Library of Congress Control Number 2004113134 ISBN-10 3-540-40841-X Springer Berlin Heidelberg NewYork ISBN-13 978-3-540-40841-3 Springer Berlin Heidelberg NewYork ISSN 1613-5318
Nai-Kong V. Cheung, MD, PhD
(e-mail: [email protected])
Department of Pediatrics, Memorial Sloan-Kettering
Cancer Center, 1275 York Avenue, New York,
NY 10021, USA
Susan L. Cohn, MD
(e-mail: [email protected]) Department of Pediatrics and the Comprehensive Robert H. Lurie Cancer Center, Northwestern University, Feinberg School of Medicine, Children's Memorial Hospital 2300 Children's Plaza, Chicago, IL 60614, USA
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The rapid advances in our understanding of the biology and treatment of neuroblastoma make it difficult to keep up to date. The clinical facets of neuroblastoma are endlessly fascinating. Its "natural history" overtly displays the difference between cancer and a truly extraordinary non-malignant proliferative disease. An interesting and potentially promising research emphasis is to unravel the difference between the "good" and "bad" forms of the disease. Our interest in neuroblastoma was kindled by clinical observations going back many decades. For example, is it likely that neuroblastoma "metastasizes" from one adrenal to the other and to the posterior mediastinum, or that malignant secondary deposits in these three unlikely sites will disappear spontaneously? Our early observations of this phenomenon were made in the days when there were no effective treatments for neuroblastoma so it was easier willy-nilly to observe the natural history.
We have seen disease wax and wane over time, such as skin lesions which became increasingly mature with each new "crop"; thus, the last one seen at 36 months was diagnosed as a neurofibroma. The results coming from the screening programs underline these concepts. They have shown that many more infants actually harbor occult neuroblastoma than are diagnosed clinically (in the nonscreened cohort population). This establishes that most such foci would have regressed spontaneously had they not been de tected through screening. Observations such as these suggest that 4S neuroblastoma could teach us more about what clonal growth implies than clonal growth teaches us about neuroblastoma.
Obviously neuroblastoma can be a relentless, malignant disease, and these children need far better therapies than we now can muster. But the future may not lie so much in new classes of compounds or even drug adjuvants. It lies, instead, in the final understanding of what makes neuroblastoma mature into ganglioneuroma or, even more importantly, what prompts it to disappear spontaneously. Success will be measured when widespread disease in children with high-risk neuroblastoma is made to vanish through molecular genetic manipulations. Then cure will have achieved its true and very special meaning: disappearance of a life-threatening malignant disease without incurring the side effects of currently available avenues of treatment.
Professor of Pediatrics, Emeritus at the University of Pennsylvania, Senior Physician, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
Giulio J. D'Angio
Professor of Radiation Oncology, Radiology and Pediatrics, Emeritus at the University of Pennsylvania, Philadelphia, Pennsylvania
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