If When parents bring their children to Dr. Caroline Piggott to have a suspicious mole on the scalp or other part of the body examined, the vast majority turn out to be benign because the incidence of melanoma is low, especially before puberty.
«Only 1 to 2% of all melanoma in the world occurs in children, so my job is primarily to provide calming,» Caroline Piggott, a pediatric dermatologist at Scripps MD Anderson Cancer Center in San Diego, said at the annual Cutaneous Malignancy Update.»There are few pediatric studies. Why? Because children are excluded from most clinical trials of melanoma. Our treatment is primarily based on the National Comprehensive Cancer Network’s guidelines for adults.»
To help parents identify melanoma, clinicians typically recommend «ABCDE» rule: asymmetry, border irregularity, color variation (especially dark or multiple colors), diameter greater than 6 mm, and development (does it change, bleed, or is it painful?) .
While Caroline Piggott believes the standard ABCDE rules are important—especially in older children and adolescents—scientists around Kelly M. Cordoro, Professor of Dermatology at the University of California, San Francisco (UCSF), modified ABCD criteria.They relied on the analysis of a cohort of 60 children who were diagnosed with melanoma at UCSF between 1984 and 2009 and 10 children who were diagnosed with unclear melanoma treated as melanoma during the same period at UCSF.
The scientists divided the patients into two groups: the 0- to 10-year-olds (19; Group A) and 11- to 19-year-olds (51; Group B). They found that 60% of children in group A and 40% of children in group B did not meet the traditional ABCDE criteria for children. Of the 60 melanoma patients, 10 died. Of these, 9 were older than 10 years, and 70% had amelanotic lesions. Based on their analysis of clinical and histopathological data and results, Dr. Cordoro and colleagues proposed additional ABCD criteria where A stands for amelanotic, B for bleeding or bumps, C for color uniformity, and D for de novo or any diameter.
«That doesn’t mean throwing the old ABCDE criteria out the window,» Caroline Piggott said.»It means using these modified criteria in conjunction with traditional ABCDE rules.»
Risk factors for childhood melanoma are similar to those in adults and include a family history of melanoma, large/huge congenital nevi, the presence of many atypical appearing nevi, Fitzpatrick’s skin types I or II, a history of blistering sunburns, and the presence of genetic abnormalities such as xeroderma pigmentosum.
According to a Analysis of datades Surveillance, Epidemiology, and End Results(SEER) program, melanomine incidence increased in to all persons in the United States aged 0 to 19 years between 1973 and 2009. Key risk factors included white race, female gender, and living in a SEER registry, which was classified as low UVB exposure.During the study period, melanomas on the face and trunk increased in boys, while melanomas on the lower limbs and hips increased in girls.
Recently, scientists evaluated data from 988,103 cases of invasive melanoma from the 2001-2015 SEER database to determine the age-specific incidenceof melanoma in the United States. In 2015, 83,362 cases of invasive melanoma were reported for all age groups. Of these, only 67 cases were younger than 10 years, 251 were between 10 and 19 years old, and 1,973 were young adults between 20 and 29 years old.
According to other results, the overall incidence of invasive melanoma increased from 200 million to 229 cases per million person-years for all age groups between 2006 and 2015. «However, there was a statistically significant decrease in melanoma incidence among 10- to 19-year-olds and 10- to 29-year-olds,» said Piggott, who was not involved in the study.»The hypothesis is that health policy efforts to prevent sun exposures and the use of solariums could influence the incidence of melanoma in younger populations. What is interesting, however, is that young adult women have twice the risk of melanoma as young adult men.»
In a separate study scientists prospectively followed 60 families at risk of melanoma over a period of up to 40 years to determine the risk of pediatric melanoma in families with and without mutations of the cyclin-dependent kinase inhibitor 2A (CDKN2A). Regardless of their CDKN2A status, the percentage of pediatric melanoma cases was 6 to 28 times higher in the families at risk of melanoma compared to the general population. In addition, families who were CDKN2A positive had a significantly higher rate of pediatric melanoma cases than families who were CDKN2A negative (11.1% versus 2.5%; P = 0.004).
As for the treatment of melanoma in children, the standard of care is similar to that of adults: usually a wide local surgical excision of the primary lesion, depending on the depth.In sentinel lymph node biopsy, doctors are usually guided by the parameters for adults, such as the depth of the lesion and ulceration.
«We know that a positive sentinel lymph node has prognostic value, but there’s a lot of debate about whether a lymph node dissection should be performed if the sentinel lymph node is positive,» Dr. Piggott said at the meeting, which was hosted by Scripps MD Anderson Cancer Center. «This will be decided on a case-by-case basis. We take into account factors such as: Are the nodes palpable? Are there signs on ultrasound? But there are no formal guidelines.»
There are few studies on systemic therapy in children, as this population is excluded from most clinical trials of melanoma. «In the past, interferon was sometimes used,» she said. «But in recent years, as with adults, we have begun to use targeted immunological therapy.This is usually carried out by an academic oncology centre with tertiary care.»
The chances of survival for children with melanoma are good if the tumors are detected early. As in adults, the stage is strongly correlated with survival, and distant metastases are associated with a poor prognosis.
In 2020, scientists published a retrospective, multicenter reviewof 38 cases of fatal pediatric melanoma between 1994 and 2017. The analysis was limited to people aged 20 years and younger who were treated at 12 academic medical centers. Of the 38 patients, 42% were male, 58% female and 57% were white. In addition, 19% were Hispanic, «which is a larger percentage than the deaths in the adult [Hispanic] population with melanoma,» according to Dr. Piggott, who was not involved in the study.
The mean age at diagnosis was 12.7 years, the average age at death was 15.6 years, and the average survival time after diagnosis was about 35 months.Of the 16 cases with known identifiable subtypes, 50% were nodular melanoma, 31% superficially scattering melanoma, and 19% were pointed melanoma. In addition, a quarter of melanomas occurred in association with congenital melanocytic nevi.
«The good news is that there are a total of only 38 cases of fatal pediatric melanoma in 12 academic centers over a 23-year period,» Piggott said. «Thank God the number is so low.»
This article originally appeared on MDedge.com, part of the Medscape Professional Network. It was translated into German by Dr. Petra Kittner.
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