This Article  • Abstract  • PDF  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on Web of Science (8)  • Contact me when this article is cited  Related Content  • Related article  • Similar articles in this journal  Topic Collections  • Dermatology  • Dermatology, Other  • Alert me on articles by topic  Social Bookmarking   What's this?

Report of 3 Cases

Benjamin W. LeSueur, BS; Nancy G. Silvis, MD; Ronald C. Hansen, MD

Arch Dermatol. 2000;136:370-372.

ABSTRACT
Background  The peak incidence of basal cell carcinoma occurs in the seventh decade of life and is rare in children. When found in the pediatric age group, basal cell carcinoma is usually associated with a genetic defect, such as basal cell nevus syndrome, xeroderma pigmentosum, or nevus sebaceus. In areas of intense UV radiation exposure, such as the southwestern United States, children may be at increased risk of developing this malignancy de novo.

Observations  Three children (2 boys, aged 8 and 16 years, and an 11-year-old girl) from Tucson, Ariz, with isolated basal cell carcinoma unassociated with any other disease or syndrome are described.

Conclusions  Basal cell carcinoma in children is probably the result of a combination of UV radiation exposure and genetic background. Early recognition in children can prevent extensive tissue destruction and excess scarring after excision. A higher index of suspicion for basal cell carcinoma may also aid in prompt diagnosis of a possible genetic disorder, such as basal cell nevus syndrome.

INTRODUCTION

Jump to Section  • Top  • Introduction  • Report of cases  • Comment  • Author information  • References

BASAL CELL carcinoma (BCC) in children is rare. Cases of BCC in the pediatric population have been reported in association with basal cell nevus syndrome,¹ xeroderma pigmentosum,² and nevus sebaceus³ and after high-dose radiotherapy.⁴ Isolated cases of BCC unrelated to one of these causes are seldom reported in pediatric patients. Consequently, clinicians often have a low index of suspicion, leading to delay in diagnosis. We report 3 cases of de novo BCC in children who presented to the dermatology clinic at the University of Arizona Medical Center, Tucson. These children had no known genetic syndromes and had not undergone radiotherapy.

REPORT OF CASES

Jump to Section  • Top  • Introduction  • Report of cases  • Comment  • Author information  • References

CASE 1

A 16-year-old Hispanic boy presented with a solitary heme-crusted nodule that had a raised ringlike border below the left orbit (Figure 1). He had first noticed the lesion 2 years earlier, when it was a much smaller papule. Since that time, the lesion had enlarged considerably. No family history of skin cancer was noted. The differential diagnosis included BCC, discoid lupus erythematosus, and deep fungal infection. A diagnostic biopsy specimen was obtained and sent for histologic evaluation. The specimen showed BCC. A dermatologic surgeon excised the lesion using the Mohs micrographic surgery technique. There were no recurrences or new primary skin cancers during 4 years of follow-up.

View larger version (65K): [in this window] [in a new window] Figure 1. Patient 1. Basal cell carcinoma on the left cheek of a 16-year-old boy.

CASE 2

An 11-year-old white girl with skin type III was seen for the evaluation and treatment of nonresponsive acne vulgaris. During the initial examination, a 3-mm dome-shaped, slightly translucent papule was noted on the left nasal labial fold. The patient's family history was negative for skin cancer. The differential diagnosis included epithelial cyst, fibrous papule, and BCC. Histologic examination of a diagnostic biopsy specimen showed nodular nests of basal neoplastic cells invading the dermis, consistent with nodular BCC. The lesion was excised using the Mohs micrographic surgery technique. Follow-up 4 years later revealed no recurrences and no new primary tumors.

CASE 3

An 8-year-old boy with a history of several severe sunburns presented with a growing, spontaneously bleeding, 3-mm papule under the left eye (Figure 2). The lesion was umbilicated and had overlying telangiectasias. The patient had no family history of skin cancer. The differential diagnosis included BCC and molluscum contagiosum. A diagnostic biopsy specimen revealed nests of basal neoplastic cells in the dermis, consistent with nodular BCC. The patient and his parents elected to have the lesion removed by electrodesiccation and curettage. Two years after treatment, the patient had no recurrences and no new skin cancers.

View larger version (82K): [in this window] [in a new window] Figure 2. Patient 3. Basal cell carcinoma under the left eye of an 8-year-old boy. Note the absence of other features of photodamage in surrounding skin.

COMMENT

Jump to Section  • Top  • Introduction  • Report of cases  • Comment  • Author information  • References

Nonmelanoma skin cancers are the most common malignant neoplasms in the United States, representing one third of all cancers diagnosed every year.^5-6 Basal cell carcinoma represents 75% of nonmelanoma skin cancers and has an estimated annual incidence of more than 700,000 cases nationally.^7-8 The US average annual incidence of BCC in whites is currently 191 per 100,000 and is increasing at a rate of 3% to 7% per year.^{7, 9}

Ultraviolet radiation exposure is partly responsible for both BCC and squamous cell carcinoma, as evidenced by their increased prevalence after chronic exposure to sunlight and the preponderance of these lesions on sun-damaged skin. Although squamous cell carcinoma is associated with cumulative sun exposure, BCC in younger patients does not show this association.^10-11 D'Errico et al¹⁰ report that BCC arising before the age of 40 years corresponds with childhood or recreational sun exposure but does not correlate directly with cumulative sun damage. Thus, in areas of the world where the UV radiation is most intense, such as the Sunbelt in the United States, childhood sun exposure is at a maximum and younger patients are at a higher risk of developing BCC.

