Correspondence

Melanoma in Children

N Engl J Med 1995; 333:255-257July 27, 1995

Article

To the Editor:

At the end of the Current Concepts article on melanoma in children (March 9 issue),1 the authors stress that “the importance of early diagnosis with prompt surgical excision of primary cutaneous melanoma cannot be overemphasized. It remains the most reliable way to cure children of this disease.” Although we obviously agree with this statement, it unfortunately does not obviate the diagnostic difficulties of clinicians who daily see pigmented skin lesions in children. These difficulties may arise from the alarming features of some benign nevi, set against the knowledge that melanoma is extremely rare in prepubertal children. Moreover, there are typical2 and atypical3 clinical forms of the disease, posing a further diagnostic challenge (e.g., nodular or pedunculate forms, and partially or totally amelanotic lesions).

To identify guidelines for managing pigmented lesions in prepubescent children, we reviewed all the cases in which lesions were observed and excised at the National Cancer Institute of Milan over a period of 16 years.4 Of the 656 lesions surgically removed, only 2 (0.30 percent) were histologically verified as cutaneous melanoma. In 31 cases, the preoperative clinical diagnosis was suspected melanoma (including the 2 that were histologically confirmed).

Diagnostic excision of a pigmented lesion in children is warranted only if there is a well-founded clinical suspicion of malignancy or if the lesion has atypical morphology. When there is uncertainty, we suggest observation only. When excision is justified, the width should be limited (to 2 mm from lesion borders) for functional and aesthetic reasons. Histologically confirmed melanoma can be more widely excised later. We have drastically reduced the number of such operations and have spared many children and parents unnecessary stress as well as some complications.

Aldo Bono, M.D.
Cesare Bartoli, M.D.
Ivan Del Prato, M.D.
Istituto Nazionale per lo Studio e la Cura dei Tumori, 20133 Milan, Italy

4 References

1
Ceballos PI, Ruiz-Maldonado R, Mihm MC Jr. Melanoma in children. N Engl J Med 1995;332:656-662
Full Text | Web of Science | Medline

2
Crotty KA, McCarthy SW, Palmer AA, et al. Malignant melanoma in childhood: a clinicopathologic study of 13 cases and comparison with Spitz nevi. World J Surg 1992;16:179-185
CrossRef | Web of Science | Medline

3
Sybert V. Six children with malignant melanoma. J Am Acad Dermatol 1991;24:666-667
CrossRef | Web of Science | Medline

4
Bono A, Bartoli C, Zurrida SM, Del Prato I, Clemente C, Cascinelli N. Let's stop worrying about pigmented skin lesions in children. Eur J Cancer 1994;30:417-417 abstract.
CrossRef | Web of Science

To the Editor:

Although Ceballos et al. summarized the clinical conditions associated with an increased risk of melanoma in children and briefly reviewed therapeutic approaches, they overlooked an opportunity to emphasize the importance of prevention and education. Although there is no definite proof that primary prevention (i.e., the avoidance of sunlight at peak hours and the use of protective clothing and sunscreens) has had a positive effect on the melanoma epidemic, it is clear that solar exposure has an important pathogenic role in melanoma.1 Harrison et al.2 reported recently that short-term and long-term sun exposure during childhood contributes substantially to the development of acquired melanocytic nevi, pigmented lesions associated with an increased risk of melanoma. Also, migration studies have shown that early childhood is a critical period for skin damage induced by ultraviolet light.1 These examples underscore the importance of establishing public-education programs to encourage risk avoidance from early childhood on. In addition, better education of parents and primary health care providers about the warning signs (asymmetry, border irregularity, color haphazard, and diameter >6 mm) and predisposing conditions (dysplastic nevi, a family history of melanoma, large numbers of banal nevi) should facilitate earlier diagnosis and treatment of pediatric melanoma.3

Alberto S. Pappo, M.D.
Charles B. Pratt, M.D.
St. Jude Children's Research Hospital, Memphis, TN 38101

3 References

1
Koh HK. Cutaneous melanoma. N Engl J Med 1991;325:171-182
Full Text | Web of Science | Medline

2
Harrison SL, MacLennan R, Speare R, Wronski I. Sun exposure and melanocytic naevi in young Australian children. Lancet 1994;344:1529-1532
CrossRef | Web of Science | Medline

3
National Institutes of Health Development Conference: diagnosis and treatment of early melanoma. NIH Consens Statement 1992;10:1-26
Medline

Author/Editor Response

The authors reply:

To the Editor: We agree with Dr. Bono and his colleagues regarding the difficulties commonly encountered during clinical assessment and management of melanocytic lesions in children. We acknowledge that many benign nevi have an alarming appearance, which is usually due to dark pigmentation.1 However, other clinical signs suggesting malignant melanoma may also be present, such as border irregularity, asymmetry, or recent change. The rare malignant melanomas with deceptively innocuous clinical features, such as absence of pigment and polypoid or verrucous architecture, often evoke suspicion because of a patient's history.2,3 Any reported change in a cutaneous lesion, such as increasing size or a change in color, shape, or surface features, should alert the clinician to the possibility of malignancy.

