Choroid plexus papilloma

Introduction

Choroid plexus papilloma is a rare, usually benign brain tumor arising from the choroid plexus tissue that produces cerebrospinal fluid (CSF). Even though it’s medically “benign,” it can cause significant health issues like hydrocephalus (fluid build-up), headaches, nausea, or developmental delays in kids. You might stumble on the term “CPP” in case reports or radiology notes. In this article, we’ll cover symptoms, causes, diagnosis, treatments, and the outlook—so you get a clear, human-friendly snapshot of living with choroid plexus papilloma.

Definition and Classification

A choroid plexus papilloma is a rare, typically benign tumor (World Health Organization grade I) that originates from the epithelial cells of the choroid plexus—those specialized structures lining brain ventricles that make CSF. Clinically, CPPs are classified as:

• Benign (grade I) papilloma
• Atypical papilloma (grade II)—showing mild atypia and slightly higher recurrence risk
• Choroid plexus carcinoma (grade III)—malignant, invasive, and far less common

CPPs predominantly affect the lateral ventricles in children, while in adults they often appear in the fourth ventricle. They’re non-invasive, slow-growing tumors, though mass effect and excess CSF production can lead to major symptoms if left untreated.

Causes and Risk Factors

The exact trigger for choroid plexus papilloma is still under investigation, but several risk factors have been identified:

• Genetic predispositions: Some cases link to Li-Fraumeni syndrome, which involves TP53 mutations, or other hereditary cancer syndromes.
• Pediatric vulnerability: About 50% of CPPs occur in children under two years old, suggesting developmental factors at play.
• Radiation exposure: Historical cranial irradiation for unrelated conditions appears to carry a slight long-term risk—though this is very uncommon now with modern protocols.
• Unknown environmental factors: There's no clear link to toxins, diet, or lifestyle, so environmental causes remain speculative.

Modifiable vs Non-modifiable risks:

• Non-modifiable: age (infants and young children), genetics (Li-Fraumeni)
• Modifiable: radiation exposure (minimized with modern imaging guidelines)

In many cases, the cause is idiopathic—doctors can’t pinpoint why a particular person develops a CPP. Ongoing research aims to uncover molecular pathways involved in epithelial cell dysregulation.

Pathophysiology (Mechanisms of Disease)

Normally, the choroid plexus lines the brain’s ventricles and secretes cerebrospinal fluid, cushioning and nourishing neural tissue. In CPP, epithelial cells proliferate abnormally, forming a frond-like tumor that can:

• Overproduce CSF, contributing to hydrocephalus
• Occupy ventricular space, causing obstructive hydrocephalus by blocking CSF flow
• Apply pressure on adjacent brain structures, leading to focal neurologic deficits

On a molecular level, alterations in tumor suppressor genes (like TP53) and growth factor pathways (e.g., FGFR1 amplification) have been implicated. As the lesion expands, intracranial pressure (ICP) rises, triggering headaches, vomiting, or visual disturbances. In infants, cranial sutures haven’t fused, so you often see head enlargement rather than headache. The tumor’s rich vascular network also raises the risk of hemorrhage into ventricle spaces.

Symptoms and Clinical Presentation

Symptoms vary by age, tumor size, and location. Here’s a rough breakdown:

• Infants and toddlers:
  • Macrocephaly (enlarged head circumference)
  • Poor feeding, irritability, developmental delays
  • Sunsetting eyes (downward gaze)
• Children and adolescents:
  • Persistent or severe headaches
  • Nausea, vomiting—especially in the morning
  • Gait disturbances or balance issues (if in fourth ventricle)
  • Visual problems (papilledema from raised ICP)
• Adults:
  • Headache and cognitive slowing
  • Memory problems, personality changes
  • Possible endocrine deficits if tumor impinges on hypothalamic regions

Warning signs requiring urgent care include sudden severe headache (“thunderclap”), seizures, rapid decline in consciousness, or acute hydrocephalus (vomiting, bradycardia, hypertension). Not everyone experiences all symptoms—some tumors are found incidentally on imaging after minor head trauma. Don’t use this as a self-diagnosis list, but if you have concerning signs, reach out to a medical provider promptly.

Diagnosis and Medical Evaluation

Diagnosing choroid plexus papilloma involves:

• Neurological exam: Basic tests of cognition, reflexes, and gait
• Imaging:
  • MRI—the gold standard; shows a vividly enhancing intraventricular mass with frond-like projections
  • CT scan—helpful if MRI contraindicated; reveals hyperdense lesion, often with calcifications
• CSF analysis: Rarely positive for tumor cells but can show high protein from overproduction
• Histopathology: Definitive diagnosis via surgical biopsy or resection; pathologist identifies typical papillary structures lined by a single layer of epithelial cells

Differential diagnoses include ependymoma, meningioma (intraventricular subtype), metastatic lesions, and subependymal giant cell astrocytoma (SEGA) in tuberous sclerosis. A multidisciplinary tumor board often reviews complex cases to guide management.

Which Doctor Should You See for Choroid Plexus Papilloma?

If you suspect a choroid plexus papilloma or get imaging suspicious for an intraventricular lesion, the first step is usually a neurologist or pediatric neurologist for kids. From there, you’ll be referred to a neurosurgeon experienced in brain tumor resection. In some centers a neuro-oncologist also guides adjuvant treatment decisions.

