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Optic Glioma in Children: A Retrospective Analysis of 101 Cases

Varan, Ali MD*; Batu, Abduselam MD*; Cila, Ayşenur MD; Soylemezoğlu, Figen MD; Balci, Serdar MD; Akalan, Nejat MD§; Zorlu, Faruk MD; Akyüz, Canan MD*; Kutluk, Tezer MD, PhD*; Büyükpamukçu, Münevver MD*

American Journal of Clinical Oncology: June 2013 - Volume 36 - Issue 3 - p 287–292
doi: 10.1097/COC.0b013e3182467efa
Original Articles: Pediatric

Aim: To evaluate the clinical characteristics and long-term outcome of pediatric patients with optic glioma.

Patients and Methods: A total of 101 patients with optic glioma newly diagnosed between 1975 and 2008 were evaluated retrospectively. COPP (cyclophosphamide, vincristine, procarbazine, prednisolone) and cisplatin plus etoposide were the most commonly used chemotherapy regimens. Radiotherapy was administered in patients with progressive or unresponsive disease.

Results: The median age at the time of diagnosis was 6 years, and the male/female ratio was 1.15. The most common referral complaint was strabismus. The most common site of optic glioma was the hypothalamic-chiasmatic region (31.7%). Fifty-three patients (52.5%) had neurofibromatosis type 1 (NF-1). Treatment consisted of surgery, radiotherapy, and chemotherapy. Forty-nine patients (48.5%) underwent surgery, which was predominantly subtotal resection, radiotherapy was administered to 39.4%, and 30 patients received chemotherapy. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 65.8% and 88.4%, respectively, and the 10-year PFS and OS were 54.2% and 83.4%, respectively, with an 8-year median follow-up. OS was significantly lower in patients with hypothalamo-chiasmatic involvement and significantly higher in patients with NF-1. The 5- and 10-year PFS rates were significantly higher in patients 10 years or older at diagnosis (P=0.0001) and in patients with intraorbital involvement (P=0.032). Eighteen patients (17.8%) died of disease.

Conclusions: Patients with NF-l and those older than 10 years have a better prognosis, whereas patients younger than 3 years and those with hypothalamic-chiasmatic optic glioma have a worse outcome. Further studies are needed to find appropriate treatment strategies.

*Department of Pediatric Oncology, Hacettepe University Institute of Oncology

Departments of Radiology

Pathology

§Neurosurgery

Radiation Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey

The authors declare no conflicts of interest.

Reprints: Ali Varan, MD, Department of Pediatric Oncology, Hacettepe University Institute of Oncology, 06100 Ankara, Turkey. E-mail: hupog@tr.net.

© 2013 by Lippincott Williams & Wilkins, Inc