Can Nhu Thuy, Tretiakova Maria S, Taxy Jerome B
1University of Chicago , Chicago, IL , USA.
Fetal Pediatr Pathol. 2015 Apr;34(2):80-90. doi: 10.3109/15513815.2014.968271. Epub 2014 Oct 29.
Recurrent respiratory papillomatosis (RRP) is a human papillomavirus (HPV) related disease in both children and adults, characterized by recurrent benign squamous papillomas of the respiratory mucosa. Malignant transformation is rare. The present report concerns the natural history of RRP in two children.
Clinical records, autopsy material and tissue from previous surgical excisions were reviewed in both cases. Select surgical and autopsy specimens were examined using p16 immunohistochemistry and in-situ hybridization for low and high risk HPV.
Both children had pulmonary involvement with incidental invasive keratinizing squamous carcinoma of the lung at autopsy. Low-risk HPV was present in the papillomas and carcinoma at autopsy in both cases.
The autopsy examinations in these two cases emphasize the serious, if uncommon, pulmonary complications of this disease. In conjunction with previously reported autopsies, destructive lung disease may be as frequent a cause of death as disseminated malignancy.
复发性呼吸道乳头状瘤病（RRP）是一种与人类乳头瘤病毒（HPV）相关的疾病，在儿童和成人中均有发生，其特征为呼吸道黏膜反复出现良性鳞状乳头状瘤。恶变罕见。本报告关注两名儿童RRP的自然病史。
回顾了两例患者的临床记录、尸检材料以及既往手术切除的组织。选取手术和尸检标本，采用p16免疫组织化学及低危和高危HPV原位杂交技术进行检测。
两名儿童均有肺部受累，尸检时意外发现肺浸润性角化鳞状细胞癌。两例患者尸检时，乳头状瘤和癌组织中均存在低危HPV。
这两例尸检结果强调了该疾病严重的肺部并发症，尽管并不常见。结合此前报告的尸检情况，破坏性肺部疾病可能与播散性恶性肿瘤一样，是常见的死亡原因。
