BACKGROUND: Pediatric rhabdoid tumor of the kidney is regarded as a distinct neoplasm, whereas rhabdoid differentiation in adult renal cell carcinoma is usually found in association with conventional (clear cell) tumor, from which it is thought to evolve. OBJECTIVE: To further characterize the rhabdoid phenotype in adult renal cell carcinoma and to determine its origin by genetic analysis. DESIGN: We performed histologic, immunophenotypic, and genetic analyses on 5 cases of adult renal cell carcinoma with rhabdoid differentiation, 3 samples of adjacent conventional (clear cell) tumor, and 6 conventional (clear cell) control tumors. RESULTS: Rhabdoid tumors differed from conventional (clear cell) carcinoma as follows: (1) macroscopically, rhabdoid tumors were solid white uniform masses; (2) microscopically, they had large rhabdoid cells with abundant eosinophilic cytoplasm, reduced lipid content, and the absence of a branching vascular pattern; and (3) biologically, they had a high metastatic potential. Despite these differences, loss of chromosome 3p in both the rhabdoid and clear cell carcinoma samples from 1 patient suggested a clonal origin. An identical mutation of the VHL gene in both rhabdoid and clear cell tumor samples from 2 patients confirmed a clonal origin for the histologically distinct tumor types in those cases. CONCLUSION: Adult rhabdoid renal cell carcinoma can in some cases arise from conventional (clear cell) renal carcinoma and should be considered a clinically important form of renal cell carcinoma separate from, but analogous to, sarcomatoid change.
BACKGROUND: Pediatric rhabdoid tumor of the kidney is regarded as a distinct neoplasm, whereas rhabdoid differentiation in adult renal cell carcinoma is usually found in association with conventional (clear cell) tumor, from which it is thought to evolve. OBJECTIVE: To further characterize the rhabdoid phenotype in adult renal cell carcinoma and to determine its origin by genetic analysis. DESIGN: We performed histologic, immunophenotypic, and genetic analyses on 5 cases of adult renal cell carcinoma with rhabdoid differentiation, 3 samples of adjacent conventional (clear cell) tumor, and 6 conventional (clear cell) control tumors. RESULTS:Rhabdoid tumors differed from conventional (clear cell) carcinoma as follows: (1) macroscopically, rhabdoid tumors were solid white uniform masses; (2) microscopically, they had large rhabdoid cells with abundant eosinophilic cytoplasm, reduced lipid content, and the absence of a branching vascular pattern; and (3) biologically, they had a high metastatic potential. Despite these differences, loss of chromosome 3p in both the rhabdoid and clear cell carcinoma samples from 1 patient suggested a clonal origin. An identical mutation of the VHL gene in both rhabdoid and clear cell tumor samples from 2 patients confirmed a clonal origin for the histologically distinct tumor types in those cases. CONCLUSION: Adult rhabdoid renal cell carcinoma can in some cases arise from conventional (clear cell) renal carcinoma and should be considered a clinically important form of renal cell carcinoma separate from, but analogous to, sarcomatoid change.
Authors: Rajesh R Singh; Paari Murugan; Lalit R Patel; Horatiu Voicu; Suk-Young Yoo; Tadeusz Majewski; Meenakshi Mehrotra; Khalida Wani; Nizar Tannir; Jose A Karam; Eric Jonasch; Christopher G Wood; Chad J Creighton; L Jeffrey Medeiros; Russell R Broaddus; Pheroze Tamboli; Keith A Baggerly; Kenneth D Aldape; Bogdan Czerniak; Rajyalakshmi Luthra; Kanishka Sircar Journal: Mod Pathol Date: 2015-06-26 Impact factor: 7.842
Authors: Jongmin Hwang; Yong Hyun Park; Kyung Un Choi; Jeong Su Kim; Ki Won Hwang; Sang Hyun Lee; Min Ku Chon; Soo Yong Lee; Dae Sung Lee Journal: J Cardiovasc Ultrasound Date: 2016-12-28