Evaluation of Neurofibromatosis Type 1 Associated Optic Pathway Gliomas

Öz

Background/Aims: Optic pathway gliomas (OPGs) are low-grade gliomas histologically represented by pilocytic astrocytoma (PA) in 90% of cases, can develop from any part of the visual pathways such as optic nerve, chiasm, optic tract, or optic radiations which frequently involve the hypothalamus. OPGs account for 3–5% of childhood central nervous system (CNS) tumors and about 2% of pediatric glial lesions. OPGs are believed to be the most prevalent intracranial tumor in patients with neurofibromatosis type 1 (NF-1) and can occur in 15–20% of NF-1 cases. The aim of this study is to evaluate the clinical features and treatment response in patients diagnosed with optic glioma and NF-1. Methods: All cases diagnosed with OPG and received treatment in the Pediatric Oncology Department, between January 2015 to January 2021 were retrospectively evaluated. Inclusion criteria include children and adolescents with OPG aged between 0 and 18 years. The medical records (gender, age, tumor entity, tumor location) of patients, as well as their treatment history and magnetic resonance imaging (MRI) scans, were examined. The diagnosis of OPG was made clinically and radiologically by the tumor board. The recommendations of the Response Assessment in Pediatric Neuro-Oncology (RAPNO) working group were used in the diagnosis and evaluation of treatment response. Patients received intravenous chemotherapy with SIOP LGG 2004 (vincristine- carboplatin) with or without bevacizumab (10 mg/kg, started every 2 weeks), therapy or vinblastine (3 mg/m2, weekly). Results: This study included 27 cases during the study period from January 2015 to January 2021. In this study there were 14 male (51.8 %) and 13 female (48.1 %) patients. The median age was 4.8 (range: 0.5–14.9) years. Biopsy was performed in three patients and the diagnosis was low-grade glioma (pilocytic astrocytoma) for all of them. Chemotherapy was administered to 22 cases in total. Twelve patients received vincristine-carboplatine, 5 patients received vincristine-carboplatin with bevacizumab and 5 patients received vinorelbine. Radiological response was evaluated in all 22 patients at 3 months MRI. No patient had a radiological complete respons, 11 patients (50%) had partial response, 2 patients (9%) presented with a progressive disease, showing an increase in measurements of 35% and 9 patients(40.9%) had stable disease at the 3-month evaluation. Conclusions: Systemic and visual problems play a significant role in the selection of treatment for pediatric patients with optic gliomas. An essential treatment option for improving symptoms and reducing tumor size is systemic chemotherapy. A crucial therapy option for enhancing vision is bevacizumab for the patients with NF-associated OPG.

Anahtar Kelimeler

• optic pathway gliomas
• pediatric cancers
• bevacizumab

Nörofibromatozis Tip 1'in Eşlik Ettiği Optik Yol Gliomlarının Değerlendirilmesi

Öz

Amaç: Optik yol gliomaları (OPG'ler), vakaların %90'ında histolojik olarak pilositik astrositom (PA) olan düşük dereceli gliomalardır ve optik sinir, kiazma, gibi görme yollarının herhangi bir kısmından hipotalamusa kadar uzanım göstererek gelişebilirler. OPG'ler çocukluk çağı merkezi sinir sistemi (CNS) tümörlerinin %3-5'ini ve pediatrik glial lezyonların yaklaşık %2'sini oluşturur. OPG'lerin, nörofibromatozis tip 1 (NF-1) hastalarında en yaygın intrakranyal tümör olduğu düşünülmektedir ve NF-1 vakalarının %15-20'sinde ortaya çıkabilmektedir. Bu çalışmanın amacı optik gliom ve NF-1 tanısı alan hastaların klinik özelliklerini ve tedavi yanıtını değerlendirmektir. Yöntem: Ocak 2015 ile Ocak 2021 tarihleri arasında Çocuk Onkoloji Bölümü'nde OPG tanısı alan ve tedavi gören tüm olgular retrospektif olarak değerlendirildi. Dahil edilme kriterleri arasında OPG'li 0 ila 18 yaş arası çocuklar ve ergenler yer almaktadır. Hastaların tıbbi kayıtları (cinsiyet, yaş, tümör varlığı, tümörün yerleşim yeri), tedavi öyküleri ve manyetik rezonans görüntüleme (MRG) tetkikleri incelendi. OPG tanısı tümör konseyi tarafından klinik ve radyolojik olarak konuldu. Tedavi yanıtının tanılanması ve değerlendirilmesinde Pediatrik Nöro-Onkolojide Yanıt Değerlendirmesi (RAPNO) çalışma grubunun önerilerinden yararlanıldı. Hastalar bevacizumab (10 mg/kg, her 2 haftada bir başlanır) ile birlikte veya bevcizumab olmadan SIOP LGG 2004 (vinkristin-karboplatin) ile intravenöz kemoterapi veya vinblastin (haftalık 3 mg/m2) aldı. Bulgular: Bu çalışmaya Ocak 2015 ile Ocak 2021 arasındaki çalışma döneminde 27 vaka dahil edildi. Bu çalışmada 14'ü erkek (%51,8) ve 13'ü kız (%48,1) hasta vardı. Ortanca yaş 4,8 (aralık: 0,5-14,9) yıldı. Üç hastaya biyopsi yapıldı ve hepsine düşük dereceli glioma (pilositik astrositom) tanısı konuldu. Toplam 22 olguya kemoterapi uygulandı. On iki hastaya vinkristin-karboplatin, 5 hastaya bevacizumab ile birlikte vinkristin-karboplatin ve 5 hastaya vinorelbin verildi. 22 hastanın tamamında 3. aydaki MRG'de radyolojik yanıt değerlendirildi. Hiçbir hastada radyolojik tam yanıt görülmedi, 11 hastada (%50) kısmi yanıt, 2 hastada (%9) ilerleyici, 9 hastada (%40.9) stabil hastalık görüldü. Sonuç: Optik gliomlu pediatrik hastalarda tedavi seçiminde sistemik ve oküler bulgular önemlidir. Sistemik kemoterapi oküler bulguların iyileştirilmesinde ve tümör boyutunun küçültülmesinde önemli bir seçenektir. Bevacizumab tedavisi tümör boyutunu küçültmese de görsel bulguları iyileştirmektedir.

Anahtar Kelimeler

• optik yol gliomları
• pediatrik kanserler
• bevacizumab

Kaynakça

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