Recurrent respiratory papillomatosis (RRP) can be a potentially life-threatening condition and a treatment challenge. Local therapies are associated with significant scarring and detrimental effects on voice and respiratory status. EGFR inhibitors have been reported to be an effective adjunctive therapy in patients with RRP.
To report our institutional experience with Erlotinib in two pediatric patients with severe RRP.
Retrospective chart review of 2 pediatric patients with severe RRP.
Patient 1 is a 19y/o female who was referred to us at the age of 11. Patient was diagnosed with respiratory papillomatosis at birth. Since age 1.5yrs, she had been undergoing micro-laryngoscopy, bronchoscopy with laser or surgical excision of the lesions, on a 1-2 monthly basis. On examination she had no skin lesions. Her voice was only a whisper. No difficulty breathing or stridor. She was pre-pubertal. Endoscopic exam 3 weeks prior to starting therapy showed severely scarred larynx, no obvious laryngeal disease, diffuse subglottic and tracheal papillomatosis with extension into the left main bronchus. Biopsy of the lesion showed squamous papilloma, EGFR positive.
Patient 2 is 14y/o who was referred to us at age 12. Patient was diagnosed at 5 months with respiratory papillomatosis of the larynx undergoing micro-laryngoscopy, bronchoscopy with laser or surgical excision of the lesions, on a 1-2 monthly basis. He developed respiratory distress post laryngoscopy and found to have 3 pulmonary lesions confirmed as papillomatosis post thoracotomy with wedge resections.
Both patients were started on an EGFR inhibitor, Erlotinib at a dose of 85mg/m2 daily and DIM (diindolylmethane) 150mg twice daily. The skin care regimen included regular moisturizers and sunscreen; topical steroids and antibiotics as needed. Therapy was well tolerated except for grade 1 skin rash in both patients and grade 1 diarrhea as well, in Patient 1.
For patient 1, within a month of therapy there was significant decrease in the lesions and by 6mo complete resolution of papillomatosis. She had 15mo of therapy. She is in remission 6 years off therapy. For patient 2, there has been decrease in lesions and the interval between laryngoscopy since start of therapy. CTs of chest confirm no recurrence of pulmonary lesions since starting Erlotinib.
Erlotinib demonstrates efficacy in respiratory papillomatosis in children and may be considered as adjunct therapy with surgical excision in this disease. DIM is an aromatase inhibitor that potentiates the effect of Erlotinib.
Abraham, Shirley; John Kuttesch; and Erica Bennett. "Erlotinib Therapy For Recurrent Respiratory Paillomatosis And Extralaryngeal Spread In Pediatrics." (2020). https://digitalrepository.unm.edu/hsc_2020_pediatric_research/15