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Presented by Dr. Pedram Argani and prepared by Austin McCuiston
This is 36 year old female with a prior history of malignancy and lung mass.
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This is 36 year old female with a prior history of malignancy and lung mass.
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Histologic Description: This neoplasm consists of nests of polygonal cells with clear to eosinophilic cytoplasm and nuclei with prominent nucleoli. The cells are somewhat discohesive within the nests, leading to an alveolar pattern. The cytoplasm is variably granular. The neoplasm was diffusely and strongly immunoreactive for TFE3. This patient previously had an alveolar soft part sarcoma of the thigh, 15 years prior to this surgery.
Differential Diagnosis: The other neoplasms listed in the differential diagnosis all can have an alveolar pattern. Renal cell carcinoma would be expected to label for PAX8 and not TFE3 unless it were Xp11 translocation carcinoma. The current neoplasm was negative for PAX8. Paraganglioma would label for neuroendocrine markers like chromogranin and synaptophysin, but not for TFE3. Granular cell tumors may show weak labeling for TFE3 but do not demonstrate TFE3 gene rearrangements, are diffusely reactive for S100 protein, and frequently label for inhibin. Only a minority of granular cell tumors demonstrate malignant behavior, whereas alveolar soft part sarcoma frequently metastasizes years after the diagnosis.
Incorrect
Histologic Description: This neoplasm consists of nests of polygonal cells with clear to eosinophilic cytoplasm and nuclei with prominent nucleoli. The cells are somewhat discohesive within the nests, leading to an alveolar pattern. The cytoplasm is variably granular. The neoplasm was diffusely and strongly immunoreactive for TFE3. This patient previously had an alveolar soft part sarcoma of the thigh, 15 years prior to this surgery.
Differential Diagnosis: The other neoplasms listed in the differential diagnosis all can have an alveolar pattern. Renal cell carcinoma would be expected to label for PAX8 and not TFE3 unless it were Xp11 translocation carcinoma. The current neoplasm was negative for PAX8. Paraganglioma would label for neuroendocrine markers like chromogranin and synaptophysin, but not for TFE3. Granular cell tumors may show weak labeling for TFE3 but do not demonstrate TFE3 gene rearrangements, are diffusely reactive for S100 protein, and frequently label for inhibin. Only a minority of granular cell tumors demonstrate malignant behavior, whereas alveolar soft part sarcoma frequently metastasizes years after the diagnosis.
