Atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system is a sporadic and highly malignant tumor that usually affects very young children and is typically deadly despite very aggressive treatment. The optimal treatment for AT/RT remains unclear, including surgery, radiotherapy, and chemotherapy. Here we report cases of AT/RT in Indonesia. Case 1: A three-year-old girl came with worsening intermittent headaches with projectile vomiting, progressive and insidious right spastic hemiparesis, and slowly progressive weight loss three months before admission. She had septate multiloculated hydrocephalus. Head MRI demonstrates a solid lobulated mass with heterogeneous enhancement and MRS shows an aggressive metabolite pattern, arising from posterior fossa extending into the cerebellum and cerebellopontine angle, causing severe obstructive hydrocephalus. She underwent tumor resection with a midline suboccipital approach, then continued with craniospinal irradiation with dose of 36 Gy in 20 fractions then followed by 18 Gy in 10 fractions booster to posterior fossa, making the total dose to posterior fossa (the tumor) to be 54 Gy. Case 2: A twenty-month-old baby with a history of recurrent seizures, tremors, and less activity in the last two months. She had spasticity with hyperreflexia. She has been referred to our center for further evaluation and management after biopsy and VP shunt surgery at the previous hospital. Head CT scan demonstrates a large solid heterogeneous mass in the right hemisphere cerebral, causing midline shift and hydrocephalus. After the VP shunt was repaired, she underwent Head Start III chemotherapy protocol cycle 1. Both pathology examinations of the patients revealed a hypercellular tumor with prominent hyperchromatic nucleoli and loss of INI-1 staining on immunohistochemistry consistent with an ATRT diagnosis. Unfortunately, both patients died due to severe sepsis after treatment. Although AT/RT has become increasingly recognized, prognosis of ATRT is generally unfavorable, especially in developing countries.

Diffuse Intrinsic Pontine Gliomas (DIPG) is a rare and highly aggressive central nervous system tumour arising from glial cells which occur mostly in children. It remains a potentially deadly cancer despite treatment, with the highest mortality rate, in which most children die within 1-year of diagnosis. Radiation therapy might improve the clinical symptoms, but the results are temporary, with tumor progression typically occurring months post radiation, whereas chemotherapy remains controversial. Here we present a case report of a patient with DIPG Indonesia and how we diagnose and currently treat it. An eight years-old boy presented with decrease level of consciousness with headache, limb weakness, slurred speech, double vision, hemifacial weakness, paraplegia, and disequilibrium which worsened two weeks prior to admission. He was initially noted to have a medially inverted left eye two months prior to admission. On physical examination, there was a N.VI and N.VII palsy, hyperreflexia, paraplegia, and nystagmus. Head MRI demonstrated a solid with minimal enhancement after contrast administration in the left pons region extending into mesencephalon with cystic component and flat floor of fourth ventricle sign, causing hydrocephalus, suggestive diffuse intrinsic pontine glioma. He was given fractionated focal intensity modulated radiation therapy (IMRT) to the tumor along with 5 mm margins with a total dose of 54 Gy. 1.8 Gy fractions, given once daily for 5 days per week over a period of 6 weeks. Supportive care in the form of corticosteroids is used to treat the peri-tumoral edema. There was a short period of motoric improvement. But then the disease progressed with the latest head CT showed hydrocephalus formation with increased size of mass. He is treated with chemotherapy using Temozolomide, and currently alive after 3 months post diagnosis. Keywords: childhood brain tumour, Diffuse Intrinsic Pontine Glioma, radiation therapy

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