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¹Göztepe Prof. Dr. Süleyman Yalçın Şehir Hastanesi, Nöroşirürji Kliniği, İstanbul, Türkiye Congenital brain tumors are defined as tumors that develop in utero and occur during the prenatal period and in the first 60 days of life. Although they are very rare, they constitute the majority of congenital tumors and make up 1.9-0.5% of all childhood tumors. The most common forms in the literature are teratomas, gliomas, embryonic tumors, and choroid plexus tumors. Teratomas constitute one-third to one-half of these and 0.5% of all intracranial tumors are seen in this age group. Macrocephaly and hydrocephalus are the most common findings at birth. Radiological methods facilitate the detailed examination of the cranial cavity in the prenatal and postnatal period and enable early diagnosis. The treatment includes maximal surgical excision and chemotherapy. Surgical difficulties in removing large tumors, life-threatening bleeding, and increased toxicity during adjuvant chemotherapy are common. Due to treatment limitations in this age group, the first series were mostly identified as autopsy findings. In recent years, the histological and molecular features have become well known. Advanced technological possibilities and new clinical series published as a result of a multidisciplinary approach have shown that the prognosis is not so bad and may be even better than in more advanced age groups for some molecular types. Anahtar Kelimeler : Congenital brain tumor, Neonatal brain tumors, Pediatric neurosurgery