Diagnosis, risk assessment and treatment of congenital melanocytic nevi (CMN), as well as melanoma, require specific strategies for pediatric patients.
Diagnosis, risk assessment and treatment of congenital melanocytic nevi (CMN), as well as melanoma, require specific strategies for pediatric patients. Elena B. Hawryluk, MD, PhD, assistant professor of dermatology at Harvard Medical School in Boston, Massachusetts, discussed recommendations for understanding how melanoma presents in pediatric cases and how to optimize outcomes during her presentation at Maui Derm Live. Maui Live was an in-person dermatology continuing medical education (CME) conference in Hawaii held concurrently with Maui Derm Connect, a virtual CME conference, from January 25 to January 29, 2021.1
ASSESSING CMN RISKS
Hawryluk first addressed CMN, noting that they increase the risk of melanoma and that the likeliness increases with the papule’s size. For small (<1.5 cm) and medium (1.5- 20 cm) nevi, the risk increases by roughly 1%. For large (>20 cm) and giant (>40 cm) nevi, the risk is 2% for large nevi and 6% to 15% for giant nevi. In CMN-associated melanoma, the risk is highest for patients with giant congenital nevi (78%) with many satellite nevi (94%).
Hawryluk added that atypical or dysplastic nevi, which are not premelanoma, can indicate an increased risk of developing melanoma. Because of this, Hawryluk recommended a thorough examination with continual monitoring.
According to Hawryluk, pediatric patients with large or giant nevi tend to have high mortality (55%), and 14% have visceral melanoma. Hawryluk also noted study results showing that patients with 2 or more nevi have an increased risk of central nervous system involvement, with 21% of patients having abnormal MRI findings.1
IS IT MELANOMA?
Hawryluk explained that various types of melanoma affect the pediatric population and generally present differently than adult patients. A 2013 study advised assessing these lesion characteristics when diagnosing pediatric cases: amelanotic, bump, bleeding, color uniformity, de novo, at any diameter. (See Table).2
Physicians can often misdiagnose amelanotic melanoma as a benign lesion. Hawryluk cited 2016 study results from the Journal of the American Academy of Dermatology that found dermatologists, following a first impression, had identified an amelanotic melanoma as a Spitz nevus, hemangioma, and acneiform lesion. To illustrate this point, she shared a case from her practice involving a girl with a suspected wart that hadn’t responded to all typical treatments.
Spitzoid melanoma can harbor kinase fusions in roughly 50% of lesions and can be challenging to diagnose and distinguish from atypical Spitz tumors, in part because nonmelanoma Spitz lesions can also harbor kinase fusions, Hawryluk added.
OPTIMIZE WORK-UPS AND MANAGEMENT OPTIONS
If there is no suspicion of melanoma, some parents prefer to simply keep the lesions under observation. According to Hawryluk, other choices include surgical excision, although no substantial evidence has been found that the treatment choices reduce the risk of melanoma; curettage; dermabrasion; chemical peels; cryotherapy; laser; and electrotherapy. These treatment options can remove CMN cells, but Hawryluk remarked that they carry the risk of scars or disfigurement and may make it more difficult to detect melanoma.
When considering whether a child needs an MRI, she recommended it for infants with 2 or more nevi, a large axial nevus, the presence of satellites, or older patients who exhibit neurologic changes. She also noted that having imaging from infancy to serve as a baseline can make it easier to discern changes.
For the work-up of patients with CMN and melanoma suspicion, she shared 2 algorithms dependent on whether the presentation was cutaneous or if it was a central nervous system manifestation.
For cutaneous presentations, a thorough clinical examination with photo documentation should occur with a review within 4 weeks. If the new mark is resolved or unchanged, the area should be monitored continually to ensure stability. If the mark progresses or has an abnormal presentation, an excision biopsy should occur, along with histopathology and hotspot genotyping.
If it’s malignant and confirmed by another expert, baseline blood, histology, staging, and further excision should occur as dictated. For new neurological symptoms, imaging should occur. If it’s normal or unchanged from the last imaging session, regular monitoring should continue. For abnormal results, a biopsy followed by histopathology and genotyping may help determine if the result is malignant or nonmalignant.
Hawryluk reported no relevant or financial disclosure.