Vascular characterization of clear cell sarcoma of the kidney in a child: a case report and review

J Pediatr Surg. 2009 Oct;44(10):2031-6. doi: 10.1016/j.jpedsurg.2009.06.023.

Abstract

Clear cell sarcoma of the kidney (CCSK) is uncommon pediatric renal tumor and can present a significant therapeutic challenge in those patients whose tumors spread beyond the kidney. Thus, identifying potential novel targets for treatment may be clinically important. Clear cell sarcoma of the kidney is characterized by a unique vascular pattern, in which nests of tumor cells are separated by regularly-spaced, fine fibrovascular septa. This distinctive histopathology raises the possibility that understanding the factors which drive angiogenesis in CCSK tumors may suggest new therapeutic targets. Here, we describe a case of CCSK and present immunohistochemical studies of its vasculature.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Blood Vessels / pathology*
  • Child, Preschool
  • Endothelial Growth Factors / genetics*
  • Endothelium, Vascular / pathology*
  • Gene Expression
  • Humans
  • Immunohistochemistry / statistics & numerical data
  • Kidney / pathology
  • Kidney Neoplasms / blood supply
  • Kidney Neoplasms / genetics*
  • Kidney Neoplasms / pathology*
  • Male
  • Neovascularization, Pathologic / genetics
  • Neovascularization, Pathologic / pathology*
  • Proto-Oncogene Proteins c-kit / genetics
  • Receptors, Notch / genetics
  • Sarcoma, Clear Cell / blood supply*
  • Sarcoma, Clear Cell / genetics
  • Sarcoma, Clear Cell / pathology*

Substances

  • Endothelial Growth Factors
  • Receptors, Notch
  • Proto-Oncogene Proteins c-kit