An Infant with Prenatally Diagnosed Congenital Anaplastic Astrocytoma Who Remains Disease-Free after Proton Therapy
In January 2007, a 9-month-old boy with recurrent anaplastic astrocytoma of occipital lobe was transferred to our hospital for further management. On referral, he looked chronically ill but was adequate development for his age. Neurologically, he showed grade IV right sided weakness. He was born at 40 weeks of gestational age by normal vaginal delivery and the baby weighed 3.1 kg at birth. An obstetric ultrasound examination showed a huge mass in occipital lobe (64×46 mm) at the gestational age of 38 weeks. At birth, he had no other physical abnormalities (head circumference 34 cm, 50-75 percentile). A brain magnetic resonance imaging (MRI) () at 1 week of age revealed a huge lobulated, soft tissue mass on parietooccipital lobe. Subtotal resection of the mass done at 2 weeks after birth confirmed anaplastic astrocytoma (). At 1 month of age, he was treated with chemotherapy as per the Korean Society for Pediatric Neuro-Oncology (KSPNO) infant brain tumor protocol (vincristine, etoposide, cisplatin, cyclophosphamide, carboplatin, ifosfamide) for 4 months until a follow-up brain computed tomography (CT) showed progressive tumor of 35×34 mm-sized mass in the occipital lobe. At 5 months of age, he underwent 2nd tumor resection. The tumor histology was the same as before. Within 2 weeks after the surgery, he was placed on temozolomide with no avail. At 7 months of age, chemotherapy was changed to irinotecan, celecoxib, etoposide and thalidomide. At 9 months of age, upon showing further progression of tumor on brain MRI, he was finally referred for further management.\nUpon arrival, he underwent the 3rd gross tumor removal at the age of 11 months and had post-operative recovery for two months. He was able to walk holding with right side weakness (grade IV), make eye contact and have control over his head at that time. And treatment strategy was followed by proton therapy to tumor bed for a total dose 40 cobalt gray equivalent (CGE)/20 fractions (Fx) in 4 weeks (). At 20 months of age, he was able to walk without assistance. Neurologically, he continued to have limping gait on the right. Neurocognitive function test by Korean-Wechsler Preschool and Primary Scale of Intelligence (K-WPPSI) at 38 months of age showed verbal intelligence 109, performance intelligence 71 and total intelligence 88 and he showed adequate development and at 51 months of age showed verbal intelligence 133, performance intelligence 108 and total intelligence 125. Now at 53 months of age, he remains disease-free with adequate head size for his age and the height of 50 percentile. The most recent follow up of brain MRI (3 yr post-proton therapy) showed a large surgical defect at left parietooccipital operative bed without evidence of tumor recurrence ().