Sporadic haemangioblastoma of the kidney with rhabdoid features and focal CD10 expression: report of a case and literature review

Diagn Pathol. 2012 Apr 12:7:39. doi: 10.1186/1746-1596-7-39.

Abstract

We present here an intriguing case of sporadic renal haemangioblastoma occurring in a 61-year-old male. The tumor consisted of nests of polygonal cells and abundant capillary networks. The neoplastic cells generally showed abundant eosinophilic cytoplasm and prominent eccentric nuclei, resembling the rhabdoid cells. Pronounced intranuclear cytoplasmic pseudoinclusions were another significant feature seen. NSE, a-inhibin and S100 were positive in tumor cells and particularly, focal CD10 expressions were observed. This is possibly the first reported case of a haemangioblastoma showing a rhabdoid phenotype and CD10 immunopositivity. Malignant rhabdoid tumor and renal cell carcinoma with rhabdoid features were probably the most challenging mimics need to be differentiated. The result of focal CD10 staining in our case may further lead to confusion with renal cell carcinoma. To avoid misdiagnosis, more considerations should be attached to the rare neoplasm.

Virtual slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1068858553657049.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Biomarkers, Tumor / metabolism*
  • Carcinoma, Renal Cell / metabolism
  • Carcinoma, Renal Cell / pathology
  • Diagnosis, Differential
  • Gene Expression Regulation, Neoplastic
  • Hemangioblastoma / metabolism*
  • Hemangioblastoma / pathology*
  • Hemangioblastoma / surgery
  • Humans
  • Inhibins / metabolism
  • Kidney Neoplasms / metabolism*
  • Kidney Neoplasms / pathology*
  • Kidney Neoplasms / surgery
  • Male
  • Middle Aged
  • Nephrectomy
  • Neprilysin / metabolism*
  • Phenotype
  • Rhabdoid Tumor / pathology*
  • S100 Proteins / metabolism
  • Treatment Outcome

Substances

  • Biomarkers, Tumor
  • S100 Proteins
  • inhibin-alpha subunit
  • Inhibins
  • Neprilysin