dr james c wittig, orthopedic oncologist, new york, new jersey
About Dr. Wittig
Eosinophilic Granuloma

General Information

  • This is a benign proliferation of Langerhans cells usually accompanied with eosinophils, lymphocytes, neutrophils and scattered plasma cells.
  • There may be solitary or multiple lesions confined to bone
  • 70% of cases consist of a solitary lesion
  • Seldom leads to disseminated systemic disease
  • Viewed as disorder of immune regulation or reactive process rather than neoplasm
  • All organ systems may be affected with disseminated forms
  • Hand-Schuller-Christian Disease (1-5 years): chronic disseminated histiocytosis
  • Letterer-Siwe disease (<1 year): acute or subacute disseminated histiocytosis Uniformly fatal
  • Solitary EG is twice as common as multifocal EG
  • May arise from any bone and any site within a bone (epiphyseal, metaphyseal, diaphyseal)
  • Radiographically variable appearance: may appear benign (geographic) or malignant (permeative or moth eaten)

Hand-Schuller-Christian Disease

Triad:

  • Destructive skeletal lesions
  • Exophthalmos
  • Diabetes Insipidus
  • 10% of patients with unifocal EG develop multifocal and extraskeletal disease
  • Usually <5 years old
  • Hepatosplenomegaly, adenopathy, anemia, fever, neurological complaints
  • Fatal in 15%
  • Any bone but 90% have skull involvement

Letterer-Siwe Disease

  • Develops in 1st year of life
  • Disseminated disease and small bone lesions
  • Fatal in 95% who develop before 1 year of life

Clinical Presentation

Signs/Symptoms:

  • Pain and soft tissue swelling
  • Temporal bone disease is clinically indistinguishable from otitis media or mastoiditis
  • May have a fever
  • Mild peripheral eosinophilia (5%-10% of patients)
  • Prevalence: Male predilection (2:1)

Age:

  • 1 month – 71 years
  • Most common age 5-15 years old
  • 85% within first 3 decades
  • 60% within first decade

Sites:

  • Flat Bones (most common—70%)
  • Skull
  • Pelvis
  • Femur
  • Humerus
  • Hands and Feet are rare in solitary disease

Radiographic Presentation

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Radiology, Permeative with periosteal reaction
RADIOGRAPHIC PRESENTATION
X-Ray: Eosinophilic Granuloma of Skull
X-ray: Eosinophilic Granuloma of Femur
X-ray: Eosinophilic Granuloma of Femur X-ray: Eosinophilic Granuloma of Femur









Gross Pathology

  • Gross appearance not distinctive
  • Depending on mixture of cells, may be yellow, gray, or brown
  • Older lesions are yellow due to regression and accumulation of lipid in histiocytes and Langerhans cells
  • Intralesional hemorrhage exists
Microscopic Pathology
  • Langerhans cell is diagnostic and clonal proliferation
  • Nuclei show prominent nuclear groove (coffee-bean)
  • Also composed of eosinophils and other inflammatory cells (non diagnostic component)
  • Ratio of inflammatory cells to Langerhans cells varies
  • Mitotic activity low
  • Eosinophils dominate some areas forming diffuse sheets, excluding Langerhans cells
  • Birbeck Granules: Electron Microscopy demonstrates granules that often take the form of a tennis raquet and form from complex invaginations of the cell membrane
  • Vimentin, CD1 and S-100 positivity

Roll over the images for more information














Differential Diagnosis

  • Osteomyelitis
  • Granulomatous Inflammation
    • Tuberculosis
    • Fungus
  • Hodgkin Disease

Biological Behavior

  • Typically acts as a benign disorder
  • Individual lesions may undergo partial or complete spontaneous resolution
  • Patients with solitary lesions are at risk for developing additional bony lesions within 6 months to 2 years
  • Adult patients with more than 3 bone lesions are at risk for visceral involvement although death due to EG in adults is rare
  • Children with multiple bone lesions are at risk for visceral involvement that may cause death. Children less than 2 who develop disseminated disease are at highest risk for death

Treatment

  • Majority of patients are cured by curettage or intralesional injection of a steroid
  • Curettage and bone grafting for long bones and weight bearing bones at risk for fracture
  • Intralesional steroids for non weightbearing bones
  • Complete healing may take a year
  • Low dose radiation may be valuable for inaccessible lesions
  • Vertebral plana is braced and observed

Prognosis

  • Complete healing after surgery may take 1 year
  • Patients with intraosseous EG are at risk for developing additional lesions
  • Pateints who develop additional bony lesions are at risk for organ involvement.
  • Death is rare of adults, regardless of extend of lesions although children, especially when less than 2 years of age, more readily die from disseminated disease
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