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Presented by Dr. Argani and prepared by J. Stephen Nix
This is a 7 year old female with a thigh mass.
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1. Question
This is a 7 year old female with a thigh mass.
Correct
Answer: B
Histologic Description: This is a nested neoplasm composed of epithelioid cells with clear cytoplasm. The nuclei are hyperchromatic and have irregular contours, and mitotic figures are relatively easy to identify. The stroma is sclerotic, and there are areas of necrosis. The neoplasm demonstrated diffuse immunoreactivity for S100 protein, and patchy staining for cytokeratins Cam5.2 and AE13. Sequencing revealed and EWSR1-POU5F1 gene fusion, consistent with myoepithelial carcinoma.
Differential Diagnosis: Clear cell hidradenoma would lack the atypia seen in the current case. The lesional cells have clear cytoplasm, and should resemble those of benign urothelium. Epithelioid sarcoma would typically demonstrate a more solid architecture associated with necrosis, and would demonstrate diffuse immunoreactivity for cytokeratin. Clear cell sarcoma of tendon sheath would label for S100, but should also label more strongly for HMB45 and not label for cytokeratins.
The distinction between myoepithelioma and myoepithelial carcinoma in soft tissue is based upon cytologic atypia, not invasion as is used in the salivary gland.Incorrect
Answer: B
Histologic Description: This is a nested neoplasm composed of epithelioid cells with clear cytoplasm. The nuclei are hyperchromatic and have irregular contours, and mitotic figures are relatively easy to identify. The stroma is sclerotic, and there are areas of necrosis. The neoplasm demonstrated diffuse immunoreactivity for S100 protein, and patchy staining for cytokeratins Cam5.2 and AE13. Sequencing revealed and EWSR1-POU5F1 gene fusion, consistent with myoepithelial carcinoma.
Differential Diagnosis: Clear cell hidradenoma would lack the atypia seen in the current case. The lesional cells have clear cytoplasm, and should resemble those of benign urothelium. Epithelioid sarcoma would typically demonstrate a more solid architecture associated with necrosis, and would demonstrate diffuse immunoreactivity for cytokeratin. Clear cell sarcoma of tendon sheath would label for S100, but should also label more strongly for HMB45 and not label for cytokeratins.
The distinction between myoepithelioma and myoepithelial carcinoma in soft tissue is based upon cytologic atypia, not invasion as is used in the salivary gland.
