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Primary paranasal sinus hyalinizing clear cell carcinoma: a case report
Batool M AlAli1, Mohammed J Alyousef2,3, Ahmad Salah Kamel1,4
1Department of Pathology and Laboratory Medicine, King Fahd Hospital of University, Khobar, Saudi Arabia.
Diagnostic Pathology
|September 27, 2017
Summary
Hyalinizing clear cell carcinoma (HCCC) is a rare salivary gland tumor. This case highlights an unusual presentation of HCCC in the paranasal sinus, confirmed by EWSR1 gene rearrangement.
Area of Science:
- Oncology
- Pathology
- Head and Neck Surgery
Background:
- Hyalinizing clear cell carcinoma (HCCC) is a rare, low-grade salivary gland tumor.
- EWSR1-ATF1 fusion is a specific molecular marker for HCCC.
- Most HCCC cases originate in minor salivary glands; paranasal sinus origin is exceptionally rare.
Observation:
- A 63-year-old female presented with epistaxis due to a destructive mass in the ethmoid/maxillary sinus.
- Histopathology revealed nests of clear cells with hyalinized septa and bone destruction.
- Immunohistochemistry excluded other clear cell tumors, and FISH confirmed EWSR1 gene rearrangement.
Findings:
- The tumor exhibited clear cell morphology with specific immunohistochemical markers (CK5/6, EMA, p63).
- EWSR1 gene rearrangement was identified, confirming HCCC.
- The patient received postoperative radiation and showed no recurrence at 4 months.
Implications:
- This case expands the known locations for HCCC, emphasizing the ethmoid/maxillary sinus as a rare site.
- Awareness of HCCC in unusual head and neck locations is crucial for accurate diagnosis.
- EWSR1 gene rearrangement is a key diagnostic marker for HCCC, aiding in differential diagnosis.
