Quiz-summary
0 of 1 questions completed
Questions:
- 1
Information
Presented by Dr. Andres Matoso and prepared by Dr. Yembur Ahmad
This case talks about a 30 year old female with a kidney mass.
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
- Kidney 0%
- 1
- Answered
- Review
-
Question 1 of 1
1. Question
A 30 year old female presents with a kidney mass.
What is the diagnosis?
Correct
Correct: B
Histology: This tumor is composed of large nests with papillary structures lines by clear cells with large nuclei. There are stromal septa composed of smooth muscle separating the tumor nests.
Discussion: This is translocation RCC involving region Xp11.2, resulting in fusion of the TFE3 gene. While they represent approximately 40% of pediatric RCCs, they can also develop in adults. Microscopically, the overall features are typically those of an RCC but are very heterogeneous. Papillary structures may be prominent, and the tumor cells can be either clear or have a markedly granular eosinophilic cytoplasm; however, some cases are more solid and clear cell predominant, closely mimicking clear cell RCC. Admixed psammoma bodies may be abundant (not present in the current case). The presence of large nests with prominent papillary structures lined by clear cells and separated by thick stromal septa could be a morphologic clue to the diagnosis. By immunohistochemistry, a proportion of these tumors express PAX8 and Cathepsin-K, and can be negative or weakly positive for cytokeratins.
References:
Am J Surg Pathol. 40:723-737 2016
Am J Clin Pathol. 126 (3):332-334 2006Incorrect
Correct: B
Histology: This tumor is composed of large nests with papillary structures lines by clear cells with large nuclei. There are stromal septa composed of smooth muscle separating the tumor nests.
Discussion: This is translocation RCC involving region Xp11.2, resulting in fusion of the TFE3 gene. While they represent approximately 40% of pediatric RCCs, they can also develop in adults. Microscopically, the overall features are typically those of an RCC but are very heterogeneous. Papillary structures may be prominent, and the tumor cells can be either clear or have a markedly granular eosinophilic cytoplasm; however, some cases are more solid and clear cell predominant, closely mimicking clear cell RCC. Admixed psammoma bodies may be abundant (not present in the current case). The presence of large nests with prominent papillary structures lined by clear cells and separated by thick stromal septa could be a morphologic clue to the diagnosis. By immunohistochemistry, a proportion of these tumors express PAX8 and Cathepsin-K, and can be negative or weakly positive for cytokeratins.
References:
Am J Surg Pathol. 40:723-737 2016
Am J Clin Pathol. 126 (3):332-334 2006
