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Presented by Pedram Argani, MD and prepared by Armen Khararjian, MD
Case 2: This is a 33 year old female with a cervical mass.
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Question 1 of 1
1. Question
Week 609: Case 2
This is a 33 year old female with a cervical mass.
Correct
Answer: Alveolar Soft Part Sarcoma
Histology: The biphasic “blue” spindle and glandular tissue in this specimen represents normal proliferative endometrium. The neoplasm is a nested proliferation of polygonal epithelioid cells with prominent nucleoli and abundant granular cytoplasm. The neoplastic cells label for cathepsin k, but are negative for HMB45, Melan A and S100 protein. They demonstrated TFE3 gene rearrangement by FISH. This supports the diagnosis of alveolar soft part sarcoma.
Discussion: Differential Diagnosis: Granular cell tumors frequently show nonspecific weak immunohistochemical labeling for TFE3, but lack TFE3 gene rearrangements. Most PEComas lack TFE3 gene rearrangement. A small subset of PEComas does demonstrate TFE3 gene rearrangements; however, these neoplasms typically have epithelioid clear cells and label for melanocytic markers like HMB45. Hence, the best diagnosis for this lesion is alveolar soft part sarcoma.
Alveolar soft part sarcomas contain a unbalanced derX t(X:17) (p11.2;q25) chromosome translocation, resulting in an ASPL-TFE3 gene fusion. This results in overexpression of TFE3 fusion proteins, and can be detected by break-apart TFE3 FISH assays.
Incorrect
Answer: Alveolar Soft Part Sarcoma
Histology: The biphasic “blue” spindle and glandular tissue in this specimen represents normal proliferative endometrium. The neoplasm is a nested proliferation of polygonal epithelioid cells with prominent nucleoli and abundant granular cytoplasm. The neoplastic cells label for cathepsin k, but are negative for HMB45, Melan A and S100 protein. They demonstrated TFE3 gene rearrangement by FISH. This supports the diagnosis of alveolar soft part sarcoma.
Discussion: Differential Diagnosis: Granular cell tumors frequently show nonspecific weak immunohistochemical labeling for TFE3, but lack TFE3 gene rearrangements. Most PEComas lack TFE3 gene rearrangement. A small subset of PEComas does demonstrate TFE3 gene rearrangements; however, these neoplasms typically have epithelioid clear cells and label for melanocytic markers like HMB45. Hence, the best diagnosis for this lesion is alveolar soft part sarcoma.
Alveolar soft part sarcomas contain a unbalanced derX t(X:17) (p11.2;q25) chromosome translocation, resulting in an ASPL-TFE3 gene fusion. This results in overexpression of TFE3 fusion proteins, and can be detected by break-apart TFE3 FISH assays.
