METHODS: A retrospective review of pathology reports and microscopy glass slides of HNSFT cases diagnosed between January 2010 and August 2022 was performed.STAT6 was additionally performed on selected cases if needed. Follow up was obtained and various risk stratification models were applied.
RESULTS: Sixty seven cases of HNSFT were collected (age range from 11 to 87 years; median 42 years; M:F 1.6:1). Most common tumor sites were orbit (n = 21; 31.3 %), sinonasal tract (n = 18; 26.9 %), and oral cavity (n = 13; 19.4 %). Tumor size ranged from 1 to 16 cm (median 4cm). Apart from common histological features, tumor cells also showed focal epithelioid morphology, clear cell change and nuclear atypia in a subset of cases. Stromal findings included myxoid and lipomatous change, pseudoglandular spaces, pseudovascular spaces and multinucleated stromal giant cells. CD34 and STAT6 were expressed in 57/67 (85.1 %) and 56/56 (100 %) cases, respectively. Recurrence was observed in 4/26 (15.4 %) cases, while none (0/22) of the patients experienced distant metastasis (follow up 1-150 months; median 20.5 months). Clinical outcome was partially concordant with risk-categories of different risk stratification models.
CONCLUSIONS: Knowledge about histological diversity of HNSFT is essential for establishing correct diagnosis. Current risk stratification models do not perfectly predict outcome, and larger studies are needed to develop more accurate criteria for aggressive behavior.
方法:对2010年1月至2022年8月诊断的HNSFT病例的病理报告和显微镜载玻片进行回顾性回顾。如果需要,对选定的病例额外进行STAT6。获得随访,并应用各种风险分层模型。
结果:收集67例HNSFT(年龄范围为11至87岁;中位数42岁;M:F1.6:1)。最常见的肿瘤部位是眼眶(n=21;31.3%),鼻窦(n=18;26.9%),和口腔(n=13;19.4%)。肿瘤大小1~16cm,中位数4cm。除了常见的组织学特征,肿瘤细胞也显示局灶性上皮样形态,一部分病例的透明细胞改变和核异型性。基质发现包括粘液样和脂肪瘤样改变,假岛隙,假血管间隙和多核基质巨细胞。CD34和STAT6在57/67例(85.1%)和56/56例(100%)中表达,分别。4/26(15.4%)例复发,而无患者(0/22)发生远处转移(随访1-150个月;中位数20.5个月)。临床结果与不同风险分层模型的风险类别部分一致。
结论:了解HNSFT的组织学多样性对于建立正确的诊断至关重要。目前的风险分层模型不能完全预测结果,需要更大规模的研究来制定更准确的攻击行为标准。