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Neuroblastoma

Neuroblastoma is the most common extracranial solid cancer in infancy and childhood. It arises from any neural crest element of the sympathetic nervous system. more...

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Differentiation

Other tumors also have similar origins and show a wide pattern of differentiation ranging from benign ganglioneuroma to partially differentiated ganglioneuroblastoma to highly malignant neuroblastoma.

Neuroblastoma is one of the rare human malignancies known to demonstrate spontaneous regression from an undifferentiated state to a completely benign cellular appearance.

Treatment

When the lesion is localized, it is generally curable. However, long-term survival for children with advanced disease is poor despite aggressive multimodality therapy.

Recent biologic and genetic characteristics have been identified, which, when added to classic clinical staging, has allowed accurate patient assignment to risk groups so that treatment strategies can be more effectively undertaken.

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Differentiating agents in pediatric malignancies: retinoids in neuroblastoma
From Alternative Medicine Review, 2/1/01 by Julie Jurenka

Differentiating agents in pediatric malignancies: retinoids in neuroblastoma. Reynolds CP. Curr Oncol Rep 2000;2:511-518.

Retinoids are derivatives of vitamin A that include all- transretinoic acid (ATRA), 13-cis-retinoic acid, (13-cis-RA), and fenretinide (4-HPR). High levels of either ATRA or 13-cis-RA can cause arrest of cell growth and morphologic differentiation of human neuroblastoma cell lines. Phase I trials have shown that higher and more sustained drug levels were obtained with 13-cis-RA relative to ATRA. A phase III randomized trial showed that high-dose pulse therapy with 13-cis-RA given after completion of intensive chemoradiotherapy (with or without autologous bone marrow transplantation) significantly improves event-free survival in high-risk neuroblastoma. Because 4-HPR achieves multi-log cell kills in neuroblastoma cell lines that are resistant to ATRA and 13-cis-RA, a pediatric phase I trial is in progress to determine the maximum tolerated dose of 4-HPR, with a view toward giving 4-HPR after completion of myeloablative therapy and 13-cis-RA.

COPYRIGHT 2001 Thorne Research Inc.
COPYRIGHT 2001 Gale Group

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