Choroid plexus papillomas are extremely rare vascular tumors that are often located in the ventricular areas of
infants and children of both sexes. Although the lateral ventricles are the most common location, these tumors can present in the third or fourth ventricles, especially in adults. Although some believe the childhood predilection suggests that these tumors are congenitally derived, no risk factors are known.
Patients commonly present with symptoms of increased intracranial pressure. In infants and small children these manifestations can include changes in behavior and decreased feeding. Seizures are not uncommon, and hemorrhages are known to occur. The main differential factor is that of malignant degeneration to a choroid carcinoma or an ependymoma. Choroid plexus carcinomas are more common in children, whereas in adults the differentiation of a choroid plexus carcinoma from a metastatic carcinoma may be difficulty Occasionally, these tumors can be confused with colloid cysts, although the latter lesions have much different imaging characteristics as discussed previously.
Evaluation usually begins with CT or MRI imaging. CT imaging reveals hydrocephalus with a brightly enhancing rounded mass located within or near the ventricular system. MRI imaging usually establishes the intraventricular location and may identify leptomeningeal seeding of malignant forms. Calcifications may be present. These tumors can be extremely vascular, necessitating angiography prior to surgery.
Shunting may be required prior to surgery to allow time for thorough surgical preparation. Since papillomas are usually well-demarcated lobules that shell out easily, these tumors can be cured with surgical resection. Those that are more adherent suggest a more malignant type. Choroid plexus papillomas require no further treatment beyond serial neuroradiographic studies to survey for early asymptomatic regrowth. In patients with choroid plexus carcinomas, further treatment is important. Patients require evaluation of the neuroaxis of any evidence of seeding by craniospinal MRI imaging and CSF evaluation. Since radiotherapy is not favored in young children, chemotherapy is the mainstay of treatment for choroid plexus carcinomas that are not completely resected, those that show evidence of regrowth, and those that have malignant potential. No large series exist in regard to the choice of optimal chemotherapeutic agents, although carboplatin and etoposide are commonly used. Some advocate presurgical chemotherapy in patients who require reoperation.
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