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Reappraising hyalinizing clear cell carcinoma: A population-based study with molecular confirmation
Juan C Hernandez-Prera1, Ricky Kwan1, Joseph Tripodi1
1Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York.
Head & Neck
|November 30, 2016
Summary
Hyalinizing clear cell carcinoma (HCCC) is a rare salivary gland cancer. This study found HCCCs have a high risk of local recurrence and metastasis, despite being indolent.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Hyalinizing clear cell carcinoma (HCCC) is a rare malignancy.
- HCCC is characterized by the EWSR1-ATF1 gene fusion.
- Its clinical behavior has been poorly understood, often being a diagnosis of exclusion.
Purpose of the Study:
- To investigate the characteristics and behavior of HCCC.
- To determine the incidence of HCCC within a salivary gland carcinoma cohort.
- To assess the risks of local recurrence and metastasis in HCCC.
Main Methods:
- Review of 594 salivary gland carcinomas from institutional archives.
- Confirmation of HCCC diagnosis using fluorescence in situ hybridization (FISH) for EWSR1 rearrangement.
- Comprehensive literature review of HCCC cases.
Main Results:
- 15 HCCC cases identified, predominantly in the oral cavity and base of tongue.
- 13 of 15 cases showed EWSR1 rearrangement.
- Combined data indicated a 10-year risk of local recurrence at 49% and locoregional nodal metastasis at 15%.
Conclusions:
- Molecularly confirmed HCCC constitutes 2.5% of salivary gland malignancies.
- HCCCs are indolent tumors with a significant propensity for locoregional recurrence.
- Understanding HCCC behavior is crucial for patient management.
