Pediatric choroid plexus tumors: epidemiology, treatments, and outcome analysis on 202 children from the SEER database

Abstract

Choroid plexus papillomas (CPPs) and carcinomas (CPCs) are rare neoplasms that affect mostly children. Due to their rarity, their epidemiology and outcomes are incompletely understood. The National Cancer Institute's Surveillance, Epidemiology and End Results (SEER) Program is a well-established population-based group of registries that collects and publishes cancer incidence and survival data representing approximately 28 % of the US population. SEER-STAT v8.1.2 was used to identify patients with ICD-O-3 codes for choroid plexus tumors in patients aged 0-19. Demographics, initial treatment, and follow-up data were collected. Statistical methods including Kaplan-Meier curves, log rank tests, and Cox proportional hazards regression were used to estimate associations between independent variables and survival. The SEER registries contained 107 CPPs (2004-2010) and 95 CPCs (1978-2010). Median follow-up was 38 and 40 months, respectively. More than 75 % of CPCs were diagnosed before the age of 5 years, versus 48 % for CPPs. Sixty-five percent of CPCs and 57 % of CPPs occurred in males. In both groups at least 90 % of children underwent surgical resection. Gross total resection (GTR) was achieved in 67.0 % of CPCs and 63.6 % of CPPs. Almost 17 % of CPCs were treated with radiation versus only 0.9 % of CPPs. More than 98 % of patients with CPP were alive at the last follow-up, versus 62 % of CPC patients. For CPC, surgery was significantly associated with increased overall survival, but contrary to previous reports, extent of surgical resection was not associated with survival. Age, sex, race, and radiation treatment also had no effect on survival. This report, using the SEER datasets, corroborates many findings of previous smaller studies on CPTs. CPC occurs in younger children, with a male predominance, and a much worse prognosis than CPP. As such, these tumors have been treated aggressively with high rates of GTR and radiation treatment. Despite these treatments, overall survival for CPC remains poor.

Pediatric choroid plexus carcinoma: Biologically and clinically in need of new perspectives

“…provided evidence for the benefit of complete surgical resection in the treatment of patients with CP carcinoma . Despite the well‐recognized prognostic value of the extent of resection in CP carcinomas, some reported series have failed to confirm the survival benefit associated with GTR …”

Co‐occurrence of histone H3 K27M and BRAF V600E mutations in paediatric midline grade I ganglioglioma

“…Grade I GG accounts for 1%–5% of all central nervous system neoplasms in children; most are found in the cortex, generally in the temporal lobe (>70%) . However, they can occur in the posterior fossa or spinal cord, and other midline locations have also been reported . GGs in the brainstem or spinal cord exhibit a poorer prognosis.…”

Atypical Presentation of a Pediatric Cerebellar Ganglioglioma

“…For low-grade GGs, which fortunately represent the majority of cases, gross total resection will often confer an excellent prognosis [7] . This is frequently the case for isolated cerebellar hemispheric or vermian GGs without extension into the peduncles or brain stem.…”

“…Within the pediatric population, GGs typically have a mean age of onset between 10 and 11 years of age with a slightly increased male-to-female ratio [7] . Presenting symptoms of GGs vary but often include epileptic seizures, deficits due to a local mass effect and signs of increased intracranial pressure (ICP) [8] .…”