title: Sarcomatoid carcinoma of the kidney in a MEN1 patient: case report and genetic profile.
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.
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Florence, Italy.
authors.forename: Tiziana
authors.initials: T
authors.lastname: Cavalli
authors.forename: Francesco
authors.initials: F
authors.lastname: Giudici
authors.forename: Gabriella
authors.initials: G
authors.lastname: Nesi
authors.forename: Francesca
authors.initials: F
authors.lastname: Marini
authors.forename: Francesca
authors.initials: F
authors.lastname: Giusti
authors.forename: Loredana
authors.initials: L
authors.lastname: Cavalli
authors.forename: Maria Luisa
authors.initials: ML
authors.lastname: Brandi
authors.forename: Francesco
authors.initials: F
authors.lastname: Tonelli
mesh_terms: D000236:Adenoma / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D000328:Adult; D002292:Carcinoma, Renal Cell / Q000150:complications / Q000175:diagnosis* / Q000235:genetics; D004252:DNA Mutational Analysis; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000235:genetics; D008297:Male; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications / Q000175:diagnosis* / Q000235:genetics; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis / Q000235:genetics; D011518:Proto-Oncogene Proteins / Q000235:genetics*
chemical_list: C105373:MEN1 protein, human; D011518:Proto-Oncogene Proteins