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Pediatric Melanoma Rare, With Puzzling Features : Children diagnosed with the disease represent many skin types and have few traditional risk factors.

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“It makes you wonder if all of the lesions they were calling melanoma really were melanomas,” Dr. Orlow said.

The SEER database cited survival rates of 89% and 92% in children under age 10 and aged 10–19, respectively, raising similar questions.

Indeed, a study published in 1996 highlighted the difficulty of interpreting melanoma statistics in children (Int. J. Cancer 1996;68:317–24), he said.

In this review, 42 of 60 “melanoma” lesions diagnosed in children under 16 years old in five Western European countries over a 33-year period were later reclassified as nevi.

The 5-year survival rate for the patients who had true melanoma lesions was 84%.

Case Illustrates Elusive Diagnosis

Melanoma in children is rare and often unheralded by a precursor lesion or melanocytic pigmentation, and it can elude diagnosis, Dr. Orlow said.

“The real thing you have to be on the lookout for is rapid and unexpected growth,” he emphasized.

He described the case of a boy who first presented at age 2 years with a 2-mm papule on his right ear. The lesion was treated with liquid nitrogen.

The lesion returned and measured 4 mm at age 5, 8 mm at age 6 (when it was biopsied and diagnosed as a Spitz nevus), and 10 mm at age 7, when a recurrence was excised and a second biopsy revealed “Spitz nevus with moderate atypia.”

“They were good at measuring it,” Dr. Orlow quipped.

The boy was referred to New York University at age 7. A work-up revealed cervical adenopathy, and a lymph node biopsy detected metastatic melanoma.

Although the child went into apparent remission after 9 months of biochemotherapy, a follow-up positron emission tomography scan at age 11 revealed abnormal foci in the liver, bilateral cervical triangle, and right paratracheal areas.

Despite the ominous course of this case study, Dr. Orlow recommends “restraint in biopsying” when a lesion first presents in a child.

After all, he reminded the audience, any patient destined to have 30 nevi in adulthood will be developing new, completely normal lesions at ages 8, 9, and 10.

There are lesions, like the one in this child, that should have been biopsied “much earlier,” he said. But there are many more “that show up in patients you wouldn't expect … when reasonable people couldn't have guessed it was anything like a melanoma until they took it out and discovered it was.”

An annual examination makes sense for children older than 12 years who have multiple nevi—particularly if they are atypical—and a family history of melanoma, he said.

In children under age 12, though, those factors do not seem to clearly confer elevated risk.

It all gets back to observation, so he is ever on the lookout “for peculiar lesions demonstrating unexpected growth, especially things you can't quite characterize, like a pink, eroded papule that doesn't quite look like a pyogenic granuloma,” he said.

Spotting Pediatric Melanoma

▸ Family history may be negative.

▸ Patient may be nonwhite.

▸ Child may have history of other malignancy.

▸ There is usually no precursor lesion.

▸ Amelanotic, nodular lesions are common.

▸ Rapid, unexpected growth is a red flag.

Source: Dr. Orlow