Published online Jan 16, 2015. doi: 10.12998/wjcc.v3.i1.77
Peer-review started: July 1, 2014
First decision: August 14, 2014
Revised: October 14, 2014
Accepted: October 23, 2014
Article in press: December 23, 2014
Published online: January 16, 2015
Xanthogranulomas (XG) are benign proliferative disorder of histiocytes, a non-Langerhans cell histiocytosis. Whose etiology is unknown. The nature of these lesions is controversial and could be either reactive or neoplastic; the presence of monoclonal cells does, however, favor the second hypothesis. Xanthogranuloma is frequently found in young adults and children (under 20 years old), mainly in the skin. In about 5%-10% of all Juvenile XG (JXG) cases xanthogranuloma are extracutaneous. Within this group, the site most frequently involved is the eye. Other involved organs are heart, liver, adrenals, oropharynx, lung, spleen, central nervous system and subcutaneous tissue, although involvement of the spine is uncommon. Isolated lesions involving the sacral region are extremely rare. To date, this is the first reported case of a giant JXG arising from S1 with extension into the pelvic region in an adult spine.
Core tip: Xanthogranulomas (XG) are benign lesions derived by histiocytes, also called histiocytosis with non-Langerhans cell. Isolated xanthogranuloma involving the sacral region are unique. We report the unique giant Juvenile XG arising from S1 with extension into the pelvic region in an adult spine. Complete surgical removal is the goal of the treatment and usually curative even if there is not a study in the literature with a long follow up able to confirm this. If total resection is not possible, the patients must be followed by strictly clinical examination or should undergo adjuvant radiotherapy.