ATRT-18. Atypical Teratoid/Rhabdoid Tumor in Children: Case Reports from Indonesia

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.