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.