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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
Author(s) -
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 Blanca,
Kellie Stewart,
Hukin Juliette,
Rosenblum Marc,
Dunkel Ira,
Miller Douglas C.,
Allen Jeffrey,
Gardner Sharon
Publication year - 2008
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.21307
Subject(s) - medicine , etoposide , chemotherapy , vincristine , surgery , radiation therapy , medulloblastoma , metastasis , cyclophosphamide , pathology , cancer
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. Pediatr Blood Cancer 2008;50:312–318. © 2007 Wiley‐Liss, Inc.