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

Choroid plexus tumors (CPTs) are rare, intraventricular neoplasms that originate from the epithelial cells of the choroid plexus [1-4]. CPTs account for 0.4% to 0.6% of all central nervous system (CNS) tumors [1,2,5,6]. The incidence of CPTs are slightly higher within the pediatric population, as they represent 1.5%-3.9% of all CNS tumors in this age group [5]. CPTs include both benign and malignant variants. The majority of CPTs are benign choroid plexus papillomas, which exhibit excellent prognosis [7]. Choroid plexus carcinomas (CPC) are rare, malignant neoplasms that represent 20-30% of CPTs in children [5]. CPC is primarily a pediatric malignancy, as 80% of them occur in the pediatric population [2]. The median age for CPC development is 26 to 32 months old [2,6]. The World Health Organization (WHO) has classified a CPC as a WHO grade III, and prognosis is poor with a five year survival rate of approximately 40% [2,8]. Histologic features include necrotic and cystic regions, distortion of the papillary pattern, increased mitotic figures, and nuclear pleomorphism [1,2,9]. Two-thirds of CPCs spread into the cerebrospinal fluid (CSF) and disseminate throughout this pathway [5]. In children, CPCs are most commonly found in the lateral ventricles, in contrast to adults who most frequently develop these tumors in the fourth ventricle [10]. This malignancy typically presents with signs of hydrocephalus and increased intracranial pressure [7]. Although the exact mechanism is unclear, this is believed to be due to overproduction of CSF by the tumor cells, blockage of CSF flow, or impaired CSF absorption [7,8].