National Cancer Institute
Post Date: Feb 22, 2022
This evidence-based, expert-reviewed summary discusses the incidence, risk factors, clinical presentation, diagnosis, and treatment of pediatric basal cell carcinoma and squamous cell carcinoma of the skin.
Childhood Basal & Squamous Cell Carcinoma of the Skin Treatment
Incidence and Risk Factors
Nonmelanoma (basal cell carcinoma [BCC] and squamous cell carcinoma [SCC]) skin cancers are very rare in children and adolescents. In a report of 7,814 cases of primary skin cancers in individuals younger than 30 years who were recorded by the Surveillance, Epidemiology, and End Results (SEER) Program database from 2000 to 2008, carcinomas accounted for 0.008% of all cases.
In one series of 28 patients, approximately one-half of patients had predisposing conditions such as nevoid BCC syndrome (Gorlin-Goltz syndrome, basal cell nevus syndrome), and one-half of patients were exposed to iatrogenic conditions such as prolonged immunosuppression or radiation. Gorlin-Goltz syndrome is a rare disorder with a predisposition to the development of early-onset neoplasms, including BCC, ovarian fibroma, and desmoplastic medulloblastoma.
BCCs and SCCs in adults have been associated with exposure to solar ultraviolet (UV) radiation, iatrogenic immunosuppression, UVB radiation, and other risk factors.
In one retrospective follow-up study of patients with xeroderma pigmentosum, development of melanoma of the skin increased by more than 2,000-fold and development of nonmelanoma skin cancers increased by 10,000-fold. (Refer to the PDQ summary on Genetics of Skin Cancer for more information about xeroderma pigmentosum.)
- Senerchia AA, Ribeiro KB, Rodriguez-Galindo C: Trends in incidence of primary cutaneous malignancies in children, adolescents, and young adults: a population-based study. Pediatr Blood Cancer 61 (2): 211-6, 2014.
- Khosravi H, Schmidt B, Huang JT: Characteristics and outcomes of nonmelanoma skin cancer (NMSC) in children and young adults. J Am Acad Dermatol 73 (5): 785-90, 2015.
- Gorlin RJ: Nevoid basal cell carcinoma syndrome. Dermatol Clin 13 (1): 113-25, 1995.
- Kimonis VE, Goldstein AM, Pastakia B, et al.: Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet 69 (3): 299-308, 1997.
- Amlashi SF, Riffaud L, Brassier G, et al.: Nevoid basal cell carcinoma syndrome: relation with desmoplastic medulloblastoma in infancy. A population-based study and review of the literature. Cancer 98 (3): 618-24, 2003.
- Veenstra-Knol HE, Scheewe JH, van der Vlist GJ, et al.: Early recognition of basal cell naevus syndrome. Eur J Pediatr 164 (3): 126-30, 2005.
- Madan V, Lear JT, Szeimies RM: Non-melanoma skin cancer. Lancet 375 (9715): 673-85, 2010.
- Bradford PT, Goldstein AM, Tamura D, et al.: Cancer and neurologic degeneration in xeroderma pigmentosum: long term follow-up characterises the role of DNA repair. J Med Genet 48 (3): 168-76, 2011.
Basal cell carcinomas generally appear as raised lumps or ulcerated lesions, usually in areas with previous sun exposure. These tumors may be multiple and exacerbated by radiation therapy. Squamous cell carcinomas are usually reddened lesions with varying degrees of scaling or crusting, and they have an appearance similar to eczema, infections, trauma, or psoriasis.
- Efron PA, Chen MK, Glavin FL, et al.: Pediatric basal cell carcinoma: case reports and literature review. J Pediatr Surg 43 (12): 2277-80, 2008.
- Griffin JR, Cohen PR, Tschen JA, et al.: Basal cell carcinoma in childhood: case report and literature review. J Am Acad Dermatol 57 (5 Suppl): S97-102, 2007.
Biopsy or excision is necessary to determine the diagnosis of any skin cancer. Diagnosis is necessary for decisions regarding additional treatment. Basal cell carcinomas and squamous cell carcinomas are generally curable with surgery alone and further diagnostic workup is not indicated.
