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.
Sarcomatoid carcinoma of the kidney in a MEN1 patient: Case report and genetic profile / Cavalli T;Giudici F;Nesi G;Marini F;Giusti F;Cavalli L;Brandi ML;Tonelli F. - In: ENDOCRINE JOURNAL. - ISSN 0918-8959. - ELETTRONICO. - (2014).
Sarcomatoid carcinoma of the kidney in a MEN1 patient: Case report and genetic profile.
CAVALLI, TIZIANA;GIUDICI, FRANCESCO;NESI, GABRIELLA;MARINI, FRANCESCA;GIUSTI, FRANCESCA;CAVALLI, LOREDANA;BRANDI, MARIA LUISA;TONELLI, FRANCESCO
2014
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.
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