Sarcomatoid carcinoma of the kidney in a MEN1 patient: case report and genetic profile

Endocr J. 2014;61(8):781-7. doi: 10.1507/endocrj.ej14-0021. Epub 2014 May 30.

Abstract

Renal tumors are exceedingly rare in Multiple Endocrine Neoplasia type 1 (MEN1), a pleyotropic hereditary cancer disorder affecting the endocrine system. Herein we report a unique case of renal sarcomatoid carcinoma with concomitant ipsilateral non-secreting adrenal adenoma occurring in a young male MEN1 patient, previously operated for hyperparathyroidism and multiple pancreatic neuroendocrine neoplasms. Molecular analysis in the MEN1 locus at 11q13 showed loss of heterozygosity in the adrenal lesion, while kidney cancer was unrelated to MEN1 syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adenoma / complications
  • Adenoma / diagnosis
  • Adenoma / genetics
  • Adrenal Gland Neoplasms / complications
  • Adrenal Gland Neoplasms / diagnosis
  • Adrenal Gland Neoplasms / genetics
  • Adult
  • Carcinoma, Renal Cell / complications
  • Carcinoma, Renal Cell / diagnosis*
  • Carcinoma, Renal Cell / genetics
  • DNA Mutational Analysis
  • Humans
  • Kidney Neoplasms / complications
  • Kidney Neoplasms / diagnosis*
  • Kidney Neoplasms / genetics
  • Male
  • Multiple Endocrine Neoplasia Type 1 / complications
  • Multiple Endocrine Neoplasia Type 1 / diagnosis*
  • Multiple Endocrine Neoplasia Type 1 / genetics
  • Neoplasms, Multiple Primary / diagnosis
  • Neoplasms, Multiple Primary / genetics
  • Proto-Oncogene Proteins / genetics*

Substances

  • MEN1 protein, human
  • Proto-Oncogene Proteins