DEK::AFF2 Fusion Sinonasal and Skull Base Nonkeratinizing Squamous Cell Carcinoma

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.

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Publication Details

Journal
The American Journal of Surgical Pathology
Published
2024-12-25
DOI
https://doi.org/10.1097/pas.0000000000002335
Citations
11
Primary Topic
Head and Neck Surgical Oncology
Type
article
Field-Weighted Citation Impact
6.30
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article

DEK::AFF2 Fusion Sinonasal and Skull Base Nonkeratinizing Squamous Cell Carcinoma

Juan C. Hernandez‐Prera, Stephanie N. David, Nicole A. Cipriani, W. Dale Plummer et al.
11 citations
The American Journal of Surgical Pathology
Head and Neck Surgical Oncology
6.30
article

DEK::AFF2 Fusion Sinonasal and Skull Base Nonkeratinizing Squamous Cell Carcinoma

Juan C. Hernandez‐Prera, Stephanie N. David, Nicole A. Cipriani, W. Dale Plummer, James S. Lewis, Eiichi Sasaki, Michael W. Mikula, Min-Shu Hsieh, Jaylou M. Velez Torres, Rebecca D. Chernock, Ying‐Ju Kuo, Justin A. Bishop, Y. Chen, Qiuying Shi, Tra Truong, Jen‐Fan Hang, Lisa M. Rooper, Ariana Geromes, Karen Ferrer, Stephanie A Hart, Karan Saluja, William D. Dupont, Sara E. Amin
article en
11 citations

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.

The American Journal of Surgical PathologyVol. 49(2)
Sunnybrook Health Science Centre (CA), University of Miami (US), National Yang Ming Chiao Tung University (TW), Emory University (US), Washington University in St. Louis (US), John H. Stroger, Jr. Hospital of Cook County (US), Health Sciences Centre (CA), Taipei Veterans General Hospital (TW), Moffitt Cancer Center (US), Chang Gung Memorial Hospital (TW), University of Chicago (US), Southwestern Medical Center (US), Mayo Clinic in Arizona (US), National Taiwan University Hospital (TW), Aichi Cancer Center (JP), Johns Hopkins Hospital (US), Clinical Research Associates (US), Southwestern Medical Center (US), The University of Texas Southwestern Medical Center (US), Vanderbilt University Medical Center (US), The University of Texas Health Science Center at Houston (US)
Good health and well-being
Openalex Percentile: Top 3%
Head and Neck Surgical Oncology
6.30
DOISourceOpenAlex
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