Head and Neck: Practice Question

The genetic abnormality seen in hyalinizing clear cell carcinoma of salivary glands is also characteristic of which of the following neoplastic entities?
  1. Malignant melanoma
  2. Myxoid liposarcoma
  3. Clear cell sarcoma of soft tissue
  4. Dedifferentiated liposarcoma
  5. Ewing sarcoma
Show Answer and Explanations

Malignant melanoma

Incorrect. Melanoma is not associated with EWSR1-ATF1 gene fusion.

Myxoid liposarcoma

Incorrect. Myxoid liposarcoma is characterized by a pathognomonic t(12;16)(q13;p11) translocation that produces a fusion oncoprotein, FUS-CHOP.

Clear cell sarcoma of soft tissue — Correct answer

Correct. EWSR1-ATF1 gene fusion is also present in clear cell sarcoma of soft tissue.

Dedifferentiated liposarcoma

Incorrect. Dedifferentiated liposarcoma is characterized by MDM2 amplification.

Ewing sarcoma

Incorrect. Ewing sarcoma is characterized by fusions between EWSR1 and members of the ETS gene family, usually EWSR1-FLI1.

Summary

Summary: Clear cell carcinoma (hyalinizing clear cell carcinoma) of salivary glands and clear cell sarcoma of soft tissue have the EWSR1-ATF1 gene fusion.
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