Childhood Basal Cell Carcinoma and Squamous Cell Carcinoma of the Skin Treatment (PDQ®): Treatment - Health Professional Information [NCI]

Incidence and Risk Factors

Nonmelanoma skin cancers include basal cell carcinoma (BCC) and squamous cell carcinoma (SCC). These skin cancers are very rare in children and adolescents. Based on National Childhood Cancer Registry data from 2016 to 2020, the incidence rate of skin carcinomas in children younger than 20 years was 0.1 cases per 1 million.[1]

In one series of 28 patients, approximately one-half of patients had predisposing conditions such as Gorlin syndrome (also known as nevoid basal cell carcinoma syndrome or basal cell nevus syndrome), and one-half of patients were exposed to iatrogenic conditions such as prolonged immunosuppression or radiation.[2]

Gorlin syndrome is a rare autosomal dominant disorder that is associated with germline PTCH1 or SUFU pathogenic variants.[3,4] This syndrome predisposes patients to develop early-onset neoplasms, including BCCs, ovarian fibromas, and desmoplastic medulloblastomas.[5,6,7,8] For more information, see the Basal cell nevus syndrome section in Genetics of Skin Cancer.

A retrospective analysis identified 25 BCCs in 11 patients with Gorlin syndrome.[9] Eighty percent of the BCCs occurred on the head and neck, and 64% of the specimens demonstrated infundibulocystic differentiation.

BCCs and SCCs in adults have been associated with exposure to solar UV radiation, iatrogenic immunosuppression, UVB radiation, and other risk factors.[10]

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.[11] For more information about xeroderma pigmentosum, see Genetics of Skin Cancer.

References:

1. National Cancer Institute: NCCR*Explorer: An interactive website for NCCR cancer statistics. Bethesda, MD: National Cancer Institute. Available online. Last accessed August 23, 2024.
2. 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.
3. Hahn H, Wicking C, Zaphiropoulous PG, et al.: Mutations of the human homolog of Drosophila patched in the nevoid basal cell carcinoma syndrome. Cell 85 (6): 841-51, 1996.
4. Johnson RL, Rothman AL, Xie J, et al.: Human homolog of patched, a candidate gene for the basal cell nevus syndrome. Science 272 (5268): 1668-71, 1996.
5. Gorlin RJ: Nevoid basal cell carcinoma syndrome. Dermatol Clin 13 (1): 113-25, 1995.
6. 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.
7. 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.
8. 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.
9. Nguyen CV, Rubin AI, Smith A, et al.: Retrospective analysis of the histopathologic features of basal cell carcinomas in pediatric patients with basal cell nevus syndrome. J Cutan Pathol 48 (3): 390-395, 2021.
10. Madan V, Lear JT, Szeimies RM: Non-melanoma skin cancer. Lancet 375 (9715): 673-85, 2010.
11. 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.

Clinical Presentation

Basal cell carcinomas (BCCs) generally appear as raised lumps or ulcerated lesions, usually in areas with previous sun exposure.[1] Multiple BCCs may be present, and they are exacerbated by radiation therapy.[2] Squamous cell carcinomas (SCCs) are usually reddened lesions with varying degrees of scaling or crusting. SCCs have an appearance similar to eczema, infections, trauma, or psoriasis.

References:

1. 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.
2. 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.

Diagnostic Evaluation

Biopsy or excision is necessary to diagnose any skin cancer. A specific diagnosis is necessary for decisions regarding treatment. Basal cell carcinomas and squamous cell carcinomas are generally curable with surgery alone, and further diagnostic workup is not indicated.[1,2]

References:

1. Rubin AI, Chen EH, Ratner D: Basal-cell carcinoma. N Engl J Med 353 (21): 2262-9, 2005.
2. Alam M, Ratner D: Cutaneous squamous-cell carcinoma. N Engl J Med 344 (13): 975-83, 2001.

Special Considerations for the Treatment of Children With Cancer

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:

• Primary care physicians.
• Pediatric surgeons.
• Pathologists.
• Pediatric radiation oncologists.
• Pediatric medical oncologists and hematologists.
• Ophthalmologists.
• Rehabilitation specialists.
• Pediatric oncology nurses.
• Social workers.
• Child-life professionals.
• Psychologists.
• Nutritionists.

