Late recurrence of choroid plexus carcinoma
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CASE-BASED REVIEW
Late recurrence of choroid plexus carcinoma Shannon Hart 1
&
Roger Avery 1,2 & Jane Barron 1,3
Received: 15 April 2020 / Accepted: 5 May 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract 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 Neurosurgery . Cancer . Brain . Tumour . Relapse . Choroid plexus . Long-term follow-up
Introduction Choroid plexus tumours (CPTs) are rare tumours arising from the choroid plexus epithelium. They comprise 0.3–0.6% of all intracranial tumours and have an annual incidence of 0.3 per 1 million [1]. CPTs are most common in the paediatric population, with over 70% of cases occurring in children less than 2 years of age [2]. CPTs are classified into benign choroid plexus papillomas (CPP; WHO grade I), atypical choroid plexus papillomas (ACPP; WHO grade II) and malignant
* Shannon Hart [email protected] 1
Faculty of Medicine, Memorial University of Newfoundland, St. John’s, Newfoundland, Canada
2
Department of Neurosurgery, Memorial University of Newfoundland, St. John’s, Newfoundland, Canada
3
Department of Pathology, Memorial University of Newfoundland, St. John’s, Newfoundland, Canada
choroid plexus carcinomas (CPC; WHO grade III). While CPP and ACPP do have the potential to undergo malignant transformation, it is CPC that is of most concern due to the characteristic aggressive behaviour and resultant poor prognosis. Despite major medical
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