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 choroid plexus papilloma pathology outlines 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.

Choroid Plexus Papilloma Causes

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. 

Causes of Choroid Plexus Papilloma:

Risk of Recurrence

While CPP is usually treated effectively through surgical excision, the likelihood of choroid plexus papilloma recurrence is contingent on several factors, including whether the tumor was completely resected during surgery or the tumor’s aggressive properties. Regular follow up imaging is vital to catch any regrowth early on.

The real key to avoiding having the disease return is knowing the root causes of what drives inflammatory-wound-healing biology and then monitoring the patient closely. In case of recurrence, further one may require surgery, radiation therapy, or other treatment options. Accurate and early diagnosis along with multimodality management is important for optimal management of CPP with better patient outcomes.

Choroid Plexus Papilloma Symptoms 

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

Common Symptoms of Choroid Plexus Papilloma in Adults

Diagnosis of Choroid Plexus Papilloma

Radiological Assessment

Choroid plexus papilloma radiology plays a pivotal role in diagnosing choroid plexus papilloma. Magnetic Resonance Imaging (Choroid plexus papilloma MRI) is the preferred imaging modality, offering detailed visualization of the tumor'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. Choroid plexus papilloma CT can also be utilized, particularly when MRI is contraindicated, revealing calcifications within the tumor 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.

Choroid Plexus Papilloma Treatment

The main goal of Choroid Plexus Papilloma treatment is to remove the tumor completely, thereby resolving symptoms and preventing complications like hydrocephalus, which is often caused by excessive cerebrospinal fluid (CSF) production.

Surgical Resection

The primary treatment for choroid plexus papilloma is choroid plexus papilloma surgery​. Complete removal of the tumor often results in resolution of symptoms and hydrocephalus, given the tumor's benign nature. The 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.

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Complications of Choroid Plexus Papilloma

Choroid plexus papilloma (CPP) is a rare benign tumor, but if left untreated or undiagnosed, it can lead to various complications. These arise mainly due to the tumor's effect on cerebrospinal fluid (CSF) production and the pressure it exerts within the skull.

Common Complications of Choroid Plexus Papilloma

Managing Complications

Choroid plexus papilloma surgery plays a critical role in the timely diagnosis and intervention to minimize the risk of these complications. After surgery, care is directed towards maintaining the CSF balance, preventing infection, and monitoring for any recurrence. A full recovery is expected with early treatment and careful monitoring.