Bedard Mary C, de Alarcon Alessandro, Kou Yann-Fuu, Lee David, Sestito Alexandra, Duggins Angela L, Brusadelli Marion, Lane Adam, Wikenheiser-Brokamp Kathryn A, Wells Susanne I, Smith David F
Division of Oncology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
Division of Pediatric Otolaryngology-Head and Neck Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
Cancers (Basel). 2021 May 23;13(11):2556. doi: 10.3390/cancers13112556.
Juvenile-onset recurrent respiratory papillomatosis (JoRRP) is the most common benign neoplasm of the larynx in children, presenting with significant variation in clinical course and potential for progression to malignancy. Since JoRRP is driven by human papillomavirus (HPV), we evaluated viral factors in a prospective cohort to identify predictive factors of disease severity. Twenty children with JoRRP undergoing routine debridement of papillomas were recruited and followed for ≥1 year. Demographical features, clinical severity scores, and surgeries over time were tabulated. Biopsies were used to establish a tissue bank and primary cell cultures for HPV6 vs. HPV11 genotyping and evaluation of viral gene expression. We found that patients with HPV11+ disease had an earlier age at disease onset, higher frequency of surgeries, increased number of lifetime surgeries, and were more likely to progress to malignancy. However, the amplitude of viral E6/E7 gene expression did not account for increased disease severity in HPV11+ patients. Determination of HPV strain is not routinely performed in the standard of care for JoRRP patients; we demonstrate the utility and feasibility of HPV genotyping using RNA-ISH for screening of HPV11+ disease as a biomarker for disease severity and progression in JoRRP patients.
儿童期复发性呼吸道乳头状瘤病(JoRRP)是儿童喉部最常见的良性肿瘤,其临床病程差异显著,且有恶变倾向。由于JoRRP由人乳头瘤病毒(HPV)驱动,我们在一个前瞻性队列中评估了病毒因素,以确定疾病严重程度的预测因素。招募了20例接受乳头状瘤常规清创术的JoRRP患儿,并对其进行了≥1年的随访。记录了人口统计学特征、临床严重程度评分以及随时间推移的手术情况。通过活检建立了组织库和原代细胞培养物,用于HPV6与HPV11基因分型以及病毒基因表达评估。我们发现,HPV11阳性疾病患者发病年龄更早,手术频率更高,终身手术次数更多,且更有可能进展为恶性肿瘤。然而,病毒E6/E7基因表达的幅度并不能解释HPV11阳性患者疾病严重程度的增加。在JoRRP患者的标准治疗中,通常不常规进行HPV毒株的检测;我们证明了使用RNA原位杂交进行HPV基因分型以筛查HPV11阳性疾病作为JoRRP患者疾病严重程度和进展的生物标志物的实用性和可行性。