Recurrent central nervous system medulloepithelioma: Response and outcome following marrow-ablative chemotherapy with stem cell rescue Journal Article


Authors: Norris, L. S.; Snodgrass, S.; Miller, D. C.; Wisoff, J.; Garvin, J.; Rorke, L. B.; Finlay, J. L.
Article Title: Recurrent central nervous system medulloepithelioma: Response and outcome following marrow-ablative chemotherapy with stem cell rescue
Abstract: Medulloepithelioma is a rare primitive neuroectodermal tumor of the central nervous system usually developing in childhood, displaying highly malignant behavior, with early progression or recurrence. Once a recurrence develops, death reportedly follows with invariable rapidity. The purpose of this study was to evaluate the efficacy of high-dose, marrow-ablative chemotherapy with autologous hemopoietic stem cell rescue in the treatment of recurrent central nervous system medulloepithelioma. Three young children with recurrent central nervous system medulloepithelioma received high-dose marrow-ablative chemotherapy with thiotepa and etoposide either alone (one patient) or with the addition of carboplatin (two patients). This was followed by irradiation only in one patient. One child with residual radiographic tumor at the time of treatment could be evaluated for response and showed complete resolution of leptomeningeal disease after receiving marrow-ablative chemotherapy. Two children developed tumor recurrence at 2.0 and 5.5 months after receiving marrow-ablative chemotherapy. The third child continues free of tumor beyond 12 years from treatment. The authors' experience with marrow-ablative chemotherapy and autologous hemopoietic stem cell rescue suggests that this treatment strategy might be beneficially incorporated into the initial treatment approach for young children with medulloepithelioma. Copyright © 2005 by Lippincott Williams & Wilkins.
Keywords: cancer chemotherapy; preschool child; treatment outcome; child, preschool; disease course; cancer recurrence; case report; cisplatin; side effect; cancer radiotherapy; combined modality therapy; drug megadose; magnetic resonance imaging; carboplatin; neoplasm recurrence, local; etoposide; antineoplastic combined chemotherapy protocols; vincristine; stem cell transplantation; hematopoietic stem cell transplantation; thiotepa; central nervous system tumor; time factors; central nervous system neoplasms; carcinoma; neuroepithelioma; developmental disorder; alopecia; hearing loss; myeloablative chemotherapy; medulloepithelioma; recurrent brain tumor
Journal Title: Journal of Pediatric Hematology/Oncology
Volume: 27
Issue: 5
ISSN: 1077-4114
Publisher: Lippincott Williams & Wilkins  
Date Published: 2005-05-01
Start Page: 264
End Page: 266
Language: English
DOI: 10.1097/01.mph.0000165498.24989.9a
PUBMED: 15891561
PROVIDER: scopus
DOI/URL:
Notes: --- - "Cited By (since 1996): 5" - "Export Date: 24 October 2012" - "CODEN: JPHOF" - "Source: Scopus"
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