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6 photo(s) Updated on: 10/19/2022

Angiomyolipoma, oncocytoma-like

Answer: C - Angiomyolipoma, oncocytoma-like

Angiomyolipoma is a benign mesenchymal tumor composed of varying portions of mature adipose tissue, spindle cells, smooth muscle and thick-walled vessels. These tumors are considered to be members of the Perivascular epithelioid cell tumor (PEComa) family. They may be sporadic, or associated with tuberous sclerosis. They can arise in the kidney cortex or medulla, as well as in the retroperitoneal soft tissue. 

Macroscopically, the tumor is usually well-delineated, but not encapsulated. The color of the tumor is dependent of the quantity of its various components. Oncocytoma-like AML are rare tumors composed of homogeneous components of polygonal cells with strongly eosinophilic cytoplasm. Tumor cells express melanocytic markers (HMB45, Malan-A, MITF and Cathepsin K), and show variable expression of smooth muscle markers (SMA and calponin). They are negative for PAX2, PAX8 and epithelial markers. Genetic alterations of TSC1 and TSC2 have been reported. 

Regarding the other listed diagnostic options, absence of PAX8 expression does not support oncocytoma, LOT, EVT or chromophobe RCC. Among these other choices, Cathepsin-K, is only expressed in EVT; however EVT does not form a lipomatous component. Additionally, lack of CK7 expression argues against chromophobe RCC. 


Reference

WHO Classification of Tumours of the Urinary System and Male Genital Organs (5th edition). IARC: Lyon 2022. 

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