Prolonged Survival for Choroid Plexus Carcinoma with Oncocytic Changes: A Case Report

Abstract

Introduction: Choroid plexus carcinoma (CPC) is a rare central nervous system neoplasm derived from the choroid plexus epithelium. CPCs are challenging to surgically resect due to their intraventricular location and high vascularity. Tumor recurrence and dissemination are frequently seen with CPC. Due to the malignant nature of these tumors, the prognosis is poor with a five year survival rate of approximately 40%. Case Description: The authors report a pediatric patient with a CPC (WHO grade III) with oncocytic changes, who survived for seven years after her initial diagnosis. A 14-month-old female presented to the emergency department with a sudden onset change in mental status following a fall. Imaging revealed a hemorrhagic, left intraventricular heterogeneous tumor. The patient underwent a left parietal craniotomy and gross tumor resection. Pathological findings were consistent with a malignant CPC. Her treatment regimen included four cycles of etoposide, Temodar, Cytoxan and vincristine, two cycles of oral etoposide and temozolomide with vincristine, in addition to a stem cell harvest. Recurrence of our patient’s disease led to two subsequent gross total resections. This patient was in the minority who survived greater than five years with this tumor type. Conclusion: Gross total resection is the goal, and pharmacologic intervention including chemotherapy and radiation therapy remains controversial, as this treatment is not curative. We would like to share this unusual case with the medical community to increase clinical understanding in the management and treatment of this malignant tumor.