Giant congenital melanocytic nevus responds to trametinib.
A GIANT CONGENITAL MELANOCYTIC NEVUS (GCMN) in a 7-year-old girl was effectively treated with the MEK inhibitor trametinib, according to a case report published in Pediatrics.
The nevus covered most of her back and much of her torso and had thickened significantly over the years since initial presentation to the point of disfigurement, even invading the fascia and musculature of the trunk and pelvis, reported Adnan Mir, MD, PhD, of the University of Texas Southwestern Medical Center, Dallas, and his coauthors. She presented with intractable pruritus and pain that interfered with sleep and responded minimally to treatments.
There are few if any effective treatments for GCMN. Considering general theories of the mechanism of GCMN, the patient's health care team decided to try a 0.5-mg daily dose of trametinib. Her pruritus and pain resolved completely, and after 6 months of treatment with trametinib, repeat MRI "revealed decreased thickening of the dermis and near resolutions of muscular invasion." According to the patient's family, her quality of life improved dramatically.
SOURCE: Mir A et al. Pediatrics. 2019;143(3):e20182469.
BY CHRISTOPHER PALMER
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|Date:||May 1, 2019|
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