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Presented by Dr. Epstein and prepared by Dr. J. David Peske.
Case 3. A 20 year-old man underwent a radical nephrectomy for a 7.2 cm renal mass.
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Case 3. A 20 year-old man underwent a radical nephrectomy for a 7.2 cm renal mass.
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Histological Description: At low magnification there is a large lobular tumor which is poorly circumscribed with extension into the surrounding renal parenchyma. The cells are arranged in sheets with high N/C ratio, speckled chromatin and inconspicuous nucleoli. Mitotic figures are present but not very prominent. Homer Wright rosettes are identifiable consisting of differentiated tumor cells grouped around a central region containing neuropil. The tumor falls apart giving rise to a pseudopapillary growth pattern.
Answer: CDiscussion: Primary Ewing sarcoma / primitive neuroectodermal tumor (ES) of the kidney are rare. The patients have no gender preference and a wide age range from children to the elderly with an average age of around 30 years. The average tumor size at presentation is large, approximately 12 cm. (range, 5-20 cm). The tumors often invade the renal sinus or perinephric fat or renal vein and less commonly the adrenal gland. Almost all patients develop metastases, commonly in the lungs and bone with a majority of them dead of disease in a mean of 21 months, although there are some long-term survivors. On a morphological basis, the presence of Homer Wright rosettes favors PNET compared to other tumors excluding neuroblastomas. In this case, the tumor most closely resembled a solid variant of papillary renal cell carcinoma. The work up of this case demonstrated negative cytokeratin 7, AE1/3, synaptophysin, WT-1, and PAX8, ruling out renal cell carcinoma, Wilms tumor, and neuroblastoma. A CD99 immunostain was positive as was NKX2.2, verifying the diagnosis of PNET. NKX2.2 is expressed in 80% of PNETs mostly with diffuse and strong staining pattern, and a minority of other tumors including olfactory neuroblastomas, extraskeletal myxoid chondrosarcoma, mesenchymal chondrosarcoma, small cell carcinomas, and Merkel cell carcinoma. When combined with CD99, the specificity of NKX2.2 increases to 98%.
Primary Ewing Sarcoma / Primitive Neuroectodermal Tumor of the Kidney: A Clinicopathologic Study of 23 Cases. Pathol Oncol Res. 2018 Jan;24(1):153-159.
Murugan P, Rao P, Tamboli P, Czerniak B, Guo CC.Incorrect
Histological Description: At low magnification there is a large lobular tumor which is poorly circumscribed with extension into the surrounding renal parenchyma. The cells are arranged in sheets with high N/C ratio, speckled chromatin and inconspicuous nucleoli. Mitotic figures are present but not very prominent. Homer Wright rosettes are identifiable consisting of differentiated tumor cells grouped around a central region containing neuropil. The tumor falls apart giving rise to a pseudopapillary growth pattern.
Answer: CDiscussion: Primary Ewing sarcoma / primitive neuroectodermal tumor (ES) of the kidney are rare. The patients have no gender preference and a wide age range from children to the elderly with an average age of around 30 years. The average tumor size at presentation is large, approximately 12 cm. (range, 5-20 cm). The tumors often invade the renal sinus or perinephric fat or renal vein and less commonly the adrenal gland. Almost all patients develop metastases, commonly in the lungs and bone with a majority of them dead of disease in a mean of 21 months, although there are some long-term survivors. On a morphological basis, the presence of Homer Wright rosettes favors PNET compared to other tumors excluding neuroblastomas. In this case, the tumor most closely resembled a solid variant of papillary renal cell carcinoma. The work up of this case demonstrated negative cytokeratin 7, AE1/3, synaptophysin, WT-1, and PAX8, ruling out renal cell carcinoma, Wilms tumor, and neuroblastoma. A CD99 immunostain was positive as was NKX2.2, verifying the diagnosis of PNET. NKX2.2 is expressed in 80% of PNETs mostly with diffuse and strong staining pattern, and a minority of other tumors including olfactory neuroblastomas, extraskeletal myxoid chondrosarcoma, mesenchymal chondrosarcoma, small cell carcinomas, and Merkel cell carcinoma. When combined with CD99, the specificity of NKX2.2 increases to 98%.
Primary Ewing Sarcoma / Primitive Neuroectodermal Tumor of the Kidney: A Clinicopathologic Study of 23 Cases. Pathol Oncol Res. 2018 Jan;24(1):153-159.
Murugan P, Rao P, Tamboli P, Czerniak B, Guo CC.
