Overview
Choroid plexus neoplasms are rare intracranial tumors, comprising less than 1% of all brain tumors, with papillomas being more common than carcinomas. These neoplasms can occur in both pediatric and adult populations and are associated with local recurrences and potential extracranial metastases in more aggressive forms 1.Diagnosis
Clinical Presentation: Often asymptomatic initially, but may present with signs of increased intracranial pressure or focal neurological deficits 1.
Imaging: MRI is essential for diagnosis, showing characteristic enhancement patterns in the ventricular system 1.
Biopsy and Histopathology: Required for definitive diagnosis, distinguishing between papillomas and carcinomas 1.
Immunohistochemistry: Useful for differentiation; positive markers include CKER, keratin, CEA, NSE, and S100 protein 1.
Markers: CEA and S100 positivity often correlate with more aggressive behavior 1.
Metastatic Workup: Consider in suspected carcinomas due to potential for extracranial metastases 1.Management
Surgical Resection: Primary treatment for localized tumors, aiming for complete removal 1.
Radiation Therapy: Adjunctive treatment for residual or recurrent disease, particularly in carcinomas 1.
Chemotherapy: Limited evidence; may be considered in recurrent or metastatic cases, though specific regimens are not detailed in the abstract 1.
Monitoring: Regular imaging follow-ups due to high recurrence rates, especially in carcinomas 1.Special Populations
Pediatrics: More frequent occurrence in children; management focuses on minimizing neurocognitive impact 1.
Adults: Higher risk of aggressive behavior and extracranial metastases compared to pediatric cases 1.Key Recommendations
Definitive diagnosis requires histopathological examination and immunohistochemistry, particularly assessing markers like CKER, CEA, and S100 protein (Evidence: Moderate 1).
Primary treatment should involve surgical resection whenever feasible to achieve local control (Evidence: Expert opinion 1).
Regular imaging follow-ups are crucial due to the high recurrence rates, especially in cases of choroid plexus carcinomas (Evidence: Moderate 1).References
1 Coffin CM, Wick MR, Braun JT, Dehner LP. Choroid plexus neoplasms. Clinicopathologic and immunohistochemical studies. The American journal of surgical pathology 1986. link