Online consultations can help you interpret MRI reports, ask follow-up questions, or get a second opinion before traveling to a specialized center. Telemedicine is great for discussing symptom progression or clarifying post-op concerns—but remember it can’t replace a hands-on neurologic exam or urgent in-person emergency care when symptoms like seizures or sudden vomiting appear.

Treatment Options and Management

The cornerstone of managing choroid plexus papilloma is surgical removal. Gross total resection often cures CPP (grade I). Key points:

• First-line: Microsurgical resection via craniotomy; complete excision usually resolves hydrocephalus too
• Adjuvant therapies: Rarely needed for benign CPP; atypical (grade II) may warrant focal radiation
• Hydrocephalus management: Temporary external ventricular drain (EVD) or ventriculoperitoneal shunt if CSF doesn’t normalize post-op
• Supportive care: Physical therapy for balance issues, occupational therapy for developmental catch-up in kids

Limitations/side effects: Surgery carries risks—bleeding, infection, transient neurologic deficits. Shunts can malfunction or become infected. Radiation is reserved for atypical/malignant cases to avoid long-term sequelae in young brains.

Prognosis and Possible Complications

Overall prognosis for CPP (grade I) is excellent with complete resection: 5- and 10-year survival rates exceed 90%. Factors influencing outlook:

• Tumor grade: Benign (I) vs atypical (II) vs carcinoma (III)
• Extent of resection: Gross total resection yields lowest recurrence risk
• Patient age: Younger age sometimes complicates anesthesia, neurosurgery
• Preoperative hydrocephalus: Long-standing raised ICP may leave persistent neurocognitive issues

Potential complications:

• Recurrence of tumor (rare with complete removal)
• Shunt-dependence or shunt infection
• Transient or permanent neurological deficits (e.g., weakness, coordination problems)

Prevention and Risk Reduction

Since most CPPs are idiopathic, primary prevention isn’t well-defined. However, you can reduce secondary risks:

• Avoid unnecessary cranial radiation—modern imaging minimization helps
• Genetic counseling if there’s family history of Li-Fraumeni or similar cancer syndromes
• Regular pediatric check-ups for head circumference tracking in infants
• Early imaging evaluation of persistent headaches, vomiting, or developmental delay

Screening: No routine population screening for CPP. In patients with known germline TP53 mutations, some centers consider periodic MRI surveillance—though this remains controversial. The best strategy is awareness of symptoms and timely medical evaluation.

Myths and Realities

There are plenty of myths floating around choroid plexus papilloma—let’s clear them up:

• Myth: “Benign tumor = no treatment needed.” Reality: Even benign CPPs can cause hydrocephalus and require surgery.
• Myth: “Radiation cures CPP.” Reality: Surgery is mainstay; radiation reserved for atypical/malignant cases only.
• Myth: “All brain tumors originate from systemic cancer.” Reality: CPP is a primary brain tumor, not a metastasis.
• Myth: “Once removed, it never comes back.” Reality: Rare recurrences occur, especially in incomplete resections.
• Myth: “CPP only affects kids.” Reality: While common in infants, adults can develop fourth-ventricle papillomas too.

Popular media often lump CPP with more aggressive tumors—this oversimplifies prognosis and misleads families about treatment urgency and outcomes.

Conclusion

Choroid plexus papilloma is a rare but generally benign brain tumor that can cause significant symptoms through CSF overproduction and hydrocephalus. Early recognition—especially in infants with macrocephaly or older patients with persistent headaches—paired with high-quality neuroimaging leads to accurate diagnosis. Surgical removal is typically curative for grade I tumors, with excellent long-term outlook. Always partner with a skilled neurosurgeon and neuro-oncologist or neurologist. If you suspect concerning signs, don’t hesitate—timely evaluation and expert care are key.

Frequently Asked Questions (FAQ)

• 1. What is choroid plexus papilloma?
  A rare benign tumor of CSF-producing cells in brain ventricles, often causing hydrocephalus.
• 2. Who gets CPP?
  Mostly infants under two and occasionally adults; some genetic risk (e.g., Li-Fraumeni).
• 3. What are common symptoms?
  Headaches, vomiting, balance issues, enlarged head in infants.
• 4. How is CPP diagnosed?
  Neurological exam plus MRI; definitive via surgical biopsy/pathology.
• 5. Which doctor treats it?
  Neurologist/pediatric neurologist for evaluation, neurosurgeon for surgery, sometimes neuro-oncologist.
• 6. Can CPP cure itself?
  No, it requires surgical removal; watchful waiting isn’t advised once symptomatic.
• 7. Is radiation needed?
  Rarely for benign CPP; considered for atypical or malignant variants.
• 8. What’s the recovery like?
  Most recover well post-op, though some need shunts or rehab for balance.
• 9. Can it recur?
  Yes, especially if resection was incomplete; follow-up MRI recommended.
• 10. Are there non-surgical options?
  Not primary; surgery is first-line. Shunts manage hydrocephalus if surgery delayed.
• 11. How urgent is treatment?
  Promptly—untreated hydrocephalus can cause permanent brain damage.
• 12. What complications to watch for?
  Shunt malfunction, bleeding, infection, transient neurological deficits.
• 13. Is it hereditary?
  Usually sporadic; small subset linked to hereditary cancer syndromes.
• 14. How to reduce risk?
  Minimize unnecessary head radiation; monitor head growth in infants.
• 15. When to seek help?
  Persistent headaches, vomiting, vision changes, head enlargement—see a neurologist ASAP.