Management of Choroid Plexus Tumors in Infants and Young Children Up to 4 Years of Age: An Institutional Experience.

BACKGROUND: Choroid plexus tumors (CPTs) are rare tumors characterized by papillary and intraventricular growth. The young age of presentation of such tumors, especially in infants, and the lack of consensus on adjuvant therapy in case of atypical choroid plexus papilloma (aCPP) and choroid plexus carcinoma (CPC) create dilemma for the management of such tumors. We discuss the presentation, management, complications, and outcome in 15 patients (children 4 years of age and younger) and review pertinent literature.

METHODS: We retrospectively analyzed the case records of all patients with CPTs who were operated in our institute from January 2010 to March 2018. We found 15 patients in the age group of 0-4 years of age. The variables analyzed include age, sex, presentation, location, surgical approach, extent of resection, intraoperative blood loss, percentage of blood loss, blood transfused, histopathology, postoperative complications, and outcome. Images were obtained from picture archiving and communication system, and patient details and follow-up were obtained from discharge summary, operative notes, and hospital records.

RESULTS: Ten patients had choroid plexus papilloma (CPP), 2 patients had aCPP, and 3 patients had CPC. The mean age was 15.2 months, whereas the median age was 8 months (range, 40 days-4 years). The mean blood loss was 329 mL, whereas the median blood loss was 175 mL. There were a total of 5 deaths, including 3 patients with CPC and 1 each with aCPP and CPP.

CONCLUSIONS: CPTs are challenging tumors in infants and very young children because of the potential for massive blood loss. CPP is associated with lesser blood loss and favorable outcome compared with aCPP and CPC. Massive blood loss in CPC and aCPP can be life threatening as has been shown in our series. CPC has a rapid proliferation potential as shown in one of our cases. Attempts at decreasing vascularization of such tumors should be made by various methods, including preoperative embolization and neoadjuvant chemotherapy; however, a consensus on this is lacking.