Multidisciplinary Management of Craniopharyngiomas in Children: A Single Center Experience

Abstract

Background: Craniopharyngioma (CP) is a rare brain tumor involving the sellar region. The best management is still debated. Gross total resection (GTR) is considered the best option to improve recurrence­free survival, but considerable long­term sequelae with a significant impact on quality of life have been reported. Subtotal resection followed by radiotherapy achieves similar disease control compared to GTR with less complications. Methods: We retrospectively reviewed 10 pediatric patients affected by CP treated with partial resection and subsequent proton therapy (PBT). We reviewed visual, endocrinological, and neuropsychological data at baseline, after surgery, and after radiation for all patients. Results: At the time of diagnosis, visual impairment was detected in 70% of patients and endocrinological abnormalities in 50%. All patients were subject to one or more surgical procedures. Surgery had no impact on visual status; however, it caused a worsening of endocrine function in half of patients. After surgery, all patients underwent PBT, achieving a partial response in 7 out of 10 patients (70%), while stable disease was observed in the other three patients (30%) at a median follow-up of 78 months from the end of PBT. Both visual and endocrine deficits were stable after PBT, with neurocognitive performance scores unchanged from baseline. Conclusions: A conservative surgical approach followed by PBT represents a safe and effective strategy to manage CP and limit long-term sequelae.

Diabetes Insipidus Pascaoperasi pada Pasien dengan Reseksi Tumor Kraniofaringioma

“…[8][9] Penegakkan diagnosis pada pasien ini dilakukan melalui pemeriksaan MRI yang menunjukkan adanya massa kistik ekstraaksial pada regio suprasella yang mendesak struktur sinus kavernosus, medula oblongata dan ventrikel III yang mengarahkan diagnosis pada kraniofaringioma tipe adamantinoma. 10 Kadar hormon aksis hipotalamus-pituitari, termasuk hormon pertumbuhan, hormon tiroid, LH dan FSH perlu diukur bersamaan dengan kadar kortisol dan penilaian osmolalitas serum dan urine. Segala jenis abnormalitas idealnya dikoreksi preoperatif.…”