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%.[3,4,5] 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:

• 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 two cases per 1 million people. However, three additional histologies (thyroid carcinoma, melanoma, and testicular cancer) with incidences of more than two cases per 1 million people were also included in the very rare group due to a lack of knowledge and expertise in the management of these tumors.[9]
• The Children's Oncology Group defines rare pediatric cancers as those listed in the International Classification of Childhood Cancer subgroup XI, which includes thyroid cancers, melanomas and nonmelanoma skin cancers, and multiple types of carcinomas (e.g., adrenocortical carcinomas, nasopharyngeal carcinomas, and most adult-type carcinomas such as breast cancers and colorectal cancers).[10] These diagnoses account for about 5% of the cancers diagnosed in children aged 0 to 14 years and about 27% of the cancers diagnosed in adolescents aged 15 to 19 years.[4]

  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 Skin Cancer Treatment.

References:

1. 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.
2. American Academy of Pediatrics: Standards for pediatric cancer centers. Pediatrics 134 (2): 410-4, 2014. Also available online. Last accessed August 23, 2024.
3. Smith MA, Altekruse SF, Adamson PC, et al.: Declining childhood and adolescent cancer mortality. Cancer 120 (16): 2497-506, 2014.
4. National Cancer Institute: NCCR*Explorer: An interactive website for NCCR cancer statistics. Bethesda, MD: National Cancer Institute. Available online. Last accessed August 23, 2024.
5. Surveillance Research Program, National Cancer Institute: SEER*Explorer: An interactive website for SEER cancer statistics. Bethesda, MD: National Cancer Institute. Available online. Last accessed September 5, 2024.
6. 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.
7. 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.
8. DeSantis CE, Kramer JL, Jemal A: The burden of rare cancers in the United States. CA Cancer J Clin 67 (4): 261-272, 2017.
9. 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.
10. 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.

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:

1. Surgery.
2. Targeted therapy.

Surgery

Treatment for nonmelanoma skin cancer is predominantly surgical, either surgical excision or Mohs micrographic surgery.[1]

Targeted Therapy

Most BCCs have activation of the hedgehog pathway, generally resulting from variants in PTCH1. For more information about PTCH1, see Genetics of Skin Cancer.[2]

• Vismodegib. In adults, vismodegib (GDC-0449), a hedgehog pathway inhibitor, has been approved by the U.S. Food and Drug Administration (FDA) for the treatment of metastatic or advanced BCC.[3,4,5] This drug also reduces the tumor burden in patients with basal cell nevus syndrome.[6] In a case report, one child with xeroderma pigmentosum and a nodular BCC achieved a complete clinical response with vismodegib.[7]
• Cemiplimab. No prospective trials of cemiplimab (PD-1 inhibitor) have been conducted in children and adolescents. However, the FDA approved cemiplimab for use in patients with metastatic or locally advanced cutaneous SCC who are not candidates for surgery or radiation and in patients with locally advanced or metastatic BCC previously treated with a hedgehog pathway inhibitor or for whom a hedgehog pathway inhibitor is not appropriate.[8,9]

For more information, see Skin Cancer Treatment.

References:

1. 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.
2. 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.
3. 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.
4. 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.
5. 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.
6. 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.
7. Fife D, Laitinen MA, Myers DJ, et al.: Vismodegib Therapy for Basal Cell Carcinoma in an 8-Year-Old Chinese Boy with Xeroderma Pigmentosum. Pediatr Dermatol 34 (2): 163-165, 2017.
8. Stratigos AJ, Sekulic A, Peris K, et al.: Cemiplimab in locally advanced basal cell carcinoma after hedgehog inhibitor therapy: an open-label, multi-centre, single-arm, phase 2 trial. Lancet Oncol 22 (6): 848-857, 2021.
9. Migden MR, Rischin D, Schmults CD, et al.: PD-1 Blockade with Cemiplimab in Advanced Cutaneous Squamous-Cell Carcinoma. N Engl J Med 379 (4): 341-351, 2018.

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, see the ClinicalTrials.gov website.

Latest Updates to This Summary (09 / 11 / 2024)

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.

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)
• William H. Meyer, MD
• 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 <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/skin/hp/child-skin-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 31909941]

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Last Revised: 2024-09-11