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Bone Tumor Menu:
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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);
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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,
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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;
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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;
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diff dx:
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Osteomyelitis:
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Ewing's sarcoma: unlike EOG, ewing's sarcoma typically has a soft tissue extension arising from the bony lesion;
- Lymphoma
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Leukemia
- Hodgkin's disease
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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.
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sites of involvement:
- skull (most common), mandible,
spine, ribs supraacetabular region of pelvis, and diaphysis of long bones are common sites;
- femur is the most frequently involved long bone;
- scapula and clavicle are also involved frequently;
- eosinophilic granuloma of spine:
- lesions of the skull
- elicits a more lytic & scalloped appearance;
- may be associated with a palpable soft tissue mass;
- pelvic lesion: pts may note subtle hip pain exacerbated by movement and ambulation;
- hands: involved only rarely;
- Radiographs:
- small appear as punched out radiolucent lesion;
- oval radiolucent lesion surrounded by thick margin of reactive bone;
- permeative lesion that invades & destroys cortex and is localized to diaphysis;
- eosinophilic granuloma seldom elicits periosteal rxn, but when it does, it can produce an ominous spectre suggesting a malignant process;
- Bone scans:
- used to document the presence of multiple lesions ( < 10% of cases);
- intense radioisotope uptake indicates an active lesion;
- in some cases, EOG may be cold on bone scan;
- CT scan:
- is helpful in demonstrating lesion, esp in spine and hip.
- MRI:
- in this example there was an associated soft tissue mass;
- Histology:
- diagnosis may be achieved with percutaneous needle biopsy;
- there should be no evidence of cartilage or bone matrix;
- look for mixture of pale lipid filled histiocytes, eosinophils, & some giant cells, plasma cells, & neutrophils;
- Langerhan's cells: (Langerhans histiocytes);
- grooved or coffee bean shaped nucleus and abundant pale staining cytoplasm;
- large ovoid, indented nucleus and well defined cytoplasmic borders;
- electron microscopy: reveals racquet-shaped cytoplasmic inclusions (Birbeck granule);
- references:
An electron microscope study of basal melanocytes and high-level clear cells (Langerhans cells) in vitiligo.
Birbeck, M. D. et al.
J. Invest. Dermatol. 37:51, 1961.
Langerhans cell granules in eosinophilic granuloma of bone.
Friedman, B., and Hanaoka, H.
J. Bone Joint Surg. 51A:367, 1969.
- Initial Work Up:
- requires bone scan or skeletal series to rule out multiple lesions;
- skull and pelvic radiographs;
- CBC and liver enzymes;
- Treatment:
- bone lesions often resolve spontaneously and do not require treatment unless they cause symptoms (less than 10% of lesions);
- curettage provides diagnostic biopsy material & is curative;
- w/ large lesions, bone grafts may be needed;
- injection of high dose steroids is another option and tends to result in rapid resolution of the lesion (often within 2 weeks);
- typical dose of steroid is 125 mg of methylprednisolone;
- chemotherapy or radiation therapy is usually not inidcated, however, steroids
may be indicated w/ multiple lesions;
- when chemotherapy is used, prednisolone and vinblastine most common agents;
- XRT is only indicated when a symptpomatic lesion involves an inaccessible region such as the spine, skull, or pelvis;
- dose of XRT is usually between 500 to 1000 rads;
- Eosinophilic Granuloma of Spine:
Diagnosis of eosinophilic granuloma of bone by cytology, histology, and electron microscopy of transcutaneous bone-aspiration biopsy.
Treatment of Langerhans-cell histiocytosis in children:
Experience at the Children's Hospital of Nancy.
S. Sessa et al.
JBJS. Vol 76-A. 1994. p 1513-1525.
Diagnostic evaluation of patients in histiocytosis X.
RA Dimentberg and KL Brown.
J. Pediatric Orthop. Vol 10. 1990. p 733-741.
Percutaneous techniques for the diagnosis and treatment of localized Langerhans-Cell Histiocytosis(Eosinophilic Granuloma of Bone).
Bone lesions in histiocytosis X.
G. Bollini et al.
J. Pediatric Orthop.
Vol 11. 1991. p 469-477.
Langerhans Cell Histiocytosis of Bone in Children and Adolescents.
Langerhans Cell Histiocytosis: A Primary Viral Infection of Bone?: Human Herpes Virus 6 Latent Protein Detected in Lymphocytes From Tissue of Children.
Langerhans Cell Histiocytosis of the Spine in Children. Long-Term Follow-up.
Other links:
Histiocytosis X (A.K.A. Langerhan's Cell Histiocytosis) (Updated March 2006) from the Orthopaedic Care Textbook