Other factors besides sunlight are reported to influence the development of BCC. Gailani et al¹¹ note a strong association between BCC and the inactivation of a gene at chromosome 9q22, which is thought to be a tumor suppressor. Inactivation of this gene was found in tumor tissue in 68% of BCCs examined and did not correlate directly with sun exposure or age. The cause of this mutation is unknown, but possible factors may include ionizing radiation, arsenicals, and polyaromatic hydrocarbons. Basal cell nevus syndrome and xeroderma pigmentosum represent inherited genetic mutations that predispose those affected to BCC. Patients with basal cell nevus syndrome are found to have a germline mutation on chromosome 9.¹²

The peak incidence of BCC occurs in the seventh decade of life.¹³ In the pediatric age group, BCC usually occurs in the setting of a known genetic defect (Table 1). Although uncommon, isolated BCC in children without these conditions has been reported.^14-29 Price et al¹⁴ described a 17-year-old boy with a solitary BCC of the nose. The patient had a history of sunburns 1 or 2 times per year since the age of 9 years. His mother had a BCC removed at the age of 44 years. Histologically, the tumor was described as superficial BCC. Scobie and Preston¹⁷ described a 4-year-old boy with a BCC of the scalp. The patient presented with a small "cyst" on the occipital region of the scalp and a family history of skin cancer. The lesion, described histologically as well defined, recurred 8 months after excision. Excision was repeated without recurrence of tumor, based on follow-up 1 year later.¹⁷ A 12-year-old boy living in Arizona was described by Comstock et al¹⁸ with a BCC on the nose. The lesion had been present since his nose was scratched by a cat 1 year earlier. The youngest patient with BCC, a 27-month-old infant, was described by Keramidas and Anagnostou.²¹ In this case, the lesion grew rapidly and ulcerated after a 4-month delay in diagnosis.

View this table: [in this window] [in a new window] Genetic Defects Predisposing Children to Basal Cell Carcinoma (BCC)

It is debatable whether BCC is more aggressive in children. Leffell et al³⁰ defined aggressive-growth BCC as sclerosing, morpheaform, infiltrative, or invasive into nerves. Their retrospective review showed an increased occurrence of aggressive-growth BCC in patients younger than 35 years old compared with older patients. In contrast, Betti et al¹³ and Dinehart et al¹⁶ found no increase in the frequency of the morpheaform pattern in younger patients. All 3 of our patients had histologically less aggressive forms of BCC.

As total incidence rates of BCC continue to rise, childhood cases may become more common. This increase in pediatric BCC may be especially true in areas of high-level UV radiation exposure. The percentage of sunny days during the year, higher altitude, and location closer to the equator may place children in these areas at increased risk. Early recognition can prevent extensive tissue destruction and scarring after excision and aid in prompt diagnosis of a possible genetic syndrome. We recommend that clinicians have a higher index of suspicion for BCC when evaluating questionable lesions in children.

AUTHOR INFORMATION

Jump to Section  • Top  • Introduction  • Report of cases  • Comment  • Author information  • References

Accepted for publication July 11, 1999.

Reprints not available from the authors.

From the Section of Dermatology, University of Arizona College of Medicine, Tucson.

REFERENCES

Jump to Section  • Top  • Introduction  • Report of cases  • Comment  • Author information  • References

