Tumors of the larynx are rare. The most common benign tumor is subglottic hemangioma.[1] Malignant tumors, which are especially rare, may be associated with benign tumors such as polyps and papillomas.[2,3] A review of Surveillance, Epidemiology, and End Results (SEER) Program data from 1973 to 2016 identified 23 pediatric patients with laryngeal malignancies. Sixteen of the patients had squamous cell carcinoma. The other identified histologies included small cell carcinoma, mucoepidermoid carcinoma, myxosarcoma, embryonal rhabdomyosarcoma, and synovial sarcoma.[4]
These tumors may present with the following:
Squamous cell carcinoma of the larynx in children is managed by surgery and radiation therapy, as in adults with carcinoma at this site.[4,5] Outcomes of pediatric patients with squamous cell carcinoma of the larynx are similar to those reported for adult patients.[4] Laser surgery may be the initial treatment used for these lesions. For more information about the treatment of laryngeal cancer in adults, see Laryngeal Cancer Treatment.
Information about National Cancer Institute (NCI)–supported clinical trials can be found on the NCI website. For information about clinical trials sponsored by other organizations, see the ClinicalTrials.gov website.
Cancer in children and adolescents is rare, although the overall incidence has slowly increased since 1975.[1] Children and adolescents with cancer should be referred to medical centers that have a multidisciplinary team of cancer specialists with experience treating the cancers that occur during childhood and adolescence. This multidisciplinary team approach incorporates the skills of the following pediatric specialists and others to ensure that children receive treatment, supportive care, and rehabilitation to achieve optimal survival and quality of life:
For specific information about supportive care for children and adolescents with cancer, see the summaries on Supportive and Palliative Care.
The American Academy of Pediatrics has outlined guidelines for pediatric cancer centers and their role in the treatment of children and adolescents with cancer.[2] At these centers, clinical trials are available for most types of cancer that occur in children and adolescents, and the opportunity to participate is offered to most patients and their families. Clinical trials for children and adolescents diagnosed with cancer are generally designed to compare potentially better therapy with current standard therapy. Other types of clinical trials test novel therapies when there is no standard therapy for a cancer diagnosis. Most of the progress in identifying curative therapies for childhood cancers has been achieved through clinical trials. Information about ongoing clinical trials is available from the NCI website.
Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2020, childhood cancer mortality decreased by more than 50%.[3-5] Childhood and adolescent cancer survivors require close monitoring because side effects of cancer therapy may persist or develop months or years after treatment. For information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors, see Late Effects of Treatment for Childhood Cancer.
Childhood cancer is a rare disease, with about 15,000 cases diagnosed annually in the United States in individuals younger than 20 years.[6] The U.S. Rare Diseases Act of 2002 defines a rare disease as one that affects populations smaller than 200,000 people in the United States. Therefore, all pediatric cancers are considered rare.
The designation of a rare tumor is not uniform among pediatric and adult groups. In adults, rare cancers are defined as those with an annual incidence of fewer than six cases per 100,000 people. They account for up to 24% of all cancers diagnosed in the European Union and about 20% of all cancers diagnosed in the United States.[7,8] In children and adolescents, the designation of a rare tumor is not uniform among international groups, as follows:
Most cancers in subgroup XI are either melanomas or thyroid cancers, with other cancer types accounting for only 2% of the cancers diagnosed in children aged 0 to 14 years and 9.3% of the cancers diagnosed in adolescents aged 15 to 19 years.
These rare cancers are extremely challenging to study because of the relatively few patients with any individual diagnosis, the predominance of rare cancers in the adolescent population, and the small number of clinical trials for adolescents with rare cancers.
Information about these tumors may also be found in sources relevant to adults with cancer, such as Laryngeal Cancer Treatment.
