101. CRAN-06. CHILDREN WITH PINEOBLASTOMA. A SINGLE-INSTITUTION SERIES
- Author
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Iwona Filipek, Paweł Kowalczyk, Elżbieta Jurkiewicz, Wiesława Grajkowska, Monika Drogosiewicz, Bożenna Dembowska-Bagińska, Marta Perek-Polnik, and Ewa Swieszkowska
- Subjects
Pineoblastoma ,Cancer Research ,Series (stratigraphy) ,medicine.medical_specialty ,business.industry ,Chemotherapy regimen ,Abstracts ,Oncology ,Tumor progression ,Medicine ,Neurology (clinical) ,Radiology ,Single institution ,business - Abstract
INTRODUCTION: Pineoblastoma is a rare, highly aggressive supratentorial tumour, more frequently diagnosed in young children. The prognosis remains poor. AIM: To present own experience with children with pineoblastoma. PATIENTS AND METHODS: 14 pts: 8 girls and 6 boys treated between 1998 and 2017 were analyzed. 5 pts (36%) were under 3 years of age. 7 pts underwent complete tumour resection, 2-partial resection and 5- biopsy. At the onset of treatment six patients had disseminated disease. 9 pts were treated according to protocol for MB HR group (with radiotherapy), 5 pts according to own baby protocol. RESULTS: 9 pts (64%) achieved good response to treatment (CCR-4 pts, CR-4 or PR-1). Four patients experienced tumour progression. 7 out of 14 patients are alive 3 ms to 17years from diagnosis (median 3years 4ms). Seven patients died (10ms to 8years from diagnosis). 1 pt died of long term post- radiotherapy sequelae. 5 yr EFS was 23 %, OS- 57%. 10 yr EFS and OS were 23 and 30,1% consecutively. 10 out of 14 pts relapsed 7ms to 4 years 3ms from diagnosis. 9 pts received second line treatment- 4 are alive. Children younger than 3 years of age treated without radiotherapy had worse outcome with 5 yrs OS estimated at 0% vs 74,1% in older children treated with chemotherapy and radiotherapy (p=0,014). CONCLUSION: Pineoblastoma is highly malignant CNS neoplasm of childhood with dismal prognosis. In our series children below 3 years of age treated without radiotherapy had poorer responses and worse outcome.
- Published
- 2018