Background Flashcards
What is the function of the choroid plexus (CP) and where is it found?
The CP is located in the brain ventricles and is the tissue that produces CSF.
What % of intracranial neoplasms do CP tumors represent in children vs. adults?
CP tumors are more common in children, representing up to 5% of pediatric brain tumors vs. <1% of primary intracranial neoplasms in adults.
What are the most common locations of CP tumors in children vs. adults?
Children: Lat ventricles
Adults: 4th ventricle
What is the name for the benign CP variant, and how frequent is it? How about the malignant variant?
Benign variant: choroid plexus papilloma (CPP)/WHO grade I (60%–80% of cases)
Malignant variant: choroid plexus carcinoma (CPC)/WHO grade III (20%–40% of cases)
What are the pathologic features of WHO grade I papillomas vs. WHO grade II atypical papillomas vs. WHO grade III carcinomas?
WHO grade I CPPs are characterized by papillary formation and lack of mitosis. WHO grade II atypical papillomas resemble WHO grade I papillomas but have more mitoses (≥2 mitoses per high-power field). WHO grade III CPCs are characterized by nuclear atypia, pleomorphism, frequent mitoses, and invasion of brain parenchyma. (WHO Classification of Tumors of the CNS, 4th ed. 2016)
With what syndrome is CPC associated?
Li–Fraumeni, d/t p53 mutation (Tabori U et al., JCO 2010)
What proportion of children present with metastatic Dz at Dx?
One-third of children present with metastatic Dz, all typically with CPC.
What is the most common age of presentation for these tumors?
70% of pts are <2 yo.
What % of CPCs can have CSF seeding? How about CPPs?
Up to 40% of CPCs have CSF seeding; seeding is very rare for CPPs.
What are the 2 most important prognostic/predictive factors for CP tumors?
Histologic grade and extent of resection
How does age affect prognosis?
Pts >40 yrs have poorer prognosis, f/b children <10 yrs. Those in the 10–40-yo group fare the best. Sex is not a prognostic factor.
