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Papillomas and carcinomas of the choroid plexus in children
P Pencalet1, C Sainte-Rose, A Lellouch-Tubiana
1Service de Neurochirurgie Pédiatrique, Hôpital Necker-Enfants Malades, Paris, France.
Insights
Complete surgical removal offers a cure for choroid plexus papillomas in children. For choroid plexus carcinomas, surgery combined with adjuvant therapies like chemotherapy and radiation can improve outcomes.
Area of Science:
- Pediatric Neurosurgery
- Pediatric Oncology
- Neuro-oncology
Background:
- Choroid plexus tumors are rare intraventricular neoplasms primarily affecting children, accounting for 1% of intracranial tumors.
- The management of associated hydrocephalus and the optimal oncological strategies for these tumors are subjects of ongoing discussion.
- This study reviews the authors' experience in managing 38 pediatric cases of choroid plexus tumors.
Purpose of the Study:
- To analyze the clinical characteristics, surgical management, and outcomes of pediatric choroid plexus tumors.
- To evaluate the effectiveness of different treatment modalities for choroid plexus papilloma and carcinoma.
- To identify factors influencing survival rates in children diagnosed with these rare brain tumors.
Main Methods:
- Retrospective review of 38 pediatric patients with choroid plexus tumors (25 papillomas, 13 carcinomas).
- Analysis of patient demographics, hydrocephalus presentation, surgical techniques, and adjuvant therapies.
- Evaluation of surgical complications, including hemorrhage and postoperative brain collapse, and their management.
Main Results:
- Hydrocephalus was present in 33 patients, with poor correlation to tumor characteristics.
- Complete tumor excision rates were 96% for papillomas and 61.5% for carcinomas.
- Five-year survival rates were 100% for papillomas and 40% for carcinomas.
Conclusions:
- Total surgical excision is curative for choroid plexus papillomas.
- For choroid plexus carcinomas, surgical excision followed by adjuvant chemotherapy (especially in children under 3) and radiotherapy (in older children) can reduce recurrence risk.
Object:
Choroid plexus tumors are rare intraventricular tumors (1% of all intracranial tumors) that occur mainly in children. The pathophysiological characteristics of associated hydrocephalus, surgical management, and oncological issues related to these tumors remain a matter of debate. To understand more about these tumors, the authors have reviewed their experience with the management of 38 children with choroid plexus tumors.
Methods:
There were 25 cases of papilloma and 13 of carcinoma. The mean age of the patients at presentation was 22.5 months, and one-half of the patients were younger than 2 years of age. Hydrocephalus was present in 33 patients and poorly correlated with the size, site, and pathological characteristics of the tumor. In nine children, a ventriculoperitoneal shunt was required after tumor excision, calling into question the notion that cerebrospinal fluid oversecretion is the only cause of hydrocephalus. Complete excision was achieved in 96% of the cases of papilloma and 61.5% of the cases of carcinoma. These surgical procedures were complicated by the risks of intraoperative hemorrhage, which proved to be fatal in two cases, and postoperative brain collapse, which led to subdural fluid collections requiring subdural shunt placement in six patients. Preoperative embolization was partially successful in four cases and significantly assisted surgery. Preoperative controlled drainage of excessively dilated ventricles and intraoperative gluing of the cortical incision have been used to address the problem of postoperative brain collapse. Patients with carcinomas were treated postoperatively by chemotherapy alone (seven cases), radiotherapy (one case), or chemotherapy plus radiotherapy (one case). The overall 5-year survival rate was 100% for patients with papillomas and 40% for those with carcinomas.
Conclusions:
Total surgical excision is curative in cases of papillomas. For carcinomas, the most effective treatment remains total surgical excision; however, adjuvant treatment in the form of chemotherapy in patients younger than age 3 years, supplemented by radiation therapy in older children, can moderately reduce the risk of recurrence.