Laryngeal Papillomas
Recurrent Respiratory Papillomatosis

General Considerations

• Most common benign tumor of the trachea in children (60% of all benign tumors)
• 2^nd most common benign tumor of trachea in adults
  • Majority of primary tracheal tumors are malignant
• In over 80% of cases, multiple papillomas occur in the trachea
• Tracheal papilloma is associated with a laryngeal papilloma in 36%
• Because of its high recurrence rate, it is also called Recurrent Respiratory Papillomatosis (RRP)
• Caused by the human papilloma virus (HPV)
  • Most often types 6 and 11
• Also the cause of skin warts and genital warts
• Transmission of recurrent respiratory papillomatosis is believed to occur, at least in part, from mother’s infected cervix during childbirth
• HPV is responsible for most cervical cancers and about 25% of head and neck cancers
• Most common in children and then again 20-40 years of age
• Significant recurrence rate may lead to numerous operative removals
• Not the same as vocal cord polyps or nodules  

Clinical Findings
 

• Children usually present around age 2-3 with
  • Male:female ratio 1:1
• Cough, hacking in nature
• Audible wheezing
• Hemoptysis
• Stridor
• Dysphagia
• Pneumonia
• Aphonia
• Adult-onset disease
• Less severe than juvenile form
• Male:female ratio in adults is 4:1
• Painless hoarseness 

Imaging Findings

• Diagnosis is made using flexible fiberoptic laryngoscopy
• Chest radiographs are usually normal
  • Papillomas can present as multiple nodules in the chest, some of which may cavitate
• CT scans of the neck and chest will demonstrate the extent of the lesions 

Treatment
 

• Surgical excision is the current standard of care in the treatment of RRP
  • Surgery is emergent if there is airway obstruction
• Numerous surgeries may be needed because of the recurrence of these tumors (>100)
  • Adult form requires fewer surgical procedures
• Tracheostomies are relatively contraindicated because they are believed to induce spread of the papilloma down the trachea into the bronchi and lungs
• Medical therapies that are being tried include
  • Interferon
• Photodynamic therapy
• Indol-3-carbinol
• Ribavirin
• Acyclovir
• Methotrexate
• Mumps vaccine
• Cidofovir
• It is hoped that widespread use of anti-HPV vaccines (Gardisil) will eventually lead to eradication of the disease 

Prognosis
 

• Progression to squamous cell carcinoma is reported but rare
• The disease frequently becomes quiescent in adolescence

Laryngeal Papilloma. Soft tissue lateral view of the neck demonstrates a smooth, rounded soft tissue
mass arising from the posterior wall of the trachea at the level of the larynx (white arrows).
For additional information about this disease, click on this icon if seen above.
For this same photo, click here

EMedicine Recurrent Respiratory Papillomatosis  John E McClay, MD