Langerhans Cell Histiocytosis in the Sphenoid Wing

Langerhans Cell Histiocytosis (LCH) is a rare and broad-spectrum of disease caused by uncontrolled proliferation of pathologic Langerhans cell resulting in localize and systemic organ infiltration. The clinical presentation can be unspecific and varies between individuals depending on the anatomical location and organ involvement. In view of bony infiltration is the most common in LCH, clinicians have to deal with the challenge of diagnosis dilemmas such as Giant Cell Tumour, Ewing’s sarcoma, osteogenic sarcoma, plasmacytoma, osteomyelitis or bony metastasis, particularly from neuroblastoma. Hence, it requires an excellent multidisciplinary team to establish the diagnosis and start targeted treatment early to prevent fatal complications. The treatment modalities include surgery, immunosuppressive therapy, chemotherapy or in combination. Recently, a seven months old boy presented to our centre with one-week duration of left cheek swelling and left eye proptosis. After detailed clinical evaluation together with imaging and biopsy, the lesion over the bilateral sphenoid and left zygoma were concluded to be Langerhans Cell Histiocytosis (LCH).