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A French retrospective study on clinical outcome in 102 choroid plexus tumors in children
- Source :
- Journal of neuro-oncology. 135(1)
- Publication Year :
- 2017
-
Abstract
- The aim of this study was to review and describe therapeutic approaches in children with choroid plexus tumor (CPT) based on a nationwide series. The World Health Organization classification subdivides these rare tumors into three histological subtypes corresponding to three grades of malignancy: low grade (grade I) choroid plexus papilloma (CPP), intermediate grade (grade II) atypical choroid plexus papilloma (aCPP) and high grade (grade III) choroid plexus carcinoma (CPC). This retrospective study included 102 French children younger than 18 years, treated from 2000 to 2012: 54 CPP, 26 aCPP and 22 CPC. The 5 year overall survival was 100% in CPP, 96.2% in aCPP and 64.7% in CPC. In patients with localized disease, complete surgical resection was achieved in 48/52 CPP, 20/26 aCPP and 7/14 CPC. In this group, patients with complete surgical resection had better event free survival than patients with partial resection (88.9 vs. 41.6%). 28 patients (1 CPP, 6 aCPP and 22 CPC) had adjuvant chemotherapy. 2 aCPP and 9 CPC had radiotherapy. We underlined the need for a central histological review to accurately analyze clinical data; we reported a much higher overall survival for CPC than in most previous CPT series probably including atypical teratoid rhabdoid tumors. In our series, the 5 years overall survival in CPC (64.7%) was higher than event free survival (25.2%) and could be interpreted as a clue for the efficiency of adjuvant/salvage therapy even if the heterogeneity of applied treatments in this retrospective series does not allow for meaningful statistical comparisons.
- Subjects :
- Male
Cancer Research
medicine.medical_specialty
Choroid Plexus Neoplasms
Adolescent
Salvage therapy
Malignancy
Polymorphism, Single Nucleotide
03 medical and health sciences
0302 clinical medicine
medicine
Humans
Intermediate Grade
Choroid plexus tumor
Child
Rhabdoid Tumor
Retrospective Studies
business.industry
Carcinoma
Teratoma
Infant
Choroid plexus carcinoma
medicine.disease
Choroid plexus papilloma
Survival Analysis
Surgery
Treatment Outcome
Neurology
Oncology
030220 oncology & carcinogenesis
Child, Preschool
Atypical teratoid rhabdoid tumor
Choroid plexus
Female
Papilloma, Choroid Plexus
Neurology (clinical)
Radiology
France
Neoplasm Grading
business
030217 neurology & neurosurgery
Follow-Up Studies
Subjects
Details
- ISSN :
- 15737373
- Volume :
- 135
- Issue :
- 1
- Database :
- OpenAIRE
- Journal :
- Journal of neuro-oncology
- Accession number :
- edsair.doi.dedup.....0f78142ce6f8b6efc731466d45317327