Abstract:
Among the three major histological subtypes of sinonasal papillomas, inverted (ISP) and oncocytic (OSP) sinonasal papillomas tend to undergo malignant transformation to carcinoma. However, criteria determining risk of recurrence and malignant progression have not been established. Recently, EGFR and KRAS mutations were detected to be characteristic for ISP and OSP, respectively. In this study, we analyzed 137 sinonasal papilloma cases (132 ISP and 5 OSP) for clinicopathological characteristics, frequency of recurrences/malignant transformation, and histological types and genetic features of carcinoma ex Schneiderian papilloma. OSP presented at a higher age than ISP (median, 75 vs. 57 years) and affected predominantly females. Overall frequency of recurrences and malignant transformation was 23.1% and 9.5%, respectively. Rates of recurrence (33.3% vs. 22.0%) and malignant transformation (33.3% vs. 8.8%) were higher in OSP compared to ISP, respectively. Carcinomas (n = 10) occurred mostly synchronously, more frequently in females and mainly associated with ISP (n = 9). Squamous cell carcinoma (SCC) was the most frequently associated malignancy. Concordant EGFR (in ISP/associated carcinoma) and KRAS (in the OSP/associated carcinoma) mutations were detected in all successfully analyzed matching papilloma/carcinoma pairs, confirming their shared clonal origin. Results of this large study are in line with recent studies showing frequent EGFR and KRAS mutations in sinonasal carcinoma ex Schneiderian papilloma. As the papilloma component might on occasion be missed on biopsy of synchronous carcinoma ex papilloma, EGFR and KRAS mutation testing represents a promising molecular surrogate for sinonasal \"carcinoma ex papilloma\", at the same time offering an opportunity for targeting mutant EGFR in this rare cancer type.
摘要:
在鼻腔鼻窦乳头状瘤的三种主要组织学亚型中,倒置型(ISP)和嗜酸细胞性(OSP)鼻窦乳头状瘤倾向于恶性转化为癌。然而,确定复发和恶性进展风险的标准尚未建立.最近,检测到EGFR和KRAS突变是ISP和OSP的特征,分别。在这项研究中,我们分析了137例鼻窦乳头状瘤(132ISP和5OSP)的临床病理特征,复发/恶性转化的频率,以及Schneiderian乳头状瘤的组织学类型和遗传特征。OSP的年龄高于ISP(中位数,75vs.57岁),主要受女性影响。复发和恶变的总频率为23.1%和9.5%,分别。复发率(33.3%vs.22.0%)和恶性转化(33.3%vs.8.8%)与ISP相比,OSP更高,分别。癌(n=10)大部分同步发生,女性更常见,主要与ISP相关(n=9)。鳞状细胞癌(SCC)是最常见的相关恶性肿瘤。在所有成功分析的匹配乳头状瘤/癌对中检测到一致的EGFR(在ISP/相关癌中)和KRAS(在OSP/相关癌中)突变,确认它们共同的克隆起源。这项大型研究的结果与最近的研究一致,这些研究表明,在Schneiderian乳头状瘤的鼻窦癌中,EGFR和KRAS突变频繁。由于乳头状瘤成分有时可能在同步癌前乳头状瘤的活检中被遗漏,EGFR和KRAS突变检测代表了鼻窦“癌乳头瘤”的有希望的分子替代,同时提供了在这种罕见癌症类型中靶向突变EGFR的机会.