Late recurrence of choroid plexus carcinoma

Childs Nerv Syst. 2020 Aug;36(8):1601-1606. doi: 10.1007/s00381-020-04663-x. Epub 2020 May 15.


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.

Keywords: Brain; Cancer; Choroid plexus; Long-term follow-up; Neurosurgery; Relapse; Tumour.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Carcinoma*
  • Choroid Plexus
  • Choroid Plexus Neoplasms* / therapy
  • Humans
  • Infant
  • Male
  • Neoplasm Recurrence, Local / therapy
  • Prognosis

Supplementary concepts

  • Choroid Plexus Carcinoma