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Adults with CNS primitive neuroectodermal tumors/pineoblastomas: results of multimodal treatment according to the
Carsten Friedrich1, Klaus Müller, Katja von Hoff
1Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Germany.
Abstract:
Central nervous system primitive neuroectodermal tumors (CNS-PNET) and pineoblastomas (PBL) are rare in adulthood. Knowledge on clinical outcome and the efficacy and toxicities of chemotherapy in addition to radiotherapy is limited. Patients older than 21 years at diagnosis were followed in the observational arm of the prospective pediatric multicenter trial HIT 2000. After surgery, craniospinal irradiation and maintenance or sandwich chemotherapy were recommended. Radiotherapy was normo- (35.2 Gy; tumor region, 55.0 Gy; metastasis, 49.6 Gy) or hyperfractionated (40.0 Gy; tumor bed, 68.0 Gy; metastasis, 50-60 Gy). Maintenance chemotherapy consisted of eight courses (vincristine, lomustine, cisplatin). Sandwich chemotherapy included two cycles of postoperative chemotherapy followed by radiotherapy, and four courses of maintenance chemotherapy. Seventeen patients (CNS-PNET, n = 7; PBL, n = 10), median age 30 years, were included. Eight patients had a postoperative residual tumor and four patients metastatic disease. The median follow-up of ten surviving patients was 41 months. The estimated rates for 3-year progression-free survival (PFS) and overall survival were 68 ± 12 and 66 ± 13%, respectively. PBL compared to CNS-PNET tended towards a better PFS, although the difference was not clear (p = 0.101). Both chemotherapeutic (maintenance, n = 6; sandwich, n = 8) protocols did not differ in their PFS and were feasible with acceptable toxicities. Intensified regimens of combined chemo- and radiotherapy are generally feasible in adults with CNS-PNET/PBL. The impact of intensified chemotherapy on survival should be further assessed.
Insights
Intensified chemo-radiotherapy is feasible for adult Central Nervous System Primitive Neuroectodermal Tumors (CNS-PNET) and Pineoblastomas (PBL). These combined treatments show acceptable toxicity and survival rates in this rare adult population.
Area of Science:
- Neuro-oncology
- Pediatric Oncology adapted for adults
- Radiation Oncology
Background:
- Central nervous system primitive neuroectodermal tumors (CNS-PNET) and pineoblastomas (PBL) are rare in adults, with limited data on treatment outcomes.
- Existing knowledge on the efficacy and toxicities of chemotherapy combined with radiotherapy in adult CNS-PNET/PBL is scarce.
Purpose of the Study:
- To evaluate the clinical outcome, efficacy, and toxicities of intensified chemo-radiotherapy in adult patients diagnosed with CNS-PNET or PBL.
- To assess the feasibility and impact of two chemotherapy protocols (maintenance vs. sandwich) in conjunction with radiotherapy.
Main Methods:
- An observational arm of the prospective pediatric multicenter trial HIT 2000 included 17 adult patients (median age 30 years) with CNS-PNET (n=7) or PBL (n=10).
- Treatment involved surgery, craniospinal irradiation (normo- or hyperfractionated), followed by either maintenance or sandwich chemotherapy regimens.
- Chemotherapy included vincristine, lomustine, and cisplatin. Patients were monitored for progression-free survival (PFS) and overall survival.
Main Results:
- The median follow-up for ten surviving patients was 41 months. Estimated 3-year PFS and overall survival rates were 68 ± 12% and 66 ± 13%, respectively.
- Both maintenance and sandwich chemotherapy protocols demonstrated similar PFS and were feasible with acceptable toxicities.
- Pineoblastoma (PBL) patients showed a trend towards better PFS compared to CNS-PNET, though not statistically significant (p=0.101).
Conclusions:
- Intensified combined chemo-radiotherapy regimens are generally feasible and well-tolerated in adult patients with CNS-PNET/PBL.
- The study suggests that these intensified treatments can achieve promising survival rates in this rare adult population.
- Further research is warranted to fully assess the impact of intensified chemotherapy on survival outcomes in adult CNS-PNET/PBL.
