Supratentorial primitive neuroectodermal tumors (S-PNET) in children: A prospective experience with adjuvant intensive chemotherapy and hyperfractionated accelerated radiotherapy

Journal Article · Wed Mar 15 04:00:00 EST 2006 · International Journal of Radiation Oncology, Biology and Physics

DOI:https://doi.org/10.1016/J.IJROBP.2005.0· OSTI ID:20793379

Massimino, Maura ^[1]; Gandola, Lorenza ^[2]; Spreafico, Filippo ^[1]; Luksch, Roberto ^[1]; Collini, Paola ^[3]; Giangaspero, Felice ^[4]; Simonetti, Fabio ^[1]; Casanova, Michela ^[1]; Cefalo, Graziella ^[1]; Pignoli, Emanuele ^[5]; Ferrari, Andrea ^[1]; Terenziani, Monica ^[1]; Podda, Marta ^[1]; Meazza, Cristina ^[1]; Polastri, Daniela ^[1]; Poggi, Geraldina ^[6]; Ravagnani, Fernando ^[7]; Fossati-Bellani, Franca ^[1]

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1. Department of Pediatric Oncology, Istituto Nazionale Tumori, Milan (Italy)
2. Department of Radiotherapy, Istituto Nazionale Tumori, Milan (Italy)
3. Department of Pathology, Istituto Nazionale Tumori, Milan (Italy)
4. Neuropathology Department, University La Sapienza, Rome (Italy)
5. Department of Physics, Istituto Nazionale Tumori, Milan (Italy)
6. Research Unit, Istituto Eugenio Medea, Bosisio Parini (Italy)
7. Department of Transfusional Units, Istituto Nazionale Tumori, Milan (Italy)

Purpose: Supratentorial primitive neuroectodermal tumors (S-PNET) are rare and have a grim prognosis, frequently taking an aggressive course with local relapse and metastatic spread. We report the results of a mono-institutional therapeutic trial. Methods and Materials: We enrolled 15 consecutive patients to preradiation chemotherapy (CT) consisting of high-dose methotrexate, high-dose etoposide, high-dose cyclophosphamide, and high-dose carboplatin, craniospinal irradiation (CSI) with hyperfractionated accelerated radiotherapy (HART) plus focal boost, maintenance with vincristine/lomustine or consolidation with high-dose thiotepa followed by autologous stem-cell rescue. Results: Median age was 9 years; 7 were male, 8 female. Site of disease was pineal in 3, elsewhere in 12. Six patients were had no evidence of disease after surgery (NED). Of those with evidence of disease after surgery (ED), 2 had central nervous system spread. Of the 9 ED patients, 2 had complete response (CR) and 2 partial response (PR) after CT, 4 stable disease, and 1 progressive disease. Of the 7 ED patients before radiotherapy, 1 had CR, 4 PR, and 2 minor response, thus obtaining a 44% CR + PR after CT and 71% after HART. Because of rapid progression in 2 of the first 5 patients, high-dose thiotepa was systematically adopted after HART in the subsequent 10 patients. Six of 15 patients relapsed (4 locally, 1 locally with dissemination, 1 with dissemination) a mean of 6 months after starting CT, 2 developed second tumors; 5 of 6 relapsers died at a median of 13 months. Three-year progression-free survival, event-free survival, and overall survival were 54%, 34%, and 61%, respectively. Conclusion: Hyperfractionated accelerated RT was the main tool in obtaining responses in S-PNET; introducing the myeloablative phase improved the prognosis (3/10 vs. 3/5 relapses), though the outcome remained unsatisfactory despite the adoption of this intensive treatment.

• OSTI ID: 20793379
• Journal Information: International Journal of Radiation Oncology, Biology and Physics, Journal Name: International Journal of Radiation Oncology, Biology and Physics Journal Issue: 4 Vol. 64; ISSN IOBPD3; ISSN 0360-3016
• Country of Publication: United States
• Language: English

Related Subjects

62 RADIOLOGY AND NUCLEAR MEDICINE
BRAIN
CHEMOTHERAPY
CHILDREN
ENDOXAN
IRRADIATION
METASTASES
METHOTREXATE
NEOPLASMS
PATIENTS
RADIATION DOSES
RADIOTHERAPY
STEM CELLS
SURGERY