Juvenile xanthogranuloma of the oral cavity in children: a clinicopathologic study

This clinicopathologic study describes the histopathologic, immunocytochemical, and electron microscopic features of oral juvenile xanthogranuloma (JXG) in children.

The study population consisted of 5 children (5 months to 10 years of age) with biopsy-proven JXGs obtained for consultation.

Lesion sites were gingiva, buccal mucosa, and tongue and were described by the clinicians as (1). irritation fibroma; (2). granulation tissue; (3). pedunculated granular nodule; (4). papilloma-like lesion; and (5). brown-red umbilicated papule. Tissue was available for histopathologic (n = 5), immunocytochemical (n = 5), and ultrastructural (n = 3) studies. Three cases showed early JXG lesions possessing abundant histiocytes, but lacking Touton giant cells. The other 2 cases had classic JXG lesions with vacuolated histiocytes and Touton giant cells. Immunocytochemical findings were (1). CD68 (KP1, PGM1), moderate to diffuse; (2). fascin, moderate to diffuse; (3). factor XIIIa, focal to diffuse; (4). alpha-1-antitrypsin, moderate to diffuse; (5). S-100 protein, focal to diffuse; and (6). CD1a, negative in all cases. Ultrastructural examination displayed histiocytic and dendritic cells lacking pentalaminar structures (Birbeck granules).

JXGs of the oral cavity vary in their clinical and histopathologic appearances and necessitate that Langerhans' cell disease (LCD) be excluded. JXG and Langerhans' cell disease may occur concurrently, before or after each other, in some children.