Eosinophilic granuloma

Posted By Wael Nemattalla
Eosinophilic granuloma

12 years old male.

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  • Wael Nemattalla 2013-12-14 09:54:08

    Skeletal manifestations of Langerhans cell histiocytosis





    The skeleton is the most commonly involved organs system in Langerhans cell histiocytosis (LCH) and is by far the most common location for single lesion, often referred to as eosinophilic granuloma (EG) (the terms are used interchangeably in this article). For a general discusion of this disease please refer to the article on Langerhans cell histiocytosis (LCH).



    Epidemiology

    The skeletal system is the commonest site of involvement of Langerhans cell histiocytosis, and in for 60-80% of cases is the only organ system involved. It primarily occurs in older children and young adults, with a male preponderance with a male to female ratio of 2:1.



    Clinical presentation

    The lesions may be asymptomatic and discovered as an incidental radiographic finding.

    When symptomatic, patients complain of pain, swelling and tenderness around the lesion. Systemic symptoms may also be present, including general malaise and, on occasion, fever with leukocytosis.



    Pathology

    There is proliferation of Langerhans cells with an abundance of eosinophils, lymphocytes and neutrophils. These cells produce prostaglandins which result in medullary bone resorption: it is this that causes the symptoms.



    Location / distribution

    Patients may have one or many lesions. The most common locations are 5-6:

    • skull: 49%

    • pelvis: 23%

    • femur: 17%

    • ribs: 8% (most common in adults)

    • humerus: 7%

    • mandible: 7%





    Radiographic features

    Plain Film

    Skull

    • solitary or multiple punched out lytic lesions with or without sclerotic rim

    • double contour or beveled edge appearance may be seen. Greater involvement of the inner than the outer table.

    • button sequestrum representing residual bone

    • geographic skull

    Mandible

    • irregular radiolucent areas mostly involving superficial alveolar bone

    • floating tooth: loss of lamina dura.

    Spine

    • vertebra plana, more often in thoracic spine

    Long bones

    • mainly involves diaphysis and respect growth plates

    • endosteal scalloping, periosteal reaction, cortical thinning and intracortical tunneling

    CT

    Similar to plain film findings with better demonstration of cortical erosion and soft tissue involvement. Excellent for biopsy and surgical planning.



    MRI

    Signal characteristics include

    • T1 - typically low signal

    • T2 - isointense to hyperintense

    • T1 C+ (Gd) - often shows contrast enhancement

    Bone Scan

    Variable appearance on bone scintigraphy with lesions showing an increased or decreased tracer uptake depending on the histological picture. Nonetheless bone scans are helpful in other asymptomatic lesions.





    Treatment and prognosis

    The prognosis is excellent when disease is confined to the skeleton, especially if it is a solitary lesion, with the majority of such lesions spontaneously resolving by fibrosis within 1-2 years. However, where symptoms persist, other treatment options may be considered.

    • excision and curettage 3

    • steroid therapy

    • chemotherapy

    • radiofrequency ablation 4

    Etymology

    The term eosinpophilic granuloma was coined by Lichenstein and Jaffe in 1940 2



    Differential diagnosis

    General imaging differential considerations include

    • osteomyelitis

    • round blue cell tumours

    • metastases

    • primary bone tumours

    • lymphoma / leukemia



    source:

    http://radiopaedia.org/articles/skeletal-manifestations-of-langerhans-cell-histiocytosis

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