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[Choroid plexus carcinoma: report of 15 cases]
L F Bleggi-Torres1, L A Urban, A Antoniuk
1Seção de Microscopia Eletrônica e Neuropatologia, Serviço de Anatomia Patológica, Hospital de Clínicas, Universidade Federal do Paraná.
Insights
Choroid plexus carcinoma (CPC), a rare brain tumor in young children, has a poor prognosis. This study highlights the high recurrence and mortality rates associated with CPC, emphasizing the need for improved treatments.
Area of Science:
- Neuro-oncology
- Pediatric oncology
- Central nervous system (CNS) tumors
Context:
- Choroid plexus carcinoma (CPC) are rare CNS tumors originating from the choroid plexus epithelium.
- These tumors predominantly affect children under 3 years old.
- Understanding the clinical, epidemiological, and histopathological characteristics of CPC is crucial for improving patient outcomes.
Purpose:
- To present a clinical, epidemiological, and histopathological analysis of 15 cases of choroid plexus carcinoma.
- To evaluate treatment modalities and their efficacy in managing CPC.
- To assess the prognosis and survival rates associated with CPC.
Summary:
- This study analyzed 15 CPC cases, with a male predominance and a mean age of 3.4 years.
- Lateral ventricles were the most common site (73.3%), with hydrocephalus and intracranial hypertension as primary symptoms.
- Surgery, often partial resection (75%), was the main treatment, but high recurrence rates (85.7%) and a low 5-year survival rate (one patient) indicate a poor prognosis.
Impact:
- The findings underscore the aggressive nature of CPC and its associated high mortality rate.
- This research highlights the urgent need for novel therapeutic strategies to improve survival in pediatric CPC patients.
- The study provides valuable data for future research and clinical management guidelines for this rare CNS malignancy.
Abstract:
Choroid plexus carcinoma (CPC) are rare central nervous system tumours derived from choroid plexus epithelium, affecting mainly children under 3 years of age. We present a clinical, epidemiological and histopathological study of 15 cases of CPC. Ten patients were male. Age ranged from 4 months to 21 years (mean=3,4). The lateral ventricles were affected in 73,3% of cases. Main symptom were: hydrocephalus (62.5%), intracranial hypertension (25%) and convulsion (12.5%). The patients were treated by surgery with partial resection in 75% of cases and total resection in 25%. There was one death due to surgical complication, 85.7% of patients had recurrence of tumours with mean survival rate of 13.6% months after diagnosis. Only one patient remain alive 5 years after initial treatment. These results support the poor prognosis and high mortality rate of CPC.