Year: 2018
Type: conference-abstract
Abstract: Pineoblastoma is a rare malignant pineal region tumor, the optimal management of which in young children remains unclear. We report the outcomes of 23 children with pineoblastoma prospectively enrolled on three sequential Head Start (HS) trials between 1990 and 2009. The HS treatment strategy included maximal surgical resection followed by five cycles of intensive induction chemotherapy and consolidation with marrow-ablative chemotherapy and autologous hematopoietic cell rescue (HDCx/AuHCR). Irradiation following recovery from consolidation was reserved for children over six years old or with residual tumor. Data on 23 children with pineoblastoma including two with trilateral retinoblastoma aged 0.44–5.72 (median 3.12) years were analyzed. Median overall survival (OS) was 12 months (95% CI: 7.6–29.7 months). The 3-year progression-free survival (PFS) and OS were 14.5% (95% CI: 5.1–41.2%) and 17.4% (95% CI: 7.1–42.4%), respectively. Three patients were long-term survivors beyond 5 years. Eight patients experienced progressive disease (PD) during induction chemotherapy, six following the fourth and fifth cycles. Ten patients proceeded to consolidation with HDCx/AuHCR; eight experienced PD post-consolidation. Seven patients received craniospinal irradiation with boost(s) (median dose 20.7 (18–36) Gy), three patients as adjuvant therapy and four upon progression/recurrence. Favorable prognostic factors were administration of radiotherapy (hazard ratio (HR) for OS=0.30 (0.11–0.86), p<0.025), and undergoing HDCx/AuHCR (HR for OS=0.40 (0.16–0.99), p<0.047). There were no statistically significant associations between age, metastasis and extent of surgical resection and OS. CONCLUSIONS: Radiotherapy and HDCx/AuHCR were the sole driving factors for positive impact on survival. No benefit of high-dose methotrexate during induction was demonstrable. The high PD rate during later induction cycles as well as following consolidation chemotherapy warrant consideration of (1) fewer induction cycles prior to consolidation, (b) intensification of consolidation with multiple cycles of marrow-ablative chemotherapy and (c) judicious post-consolidation irradiation, in order to improve outcomes.
Source: Neuro-Oncology
Language: English
Subfield: Genetics
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W2900399002