicals; 616.99481
• DOI: 10.1093/neuonc/noy059.364
• Languages: English
• ISSNs: 1522-8517

Abstract

Abstract: OBJECTIVE: Recommendations for primary treatment of pediatric CHG are well established, but comprehensive therapeutic strategies for subsequent progressive disease are needed. We evaluated salvage therapies applied within the German/Swiss SIOP-LGG-2004/LGG-register cohort. PATIENTS: 446 patients with CHG among 2589 patients with low-grade glioma were registered between 2004 and 2015. Neurofibromatosis type 1: 209; histology: 159 pilocytic astrocytomas, 18 pilomyxoid astrocytomas, 27 other histologies and 242 radiological diagnosis. RESULTS: Resection (complete/subtotal/partial) was achieved in 115, biopsy in 89 patients. Only 131/446 patients remained observed without treatment for up to12.8 years. First non-surgical treatment was chemotherapy (ChT) for 268 (263 vincristine/carboplatin ±etoposide) and radiotherapy (RT) for 47. After primary treatment 136/315 patients progressed (median time 2.2 years), of whom 107 received second- (85ChT, 22RT), 54 third- (39ChT, 15RT), 15 fourth- (11ChT, 4RT) and 5 fifth-line therapy (2ChT, 3RT). Five-year overall survival (OS) of the complete cohort is 96.8%. Although 23/53 infants (age <1year) and 21/44 patients with dissemination needed 3-5 therapies, 5-year-OS for infants is still 83.7% and 84.9% for dissemination, respectively. While successive treatments mirrored the distribution of general options, the percentage of patients receiving RT increased up to 30-60% for fourth and fifth therapy. Multiple chemotherapies do not seem to hamper the effect of subsequent radiotherapy. Following vincristine/carboplatin ±etoposide second-line platinum-based ChT achieved 53.4% 2-year progression-free survival vs. 30.3% with vinblastine. CONCLUSION: Progression/relapse of pediatric CHG following first-line standard treatment still allows disease control for most patients with multiple treatment lines. Future studies should focus upon the effect on vision and integrate targeted treatment.