Special Considerations for the Treatment of Children With Cancer
Cancer in children and adolescents is rare, although the overall incidence has been slowly increasing since 1975. Referral to medical centers with multidisciplinary teams of cancer specialists experienced in treating cancers that occur in childhood and adolescence should be considered. This multidisciplinary team approach incorporates the skills of the following health care professionals and others to ensure that children receive treatment, supportive care, and rehabilitation that will achieve optimal survival and quality of life:
- Primary care physicians.
- Pediatric surgeons.
- Radiation oncologists.
- Pediatric medical oncologists/hematologists.
- Rehabilitation specialists.
- Pediatric nurse specialists.
- Social workers.
- Child-life professionals.
(Refer to the PDQ Supportive and Palliative Care summaries for specific information about supportive care for children and adolescents with cancer.)
The American Academy of Pediatrics has outlined guidelines for pediatric cancer centers and their role in the treatment of pediatric patients with cancer. At these pediatric cancer 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. Most of the progress made in identifying curative therapy 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 2010, childhood cancer mortality decreased by more than 50%. Childhood and adolescent cancer survivors require close monitoring because side effects of cancer therapy may persist or develop months or years after treatment. (Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.)
Childhood cancer is a rare disease, with about 15,000 cases diagnosed annually in the United States in individuals younger than 20 years. The U.S. Rare Diseases Act of 2002 defines a rare disease as one that affects populations smaller than 200,000 people. 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. Also, the designation of a pediatric rare tumor is not uniform among international groups, as follows:
- A consensus effort between the European Union Joint Action on Rare Cancers and the European Cooperative Study Group for Rare Pediatric Cancers estimated that 11% of all cancers in patients younger than 20 years could be categorized as very rare. This consensus group defined very rare cancers as those with annual incidences of fewer than 2 cases per 1 million people. However, three additional histologies (thyroid carcinoma, melanoma, and testicular cancer) with incidences of more than 2 cases per 1 million people were also included in the very rare group because there is a lack of knowledge and expertise in the management of these tumors.
- The Children's Oncology Group defines rare pediatric cancers as those listed in the International Classification of Childhood Cancer subgroup XI, which includes thyroid cancer, melanoma and nonmelanoma skin cancers, and multiple types of carcinomas (e.g., adrenocortical carcinoma, nasopharyngeal carcinoma, and most adult-type carcinomas such as breast cancer, colorectal cancer, etc.). These diagnoses account for about 4% of cancers diagnosed in children aged 0 to 14 years, compared with about 20% of cancers diagnosed in adolescents aged 15 to 19 years.
Most cancers in subgroup XI are either melanomas or thyroid cancer, with other types accounting for only 1.3% of cancers in children aged 0 to 14 years and 5.3% of cancers in adolescents aged 15 to 19 years.
These rare cancers are extremely challenging to study because of the low number of patients with any individual diagnosis, the predominance of rare cancers in the adolescent population, and the lack 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 the PDQ summary on Skin Cancer Treatment.
- Smith MA, Seibel NL, Altekruse SF, et al.: Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J Clin Oncol 28 (15): 2625-34, 2010.
- American Academy of Pediatrics: Standards for pediatric cancer centers. Pediatrics 134 (2): 410-4, 2014. Also available online. Last accessed February 1, 2022.
- Smith MA, Altekruse SF, Adamson PC, et al.: Declining childhood and adolescent cancer mortality. Cancer 120 (16): 2497-506, 2014.
- Ward E, DeSantis C, Robbins A, et al.: Childhood and adolescent cancer statistics, 2014. CA Cancer J Clin 64 (2): 83-103, 2014 Mar-Apr.
- Gatta G, Capocaccia R, Botta L, et al.: Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet-a population-based study. Lancet Oncol 18 (8): 1022-1039, 2017.
- DeSantis CE, Kramer JL, Jemal A: The burden of rare cancers in the United States. CA Cancer J Clin 67 (4): 261-272, 2017.
- Ferrari A, Brecht IB, Gatta G, et al.: Defining and listing very rare cancers of paediatric age: consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors. Eur J Cancer 110: 120-126, 2019.
- Pappo AS, Krailo M, Chen Z, et al.: Infrequent tumor initiative of the Children's Oncology Group: initial lessons learned and their impact on future plans. J Clin Oncol 28 (33): 5011-6, 2010.
- Howlader N, Noone AM, Krapcho M, et al., eds.: SEER Cancer Statistics Review, 1975-2012. National Cancer Institute, 2015. Also available online. Last accessed June 22, 2021.
