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Presented by Dr. Jonathan Epstein and prepared by Dr. Monica Butcher
A 43 year old woman underwent a resection for a 1.5 cm mass in the kidney.
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1. Question
A 43 year old woman underwent a resection for a 1.5 cm mass in the kidney.
Choose the correct diagnosis:
Correct
Answer: C
Histological description: There is a circumscribed lesion with small solid sheets and nests of cells with granular eosinophilic cells with numerous vacuoles in a myxoid background. Whereas some nuclei are round, others are variably shaped and some have notches with all nuclei having relatively prominent nucleoli.
Discussion: This entity has also been described as “high-grade oncocytic tumor (HOT)”, based on the cytology. However since this tumor has an indolent course based on the limited number of cases reported to date, this terminology is misleading and the more descriptive term EVT is preferred. The neoplastic cells are immunoreactive for PAX8, AE1/ AE3, and often for cathepsin K. CK7 is variable, from negative to focally positive. EVT often demonstrates CD117+/CK 20- profile, different from the CD117−/CK20+ profile seen in ESC RCC. The tumor has more irregular nuclei than oncocytoma and also lacks the clumped cytoplasmic granules and frequent cystic features seen in ESC. However, there are some similarities with ESC in that they share losses of TSC2 and TSC1 and activation of the MTOR pathway.
Incorrect
Answer: C
Histological description: There is a circumscribed lesion with small solid sheets and nests of cells with granular eosinophilic cells with numerous vacuoles in a myxoid background. Whereas some nuclei are round, others are variably shaped and some have notches with all nuclei having relatively prominent nucleoli.
Discussion: This entity has also been described as “high-grade oncocytic tumor (HOT)”, based on the cytology. However since this tumor has an indolent course based on the limited number of cases reported to date, this terminology is misleading and the more descriptive term EVT is preferred. The neoplastic cells are immunoreactive for PAX8, AE1/ AE3, and often for cathepsin K. CK7 is variable, from negative to focally positive. EVT often demonstrates CD117+/CK 20- profile, different from the CD117−/CK20+ profile seen in ESC RCC. The tumor has more irregular nuclei than oncocytoma and also lacks the clumped cytoplasmic granules and frequent cystic features seen in ESC. However, there are some similarities with ESC in that they share losses of TSC2 and TSC1 and activation of the MTOR pathway.
