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Presented by Dr. Pedram Argani and prepared by Dr. Robby Jones
Case 2: A 24 year old male with a renal mass.
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1. Question
Case 2: A 24 year old male with a renal mass.
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Answer: C. Inflammatory myofibroblastic tumor
Histologic Description: This is a spindle cell lesion composed of relatively uniform spindle cells with abundant admixed lymphocytes and plasma cells. The spindle cells form relatively loosely fascicles, with abundant intervening inflammation. These are the typical morphologic features of inflammatory myofibroblastic tumor.
Differential Diagnosis: Sarcomatoid carcinoma would be expected to demonstrate greater pleomorphism, be associated with identifiable epithelial component, and demonstrate immunoreactivity for cytokeratin. Leiomyosarcoma would form tighter fascicles, demonstrate more pleomorphism, and demonstrate densely eosinophilic cytoplasm. Xanthogranulomatous pyelonephritis typically has a more epithelioid appearance, with the histiocytes mimicking the clear cells of clear cell RCC.
Not all inflammatory myofibroblastic tumors demonstrate ALK gene rearrangements. A subset will instead demonstrate the ETV6-NTRK3 gene fusion found in infantile fibrosarcoma, ROS1 gene fusions, and other alterations.
Reference Am J Surg Pathol 2016; 40:1051-1061.Incorrect
Answer: C. Inflammatory myofibroblastic tumor
Histologic Description: This is a spindle cell lesion composed of relatively uniform spindle cells with abundant admixed lymphocytes and plasma cells. The spindle cells form relatively loosely fascicles, with abundant intervening inflammation. These are the typical morphologic features of inflammatory myofibroblastic tumor.
Differential Diagnosis: Sarcomatoid carcinoma would be expected to demonstrate greater pleomorphism, be associated with identifiable epithelial component, and demonstrate immunoreactivity for cytokeratin. Leiomyosarcoma would form tighter fascicles, demonstrate more pleomorphism, and demonstrate densely eosinophilic cytoplasm. Xanthogranulomatous pyelonephritis typically has a more epithelioid appearance, with the histiocytes mimicking the clear cells of clear cell RCC.
Not all inflammatory myofibroblastic tumors demonstrate ALK gene rearrangements. A subset will instead demonstrate the ETV6-NTRK3 gene fusion found in infantile fibrosarcoma, ROS1 gene fusions, and other alterations.
Reference Am J Surg Pathol 2016; 40:1051-1061.
