Presentation
Otherwise healthy developmental delayed male presenting after multiple, incidentally discovered radiolucencies on Panorex (dental films).
Patient Data
Multiple lucent lesions are seen in the mandible and maxilla with the largest located on the anterior aspect of the mandible.
Incisional biopsy of the lesions was performed. Immunohistochemical staining demonstrated Langerhan cells staining positive for CD1a and S100. The patient was subsequently treated with teeth extraction and curettage of the lesions, in addition to 6 cycles of cytarabine.
There are destructive lytic lesions in the patient's maxilla, with a new lesion in ramus of the left mandible demonstrating associated FDG uptake. There is a 1 cm nodule in the left upper lobe of the lung with faint FDG uptake indicating pulmonary involvement, in addition to multiple pulmonary micronodules. No evidence of intra-abdominal, retroperitoneal, or lymphatic disease.
There is an expansile 6 cm osteolytic lesion in the metaphysis of the left femur resulting in cortical thinning with adjacent periostitis.
Case Discussion
This is a case of Langerhans cell histiocytosis presenting with destructive lesions in the maxilla and mandible. Bone biopsy of the lesion with immunohistochemical staining showed Langerhan cells positive for CD1a and S100. The patient was treated with bone curettage of the lesions and 6 cycles of cytarabine. Subsequent scans demonstrate recurrent facial bone lesions, new lytic lesion in the femur, and pulmonary involvement.
This case was submitted with supervision and input from:
Diego Davila III, M.D.
Soni C. Chawla, M.D.
Associate Professor
Department of Radiological Sciences
David Geffen School of Medicine at UCLA
Olive View - UCLA Medical Center