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Intensive chemotherapy followed by consolidative myeloablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) in young children with newly diagnosed supratentorial primitive neuroectodermal tumors (sPNETs): report of the Head Start I and II experience.
Pediatr Blood Cancer. 2008 Feb; 50(2):312-8.PB

Abstract

BACKGROUND

Children with newly diagnosed supratentorial primitive neuroectodermal tumors (sPNET) have poor outcomes compared to medulloblastoma patients, despite similar treatments. In an effort to improve overall survival (OS) and event-free survival (EFS) and to decrease radiation exposure, the Head Start (HS) protocols treated children with newly diagnosed sPNET utilizing intensified induction chemotherapy (ICHT) followed by consolidation with myeloablative chemotherapy and autologous hematopoietic cell rescue (AuHCR).

PROCEDURES

Between 1991 and 2002, 43 children with sPNET were prospectively treated on two serial studies (HS I and II). After maximal safe surgical resection, patients on HS I and patients with localized disease on HS II were treated with five cycles of ICHT (vincristine, cisplatin, cyclophosphamide, and etoposide). Patients on HS II with disseminated disease received high-dose methotrexate during ICHT. If the disease remained stable or in response, patients received a single cycle of high-dose myeloablative chemotherapy followed by AuHCR.

RESULTS

Five-year EFS and OS were 39% (95%CI: 24%, 53%) and 49 (95%CI: 33%, 62%), respectively. Non-pineal sPNET patients faired significantly better than those patients with pineal sPNETs. Metastasis at diagnosis, age, and extent of resection were not significant prognostic factors. Sixty percent of survivors (12 of 20) are alive without exposure to radiation therapy.

CONCLUSIONS

ICHT followed by AuHCR in young patients with newly diagnosed sPNET appears to not only provide an improved EFS and OS for patients who typically have a poor prognosis, but also it successfully permitted deferral and elimination of radiation therapy in a significant proportion of patients.

Authors+Show Affiliations

Children's Hospital Los Angeles, Los Angeles, California, USA. jfangusaro@childrensmemorial.orgNo 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 availableNo affiliation info availableNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

17668858

Citation

Fangusaro, Jason, et al. "Intensive Chemotherapy Followed By Consolidative Myeloablative Chemotherapy With Autologous Hematopoietic Cell Rescue (AuHCR) in Young Children With Newly Diagnosed Supratentorial Primitive Neuroectodermal Tumors (sPNETs): Report of the Head Start I and II Experience." Pediatric Blood & Cancer, vol. 50, no. 2, 2008, pp. 312-8.
Fangusaro J, Finlay J, Sposto R, et al. Intensive chemotherapy followed by consolidative myeloablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) in young children with newly diagnosed supratentorial primitive neuroectodermal tumors (sPNETs): report of the Head Start I and II experience. Pediatr Blood Cancer. 2008;50(2):312-8.
Fangusaro, J., Finlay, J., Sposto, R., Ji, L., Saly, M., Zacharoulis, S., Asgharzadeh, S., Abromowitch, M., Olshefski, R., Halpern, S., Dubowy, R., Comito, M., Diez, B., Kellie, S., Hukin, J., Rosenblum, M., Dunkel, I., Miller, D. C., Allen, J., & Gardner, S. (2008). Intensive chemotherapy followed by consolidative myeloablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) in young children with newly diagnosed supratentorial primitive neuroectodermal tumors (sPNETs): report of the Head Start I and II experience. Pediatric Blood & Cancer, 50(2), 312-8.
Fangusaro J, et al. Intensive Chemotherapy Followed By Consolidative Myeloablative Chemotherapy With Autologous Hematopoietic Cell Rescue (AuHCR) in Young Children With Newly Diagnosed Supratentorial Primitive Neuroectodermal Tumors (sPNETs): Report of the Head Start I and II Experience. Pediatr Blood Cancer. 2008;50(2):312-8. PubMed PMID: 17668858.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Intensive chemotherapy followed by consolidative myeloablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) in young children with newly diagnosed supratentorial primitive neuroectodermal tumors (sPNETs): report of the Head Start I and II experience. AU - Fangusaro,Jason, AU - Finlay,Jonathan, AU - Sposto,Richard, AU - Ji,Lingyun, AU - Saly,Monirath, AU - Zacharoulis,Stergios, AU - Asgharzadeh,Shahab, AU - Abromowitch,Minnie, AU - Olshefski,Randal, AU - Halpern,Steven, AU - Dubowy,Ronald, AU - Comito,Melanie, AU - Diez,Blanca, AU - Kellie,Stewart, AU - Hukin,Juliette, AU - Rosenblum,Marc, AU - Dunkel,Ira, AU - Miller,Douglas C, AU - Allen,Jeffrey, AU - Gardner,Sharon, PY - 2007/8/3/pubmed PY - 2008/1/11/medline PY - 2007/8/3/entrez SP - 312 EP - 8 JF - Pediatric blood & cancer JO - Pediatr Blood Cancer VL - 50 IS - 2 N2 - BACKGROUND: Children with newly diagnosed supratentorial primitive neuroectodermal tumors (sPNET) have poor outcomes compared to medulloblastoma patients, despite similar treatments. In an effort to improve overall survival (OS) and event-free survival (EFS) and to decrease radiation exposure, the Head Start (HS) protocols treated children with newly diagnosed sPNET utilizing intensified induction chemotherapy (ICHT) followed by consolidation with myeloablative chemotherapy and autologous hematopoietic cell rescue (AuHCR). PROCEDURES: Between 1991 and 2002, 43 children with sPNET were prospectively treated on two serial studies (HS I and II). After maximal safe surgical resection, patients on HS I and patients with localized disease on HS II were treated with five cycles of ICHT (vincristine, cisplatin, cyclophosphamide, and etoposide). Patients on HS II with disseminated disease received high-dose methotrexate during ICHT. If the disease remained stable or in response, patients received a single cycle of high-dose myeloablative chemotherapy followed by AuHCR. RESULTS: Five-year EFS and OS were 39% (95%CI: 24%, 53%) and 49 (95%CI: 33%, 62%), respectively. Non-pineal sPNET patients faired significantly better than those patients with pineal sPNETs. Metastasis at diagnosis, age, and extent of resection were not significant prognostic factors. Sixty percent of survivors (12 of 20) are alive without exposure to radiation therapy. CONCLUSIONS: ICHT followed by AuHCR in young patients with newly diagnosed sPNET appears to not only provide an improved EFS and OS for patients who typically have a poor prognosis, but also it successfully permitted deferral and elimination of radiation therapy in a significant proportion of patients. SN - 1545-5017 UR - https://www.unboundmedicine.com/medline/citation/17668858/Intensive_chemotherapy_followed_by_consolidative_myeloablative_chemotherapy_with_autologous_hematopoietic_cell_rescue__AuHCR__in_young_children_with_newly_diagnosed_supratentorial_primitive_neuroectodermal_tumors__sPNETs_:_report_of_the_Head_Start_I_and_II_experience_ DB - PRIME DP - Unbound Medicine ER -