Recurrent respiratory papillomatosis is the most common benign laryngeal tumor in children, and it is associated with human papillomavirus (HPV) infection, most commonly HPV-6 and HPV-11.[1,2] The presence of HPV-11 appears to correlate with a more aggressive clinical course than does the presence of HPV-6.[3] An Australian survey of pediatric otorhinolaryngologists documented a decline in the incidence of laryngeal papillomatosis after the introduction of HPV vaccinations for adolescent girls and young women aged 12 to 26 years.[4] In another study of patients younger than 18 years with laryngeal papillomatosis, the incidence decreased from 165 cases in children born between 2004 and 2005 to 36 cases in children born between 2012 and 2013. The authors of the study attribute the decline in incidence to the widespread use of the HPV vaccine, which was released in 2006.[5]
These tumors can cause hoarseness because of their association with wart-like nodules on the vocal cords, and they may rarely extend into the lung, producing significant morbidity.[6] Malignant degeneration may occur, with development of laryngeal carcinoma and squamous cell lung cancer, generally reported at rates of 2% to 10% in the pediatric population.[7]
A multi-institutional registry study identified children with juvenile-onset recurrent respiratory papillomatosis from 23 states between January 2015 and August 2020.[8] Of the 215 children with juvenile-onset recurrent respiratory papillomatosis, 88.8% were delivered vaginally. Among 190 mothers, the median age at the time of delivery was 22 years. Of 114 mothers (60.0%) who were age-eligible for the HPV vaccination, 16 (14.0%) were vaccinated, 1 (0.9%) of whom was vaccinated before delivery. Of 162 tested biopsy specimens, 157 (96.9%) had detectable HPV. All 157 specimens had a vaccine-preventable HPV type.
Primary treatment for papillomatosis is surgical ablation with laser vaporization.[9] Frequent recurrences are common. Lung involvement, although rare, can occur.[6]
Evidence (surgery):
If a patient requires more than four surgical procedures per year, other interventions may be necessary, including the following:
The effectiveness of intralesional cidofovir has not been conclusively demonstrated.[14] Intralesional bevacizumab has also been used in some patients. In a pilot study, ten patients with severe respiratory papillomatosis received intralesional bevacizumab. With this treatment, the number of surgical procedures per year decreased and quality-of-life scores improved.[15]
The role of checkpoint inhibitors, such as PD-1 inhibitors, is currently being investigated.[16] Reports (with small numbers of patients) have documented that in selected cases, the administration of a quadrivalent HPV vaccine can be associated with a complete remission and an increase in the intersurgical interval.[17,18] In contrast, other reports have not documented a therapeutic effect of the quadrivalent HPV vaccine.[19]
In a report of two patients with aggressive recurrent respiratory papillomatosis, treatment with systemic bevacizumab produced good results with minimal toxicity.[20] In another report of seven children who were treated with bevacizumab, continued responses were noted and subsequent surgical debridement was avoided in most patients. Of the seven patients, five have not required surgical debridement after initiation of bevacizumab. Four of these five patients had previously required between four to ten debridements per year. Follow-up for these patients was between 8 months and 3.5 years. No serious adverse events were reported.[21] In a study of 24 patients with recurrent respiratory papillomatosis, 15 had pediatric-onset disease. Patients were treated with systemic bevacizumab (7.5–10 mg/kg) every 3 to 4 weeks. All patients had a reduction in the number and size of lesions after three doses, excluding one patient who was lost to follow-up. Voice outcomes were improved in 87.5% of patients, as measured by Voice Handicap Index-30 (VHI) or pediatric VHI. No grade 3 Common Terminology Criteria for Adverse Events were reported. However, follow-up was limited to a maximum of 14 months after initiation of therapy and 10 months after discontinuation of bevacizumab.[22]
Information about National Cancer Institute (NCI)–supported clinical trials can be found on the NCI website. For information about clinical trials sponsored by other organizations, see the ClinicalTrials.gov website.
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
This summary was comprehensively reviewed.
This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® Cancer Information for Health Professionals pages.
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood laryngeal cancer and papillomatosis. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.
This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).
Board members review recently published articles each month to determine whether an article should:
Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.
The lead reviewers for Childhood Laryngeal Tumors Treatment are:
Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.
Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Pediatric Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.
PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”
The preferred citation for this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Laryngeal Tumors Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/head-and-neck/hp/child/laryngeal-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 29337477]
Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.
Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s Email Us.