Reduced-dose craniospinal radiotherapy followed by high-dose chemotherapy and autologous stem cell rescue for children with newly diagnosed high-risk medulloblastoma or supratentorial primitive neuroectodermal tumor

BACKGROUND: In this study, we investigated the effects of reduced-dose craniospinal radiotherapy (CSRT) followed by tandem high-dose chemotherapy (HDCT) with autologous stem cell rescue (ASCR) in children with a newly diagnosed high-risk medulloblastoma (MB) or supratentorial primitive neuroectodermal tumor (sPNET). METHODS: Between March 2005 and April 2007, patients older than 3 years with a newly diagnosed high-risk MB or sPNET were enrolled. The patients received two cycles of pre-RT chemotherapy consisting of cisplatin, etoposide, vincristine, and cyclophosphamide (cycle A), and carboplatin, etoposide, vincristine, and ifosphamide (cycle B), followed by CSRT with 23.4 Gy and local RT with 30.6 Gy. After four cycles of post-RT chemotherapy (cycles A, B, A, and B), tandem double HDCT with ASCR was performed. RESULTS: A total of 13 patients (MB=11, sPNET=2) were enrolled. Of these, one patient progressed, one patient died of septic shock after the second cycle of B, and one patient relapsed after the third cycle of B. The 3-year event-free survival (EFS) rate of the patients intended for HDCT was 76.9%, whereas the 3-year EFS rate of the patients who received HDCT was 100%. No treatment-related mortality occurred during HDCT. CONCLUSION: Although the follow-up period was short and the patient cohort was small in size, the results of this study are encouraging. The limited toxicity and favorable EFS rate observed in children treated with reduced-dose CSRT followed by HDCT and ASCR warrant further exploration in a larger study population.

Therapeutic Difficulty in a Case of Supratentorial Primitive Neuroectodermal Tumor Diagnosed during Pregnancy

We report a supratentorial primitive neuroectodermal tumor (sPNET) in 17-year-old primipara in the second trimester her pregnancy. Magnetic resonance imaging revealed a left frontoparietal mass with solid and cystic component. Gross-total resection was achieved via a left frontoparietal craniotomy. It was decided to suspend the radiotherapy and chemotherapy until the 30 weeks of gestation. But, a sudden uncal herniation was developed due to the reccurrence of the tumor and bleeding into the tumor at the 25 weeks of gestation and the patient died after urgent decompressive surgery. sPNETs is an extremely rare brain tumor in pregnancy and only two cases were reported in the literature to date. There is no universally agreed treatment protocol for sPNETs during pregnancy and a multidisciplinary approach is required in treatment. In the present study, the clinical, histopathological features and therapeutical difficulties of sPNETs diagnosed during pregnancy was discussed with the literature review.

Survival Rates and Prognostic Factors in Childhood Medulloblastoma and Supratentorial Primitive Neuroectodermal Tumor / 대한소아혈액종양학회지

PURPOSE: Children under 3 years old with medulloblastoma (MB) or supratentorial primitive neuroectodermal tumor (SPNET) have a poor prognosis. They are more susceptible to the deleterious effects of craniospinal radiation (CSR). This study was undertaken to determine the survival rates and prognostic factors of currently used treatment. METHPDS: Retrospective review of 19 children (MB 16, SPNET 3) who were newly diagnosed as MB or SPNET at Seoul National University Children's Hospital from April 1996 to August 2000 was done. Eligible Patients were treated with postoperative induction chemotherapy using Children's Cancer Group (CCG) 9921A or 9931. Patients over 3 year of age received CSR as well. RESULTS: Three Patients with SPNET are all alive without disease. However, 6 patients under 3 years old at diagnosis with MB were all dead whereas only one patient had an event among 10 patients over 3 years old at diagnosis. Among patients with MB, age under 3 years at diagnosis or residual tumors after induction chemotherapy correlated with poor outcome. Metastasis at diagnosis, postoperative residual disease or extent of excision did not have a significant effect on survival statistically. Three-year event-free survival (EFS) of patients with MB was 53%. Three-year EFS of patients over 3 years old at diagnosis with MB was 85.7% and 1-year EFS of under 3 years old at diagnosis with MB was 16.7%. CONCLUSION: The combined use of chemotherapy and CSR in the treatment of MB and SPNET improved survival in children over 3 years old, whereas any treatment failed to improve survival in children under 3 years old. So, there's a need for a new treatment modality to improve survival of children under 3 year of age.