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Presented by Dr. Pedram Argani and prepared by Dr. J. Judd Fite.
This is a 46 year old female with a cystic renal tumor.
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1. Question
This is a 46 year old female with a cystic renal tumor.
Correct
Answer: A. Epithelioid angiomyolipoma with epithelial cysts
Histologic Description: This is an extremely unusual case. The lesion is extensively cystic, with the cysts lined by entrapped native tubular epithelium that labels for PAX8. The cells in between the epithelial cysts demonstrate pleomorphism, but minimal mitotic activity. They are associated with dysplastic blood vessels. Focally, beneath the epithelium, one can appreciate a more primitive appearing “bluer” component. These are the typical features of angiomyolipoma with epithelial cysts (AMLEC). The current case is unusual in that the angiomyolipoma component is predominantly epithelioid and demonstrates cytologic atypia. Therefore, this lesion has potential for aggressive behavior, and the patient should be followed closely.
Differential Diagnosis: Renal synovial sarcoma can be extensively cystic like the current case. However, synovial sarcoma features monomorphic bland spindle cells, and would not label for HMB45 like the current lesion. Mixed epithelial stromal tumor could also have extensively cystic architecture like the current lesion. However, the intervening cellular stroma is predominantly composed of bland smooth muscle cells or fibrous tissue, and would not demonstrate the features of angiomyolipoma such as dysplastic vessels and melanocytic marker immunoreactivity. Tubulocystic carcinoma is an extensively cystic low grade renal carcinoma with a characteristic “bubble-wrap” appearance. This lesion is extensively cystic, the cysts are lined by oncocytic cells demonstrating nuclear irregularities, and the stroma has a fibroelastotic appearance, unlike the cellular stroma with atypia seen in the current lesion.
Angiomyolipoma with epithelial cysts contains 3 components. First there is a peripheral muscle prominent angiomyolipoma that stains strongly for muscle markers like actin and focally for HMB45. Second there is an inner cellular stroma which demonstrate stronger immunoreactivity for HMB45 and ER/PR. Finally, there is entrapped renal tubular epithelium that labels for PAX8.
Reference Fine S et a.al Am J Surg Pathol 2006; 30:593-599.
Incorrect
Answer: A. Epithelioid angiomyolipoma with epithelial cysts
Histologic Description: This is an extremely unusual case. The lesion is extensively cystic, with the cysts lined by entrapped native tubular epithelium that labels for PAX8. The cells in between the epithelial cysts demonstrate pleomorphism, but minimal mitotic activity. They are associated with dysplastic blood vessels. Focally, beneath the epithelium, one can appreciate a more primitive appearing “bluer” component. These are the typical features of angiomyolipoma with epithelial cysts (AMLEC). The current case is unusual in that the angiomyolipoma component is predominantly epithelioid and demonstrates cytologic atypia. Therefore, this lesion has potential for aggressive behavior, and the patient should be followed closely.
Differential Diagnosis: Renal synovial sarcoma can be extensively cystic like the current case. However, synovial sarcoma features monomorphic bland spindle cells, and would not label for HMB45 like the current lesion. Mixed epithelial stromal tumor could also have extensively cystic architecture like the current lesion. However, the intervening cellular stroma is predominantly composed of bland smooth muscle cells or fibrous tissue, and would not demonstrate the features of angiomyolipoma such as dysplastic vessels and melanocytic marker immunoreactivity. Tubulocystic carcinoma is an extensively cystic low grade renal carcinoma with a characteristic “bubble-wrap” appearance. This lesion is extensively cystic, the cysts are lined by oncocytic cells demonstrating nuclear irregularities, and the stroma has a fibroelastotic appearance, unlike the cellular stroma with atypia seen in the current lesion.
Angiomyolipoma with epithelial cysts contains 3 components. First there is a peripheral muscle prominent angiomyolipoma that stains strongly for muscle markers like actin and focally for HMB45. Second there is an inner cellular stroma which demonstrate stronger immunoreactivity for HMB45 and ER/PR. Finally, there is entrapped renal tubular epithelium that labels for PAX8.
Reference Fine S et a.al Am J Surg Pathol 2006; 30:593-599.
