Presentation
15 year old male with papillomatosis, initially diagnosed during first year of life.
Patient Data
There are are multifocal nodular opacities involving both lower lobes. On the lateral view, the opacities are localized to a posterior segment lower lobe and have a mass-like appearance.
Fine undulations of the tracheal contour are suggestive of mural growths, and consistent with the patient's known tracheal papilloma.
There are numerous polypoid masses along the tracheal wall, consistent with known papillomatosis.
Within the lungs, there are scattered cystic lesions with thickened periphery, likely representing papillomas with cavitation. In the lower lobes, there mass-like consolidationes associated with areas of central saccular bronchiectasis. There is no pleural effusion or focal pleural thickening.
Bronchoscopic evaluation shows diffuse papillomatous change throughout the subglottic trachea.
Case Discussion
This is an advanced case of recurrent respiratory papillomatosis, with distal airway and lung involvement. The patient was diagnosed during the first year of life, and thus the disease may be characterized as juvenile-form RRP.
Extensive papillomatosis of the upper airway is the classic finding, and can even be visualized on the radiograph. Endoscopic evaluation showed a mixture of sessile and polypoid morphology of the lesions.
What makes this presentation more unusual is the extent of lower-airway involvement. The isolated pulmonary cystic lesions likely represent bronchiolar papillomas with surrounding cavitation. Bronchial dilatation and bronchiectasis are the result of chronic bronchial obstruction. The lower lobe masses likely represent papillomas with associated post-obstructive pulmonary consolidation.
This patient had numerous endoscopic excisions and biopsies throughout the disease course. A typical pathology result would read:
- Sections show a squamous papilloma with marked hyperplasia and scattered mitotic figures. The hyperplasia and mitotic activity imply an actively proliferating lesion, but it lacks any dysplastic changes.
- In Situ Hybridization testing for Human Papilloma Virus shows:
- Positive for one or more of the following types: 6 and 11.
- Negative for types 16,18, 31, 33, 35, 39, 45, 51, 52, 56, 58 and 66.
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