Late recurrence of choroid plexus carcinoma
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Shannon Hart1, Roger Avery2,3, Jane Barron2,4
1Faculty of Medicine, Memorial University of Newfoundland, St. John's, Newfoundland, Canada. sh8062@mun.ca.
Insights
This study highlights a rare case of delayed choroid plexus carcinoma (CPC) recurrence 10 years after initial treatment. Long-term surveillance is crucial for detecting late relapses in pediatric brain tumor patients.
Area of Science:
- Neuro-oncology
- Pediatric oncology
- Rare tumor research
Background:
- Choroid plexus carcinomas (CPC) are rare, aggressive brain tumors primarily affecting children under two years old.
- Common symptoms include headaches, vision problems, and signs of increased intracranial pressure.
- Diagnosis relies on radiological and histological evaluation.
Observation:
- A 2-month-old male diagnosed with CPC underwent gross total resection (GTR), chemotherapy, and stem cell transplant.
- The patient later presented with a recurrence of CPC 10 years post-initial diagnosis and treatment.
Findings:
- Gross total resection (GTR) offers the best survival but prognosis remains poor for CPC.
- Chemotherapy and radiation efficacy in CPC management are debated, with no standard regimen established.
- While early recurrence is common, delayed recurrence is exceptionally rare.
Implications:
- This case underscores the necessity of long-term patient surveillance for choroid plexus carcinomas.
- It prompts further investigation into the natural history and risk factors for late CPC recurrence.
- Research should focus on identifying factors predicting late relapse and the role of adjuvant therapies.
Background:
Choroid plexus carcinomas (CPC) are rare malignant brain tumours arising from the choroid plexus epithelium. CPC are most common in the paediatric population, particularly those under 2 years of age. Common presentations include headache, diplopia and signs of increased intracranial pressure such as nausea and vomiting. Infants may present with increased head circumference, bulging fontanelles, splayed cranial sutures and/or neurological delay. Diagnosis is made via radiological and histological analysis.
Management And Prognosis:
Gross total resection (GTR) is the preferred treatment and infers the best survival rate, but despite this, prognosis remains poor. The utility of chemotherapy and/or radiation in CPC management remains controversial, and an optimal treatment regimen has not been identified. Even with GTR, recurrence is common and usually occurs within months after resection. Delayed recurrence is exquisitely rare and has been reported very few times to date.
Case Presentation:
Here, we present a rare case of delayed CPC recurrence 10 years after initial presentation. A 2-month-old male was diagnosed with CPC and received GTR, chemotherapy and stem cell transplant. The patient presented with a recurrent CPC 10 years after the initial diagnosis.
Conclusions:
This case demonstrates the importance of long-term surveillance and raises questions regarding the natural history, recurrence patterns and factors contributing to long-term relapse in CPC. Further research should be targeted at identifying patient factors contributing to increased risk of late recurrence and whether adjuvant treatments play any role in decreasing this.
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