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Uncommon Malignancies

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Fundamentals of Pediatric Surgery

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Abstract

Rare tumors in children are defined as any tumor occurring with an incidence of 2 or less per million children less than 18 years of age. In the current chapter, we will describe a selection of rare tumors including lung tumors such as pleuropulmonary blastoma, abdominal tumors including desmoplastic round blue cell tumor, pancreatoblastoma, solid pseudopapillary tumor of the pancreas, gastrointestinal stromal tumor, neuroendocrine tumors, and pediatric colorectal carcinomas, as well as adrenocortical carcinomas. We describe the presentation, diagnosis, and management of these tumors including some controversies that presented over the last decades. Melanomas and rare sarcomas will be described in other chapters of this book. Several collaborative groups (EXPERT, COG, different tumor registries) have emerged and are collecting data on these tumors from a biological and outcomes perspective. These initiatives have allowed significant advances in the understanding of the genetics and biology of these tumors and will allow further targeted therapies through personalized medicine.

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Further Reading

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Authors and Affiliations

  1. General and Thoracic Surgery, The Hospital for Sick Children, Toronto, ON, Canada

    Aodhnait S. Fahy & Reto M. Baertschiger

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  1. Aodhnait S. Fahy
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  2. Reto M. Baertschiger
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Correspondence to Reto M. Baertschiger .

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  1. General, Thoracic and Fetal Surgery, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Peter Mattei

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Fahy, A.S., Baertschiger, R.M. (2022). Uncommon Malignancies. In: Mattei, P. (eds) Fundamentals of Pediatric Surgery. Springer, Cham. https://doi.org/10.1007/978-3-031-07524-7_122

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