Choroid Plexus Carcinoma
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Definition
Choroid plexus carcinoma is a papillary, intraventricular tumor derived from choroid plexus epithelium that shows clear signs of malignancy. Choroid plexus papilloma corresponds to WHO Grade III glioma.
Epidemiology
Choroid plexus carcinoma accounts 30-60% of all choroids plexus tumors in children. These lesions are most common among children less than five years of age.
Localization
These tumors arise most frequently in the lateral (50%), 4th (40%) and 3rd (5%) ventricles.
Clinical presentation
Patients present with signs and symptoms of obstructive hydrocephalus.
Imaging
On MRI, choroid plexus papillomas appear as solid, cauliflower-like lesions arising from the ventricular surface. Necrosis and intra-tumoral hemorrhage are common. Evidence of frank brain invasion may be seen.
Pathology
Macroscopy
These lesions present as firm nodules of variable size, arising from the choroids plexus and bulging into the ventricular lumen. Brain invasion is typical.
Histopathology
Choroid plexus carcinoma is a malignant solid tumor. Nuclear pleomorphism, frequent mitoses, high nucleus:cytoplasm ratio, and increased cellularity are characteristic. The papillary pattern underlying the biologic origin of these tumors may be blurred by the poorly structured sheets of tumor cells, frank necrosis and often diffuse areas of brain invasion.
Molecular genetics
Choroid plexus carcinomas may occur in the setting of Li Fraumeni syndrome, and TP53 mutations are common in cases of sporadic choroid plexus carcinoma. Somatic mutations of the hSNF5/INI1 gene, a member of the SWI/SNF adenosine triphosphate-dependent chromatin-remodeling complex located on chromosome 22q11.2, have also been reported in cases of choroid plexus carcinoma.
Prognosis
The 5-year survival for choroid plexus carcinoma following surgical resection and adjuvant therapy is 40%. Meningeal dissemination and system metastasis may occur.
