Dilemmas associated with congenital ewing sarcoma family tumors

J Pediatr Hematol Oncol. 2008 Jan;30(1):4-7. doi: 10.1097/MPH.0b013e31815cf71f.

Abstract

The case report by Meazza et al describes the rapidly fatal outcome of a child born with a congenital peripheral primitive neuroectodermal tumor (pPNET). The case report prompted us to ask several questions. (1) Is there such an entity as congenital pPNET? (2) Do translocation-negative Ewing sarcoma family tumors (ESFT) exist? (3) What is the outcome for newborns with congenital ESFT and how does this affect treatment options?

Publication types

  • Review

MeSH terms

  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Neuroectodermal Tumors, Primitive, Peripheral / congenital
  • Neuroectodermal Tumors, Primitive, Peripheral / genetics
  • Neuroectodermal Tumors, Primitive, Peripheral / mortality
  • Neuroectodermal Tumors, Primitive, Peripheral / pathology
  • Neuroectodermal Tumors, Primitive, Peripheral / therapy
  • Oncogene Proteins, Fusion / genetics*
  • Sarcoma, Ewing* / congenital
  • Sarcoma, Ewing* / genetics
  • Sarcoma, Ewing* / mortality
  • Sarcoma, Ewing* / pathology
  • Sarcoma, Ewing* / therapy
  • Translocation, Genetic*
  • Treatment Outcome

Substances

  • Oncogene Proteins, Fusion