1. Gorlin RJ, Goltz RW. Multiple nevoid basal-cell epithelioma, jaw cysts and bifid rib. N Engl J Med. 1960;262:908-912. 2. Leibowitz E, Janniger CK, Schwartz RA, Lambert WC. Xeroderma pigmentosum. Cutis. 1997;60:75-77, 81-84. WEB OF SCIENCE | PUBMED 3. Goldstein GD, Whitaker DC, Argenyi ZB, Bardach J. Basal cell carcinoma arising in a sebaceous nevus during childhood. J Am Acad Dermatol. 1988;18:429-430. WEB OF SCIENCE | PUBMED 4. Garcia-Silva J, Velasco-Benito JA, Pena-Penabad C, Armijo M. Basal cell carcinoma in a girl after cobalt irradiation to the cranium for acute lymphoblastic leukemia: case report and literature review. Pediatr Dermatol. 1996;13:54-57. WEB OF SCIENCE | PUBMED 5. Silverberg E, Lubera JA. Cancer statistics, 1989. CA Cancer J Clin. 1989;39:3-20. FREE FULL TEXT 6. Boring CC, Squires TS, Tong T. Cancer statistics, 1991. CA Cancer J Clin. 1991;41:19-36. WEB OF SCIENCE | PUBMED 7. Silverberg E, Boring CC, Squires TS. Cancer statistics, 1990. CA Cancer J Clin. 1990;40:9-26. WEB OF SCIENCE | PUBMED 8. Parker SL, Tang T, Bolden S, Wingo PA. Cancer statistics, 1997. CA Cancer J Clin. 1997;47:5-27. WEB OF SCIENCE | PUBMED 9. Green A. Changing patterns in incidence of nonmelanoma skin cancer. Epithelial Cell Biol. 1992;1:47-51. PUBMED 10. D'Errico M, Calcagnile AS, Corona R, et al. p53 mutations and chromosome instability in basal cell carcinomas developed at an early or late age. Cancer Res. 1997;57:747-752. FREE FULL TEXT 11. Gailani MR, Leffell DJ, Zeigler A, Gross EG, Brash DE, Bale AE. Relationship between sunlight exposure and a key genetic alteration in basal cell carcinoma. J Natl Cancer Inst. 1996;88:349-354. FREE FULL TEXT 12. Gailani MR, Bale SJ, Leffel DJ, DiGiovanna JJ, Peck GL, Poliak S. Developmental defects in Gorlin syndrome related to a putative tumor suppressor gene on chromosome 9. Cell. 1992;69:111-117. FULL TEXT | WEB OF SCIENCE | PUBMED 13. Betti R, Inselvini E, Carducci M, Crosti C. Age and site prevalence of histologic subtypes of basal cell carcinomas. Int J Dermatol. 1995;34:174-176. WEB OF SCIENCE | PUBMED 14. Price MA, Goldberg LH, Levy ML. Juvenile basal cell carcinoma. Pediatr Dermatol. 1994;11:176-177. WEB OF SCIENCE | PUBMED 15. Cox NH. Basal cell carcinoma in young adults. Br J Dermatol. 1992;127:26-29. FULL TEXT | WEB OF SCIENCE | PUBMED 16. Dinehart SM, Dodge R, Stanley WE, Franks HH, Pollack SV. Basal cell carcinoma treated with Mohs surgery: a comparison of 54 younger patients with 1050 older patients. J Dermatol Surg Oncol. 1992;18:560-566. WEB OF SCIENCE | PUBMED 17. Scobie WG, Preston J. Basal cell carcinoma in children. J R Coll Surg Edinb. 1992;37:46-47. PUBMED 18. Comstock J, Hansen RC, Korc A. Basal cell carcinoma in a 12-year-old boy. Pediatrics. 1990;86:460-462. FREE FULL TEXT 19. Cullen KW, Bleach NR, Green DM. Juvenile basal cell carcinoma. Br J Clin Pract. 1989;43:419-420. WEB OF SCIENCE | PUBMED 20. Fliss DM, Hauben DJ, Ben-Meir P, Sion-Vardy N. Solitary basal cell carcinoma in a child. Ann Plast Surg. 1989;22:43-46. FULL TEXT | WEB OF SCIENCE | PUBMED 21. Keramidas DC, Anagnostou D. Basal cell carcinoma of the lower lid in a 27-month-old child. Z Kinderchir. 1987;42:250-251. WEB OF SCIENCE | PUBMED 22. Rahbari H, Mehregan AH. Basal cell epithelioma (carcinoma) in children and teenagers. Cancer. 1982;49:350-353. FULL TEXT | WEB OF SCIENCE | PUBMED 23. Henriksson C, Eldh J, Hersle K, Suurkula M. Basal cell carcinoma in children: case report. Scand J Plast Reconstr Surg. 1981;15:157-158. WEB OF SCIENCE | PUBMED 24. Hernandez-Perez E. Basal cell carcinoma in children. Dermatologica. 1975;150:311-315. WEB OF SCIENCE | PUBMED 25. Milstone EB, Helwig EB. Basal cell carcinoma in children. Arch Dermatol. 1973;108:523-527. FREE FULL TEXT 26. Coskey RJ, Chow C. Basal cell epitheliomas in children and young adolescents. Cutis. 1973;12:224-226. 27. Botvinick I, Mehregan AH, Weissman F. Morphea-like basal cell epithelioma in a child. Arch Dermatol. 1967;95:67-68. FREE FULL TEXT 28. Murray JE, Cannon B. Basal-cell cancer in children and young adults. N Engl J Med. 1960;262:440-443. 29. Sewell RL. Basal cell carcinoma in youth. Arch Surg. 1941;42:909-912. FREE FULL TEXT 30. Leffel DJ, Headington JT, Wong DS, Swanson NA. Aggressive-growth basal cell carcinoma in young adults. Arch Dermatol. 1991;127:1663-1667. FREE FULL TEXT

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

RELATED ARTICLE

Archives of Dermatology Reader's Choice: Continuing Medical Education
Arch Dermatol. 2000;136(3):438-439.
FULL TEXT