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Presented by Jonathan Epstein, M.D. and prepared by Dengfeng Cao, M.D. Ph.D.
Case 1: A 67-year-old man presented with a mass where it was difficult to distinguish whether it was arising from the kidney or whether it was surrounding the kidney.
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1. Question
Week 218: Case 1
A 67-year-old man presented with a mass where it was difficult to distinguish whether it was arising from the kidney or whether it was surrounding the kidney./images/DengfengCao/Cao_041105_case1a.jpg
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/images/DengfengCao/Cao_041105_case1e.jpgCorrect
Answer: Dedifferentiated liposarcoma
Histology: Surrounding the kidneys is a mass variably composed of hypocellular fibrous tissue, adipose tissue, and more cellular fibrous-appearing tissue arranged in whorls around blood vessels. Focally within the hypocellular fibrous tissue adjacent to the adipose tissue are cells with hyperchromatic enlarged nuclei. The cells within the whorls show moderate pleomorphism with scattered mitotic figures. The patchy areas of chronic inflammation are associated with foamy macrophages.
Discussion: This case was initially sampled on frozen section at the time of laparoscopic surgery where the clinicians were unsure whether the tissue was from within the kidney or extrarenal. The presence of a cellular spindle cell component with some atypia seen on frozen section raised the question of sarcomatoid renal cell carcinoma. However, given the lack of identifiable epithelial renal cell carcinoma components, the frozen section was deferred as malignant spindle cell tumor. The surgery was converted to an open radical nephrectomy where the mass was shown to be entirely perinephric, ruling out sarcomatoid renal cell carcinoma. Idiopathic retroperitoneal fibrosis may show patchy areas of chronic inflammation as seen in this case, yet it lacks the distinctive whorls of more cellular spindle cells seen in this case. Similarly, fibromatosis consists of fascicles of hypocellular fibrous tissue without prominent inflammation or areas of cellular spindle cells. The distinctive feature in this case is the perivascular whorls of spindle cells. These spindle cells show some atypia with enlarged nuclei, prominent nucleoli, and scattered mitotic figures. Once one recognizes that these hypercellular spindled to ovoid cells arranged in tight consistent whorls are typical of dedifferentiated liposarcoma, one notes that in the areas of more hypocellular fibrous tissue admixed with adipose tissue there are scattered enlarged atypical hyperchromatic cells, diagnostic of a well-differentiated liposarcoma component.
Reference(s):
– Am J Surg Pathol 1998;22:945-55 and Histopathology 1998;33:414-424.Incorrect
Answer: Dedifferentiated liposarcoma
Histology: Surrounding the kidneys is a mass variably composed of hypocellular fibrous tissue, adipose tissue, and more cellular fibrous-appearing tissue arranged in whorls around blood vessels. Focally within the hypocellular fibrous tissue adjacent to the adipose tissue are cells with hyperchromatic enlarged nuclei. The cells within the whorls show moderate pleomorphism with scattered mitotic figures. The patchy areas of chronic inflammation are associated with foamy macrophages.
Discussion: This case was initially sampled on frozen section at the time of laparoscopic surgery where the clinicians were unsure whether the tissue was from within the kidney or extrarenal. The presence of a cellular spindle cell component with some atypia seen on frozen section raised the question of sarcomatoid renal cell carcinoma. However, given the lack of identifiable epithelial renal cell carcinoma components, the frozen section was deferred as malignant spindle cell tumor. The surgery was converted to an open radical nephrectomy where the mass was shown to be entirely perinephric, ruling out sarcomatoid renal cell carcinoma. Idiopathic retroperitoneal fibrosis may show patchy areas of chronic inflammation as seen in this case, yet it lacks the distinctive whorls of more cellular spindle cells seen in this case. Similarly, fibromatosis consists of fascicles of hypocellular fibrous tissue without prominent inflammation or areas of cellular spindle cells. The distinctive feature in this case is the perivascular whorls of spindle cells. These spindle cells show some atypia with enlarged nuclei, prominent nucleoli, and scattered mitotic figures. Once one recognizes that these hypercellular spindled to ovoid cells arranged in tight consistent whorls are typical of dedifferentiated liposarcoma, one notes that in the areas of more hypocellular fibrous tissue admixed with adipose tissue there are scattered enlarged atypical hyperchromatic cells, diagnostic of a well-differentiated liposarcoma component.
Reference(s):
– Am J Surg Pathol 1998;22:945-55 and Histopathology 1998;33:414-424.