Treatment of Childhood Basal Cell Carcinoma (BCC) and Squamous Cell Carcinoma (SCC) of the Skin
Treatment options for childhood BCC and SCC of the skin include the following:
Treatment for nonmelanoma skin cancer is predominantly surgical, either surgical excision or Mohs micrographic surgery.
Most BCCs have activation of the hedgehog pathway, generally resulting from mutations in PTCH1 (refer to the PDQ summary on Genetics of Skin Cancer for more information about PTCH1). Vismodegib (GDC-0449), a hedgehog pathway inhibitor, has been approved for the treatment of adult patients with metastatic or advanced BCC. This drug also reduces the tumor burden in patients with basal cell nevus syndrome.
(Refer to the PDQ summary on adult Skin Cancer Treatment for more information.)
- Khosravi H, Schmidt B, Huang JT: Characteristics and outcomes of nonmelanoma skin cancer (NMSC) in children and young adults. J Am Acad Dermatol 73 (5): 785-90, 2015.
- Caro I, Low JA: The role of the hedgehog signaling pathway in the development of basal cell carcinoma and opportunities for treatment. Clin Cancer Res 16 (13): 3335-9, 2010.
- Von Hoff DD, LoRusso PM, Rudin CM, et al.: Inhibition of the hedgehog pathway in advanced basal-cell carcinoma. N Engl J Med 361 (12): 1164-72, 2009.
- Sekulic A, Migden MR, Oro AE, et al.: Efficacy and safety of vismodegib in advanced basal-cell carcinoma. N Engl J Med 366 (23): 2171-9, 2012.
- Basset-Séguin N, Hauschild A, Kunstfeld R, et al.: Vismodegib in patients with advanced basal cell carcinoma: Primary analysis of STEVIE, an international, open-label trial. Eur J Cancer 86: 334-348, 2017.
- Tang JY, Mackay-Wiggan JM, Aszterbaum M, et al.: Inhibiting the hedgehog pathway in patients with the basal-cell nevus syndrome. N Engl J Med 366 (23): 2180-8, 2012.
Treatment Options Under Clinical Evaluation for Childhood Basal Cell Carcinoma and Squamous Cell Carcinoma of the Skin
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, refer to the ClinicalTrials.gov website.
The following is an example of a national and/or institutional clinical trial that is currently being conducted:
- APEC1621 (NCT03155620) (Pediatric MATCH: Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients with Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders): NCI-COG Pediatric Molecular Analysis for Therapeutic Choice (MATCH), referred to as Pediatric MATCH, will match targeted agents with specific molecular changes identified in a patient's tumor (refractory or recurrent). Children and adolescents aged 1 to 21 years are eligible for the trial.
Patients with tumors that have molecular variants addressed by treatment arms included in the trial will be offered treatment on Pediatric MATCH. Additional information can be obtained on the NCI website and ClinicalTrials.gov website.
Changes to This Summary (02/23/2022)
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.
Editorial changes were made to this summary.
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® - NCI's Comprehensive Cancer Database pages.
About This PDQ Summary
Purpose of This Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of pediatric basal cell carcinoma and squamous cell carcinoma of the skin. 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.
Reviewers and Updates
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:
- be discussed at a meeting,
- be cited with text, or
- replace or update an existing article that is already cited.
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 Basal Cell Carcinoma and Squamous Cell Carcinoma of the Skin Treatment are:
- Denise Adams, MD (Children's Hospital Boston)
- Karen J. Marcus, MD, FACR (Dana-Farber Cancer Institute/Boston Children's Hospital)
- Paul A. Meyers, MD (Memorial Sloan-Kettering Cancer Center)
- Thomas A. Olson, MD (Aflac Cancer and Blood Disorders Center of Children's Healthcare of Atlanta - Egleston Campus)
- Alberto S. Pappo, MD (St. Jude Children's Research Hospital)
- Arthur Kim Ritchey, MD (Children's Hospital of Pittsburgh of UPMC)
- Carlos Rodriguez-Galindo, MD (St. Jude Children's Research Hospital)
- Stephen J. Shochat, MD (St. Jude Children's Research Hospital)
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.
Levels of Evidence
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.
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The preferred citation for this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Basal Cell Carcinoma and Squamous Cell Carcinoma of the Skin Treatment. Bethesda, MD: National Cancer Institute. Updated
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