Spheno-temporal lamgerhans cell histiocytosis: a case report

---

Résumé

Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells withinflammatory cells, characterized by the activation of histiocytic cells or theirproliferation that affects structures of the human body, including the tempor-al bone which localization is rare in children. The objective of this study is toillustrate the clinical presentation, management and prognosis of this disease,and to compare our case with previously reported series. We report the caseof a young 4-year-old patient with a history of otorrhea and recurrent otalgia,with left axillary non-blowing exophthalmia progressive since 5 months asso-ciated with indurated left temporal swelling and also an indurated swelling ofposterior face of the left shoulder on pathological fracture. A cerebro-orbitalMRI performed at home showed an extra-conical process with extension atthe temporal fossa. The patient, then, underwent an open-air biopsy of thetemporal mass and the pathological analysis returned to favor Langerhans cellhistiocytosis. The diagnosis was made by immunohistochemical findings ofthe S-100 protein and/or the CD1 antigen. The child was treated by chemo-therapy with a good outcome. Our results concord with literature. The prog-nosis is different and better in children with unifocal bone disease than thosewith a multifocal disease with a survival rate of 65% - 100

Mots-clés

Langerhans Cell, Histocytosis, temporal, sphenoid, exophthalmia