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Presented by Dr. Pedram Argani and prepared by Dr. Tricia Cottrell
This is 5 month old male with a renal mass
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1. Question
This is 5 month old male with a renal mass
Correct
Answer: C. Clear cell sarcoma of the kidney
Histology: This is a fairly uniform neoplasm composed of spindle to epithelioid cells with inconspicuous cytoplasm and prominent clear extracellular matrix material, simulating the appearance of clear cytoplasm. Cells are separated by regular branching capillary vasculature. At the edge of the lesion, the neoplasm entraps single nephrons. The cytology of the nuclei is bland, with open chromatin associated nuclear grooves. These are the typical features of clear cell sarcoma of the kidney. The patient age in this case is more typical of congenital mesoblastic nephroma, but CCSK can occur in this age group.
Discussion: Congenital mesoblastic nephroma is divided into classic (fibromatous like) or cellular (fibrosarcoma like) types. Aside from differences in cytology, classic CMN has a widely infiltrative broader, whereas cellular CMN is typically associated with high mitotic activity and necrosis. Rhabdoid tumor of the kidney typically features a characteristic cytologic triad; vesicular chromatin, prominent nucleus, and hyaline cytoplasmic inclusions. Rhabdoid tumor of the kidney demonstrates loss if INI1 protein.
Clear cell sarcoma of the kidney is characterized by internal tandem duplication of the BCOR gene in the majority of cases. Approximately 5-10% of CCSKs harbor the recurring chromosome translocation t(10;17) (q22;p13), resulting YWHAE-FAM22 gene fusion. The same gene fusion is also found in a subset of high grade endometrial stromal sarcomas.
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Answer: C. Clear cell sarcoma of the kidney
Histology: This is a fairly uniform neoplasm composed of spindle to epithelioid cells with inconspicuous cytoplasm and prominent clear extracellular matrix material, simulating the appearance of clear cytoplasm. Cells are separated by regular branching capillary vasculature. At the edge of the lesion, the neoplasm entraps single nephrons. The cytology of the nuclei is bland, with open chromatin associated nuclear grooves. These are the typical features of clear cell sarcoma of the kidney. The patient age in this case is more typical of congenital mesoblastic nephroma, but CCSK can occur in this age group.
Discussion: Congenital mesoblastic nephroma is divided into classic (fibromatous like) or cellular (fibrosarcoma like) types. Aside from differences in cytology, classic CMN has a widely infiltrative broader, whereas cellular CMN is typically associated with high mitotic activity and necrosis. Rhabdoid tumor of the kidney typically features a characteristic cytologic triad; vesicular chromatin, prominent nucleus, and hyaline cytoplasmic inclusions. Rhabdoid tumor of the kidney demonstrates loss if INI1 protein.
Clear cell sarcoma of the kidney is characterized by internal tandem duplication of the BCOR gene in the majority of cases. Approximately 5-10% of CCSKs harbor the recurring chromosome translocation t(10;17) (q22;p13), resulting YWHAE-FAM22 gene fusion. The same gene fusion is also found in a subset of high grade endometrial stromal sarcomas.
