Choroid plexus carcinoma

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Choroid plexus carcinoma
Synonyms	Choroid plexus cancer
Pronounce	N/A
Specialty	N/A
Symptoms	Headache, nausea, vomiting, seizures, hydrocephalus
Complications	Metastasis, neurological deficits
Onset	Most common in children
Duration	Variable
Types
Causes	Unknown, possibly genetic mutations
Risks	Li-Fraumeni syndrome, Aicardi syndrome
Diagnosis	MRI, CT scan, biopsy
Differential diagnosis	Choroid plexus papilloma, medulloblastoma, ependymoma
Prevention	None known
Treatment	Surgery, chemotherapy, radiation therapy
Medication
Prognosis	Variable, generally poor
Frequency	Rare
Deaths

Choroid Plexus Carcinoma is a rare, malignant tumor that arises from the choroid plexus in the ventricular system of the brain. It is classified as a central nervous system tumor and is most commonly found in children, although it can occur in adults.

Anatomy and Function of the Choroid Plexus

The choroid plexus is a network of cells that produces cerebrospinal fluid (CSF) in the ventricles of the brain. It plays a crucial role in maintaining the homeostasis of the central nervous system by regulating the composition of the CSF, which cushions the brain and spinal cord, removes waste, and provides nutrients.

Pathophysiology

Choroid Plexus Carcinoma is characterized by the uncontrolled proliferation of epithelial cells of the choroid plexus. This leads to the formation of a mass that can obstruct the flow of CSF, causing hydrocephalus and increased intracranial pressure. The tumor is highly aggressive and can metastasize to other parts of the central nervous system.

Clinical Presentation

Patients with Choroid Plexus Carcinoma may present with symptoms of increased intracranial pressure, such as headache, nausea, vomiting, and papilledema. In infants, signs may include macrocephaly and irritability. Neurological deficits may occur depending on the location and size of the tumor.

Diagnosis

Diagnosis of Choroid Plexus Carcinoma typically involves neuroimaging techniques such as MRI and CT scan to visualize the tumor. A definitive diagnosis is made through histopathological examination of a biopsy sample, which reveals atypical epithelial cells with high mitotic activity.

Treatment

The primary treatment for Choroid Plexus Carcinoma is surgical resection. Complete removal of the tumor is the goal, but it may be challenging due to the tumor's location and potential involvement with critical brain structures. Adjuvant therapy such as radiation therapy and chemotherapy may be employed to manage residual disease and reduce the risk of recurrence.

Prognosis

The prognosis for patients with Choroid Plexus Carcinoma varies depending on factors such as the extent of surgical resection, age at diagnosis, and response to adjuvant therapy. Overall, the prognosis is poor due to the aggressive nature of the tumor and its tendency to recur.

See also

• Choroid plexus
• Central nervous system tumor
• Hydrocephalus
• Neuroimaging