title: Renal cell carcinoma with rhabdoid features: an aggressive neoplasm with overexpression of p53.
abstract: BACKGROUND
  Adult renal cell carcinoma (RCC) with rhabdoid features is a recently recognized morphologic variant of kidney carcinoma. To date, only very few studies have been published on this subject and p53 was not previously studied.
  
  
  OBJECTIVE
  To evaluate clinical attributes, morphology, and immunohistochemistry in RCC with rhabdoid component.
  
  
  METHODS
  Reviewing a consecutive series of 310 RCCs, we identified 14 cases of RCC with rhabdoid features. All cases were reviewed and subjected to detailed clinical and pathologic studies with immunohistochemical evaluation of p53.
  
  
  RESULTS
  All tumors were clear RCCs with rhabdoid component representing from 5% to 50% of the tumor volume. Rhabdoid cells were large with a central eosinophilic intracytoplasmic inclusion and an eccentric atypical nucleus. Tumor necrosis was common (13/14) and sometimes extensive. Nine of 14 tumors were staged pT3, 4 of 14 were pT2, and only 1 tumor was pT1. On immunohistochemistry, rhabdoid cells were positive for vimentin (14/ 14), epithelial membrane antigen (11/14), and cytokeratin (9/14). Desmin and smooth muscle actin were always negative. p53 was positive in 10 of 14 tumors in the rhabdoid areas (5%-50% of tumor cells stained) but only in 5 of 14 cases in usual clear renal cell areas. In the follow-up, 10 of 14 patients developed metastases and 6 of 14 died of the disease. The median of survival was 8 months.
  
  
  CONCLUSIONS
  We showed that RCC with rhabdoid features is a very aggressive neoplasm with a poor prognosis. We observed an overexpression of p53 in the rhabdoid component that may be implicated in the tumor dedifferentiation.
authors.affiliation: Centre Hospitalier Universitaire de Lille, Department of Pathology, Parc Eurasante, Lille, nord 59037 France. x-leroy@chru-lille.fr
authors.forename: Xavier
authors.initials: X
authors.lastname: Leroy
authors.forename: Laurent
authors.initials: L
authors.lastname: Zini
authors.forename: David
authors.initials: D
authors.lastname: Buob
authors.forename: Charles
authors.initials: C
authors.lastname: Ballereau
authors.forename: Arnauld
authors.initials: A
authors.lastname: Villers
authors.forename: Sebastien
authors.initials: S
authors.lastname: Aubert
mesh_terms: D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000378:metabolism; D002292:Carcinoma, Renal Cell / Q000175:diagnosis / Q000378:metabolism* / Q000473:pathology*; D002454:Cell Differentiation; D005260:Female; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D007680:Kidney Neoplasms / Q000175:diagnosis / Q000378:metabolism* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D018396:Mucin-1 / Q000378:metabolism; D009367:Neoplasm Staging; D011379:Prognosis; D018335:Rhabdoid Tumor / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology; D016159:Tumor Suppressor Protein p53 / Q000378:metabolism*; D014746:Vimentin / Q000235:genetics / Q000378:metabolism
chemical_list: D014408:Biomarkers, Tumor; D018396:Mucin-1; D016159:Tumor Suppressor Protein p53; D014746:Vimentin