Choroid plexus carcinoma
Choroid plexus carcinoma | |
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Photomicrograph of hematoxylin-eosin stained section of a choroid plexus carcinoma (grade III WHO) at 400x magnification | |
Specialty | Neuro-oncology |
A choroid plexus carcinoma (
Signs and symptoms
The symptoms of choroid plexus carcinoma are similar to those of other brain tumors. They include:[4]
- Persistent or new onset headaches
- fontanelsin infants.
- Loss of appetite (refusal to take food in infants)
- Papilledema
- emesis
- Ataxia
- Strabismus
- Developmental delays
- Altered mental status
Cause
The cause of choroid plexus carcinomas are relatively unknown, although hereditary factors are suspected. They sometimes occur in conjunction with other hereditary cancers, including Li–Fraumeni syndrome and malignant rhabdoid tumors. A mutation in the tumor suppressor gene TP53 is usually characterized in this disease.[5]
Pathophysiology
Choroid plexus carcinomas typically occur in the
The tumors most frequently spread through the CSF. As a result, metastases frequently occur along the
Diagnosis
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Treatment
Treatment of choroid plexus carcinoma depends on the location and severity of the tumor. Possible interventions include inserting
Incidence
Choroid plexus tumors have an annual incidence of about 0.3 per 1 million cases.[7] It is seen mainly in children under the age of 5,[4] representing 5% of all pediatric tumors and 20% of tumors in children less than 1 year old.[6] There has been no link between sex and occurrence.[10]
Although choroid plexus carcinomas are significantly more aggressive and have half the survival rate as choroid plexus papillomas, they are outnumbered in incidence by 5:1 in all age groups.[10][11] Clinical studies have shown that patients who receive a total resection of a tumor have an 86% survival rate, while patients who only receive a partial resection have a 26% 5-year survival rate. Many incomplete resections result in recurrence within 2 years of primary surgery.[12]