What is it?
Our patient had juvenile laryngeal papillomatosis. This is caused by a human papillomavirus infection of the throat (types 6 and 11). HPV can activate the epidermal growth factor receptor pathway and inactivate tumor-suppressor proteins resulting in ‘cauliflower-like’ growths in the airway. Without treatment it is potentially fatal and often requires repetitive surgery to prevent airway obstruction. The disease is most commonly found in children and may be caused by an infant contracting HPV from the mother in the birth canal during vaginal delivery. It is often misdiagnosed as asthma in children. Since the HPV vaccine includes HPV 6 and 11, it is expected to prevent genital infections among women and thus prevent secondary laryngeal infections to newborns which can lead to laryngeal papillomatosis. A second form of papillomatosis occurs in adults usually in their 30s which appears to be less aggressive.
Papillomatosis has the potential for malignant transformation to squamous lung carcinoma with a dismal prognosis. HPV11 is associated with greater risk of producing lung ca than HPV 6. These are often manifested by “drop metastases” where the virus drops into the airway and lodges in the lung where it transforms into a carcinoma. Fortunately, only 1% of papillomas undergo this transformation to squamous cell cancer.
The tumors can be treated with photodynamic therapy where physicians inject a light-sensitive dye that is only absorbed by the tumors and then the physician activates the dye using a bright light which destroys the tumors. Antiangiogenic therapy is also used. Atopical treatment with anti=vascular endothelial growth factor (VEGF) antibody bevacizumab is used to paint the tumors and since many express VEGF-A there is a significant reduction in tumor burden.
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