Juvenile xanthogranuloma clinicopathologic analysis and immunohistochemical study of 57 patients

Abstract

Fifty-seven cases of juvenile xanthogranuloma that fulfilled the classic description of histologic findings of the disease were analyzed clinicopathologically and immunohistochemically. Two forms could be distinguished: 47 cases of the infantile form and 10 of the adolescent and young adult form. The infantile lesion was found at birth in 8 patients (17%), and was noted within 1 year after birth in 33 (70%). Twenty-two had multiple lesions and five of the six for whom follow-up was feasible had spontaneous involution. About half of the lesions were located on the head and neck, 30% on the trunk, and 20% on the extremities. All six adolescents had a solitary tumor located in the head and neck region. Comparison of the latter form with reticulohistiocytoma and cutaneous fibrous histiocytoma was established from a differential point of view. Immunohistochemically, most lesions of juvenile xanthogranuloma displayed a positive reaction for lysozyme and alpha-1-antichymotrypsin and were negative for S-100 protein, thereby suggesting that the essential constituents of this lesion would derive from the mononuclear phagocyte system.

Bilateral multifocal uveal juvenile xanthogranuloma in a young boy with systemic disease

“…The diagnosis is confirmed by histology and immunohistochemical analysis [6,9]. Excluding isolated oculo-orbital disease, less than 50 cases of disseminated systemic JXG have been reported, and only two of them included an iris lesion [1,5].…”

Xantogranuloma juvenil gigante do limbo corneoescleral

“…5 A maioria dessas células é fortemente marcada com o marcador de macrófagos CD68, porém não imunorreativa à neuroproteína S-100 ou à glicoproteína da superfície celular CD1a, ambas marcadores de histiócitos de Langerhans. [6][7][8] Neste relato, apresentamos o caso de uma criança com um XGJ ocular, manifesto por uma lesão nodular antiestética de grandes proporções no limbo corneoescleral do olho esquerdo.…”

An Unusual Juvenile Xanthogranulomaon a Finger MCP Joint

“…Juvenile xanthogranuloma (JXG) is a benign cutaneous histiocytic proliferation, and was first described by Helwig and Hackney in 1954 1 . The pathophysiology of JXG is not well understood, although it is thought to originate from a histiocytic granulomatous reaction 2,3 .…”