Langerhans cell histocytosis presenting as chondrosarcoma of the clivus at intraoperative consultation in a young patient

Abstract

Langerhans cell histiocytosis is a rare disorder which mimics various clinical and radiological presentations including infection or malignancy; and symptoms can be mild or life threatening.1 It is a clonal proliferation of myeloid dendritic cells in various sites of the body, caused by alterations in the MAPKinase pathway and BRAF V600 E. The literature reveals common sites being skin and bones, lymph nodes, brain, lung, liver, spleen.2,3 Although skull is a common site for LCH, because of its rarity, this diagnosis was not considered clinically in this case, when it presented to us at the time of intraoperative consultation of a chondrosarcoma.