Uncommon Malignancies

Aodhnait S. Fahy, Reto M. Baertschiger

Research output: Chapter in Book/Report/Conference proceedingChapter


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.

Original languageEnglish (US)
Title of host publicationFundamentals of Pediatric Surgery, Third Edition
PublisherSpringer International Publishing
Number of pages12
ISBN (Electronic)9783031075247
ISBN (Print)9783031075230
StatePublished - Jan 1 2022

All Science Journal Classification (ASJC) codes

  • General Medicine
  • General Nursing


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