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

Jason Fangusaro, Jonathan Finlay, Richard Sposto, Lingyun Ji, Monirath Saly, Stergios Zacharoulis, Shahab Asgharzadeh, Minnie Abromowitch, Randal Olshefski, Steven Halpern, Ronald Dubowy, Melanie Comito, Bianca Diez, Stewart Kellie, Juliette Hukin, Marc Rosenblum, Ira Dunkel, Douglas C. Miller, Jeffrey Allen, Sharon Gardner

Research output: Contribution to journalArticle

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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.

Original languageEnglish (US)
Pages (from-to)312-318
Number of pages7
JournalPediatric Blood and Cancer
Volume50
Issue number2
DOIs
StatePublished - Feb 1 2008

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Primitive Neuroectodermal Tumors
Drug Therapy
Induction Chemotherapy
Disease-Free Survival
Survival
Radiotherapy
Medulloblastoma
Vincristine
Etoposide
Methotrexate
Cyclophosphamide
Cisplatin
Survivors
Neoplasm Metastasis

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

Fangusaro, Jason ; Finlay, Jonathan ; Sposto, Richard ; Ji, Lingyun ; Saly, Monirath ; Zacharoulis, Stergios ; Asgharzadeh, Shahab ; Abromowitch, Minnie ; Olshefski, Randal ; Halpern, Steven ; Dubowy, Ronald ; Comito, Melanie ; Diez, Bianca ; Kellie, Stewart ; Hukin, Juliette ; Rosenblum, Marc ; Dunkel, Ira ; Miller, Douglas C. ; Allen, Jeffrey ; Gardner, Sharon. / 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. In: Pediatric Blood and Cancer. 2008 ; Vol. 50, No. 2. pp. 312-318.
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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.",
author = "Jason Fangusaro and Jonathan Finlay and Richard Sposto and Lingyun Ji and Monirath Saly and Stergios Zacharoulis and Shahab Asgharzadeh and Minnie Abromowitch and Randal Olshefski and Steven Halpern and Ronald Dubowy and Melanie Comito and Bianca Diez and Stewart Kellie and Juliette Hukin and Marc Rosenblum and Ira Dunkel and Miller, {Douglas C.} and Jeffrey Allen and Sharon Gardner",
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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, DC, 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 and Cancer, vol. 50, no. 2, pp. 312-318. https://doi.org/10.1002/pbc.21307

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. / Fangusaro, Jason; Finlay, Jonathan; Sposto, Richard; Ji, Lingyun; Saly, Monirath; Zacharoulis, Stergios; Asgharzadeh, Shahab; Abromowitch, Minnie; Olshefski, Randal; Halpern, Steven; Dubowy, Ronald; Comito, Melanie; Diez, Bianca; Kellie, Stewart; Hukin, Juliette; Rosenblum, Marc; Dunkel, Ira; Miller, Douglas C.; Allen, Jeffrey; Gardner, Sharon.

In: Pediatric Blood and Cancer, Vol. 50, No. 2, 01.02.2008, p. 312-318.

Research output: Contribution to journalArticle

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)

T2 - 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, Bianca

AU - Kellie, Stewart

AU - Hukin, Juliette

AU - Rosenblum, Marc

AU - Dunkel, Ira

AU - Miller, Douglas C.

AU - Allen, Jeffrey

AU - Gardner, Sharon

PY - 2008/2/1

Y1 - 2008/2/1

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.

AB - 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.

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U2 - 10.1002/pbc.21307

DO - 10.1002/pbc.21307

M3 - Article

VL - 50

SP - 312

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JO - Pediatric Blood and Cancer

JF - Pediatric Blood and Cancer

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