CR-16
Title: An astronomical finding: an unusual case of hydrocephalus in a 9 year old female
1Tolia S, 2Sharma E, 2Molinos N
1Nova Southeastern University, Fort Lauderdale, FL, United states; 2Broward Health Medical Center, Fort Lauderdale, FL, United states
Introduction: Primary malignant central nervous system tumors are the second most common pediatric malignancy and are the most common pediatric solid tumor. They account for 20 percent of childhood cancers and are the leading cause of cancer death in in children 0-14 years old. Gliomas account for the great majority of primary brain tumors that arise within the brain parenchyma. Previously, classification of brain tumors was only by histologic grade determined by light microscopy. Recently, the World Health Organization published new classification guidelines based on both histopathologic appearance and on well-established molecular patterns.
Case Description: We present a 9 y.o. female patient with no significant past medical history who presented to the Emergency Department complaining of a three week history of headaches and a three day history of vomiting. Patient denied any weight loss, fever, night sweats, chills, changes in gait and vision, numbness or tingling. On physical examination, her cranial nerves 2-12 were intact, sensory and gait examination were normal. The patient previously visited her PCP a week prior to coming to the ED, and MRI was ordered. The MRI showed evidence of a well circumscribed mass in the epicenter at the left thalamus causing mild ventriculomegaly consistent with obstructive hydrocephalus due to third ventricular obstruction. Patient underwent a procedure for a ventriculoperitoneal shunt to relieve ICP pressure and obtain a biopsy of the mass. Biopsy of the mass showed cellular lesion is composed of hyperchromatic, pleomorphic cells with frequent mitotic figures and scattered apoptotic bodies. Mass revealed to be: GFAP positive, CD45 negative, Ki-67 positive. The final diagnosis of this tumor was anaplastic astrocytoma Grade III, classified by WHO. The specific histology of this tumor was determined to be ATRX wild type, IDHI R132H wild type, EFGR amplification, TP53 mutation and MYCN copy number gain. This tumor was determined to be unresectable. Once patient was stable postoperatively, patient was discharged and is currently undergoing chemotherapy and proton therapy.
Discussion: Classification of CNS tumors with both cytogenetics and histopathology can alter treatment approach and course of therapy than if the tumor had been classified with histology alone. However, complexities can arise when multiple different molecular patterns are identified, each associated with different risk stratifications. Mainstays of treatment of high grade glioma tumors include resection while balancing preservation of neurologic function. Radiation and chemotherapy as indicated by age, grade, and risk stratification.
References: Blaney SM, Hass-Kogan D, Poussaint TY, et al. Gliomas, ependymomas, and other nonembryonal tumors of the central nervous system. In: Principles and practice of pediatric oncology, 6th ed, Pizzo P, Poplack D (Eds), Lippincott Williams & Wilkins, Philadelphia 2011. p.717
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