Journal of Clinical Oncology, Vol 12, 1212-1216, Copyright © 1994 by American Society of Clinical Oncology

ARTICLES

Medulloblastoma in very young children: outcome of definitive craniospinal irradiation following incomplete response to chemotherapy

A Gajjar, RK Mulhern, RL Heideman, RA Sanford, EC Douglass, EH Kovnar, JA Langston, JJ Jenkins and LE Kun
St Jude Children's Research Hospital/LeBonheur Children's Medical Center Brain Tumor Team, Memphis, TN 38101.

PURPOSE: To evaluate survival and neurodevelopmental outcomes following radiation therapy in infants and young children with residual or progressive medulloblastoma after primary chemotherapy. PATIENTS AND METHODS: Thirteen young patients (< or = 36 months old) with medulloblastoma were treated with preirradiation multiagent chemotherapy and maximal surgical resection. Patients were scheduled to receive radiation therapy at the time of documented disease progression or upon completion of chemotherapy with residual disease. All patients underwent neurodevelopmental evaluation at the time of diagnosis, before receiving radiation therapy, and at yearly intervals posttreatment. RESULTS: Two patients completed the scheduled chemotherapy with residual disease and received delayed radiation therapy. The remaining 11 patients had either local or leptomeningeal progression during chemotherapy (median time to progression, 5 months). Six patients had a complete response (CR) to radiation therapy, and three of these children are alive 48 to 104 months postdiagnosis. Of the five patients who had progressive disease (PD) during radiation therapy or residual imaging abnormalities after treatment, only one is alive (with stable enhancing leptomeningeal abnormalities) 48 months postirradiation. Two additional survivors were rendered disease-free by surgical resection before radiation therapy and are without evidence of disease at 91 and 107 months after diagnosis. Thus, six of 13 patients are alive at 48 to 107 months postdiagnosis. Neurodevelopmental scores tended to be below age norms at diagnosis; scores improved during chemotherapy, but then decreased during posttreatment follow-up evaluation. CONCLUSION: Radiation therapy appears to produce long-term disease-free survival in a proportion of very young patients who have progressive or residual medulloblastoma during or after primary chemotherapy. However, neurodevelopmental deficits are frequent among long-term survivors.
CiteULike    Complore    Connotea    Del.icio.us    Digg    Facebook    Reddit    Technorati    Twitter    What's this?

This article has been cited by other articles:

				N. G. Gottardo and A. Gajjar Chemotherapy for Malignant Brain Tumors of Childhood J Child Neurol, October 1, 2008; 23(10): 1149 - 1159. [Abstract] [PDF]

				J. C. Barredo, M. Devidas, S. J. Lauer, A. Billett, M. Marymont, J. Pullen, B. Camitta, N. Winick, W. Carroll, and A. K. Ritchey Isolated CNS Relapse of Acute Lymphoblastic Leukemia Treated With Intensive Systemic Chemotherapy and Delayed CNS Radiation: A Pediatric Oncology Group Study J. Clin. Oncol., July 1, 2006; 24(19): 3142 - 3149. [Abstract] [Full Text] [PDF]

				R. K. Mulhern, S. L. Palmer, W. E. Reddick, J. O. Glass, L. E. Kun, J. Taylor, J. Langston, and A. Gajjar Risks of Young Age for Selected Neurocognitive Deficits in Medulloblastoma Are Associated With White Matter Loss J. Clin. Oncol., January 15, 2001; 19(2): 472 - 479. [Abstract] [Full Text] [PDF]

				A. W. Walter, R. K. Mulhern, A. Gajjar, R. L. Heideman, D. Reardon, R. A. Sanford, X. Xiong, and L. E. Kun Survival and Neurodevelopmental Outcome of Young Children With Medulloblastoma at St Jude Children's Research Hospital J. Clin. Oncol., December 1, 1999; 17(12): 3720 - 3728. [Abstract] [Full Text] [PDF]

				P. K. Duffner, M. E. Horowitz, J. P. Krischer, P. C. Burger, M. E. Cohen, R. A. Sanford, H. S. Friedman, L. E. Kun, and the Pediatric Oncology Group (POG) The treatment of malignant brain tumors in infants and very young children: An update of the Pediatric Oncology Group experience Neuro-oncol, April 1, 1999; 1(2): 152 - 161. [PDF]