Eosinophilic Granuloma

eosinophilic granuloma of ribs

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 Langerhan’s 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 oblique rib view demonstrates two lytic lesions with sclerotic margins (click image for arrows). The lesions are expansile, with a broad zone of transition and no appreciable cortical breakthrough or periosteal reaction. There is no associated soft tissue mass.

Langerhan’s 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.

Reference: Dähnert W. Radiology Review Manual, 5th edition. Lippincott, Williams & Wilkins, 2003.

Credit: Dr Laughlin Dawes