Choroid plexus tumors in infants and young children - Lessons learnt from an institutional review

OBJECTIVES: Choroid plexus tumors (CPT) are rare tumors characterized by papillary and intraventricular growth. The young age of presentation of such tumors as well as the lack of consensus on adjuvant therapy in cases of... [ view full abstract ]

OBJECTIVES: Choroid plexus tumors (CPT) are rare tumors characterized by papillary and intraventricular growth. The young age of presentation of such tumors as well as the lack of consensus on adjuvant therapy in cases of atypical choroid plexus papillomas (aCPP) and choroid plexus carcinomas (CPC) create a dilemma in their management. METHODS: We retrospectively analyzed the case records of all patients in the age group 0 to 5 years with CPTs who were operated at our institute from January 2010 to July 2015. The variables analyzed included 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 PACS, patient details and follow up were obtained from discharge summaries, operative notes and hospital records. RESULTS: 11 children were included in our study. 7 patients had choroid plexus papillomas (CPP), two patients had aCPP and two patients had CPC. The median age was 8 months (range 40 days - 4 years). The mean blood loss was 285.7 ml whereas the median blood loss was 130 ml. Out of 3 deaths, 2 deaths occurred on post operative day 1 (POD1) (one patient with aCPP and one patient with CPC) where as one patient (CPC) died four months after surgery. CONCLUSIONS: CPT's are challenging tumors in infants and very young children because of potential for massive blood loss in such patients. CPP are associated with lesser blood loss and favorable outcome as compared to aCPP and CPC, where massive blood loss can be life threatening. CPC have a rapid proliferation potential as shown in one of our cases. Attempts at decreasing vascularisation of such tumors should be made by various methods which includes preoperative embolization and neo-adjuvant chemotherapy, although a consensus on this is lacking.