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Choroid plexus papilloma (CPP) ia a non-cancerous (benign) tumor of the choroid plexus, a network of blood vessels in the brain which surrounds the ventricles and produces the fluid surrounding the brain and spinal cord (cerebrospinal fluid, or CSF). CPPs most commonly occur in children but may occur in adults. Symptoms are generally due to increased secretion of CSF by tumor cells, causing hydrocephalus and subsequent intracranial pressure. Affected individuals may experience headaches, nausea and vomiting, drowsiness, ocular or gaze palsies, optic nerve swelling (papilledema), visual disturbances, and possible blindness. Infants, especially those with a tumor in the third ventricle, can present with hydrocephalus or macrocephaly. Some cases of CPP may result from a mutation in the TP53 gene. Complete tumor removal often cures the condition and may also relieve the hydrocephalus.
Source: GARD Last updated on 05-01-20
Hydrocephalus is characterized by excessive accumulation of fluid (cerebrospinal fluid, or CSF) in the brain. Accumulation results in the abnormal widening (dilation) of spaces in the brain called ventricles. This can create harmful pressure on the tissues of the brain. The brain can accommodate ventricular dilatation to a certain extent without significant neuronal damage, but as the process continues, irreversible brain damage can occur.
The effects of hydrocephalus can be difficult to predict and partially depend on the severity, timeliness of the diagnosis and the success of treatment. Early diagnosis and treatment improves the chance of a good recovery, but the degree to which relief of CSF pressure after treatment can minimize or reverse damage is not well understood. Hydrocephalus causes risks to cognitive and physical development, but many affected individuals benefit from rehabilitation and educational intervention, leading lives with few limitations. If hydrocephalus is left untreated, it may be fatal.
In general, there is a greater chance for an abnormal outcome with fetal hydrocephalus (present in utero) compared to neonatal hydrocephalus (beginning shortly after birth) because of the period it is left untreated in utero. Prenatal factors such as progression, degree of cortical mantle thinning to less than 1.5 cm, and in-utero duration of greater than four weeks are associated with a poorer prognosis.
Last updated on 05-01-20
The prognosis for choroid plexus papilloma (CPP) is determined mainly by how completely the lesion is removed during surgery. Complete removal of intraventricular CPPs almost always cures the condition and it has been estimated that the survival rate at 5 years for CPPs after removal is approximately 100%. Examples of rare complications have been described and generally involve neurologic deficits from the surgical procedure.
Although there may not be residual effects of the CPP specifically, the extent and duration of the presence of hydrocephaly can affect cognitive and/or physical development in affected individuals. Hydrocephalus may continue after surgery in some cases and is then often managed by CSF shunting. Complications that result in neurological or psychological problems may influence overall outcome.
Last updated on 05-01-20
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