Bono et al. point out that 2 of 31 cases (6 percent) in which malignant melanoma was suspected clinically were in fact malignant melanoma on histologic examination.1 Although we agree that malignant melanoma is extremely rare in childhood, an incidence of 6 percent among clinically worrisome cases is substantial, considering the potentially devastating consequences of delaying this diagnosis. We strongly recommend that suspect lesions be conservatively removed by surgical excision as early as possible and that the margins of the excision be widened if malignant melanoma is histologically confirmed. In “uncertain” cases in which clinical criteria are lacking, we recommend close clinical follow-up with periodic photographic documentation. If a lesion undergoes a change in appearance, biopsy or excision should not be delayed.

Drs. Pappo and Pratt rightly remind us of the importance of public education in the prevention of malignant melanoma. We agree that public education should begin reinforcing responsible attitudes and behavior in early childhood. In this regard, the importance of averting blistering or painful sunburns during childhood and adolescence and of recognizing the increased risk of melanoma associated with poor tanning ability in adolescence cannot be overemphasized.4 With respect to the report by Harrison et al. that Pappo and Pratt cite, we wonder whether there is a correlation between higher counts of melanocytic nevi and cutaneous malignant melanoma in children and adolescents5 similar to that in adults.

Although we recognize the usefulness of the A-B-C-D criteria in the clinical evaluation of pigmented lesions, we question the validity of a requisite lesional diameter greater than 6 mm, considering that many malignant melanomas are substantially smaller.6 Accordingly, small size should not preclude total excision or biopsy, especially if other signs or symptoms suggesting malignant melanoma are present.

Patricia I. Ceballos, M.D.
New York University Medical Center, New York, NY 10016

Ramon Ruiz-Maldonado, M.D.
National Institute of Pediatrics, Mexico City, Mexico 04530

Martin C. Mihm, Jr., M.D.
Albany Medical College, Albany, NY 12208-3479

6 References

1
Bono A, Bartoli C, Zurrida SM, Del Prato I, Clemente C, Cascinelli N. Let's stop worrying about pigmented skin lesions in children. Eur J Cancer 1994;30:417-417 abstract.
CrossRef | Web of Science

2
Roth ME, Grant-Kels JM, Kuhn MK, Greenberg RD, Hurwitz S. Melanoma in children. J Am Acad Dermatol 1990;22:265-274
CrossRef | Web of Science | Medline

3
Pratt CB, Palmer MK, Thatcher N, Crowther D. Malignant melanoma in children and adolescents. Cancer 1981;47:392-397
CrossRef | Web of Science | Medline

4
Lew RA, Sober AJ, Cook N, Marvell R, Fitzpatrick TB. Sun exposure habits in patients with cutaneous melanoma: a case control study. J Dermatol Surg Oncol 1983;9:981-986
Medline

5
Holly EA, Kelly JW, Shpall SN, Chiu S-H. Number of melanocytic nevi as a major risk factor for malignant melanoma. J Am Acad Dermatol 1987;17:459-468
CrossRef | Web of Science | Medline

6
Kamino H, Kiryu H, Ratech H. Small malignant melanomas: clinicopathologic correlation and DNA ploidy analysis. J Am Acad Dermatol 1990;22:1032-1038
CrossRef | Web of Science | Medline

Citing Articles (5)

Citing Articles

1
S. A. Oliveria, J. M. Satagopan, A. C. Geller, S. W. Dusza, M. A. Weinstock, M. Berwick, M. Bishop, M. K. Heneghan, A. C. Halpern. (2008) Study of Nevi in Children (SONIC): Baseline Findings and Predictors of Nevus Count. American Journal of Epidemiology 169:1, 41-53
CrossRef
2
A MARGHOOB, J BORREGO, A HALPERN. (2007) Congenital Melanocytic Nevi: Treatment Modalities and Management Options. Seminars in Cutaneous Medicine and Surgery 26:4, 231-240
CrossRef
3
A. Rütten. (2007) Maligne Melanome bei Kindern und Jugendlichen. Der Pathologe 28:6, 437-444
CrossRef
4
Aldo Bono, Andrea Ferrari. (2005) Early diagnosis remains the most reliable way to cure children with melanoma. Pediatric Blood & Cancer 45:3, 355-355
CrossRef
5
A MARGHOOB, J BORREGO, A HALPERN. (2003) Congenital melanocytic nevi: Treatment modalities and management options. Seminars in Cutaneous Medicine and Surgery 22:1, 21-32
CrossRef