- See:
- Bone Tumor Menu:
- Eosinophilic Granuloma of Spine:
- Discussion:
- includes a group of disorders characterized by a variety of tumor like lesions, which arise from from clonal proliferation of Langerhans-
type histiocytes;
- most common in children less than 12 years of age (but can occur in young adults);
- etiology:
- occurs as a result of metabolic defects in the reticuloendothelia system;
- it is sometimes non painful, unless of course a fracture occurs;
- hallmark is presence of an osseous lesion (70-90%) most frequently arising in skull and femur;
- osseous lesions may produce rapidly destructive bone lesions;
- soft tissue masses associated w/ skeletal lesions rarely are seen,
- disease sub-types:
- note that intermediate forms of the disease occur and individual disease presentations may change from one form to another;
- Histiocytosis X;
- Hand Schuller Christian disease
- occurs in children > 3 yrs;
- traid of skull lesions, exophthalmos, & diabetes insipidus;
- a minority of patients will have wide spread viceral involvement;
- cranial lesions are always present in this disease;
- Letterer Siwe dz;
- represents more acute manifestations of histiocytosis & generally has an age of onset of less than 3 yrs;
- look for recurrent bacteremia, diffuse lymphadenopathy, & skin lesions;
- disease is commonly fatal;
- poor prognostic signs:
- involvement at young age, rapid disease progression, organ involvement (eg pituitary, lung, hematopoietic, or liver involvement);
- organ dysfunction carries an especially poor prognosis;
- diff dx:
- Osteomyelitis:
- Ewing's sarcoma: unlike EOG, ewing's sarcoma typically has a soft tissue extension arising from the bony lesion;
- Lymphoma
- Leukemia
- Hodgkin's disease
- Myeloma:
- Intraosseous hemangioma
- Fibrous dysplasia
- Clinical Manifestation:
- tends to occur as solitary, minimally symtomatic lesion in young child;
- low grade fever, elevated sed rate, & mild peripheral eosinophilia are occassionally associatted findings.
- sites of involvement:
- skull (most common), mandible