LGG-39. Ascites in a medullary and leptomeningeal ganglioglioma patient following cisplatin treatment necessitating cessation of therapy and conversion to VA shunt

Abstract

INTRODUCTION: Ganglioglioma is a low grade neoplasm consisting of dysplastic neuronal and neoplastic glial cells and accounts for 5% of pediatric CNS tumors. Management often includes CNS diversion. There have been case reports in which platinum containing chemotherapy has been thought to contribute to CSF malabsorption leading to ascites. CASE: A 13 month old male developed progressive macrocephaly, developmental delay, chronic emesis, and intermittent bilateral cranial nerve VI palsy over the 5 months prior to presentation. MRI brain/spine was significant for an enhancing nodule in the left posterior lateral medulla, nodular thickening and enhancement along the brainstem down to the conus medullaris and in the tentorium, with associated hydrocephalus. Biopsy of the medullary nodule and of the enhancement were consistent with ganglioglioma with BRAF-KIAA1549 fusion, equivocal MYCN amplification, and no BRAF V600E mutation. A ventriculo-peritoneal shunt was placed at the time of biopsy. Therapy was initiated with vincristine (1.5 mg/m2) and carboplatin (175 mg/m2.) Following the 12 week induction phase of therapy, he developed increasing diarrhea, emesis, and abdominal ascites. Peritoneal fluid analysis had no malignant cells and low protein compared to CSF. Ascites was responsive to drainage but would rapidly re-accumulate. Ultimately the patient’s chemotherapy was discontinued after 2 maintenance cycles due to continued symptoms. Acetazolamide was trialed but discontinued due to side effects, so its efficacy could not be determined. He underwent shunt externalization followed by venticulo-atrial (VA) shunt re-internalization. He has not had ascites since that time, at 4 months from surgery. His CNS disease burden has been stable at 6 months off therapy. DISCUSSION: Ascites was most likely due to CSF malabsorption in the abdomen with a possible contribution from platinum containing chemotherapy and less likely secondary to malignant peritoneal cells. Resolution since VA shunt internalization makes alternate explanations less likely.