1. DEK :: AFF2 Fusion Sinonasal and Skull Base Nonkeratinizing Squamous Cell Carcinoma : A Clinical Outcome Study Compared With Conventional Sinonasal Squamous Cell Carcinoma.
- Author
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Hart SA, Hang JF, Chernock RD, Mikula MW, Rooper L, Amin SE, Saluja K, Bishop JA, Chen YH, Cipriani NA, David SN, Dupont WD, Plummer WD, Ferrer KT, Geromes A, Hsieh MS, Hernandez-Prera JC, Kuo YJ, Sasaki E, Shi Q, Truong T, Velez Torres JM, and Lewis JS Jr
- Subjects
- Humans, Male, Middle Aged, Female, Aged, Adult, Chromosomal Proteins, Non-Histone analysis, Chromosomal Proteins, Non-Histone genetics, Squamous Cell Carcinoma of Head and Neck pathology, Squamous Cell Carcinoma of Head and Neck mortality, Squamous Cell Carcinoma of Head and Neck chemistry, Squamous Cell Carcinoma of Head and Neck virology, Immunohistochemistry, Ki-67 Antigen analysis, Aged, 80 and over, Carcinoma, Squamous Cell pathology, Carcinoma, Squamous Cell mortality, Carcinoma, Squamous Cell chemistry, Carcinoma, Squamous Cell virology, Mitotic Index, Treatment Outcome, Cell Proliferation, Disease-Free Survival, Gene Fusion, Poly-ADP-Ribose Binding Proteins genetics, Biomarkers, Tumor analysis, Paranasal Sinus Neoplasms pathology, Paranasal Sinus Neoplasms mortality, Paranasal Sinus Neoplasms virology, Paranasal Sinus Neoplasms chemistry, Paranasal Sinus Neoplasms genetics, Paranasal Sinus Neoplasms therapy, Oncogene Proteins genetics, Oncogene Proteins analysis, Skull Base Neoplasms pathology, Skull Base Neoplasms mortality, Skull Base Neoplasms chemistry, Skull Base Neoplasms therapy
- Abstract
DEK :: AFF2 fusion nonkeratinizing squamous cell carcinoma (NKSCC) is an emerging entity in the sinonasal tract, temporal bone, and skull base. However, the clinical behavior of these tumors has not been well studied. Here, we report the largest cohort of DEK :: AFF2 carcinomas to determine if morphology, mitotic rate, and/or Ki-67 IHC are associated with patient outcomes, including a comparison with high-risk human papillomavirus (HPV)-associated and independent patients. We solicited cases of molecularly or AFF2 immunohistochemistry (IHC) proven DEK :: AFF2 SCC from surgical pathologists to collect patient demographic, clinical, and outcome data. Using representative H&E slides, we characterized the morphology and counted mitoses. Ki-67 immunohistochemistry was performed. We also compared the DEK :: AFF2 survival rates to those in a cohort of AFF2 IHC-negative HPV-associated and HPV-independent SCC. DEK :: AFF2 carcinomas most commonly arose in the nasal cavity (13/30, 43%), and the average number of recurrences was 1.8 (range: 0 to 10). At the last follow-up, most patients were disease free (19/30, 63%) or were alive with disease (9/30, 30%). There was an average mitotic rate of 2 per 2 mm 2 (range: 0 to 9) and Ki-67 proliferation rate of 26% (range: 3% to 60%). Local recurrence was common, but morphology, mitotic activity, and Ki-67 index were not associated with recurrence or survival. On Kaplan-Meier survival analysis, DEK :: AFF2 patients had lower disease-free survival but otherwise had similar outcomes to conventional SCC patients. Our multi-institutional study shows that local recurrence is common in DEK :: AFF2 fusion nonkeratinizing SCC patients, but patients have survival rates similar to conventional SCC. Despite showing a range of different features and proliferation rates, traditional grading by morphology, mitotic rate, and/or Ki-67 activity does not seem to be predictive of outcome., Competing Interests: Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)
- Published
- 2025
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