Eosinophilic Granuloma
Editors: Frassica, Frank J.; Sponseller, Paul D.; Wilckens, John H.
Title: 5-Minute Orthopaedic Consult, 2nd Edition
Copyright ©2007 Lippincott Williams & Wilkins
> Table of Contents > Eosinophilic Granuloma
Eosinophilic Granuloma
Frank J. Frassica MD
Basics
Description
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EOG is the bony, and most common, manifestation of a group of nonneoplastic disorders known as “LCH.”
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3 basic scenarios occur:
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Solitary site bone disease
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Multiple bone lesions without visceral disease
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Multiple bone lesions and visceral disease
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EOG commonly affects the skull, ribs, pelvis, spine, diaphysis of long bones, and mandible, but any bone may be involved.
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It more commonly affects a single bone rather than multiple bones.
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LCH:
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Spectrum of disease involvement rather than separate specific entries
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In the past, divided into 3 separate conditions:
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EOG: Single intramedullary site of bone disease
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Hand-Schüller Christian disease: Multiple
sites of bone disease and visceral disease (skin, lymph nodes, liver,
spleen, etc.); the classic triad (Christian triad) (uncommon by itself)
involves lytic skull disease, exophthalmos, and diabetes insipidus.
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Letter-Siwe disease: Fulminant condition
in young children (<2 years old) with widespread involvement that
often results in death (lymphadenopathy, hepatosplenomegaly, and
extensive pulmonary disease)
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Synonyms: Histiocytosis X; Langerhans cell histiocytosis; Reticuloendotheliosis
Epidemiology
Incidence
Rare
Prevalence
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Usually seen in patients <30 years old, with a peak incidence at age 5–10 years (1)
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Male:Female ratio of 2:1 (1)
Etiology
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The cause of the accumulation of abnormal metabolic products in the reticuloendothelial cells is unknown.
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An inflammatory response occurs around these cells and produces the lytic destruction of bone.
Diagnosis
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3 diagnostic criteria for LCH of the spine (2)
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Percutaneous needle biopsy is a very effective method of establishing the diagnosis (3).
Signs and Symptoms
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Local pain
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Swelling, tenderness
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Warmth at the site of involvement
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Occasional fever
Physical Exam
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The skull and skeleton should be palpated for areas of tenderness.
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Note the position of the eyes within the orbit.
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The spine should be percussed for tenderness.
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The patient’s gait should be observed for the presence of a limp.
Tests
Lab
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Elevated ESR
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Peripheral eosinophilia
Imaging
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Radiography:
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Plain radiographs show sharply circumscribed, “punched-out” lytic lesions.
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As the lesion heals, a thick rim of reactive bone forms around the periphery.
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The cortex may be destroyed with endosteal scalloping, periosteal reaction, and expansion of the bone.
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If the cortex is destroyed unevenly (EOG
attacks cortex from within the canal, but 1 side of the cortex may be
more involved than the other), a “hole within a hole” appearance ensues. -
In the vertebra, the body may collapse to a slender sclerotic wafer of bone called vertebra plana.
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In the mandible and maxilla, the lytic lesion appears as a “floating tooth.”
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Bone scanning usually is not recommended because the lesions may not be “hot” on bone scan.
Pathological Findings
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Sheets of “foamy” (lipid-filled) histiocytes:
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Coffee-bean-shaped nucleus
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Crisp nuclear membrane
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Abundant pale eosinophilic cytoplasm
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Staining with S-100 and Cd1a stains (1)
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Inflammatory cells found around these histiocytes:
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Predominantly eosinophils, but also a few lymphocytes, neutrophils, and giant cells
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P.119
Differential Diagnosis
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Ewing sarcoma
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Lymphoma
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Osteomyelitis
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EOG appropriately is called the “great imitator,” because it may mimic infection or neoplasm.
Treatment
General Measures
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Most lesions are self-limiting and resolve spontaneously.
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Options include:
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Observation
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Curettage and bone grafting
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Injection with steroids
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If a pathologic fracture is impending, or the articular surface is in danger, curettage and bone grafting are necessary.
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Biopsy often is necessary except for characteristic spine lesions.
Medication
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Methylprednisolone acetate injected into the lesion is effective in >90% of patients, with excellent bone healing (3).
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For patients with systemic disease with
constitutional symptoms, chemotherapy with methylprednisolone,
methotrexate, doxorubicin (Adriamycin), and other agents is indicated. -
Vertebra plana usually heals itself, and vertebral body height is restored with time.
Surgery
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Surgery is unnecessary in most cases.
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However, curettage and bone grafting with
or without internal fixation are indicated if nonoperative measures
fail and pathologic fracture seems imminent.
Follow-up
Complications
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Pathologic fractures may occur.
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Usually heal well with closed or operative treatment
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Patient Monitoring
Observation with plain radiographs is indicated if the
diagnosis is clear-cut and the patient has no impending fracture, until
lesions resolve (usually 6 months).
diagnosis is clear-cut and the patient has no impending fracture, until
lesions resolve (usually 6 months).
References
1. Dorfman HD, Czerniak B. Immunohematopoietic tumors. In: Bone Tumors. St. Louis: Mosby, 1998: 664–728.
2. Garg S, Mehta S, Dormans JP. Langerhans cell histiocytosis of the spine in children. Long-term follow-up. J Bone Joint Surg 2004;86A: 1740–1750.
3. Yasko
AW, Fanning CV, Ayala AG, et al. Percutaneous techniques for the
diagnosis and treatment of localized Langerhans-cell histiocytosis
(eosinophilic granuloma of bone). J Bone Joint Surg 1998;80A:219–228.
AW, Fanning CV, Ayala AG, et al. Percutaneous techniques for the
diagnosis and treatment of localized Langerhans-cell histiocytosis
(eosinophilic granuloma of bone). J Bone Joint Surg 1998;80A:219–228.
Additional Reading
McCarthy EF, Frassica FJ. Skeletal manifestations of systemic disease. In: Pathology of Bone and Joint Disorders: With Clinical and Radiographic Correlation. Philadelphia: WB Saunders, 1998:119–133.
Miscellaneous
Codes
ICD9-CM
277.8 EOG
Patient Teaching
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Reassure the patient and family about the self-limiting nature of most lesions.
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Make sure that the child and parents understand the need for close follow-up care.
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Make sure disease progression is not occurring.
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Ensure that this is not the initial presentation of widespread disease.
Activity
No restrictions are placed on activity unless the patient has an impending pathologic fracture.
FAQ
Q: Is EOG or LCH a malignant condition?
A: No. It is a self-limited inflammatory process.
Q: What is the prognosis when a single bone is involved?
A: The prognosis is excellent.
Q: Do patients with complete vertebral collapse need surgery?
A:
Patients with complete vertebral collapse can be treated
symptomatically, and only ~10% of patients with LCH of the spine need
reconstructive surgery at long-term follow-up.
Patients with complete vertebral collapse can be treated
symptomatically, and only ~10% of patients with LCH of the spine need
reconstructive surgery at long-term follow-up.