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Managing isolated subglottic juvenile xanthogranuloma without tracheostomy: Case report and review of literature
Author(s) -
Somorai Marta,
Goldstein Nira A.,
Alexis Richard,
Giusti Robert J.
Publication year - 2007
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.20544
Subject(s) - juvenile xanthogranuloma , medicine , histiocytosis , histiocyte , respiratory distress , lesion , pathology , dermatology , surgery , disease
Juvenile Xanthogranuloma (JXG) is a dendritic cell related histiocytic disorder which usually presents in the first year of life as a solitary cutaneous granuloma. Isolated presentation in the upper airway is very rare but can result in severe respiratory distress, especially in young children. We present the case of a 5‐month‐old male with an isolated subglottic JXG lesion. Endoscopic excision provided symptomatic relief and avoided the need for tracheostomy. The lesion has completely resolved 17 months later. Surgical excision without tracheostomy was the treatment of choice in two of the four additional cases of upper airway JXG presented in the literature. JXG has an excellent prognosis with spontaneous regression over time. Histology alone is frequently inadequate to differentiate JXG from the more common Langerhans Cell Histiocytosis (LCH), which carries a much less favorable prognosis. The evolving field of immunohistochemistry provides an essential tool to establish the correct diagnosis. The typical phenotype of JXG is Factor XIIIa+/Fascin+/CD68+/CD163+/CD14+/CD1a−/S100−. Pediatr Pulmonol. 2007; 42:181–185. © 2006 Wiley‐Liss, Inc.