Atypical papilloma of choroid plexus

Atypical papilloma of the choroid plexus (also known as atypical choroid plexus papilloma) is a rare brain tumor classified as WHO grade II, representing an intermediate form between the benign choroid plexus papilloma (WHO grade I) and the malignant choroid plexus carcinoma (WHO grade III). The choroid plexus is a network of cells located within the ventricles of the brain that produces cerebrospinal fluid (CSF). This tumor arises from the epithelial cells of the choroid plexus and is characterized by increased mitotic activity compared to typical papillomas, which confers a higher risk of recurrence, though it generally carries a more favorable prognosis than choroid plexus carcinoma. The tumor primarily affects the central nervous system. Because it grows within the brain's ventricular system, it can obstruct the normal flow of cerebrospinal fluid, leading to hydrocephalus (abnormal accumulation of fluid in the brain). Key symptoms often include signs of increased intracranial pressure such as headaches, nausea, vomiting, visual disturbances, and in young children, rapid head enlargement and bulging fontanelles. Seizures and developmental delays may also occur. Atypical choroid plexus papillomas are most commonly diagnosed in young children, particularly in the first few years of life, though they can occur at any age. In children, the tumor tends to arise in the lateral ventricles, while in adults it more frequently occurs in the fourth ventricle. The primary treatment for atypical choroid plexus papilloma is surgical resection, with gross total removal being the most important prognostic factor. When complete surgical excision is achieved, outcomes are generally favorable. In cases of incomplete resection or tumor recurrence, adjuvant therapies such as radiation therapy or chemotherapy may be considered, though their roles remain under investigation. Long-term follow-up with neuroimaging is recommended due to the increased recurrence risk associated with the atypical histological features. Management of associated hydrocephalus, which may require CSF diversion procedures such as ventriculoperitoneal shunting, is also an important component of care.

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