Authors: | 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. |
Article Title: | 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 |
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. © 2007 Wiley-Liss, Inc. |
Keywords: | cancer chemotherapy; cancer survival; child; clinical article; treatment response; child, preschool; disease-free survival; cancer surgery; surgical technique; survival rate; overall survival; cancer localization; cisplatin; disease free survival; combined modality therapy; methotrexate; drug megadose; brain tumor; cancer diagnosis; carboplatin; metastasis; multiple cycle treatment; etoposide; blood toxicity; mucosa inflammation; antineoplastic combined chemotherapy protocols; combination chemotherapy; cyclophosphamide; vincristine; hematopoietic stem cell transplantation; thiotepa; childhood cancer; radiation exposure; cancer survivor; febrile neutropenia; confidence interval; myeloablative conditioning; folinic acid; newborn; supratentorial neoplasms; virus infection; mesna; children; time series analysis; granulocyte colony stimulating factor; granulocyte colony-stimulating factor; neuroectoderm tumor; autologous hematopoietic stem cell transplantation; hearing loss; streptococcus infection; neuroectodermal tumors, primitive; adult respiratory distress syndrome; brain tumors; supratentorial primitive neuroectodermal tumor; autologous hematopoietic cell rescue; primitive neuroectodermal tumor (pnet); supratentorial |
Journal Title: | Pediatric Blood and Cancer |
Volume: | 50 |
Issue: | 2 |
ISSN: | 1545-5009 |
Publisher: | Wiley Periodicals, Inc |
Date Published: | 2008-02-01 |
Start Page: | 312 |
End Page: | 318 |
Language: | English |
DOI: | 10.1002/pbc.21307 |
PUBMED: | 17668858 |
PROVIDER: | scopus |
DOI/URL: | |
Notes: | --- - "Cited By (since 1996): 19" - "Export Date: 17 November 2011" - "CODEN: PBCEA" - "Source: Scopus" |