MP78-11 DO SARCOMATOID AND RHABDOID DIFFERENTIATION HAVE SIMILAR PROGNOSTIC EFFECTS FOR PATIENTS WITH GRADE 4 RENAL CELL CARCINOMA?

494 Background: Renal cell carcinoma (RCC) with rhabdoid differentiation is thought to portend a poor prognosis, similar to RCC with sarcomatoid differentiation. Both rhabdoid and sarcomatoid differentiation are classified as grade 4 RCC based on the most recent International Society of Urological Pathology (ISUP) grading system. We sought to determine the prognostic value of rhabdoid differentiation in comparison to RCC with sarcomatoid differentiation, grade 4 RCC without rhabdoid or sarcomatoid differentiation, and grade 3 RCC. Methods: Using the Mayo Clinic Nephrectomy Registry, we identified 406 patients with ISUP grade 4 RCC and 1,758 patients with grade 3 RCC. A urologic pathologist reviewed all specimens to determine the presence of both rhabdoid and sarcomatoid differentiation. Associations of clinical and pathologic features with death from RCC were evaluated using Cox models. Results: Among the 406 grade 4 RCC tumors, 111 (27%) had rhabdoid differentiation and 189 (47%) had sarcomatoid differentiation, although only 28 (7%) demonstrated both rhabdoid and sarcomatoid differentiation. In multivariable analysis of grade 4 RCC tumors, the presence of rhabdoid differentiation was not associated with death from RCC (HR 0.95, p=0.75); in contrast, sarcomatoid differentiation was significantly associated with death from RCC (HR 1.63, p<0.001). Patients with RCC with rhabdoid differentiation were significantly more likely to die of RCC than patients with grade 3 RCC (HR 2.45, p<0.001) and grade 3 RCC with necrosis (HR 1.62; p<0.001). Conclusions: This study confirms that RCC with rhabdoid differentiation is appropriately classified as grade 4. However, unlike sarcomatoid differentiation, the presence of rhabdoid differentiation in grade 4 RCC is not associated with an increased risk of death from RCC. Therefore, rhabdoid and sarcomatoid differentiation should not be grouped together when assessing risk in a patient with grade 4 RCC.

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Chromophobe renal cell carcinoma with rhabdoid differentiation in an adult

Wiener klinische Wochenschrift ◽

10.1007/s00508-012-0189-y ◽

2012 ◽

Vol 124 (11-12) ◽

pp. 419-421 ◽

Cited By ~ 7

Author(s):

Iva Brčić ◽

Borislav Spajić ◽

Božo Krušlin

Keyword(s):

Renal Cell Carcinoma ◽

Cell Carcinoma ◽

Renal Cell ◽

Chromophobe Renal Cell Carcinoma ◽

Rhabdoid Differentiation

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Adult Rhabdoid Renal Cell Carcinoma

Archives of Pathology & Laboratory Medicine ◽

10.5858/2002-126-1506-arrcc ◽

2002 ◽

Vol 126 (12) ◽

pp. 1506-1510 ◽

Cited By ~ 4

Author(s):

Beverley Shannon ◽

Zdzisilaw Stan Wisniewski ◽

Jacqueline Bentel ◽

Ronald J. Cohen

Keyword(s):

Renal Cell Carcinoma ◽

Cell Carcinoma ◽

Renal Cell ◽

Clear Cell ◽

Clear Cell Carcinoma ◽

Cell Tumor ◽

Clear Cell Tumor ◽

Clonal Origin ◽

Rhabdoid Tumors ◽

Rhabdoid Differentiation

Abstract 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.

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