An 11-year-old male with no past medical history presented to the emergency department (ED) for headache and vomiting. His symptoms began four days prior to arrival with headache and neck pain first that gradually worsened with subsequent development of non-bloody, non-bilious emesis one day prior to arrival to the ED. He localized his headache to the frontal midline and reported accompanying photophobia. He denied recent illness, foreign travel, fever, chills, weakness, dizziness, or changes in hearing, vision, strength, or sensation. Physical examination was only remarkable for tenderness to palpation at the posterior neck. Laboratory blood counts and metabolic panels were unremarkable for acute pathology. However, a head computer tomography (CT) scan without contrast showed an irregular hyperattenuating mass at the midline spanning the bilateral lateral ventricles measuring 2.8 x 2.7 x 2.1 cm with 4 mm left-to-right mass effect on corpus collosum. Brain and spinal magnetic resonance imaging (MRI) with and without contrast demonstrated a heterogeneous, lobulated, and predominantly intraventricular enhancing mass centered in the left atrium abutting the choroid plexus crossing the midline to the contralateral lateral ventricle and extending superiorly with invasion into the posterior body of the corpus callosum and cingulate gyrus bilaterally without metastases. No hydrocephalus or significant transependymal flow were noted. The patient was admitted to the Pediatric Intensive Care Unit (PICU) with resolution of headache and pain with intravenous dexamethasone among other supportive medications and management. On hospital day #6, the patient underwent a left parietal neuroendoscopic approach with stereotactic navigation for intraventricular brain tumor biopsy and debulking. On hospital day #8, the patient was discharged on a dexamethasone taper with pathology report pending and outpatient follow-up with neurosurgery.

The patient returned to the ED on two subsequent occasions. His second visit was within one week of being discharged due to recurrence of intractable headaches and vomiting again with head CT in the ED showing a small hemorrhage within the tumor bed with readmission to PICU due to concern for elevated Intracranial Pressure (ICP) with resumption of IV dexamethasone and supportive management and eventual discharge after 7 days with steroid taper. The patient presented again to ED 10 days after his last discharge due to a recurrence of headaches without vomiting again with resumption of IV dexamethasone and supportive management. On hospital day #4, he underwent a transcortical approach left parietal craniotomy with tumor debulking and placement of an External Ventricular Drain (EVD). Due to its high vascularization, only 75% of the tumor could be respected. Patient tolerated the procedure well and EVD was eventually removed due to resolution of headaches and minimal output with repeat head CT showing no intracranial edema or fluid collections. The patient’s tumor pathology report finally yielded a diagnosis of a High-Grade Glioma (HGG) composed of cells with angulated, hyperchromatic, and pleomorphic nuclei evidencing mitotic activity and immunohistochemistry negative for IDH1/2 mutations indicating IDH-wildtype genealogy and negative for BRAF, EGFR, or FGFR1/2/3 mutations as well. Unfortunately, further molecular testing could not differentiate a more specific tumor classification due to lack of sufficient tissue from biopsy. Currently, the patient’s case is scheduled to be discussed by an interdisciplinary tumor board.