and systemic lesions, ocular lesions not spontaneously regress and may require intervention On occasion, ocular lesions have been misdiagnosed as retinoblastoma Other noncutaneous lesions in systemic JXG may involve the gastrointestinal tract (most commonly the liver), central nervous system, bone marrow, lymph nodes, heart, kidney, adrenal glands, and lungs Not all children with systemic JXG have concomitant cutaneous lesions Children who have both JXG and neurofibromatosis are at a much higher risk for unusual forms of leukemia and thus should be appropriately monitored Unlike children with disseminated xanthomas, there is no relationship between JXG and lipid abnormalities All children with suspected xanthogranuloma warrant consideration of an ophthalmologic evaluation, and careful evaluation for evidence of systemic involvement FIGURE 120.20 Yellow nodular lesion of juvenile xanthogranuloma (Reprinted with permission from Burkhart C, Morrell D, Goldsmith LA, et al VisualDx: Essential Pediatric Dermatology Philadelphia, PA: Lippincott Williams & Wilkins; 2009.) Neurofibroma A localized neurofibroma may present as a solitary lesion in an otherwise normal patient or as a feature of neurofibromatosis type I Cutaneous neurofibromas arise from nerve sheath cells located in the dermis They appear as pink or fleshcolored nodules that are soft and range in size from 0.5 to cm They are