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Supratentorial primitive neuroectodermal tumors (S-PNET) in children: A prospective experience with adjuvant intensive chemotherapy and hyperfractionated accelerated radiotherapy.
Int J Radiat Oncol Biol Phys. 2006 Mar 15; 64(4):1031-7.IJ

Abstract

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.

Authors+Show Affiliations

Neuro-Oncology Functional Unit, Department of Pediatric Oncology, Istituto Nazionale Tumori, Milan, Italy. maura.massimino@istitutotumori.mi.itNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

16343801

Citation

Massimino, Maura, et al. "Supratentorial Primitive Neuroectodermal Tumors (S-PNET) in Children: a Prospective Experience With Adjuvant Intensive Chemotherapy and Hyperfractionated Accelerated Radiotherapy." International Journal of Radiation Oncology, Biology, Physics, vol. 64, no. 4, 2006, pp. 1031-7.
Massimino M, Gandola L, Spreafico F, et al. Supratentorial primitive neuroectodermal tumors (S-PNET) in children: A prospective experience with adjuvant intensive chemotherapy and hyperfractionated accelerated radiotherapy. Int J Radiat Oncol Biol Phys. 2006;64(4):1031-7.
Massimino, M., Gandola, L., Spreafico, F., Luksch, R., Collini, P., Giangaspero, F., Simonetti, F., Casanova, M., Cefalo, G., Pignoli, E., Ferrari, A., Terenziani, M., Podda, M., Meazza, C., Polastri, D., Poggi, G., Ravagnani, F., & Fossati-Bellani, F. (2006). Supratentorial primitive neuroectodermal tumors (S-PNET) in children: A prospective experience with adjuvant intensive chemotherapy and hyperfractionated accelerated radiotherapy. International Journal of Radiation Oncology, Biology, Physics, 64(4), 1031-7.
Massimino M, et al. Supratentorial Primitive Neuroectodermal Tumors (S-PNET) in Children: a Prospective Experience With Adjuvant Intensive Chemotherapy and Hyperfractionated Accelerated Radiotherapy. Int J Radiat Oncol Biol Phys. 2006 Mar 15;64(4):1031-7. PubMed PMID: 16343801.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Supratentorial primitive neuroectodermal tumors (S-PNET) in children: A prospective experience with adjuvant intensive chemotherapy and hyperfractionated accelerated radiotherapy. AU - Massimino,Maura, AU - Gandola,Lorenza, AU - Spreafico,Filippo, AU - Luksch,Roberto, AU - Collini,Paola, AU - Giangaspero,Felice, AU - Simonetti,Fabio, AU - Casanova,Michela, AU - Cefalo,Graziella, AU - Pignoli,Emanuele, AU - Ferrari,Andrea, AU - Terenziani,Monica, AU - Podda,Marta, AU - Meazza,Cristina, AU - Polastri,Daniela, AU - Poggi,Geraldina, AU - Ravagnani,Fernando, AU - Fossati-Bellani,Franca, Y1 - 2005/12/15/ PY - 2005/07/04/received PY - 2005/08/22/revised PY - 2005/09/13/accepted PY - 2005/12/14/pubmed PY - 2006/4/21/medline PY - 2005/12/14/entrez SP - 1031 EP - 7 JF - International journal of radiation oncology, biology, physics JO - Int J Radiat Oncol Biol Phys VL - 64 IS - 4 N2 - 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. SN - 0360-3016 UR - https://www.unboundmedicine.com/medline/citation/16343801/Supratentorial_primitive_neuroectodermal_tumors__S_PNET__in_children:_A_prospective_experience_with_adjuvant_intensive_chemotherapy_and_hyperfractionated_accelerated_radiotherapy_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0360-3016(05)02717-3 DB - PRIME DP - Unbound Medicine ER -