Eosinophilic Granuloma

- 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

Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Wednesday, September 14, 2011 3:28 pm