Hart Stephanie A, Hang Jen-Fan, Chernock Rebecca D, Mikula Michael W, Rooper Lisa, Amin Sara E, Saluja Karan, Bishop Justin A, Chen Yu Hsiu, Cipriani Nicole A, David Stephanie N, Dupont William D, Plummer W Dale, Ferrer Karen T, Geromes Ariana, Hsieh Min-Shu, Hernandez-Prera Juan C, Kuo Ying-Ju, Sasaki Eiichi, Shi Qiuying, Truong Tra, Velez Torres Jaylou M, Lewis James S
Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center.
Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, MO.
Am J Surg Pathol. 2025 Feb 1;49(2):130-137. doi: 10.1097/PAS.0000000000002335. Epub 2024 Dec 25.
: 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.
:AFF2融合性非角化鳞状细胞癌（NKSCC）是鼻窦、颞骨和颅底出现的一种新肿瘤类型。然而，这些肿瘤的临床行为尚未得到充分研究。在此，我们报告了最大规模的DEK::AFF2癌病例队列，以确定形态学、有丝分裂率和/或Ki-67免疫组化是否与患者预后相关，包括与高危人乳头瘤病毒（HPV）相关和不相关患者的比较。我们向外科病理学家征集分子检测或AFF2免疫组化（IHC）证实的DEK::AFF2鳞状细胞癌病例，以收集患者的人口统计学、临床和预后数据。使用代表性的苏木精-伊红（H&E）切片，我们对形态学进行了特征描述并计数有丝分裂。进行了Ki-67免疫组化检测。我们还将DEK::AFF2的生存率与一组AFF2免疫组化阴性的HPV相关和HPV不相关鳞状细胞癌患者的生存率进行了比较。DEK::AFF2癌最常见于鼻腔（13/30，43%），平均复发次数为1.8次（范围：0至10次）。在最后一次随访时，大多数患者无疾病（19/30，63%）或带瘤生存（9/30，30%）。平均每2平方毫米有丝分裂率为2次（范围：0至9次），Ki-67增殖率为26%（范围：3%至60%）。局部复发很常见，但形态学、有丝分裂活性和Ki-67指数与复发或生存无关。在Kaplan-Meier生存分析中，DEK::AFF2患者的无病生存率较低，但在其他方面与传统鳞状细胞癌患者的预后相似。我们的多机构研究表明，DEK::AFF2融合性非角化鳞状细胞癌患者局部复发很常见，但患者生存率与传统鳞状细胞癌相似。尽管表现出一系列不同特征和增殖率，但通过形态学、有丝分裂率和/或Ki-67活性进行的传统分级似乎并不能预测预后。
