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Presented by Dr. Pedram Argani and prepared by Dr. Sintawat Wangsiricharoen.
This is a 40-year-old male with a large renal mass growing into the renal vein.
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Answer: A. Clear cell renal cell carcinoma
Histologic Description: This is a clear cell neoplasm with nested and papillary architecture. The cells have low grade nuclei, clear cytoplasm, and prominent subnuclear vacuolization, which suggest the diagnosis of clear cell papillary RCC. However, one can see on the section that the neoplastic cells are protruding into the renal sinus, and clinically were growing in the renal vein. Other portions of this tumor demonstrate solid acinar growth with a highly vascularized stroma with prominent septal capillaries, along with extruded blood and fibrin, which is much more typical of clear cell RCC. By immunohistochemistry, the neoplastic cells were diffusely positive for CA-IX and CD10, minimally positive for cytokeratin 7, and negative for TFE3 and cytokeratin 903. These results support the diagnosis of clear cell RCC which has areas mimicking clear cell papillary RCC.
Differential Diagnosis: Clear cell papillary RCC is typically an indolent neoplasm, and it would be very unusual for it to have renal vein involvement. Clear cell papillary RCC would label for cytokeratin 7 and would have a cup-shaped distribution of CA-IX labeling, and typically does not label much for CD10 but does label for cytokeratin 903. Xp11 translocation RCC (particularly those with the SFPQ or NONO gene TFE3 fusions) may closely mimic clear papillary RCC in that they demonstrate subnuclear vacuoles and clear cells with papillary architecture; however, these neoplasms should demonstrate TFE3 labeling, and should not show diffuse CA-IX labeling. Papillary RCC may have tubular areas, but would not have the water clear cytoplasm of the current lesions, and would label for cytokeratin 7.
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Answer: A. Clear cell renal cell carcinoma
Histologic Description: This is a clear cell neoplasm with nested and papillary architecture. The cells have low grade nuclei, clear cytoplasm, and prominent subnuclear vacuolization, which suggest the diagnosis of clear cell papillary RCC. However, one can see on the section that the neoplastic cells are protruding into the renal sinus, and clinically were growing in the renal vein. Other portions of this tumor demonstrate solid acinar growth with a highly vascularized stroma with prominent septal capillaries, along with extruded blood and fibrin, which is much more typical of clear cell RCC. By immunohistochemistry, the neoplastic cells were diffusely positive for CA-IX and CD10, minimally positive for cytokeratin 7, and negative for TFE3 and cytokeratin 903. These results support the diagnosis of clear cell RCC which has areas mimicking clear cell papillary RCC.
Differential Diagnosis: Clear cell papillary RCC is typically an indolent neoplasm, and it would be very unusual for it to have renal vein involvement. Clear cell papillary RCC would label for cytokeratin 7 and would have a cup-shaped distribution of CA-IX labeling, and typically does not label much for CD10 but does label for cytokeratin 903. Xp11 translocation RCC (particularly those with the SFPQ or NONO gene TFE3 fusions) may closely mimic clear papillary RCC in that they demonstrate subnuclear vacuoles and clear cells with papillary architecture; however, these neoplasms should demonstrate TFE3 labeling, and should not show diffuse CA-IX labeling. Papillary RCC may have tubular areas, but would not have the water clear cytoplasm of the current lesions, and would label for cytokeratin 7.