Eosinophilic granuloma of the femur
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This male patient presented at age 3 with a mandibular mass. Biopsy showed a dense infiltrate of cells with eosinophilic cytoplasm and grooved nuclei, typical of Langerhans cells. There were also numerous eosinophils. A subsequent skeletal survey demonstrated no other lesions. Two years later, a repeat skeletal survey shows characteristic lesions in femur, ribs and skull. This AP x-ray of the right femur demonstrates a well-defined lytic diaphyseal lesion. There is associated dense consolidated periosteal reaction. There is no cortical breach or soft-tissue mass.
Langerhans cell histiocytosis is a heterogeneous group of disorders, including Letterer-Siwe disease (disseminated fulminant disease, typically infants); Hand-Schuller-Christian disease (chronic disseminated disease, typically children); and eosinophilic granuloma of bone (70% of cases).
Eosinophilic granuloma of bone is polyostotic in 25-50% of cases, which does not imply dissemination beyond bone. Disseminated forms of LCH may involve lymph nodes, liver, spleen, thymus, gastrointestinal tract and endocrine system. The prognosis for disease limited to bone is good. Disseminated disease is more aggressive, with 15% mortality in Hand-Schuller-Christian, and 70% mortality in Letterer-Siwe.