Choroid Plexus Papilloma: Symptoms and Treatment

Choroid Plexus Papilloma (CPP) is a rare, benign tumour arising from the choroid plexus, a network of cells responsible for cerebrospinal fluid production within the brain's ventricular system. Although rare, this condition primarily affects children, representing a significant portion of pediatric brain tumours. 

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What is Choroid Plexus Papilloma?

Choroid Plexus Papilloma is a slow-growing neoplasm classified as a benign tumour by the World Health Organization (WHO). It typically occurs in the lateral ventricles in children and the fourth ventricle in adults. Despite its benign nature, CPP can cause significant clinical symptoms due to its location and the resultant obstruction of cerebrospinal fluid (CSF) flow, leading to increased intracranial pressure.

Choroid Plexus Papilloma Pathology

Histologically, CPPs are characterized by papillary structures lined by a single layer of uniform epithelial cells. The pathology outlines of choroid plexus papilloma reveal fibrovascular cores surrounded by neoplastic epithelial cells, which resemble the normal choroid plexus epithelium. Mitotic activity is generally low, reflecting its benign nature; however, atypical forms exist, which may exhibit more aggressive behaviour.

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Symptoms of Choroid Plexus Papilloma

The clinical presentation of choroid plexus papilloma varies based on tumour size, location, and the degree of CSF obstruction. Common symptoms include:

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Diagnosis of Choroid Plexus Papilloma

Radiological Assessment

Radiology plays a pivotal role in diagnosing choroid plexus papilloma. Magnetic Resonance Imaging (MRI) is the preferred imaging modality, offering detailed visualization of the tumour's size, location, and effect on surrounding structures. CPPs typically appear as well-circumscribed, lobulated masses with vivid enhancement post-contrast administration on MRI scans. Computed Tomography (CT) can also be utilized, particularly when MRI is contraindicated, revealing calcifications within the tumour in some cases.

Differential Diagnosis

Differential diagnosis for CPP includes other intraventricular tumors such as ependymomas, medulloblastomas, and central neurocytomas. Distinguishing between these entities is crucial, as treatment and prognosis differ significantly.

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Causes and Risk Factors

The exact aetiology of choroid plexus papilloma remains unclear. However, genetic predisposition may play a role, with associations noted in certain hereditary cancer syndromes. Further research is ongoing to elucidate the molecular pathways involved in CPP pathogenesis.

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Treatment Options for Choroid Plexus Papilloma

Surgical Resection

The primary treatment for choroid plexus papilloma is surgical resection. Complete removal of the tumor often results in resolution of symptoms and hydrocephalus, given the tumor's benign nature. Surgical approach varies based on tumor location, with considerations for minimizing neurological damage while achieving total resection.

Adjuvant Therapies

In cases where complete surgical resection is not feasible or in atypical CPPs with aggressive features, adjuvant therapies such as radiation therapy may be considered. Chemotherapy is rarely used, given the tumour's low-grade nature.

Postoperative Care and Monitoring

Postoperative care includes monitoring for complications such as infection, haemorrhage, and persistent hydrocephalus. Long-term follow-up with regular imaging is essential to ensure no recurrence, particularly in cases of subtotal resection.

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Prognosis of Choroid Plexus Papilloma

The prognosis for patients with choroid plexus papilloma is generally favourable, especially with complete surgical resection. The five-year survival rate is high, exceeding 90% in most studies. Atypical CPPs may have a less favourable prognosis, necessitating closer monitoring and potential adjuvant therapy.