Langerhans cell histiocytosis (skeletal manifestations)
Updates to Article Attributes
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 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 the skull and long bones 5, 6:
- skull: ~50%
- pelvis: 23%
- femur: 17%
- ribs: 8% (most common in adults)
- humerus: 7%
- mandible: 7%
- spine
Radiographic features
Plain radiographs
Skull
- solitary or multiple punched out lytic lesions without sclerotic rim
- double contour or beveled edge appearance may be seen due to greater involvement of the inner than the outer table (hole within a hole) sign
- 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: most common cause of vertebra plana in children; more often in thoracic spine
Long bones
- depends on phase of disease which is imaged
- permeative and aggressive appearing lesion
- mainly involves diaphysis or metadiaphysis and respect growth plates
- endosteal scalloping, periosteal reaction (in healing phase it can appear as solid benign periosteal reaction), cortical thinning, intracortical tunneling, and associated soft tissue mass
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
- STIR: hyperintense
- T1 C+ (Gd): often shows contrast enhancement
Nuclear medicine
There is a 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: intralesional injection
- chemotherapy
- radiofrequency ablation 4
- radiotherapy for spinal lesion
History and etymology
The term eosinpophilic granuloma was coined by Lichtenstein and Jaffe in 1940 2.
Differential diagnosis
General imaging differential considerations include:
-<p>The <strong>skeleton </strong>is the most commonly involved organs system in<strong> Langerhans cell histiocytosis (LCH) </strong>and is by far the most common location for single lesion, often referred to as<strong> eosinophilic granuloma (EG)</strong> (the terms are used interchangeably in this article). For a general discusion of this disease please refer to the article on <a href="/articles/langerhans-cell-histiocytosis">Langerhans cell histiocytosis (LCH)</a>. </p><h4>Epidemiology</h4><p>The skeletal system is the commonest site of involvement of <a href="/articles/langerhans-cell-histiocytosis">Langerhans cell histiocytosis</a>, 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 to female ratio of 2:1.</p><h4>Clinical presentation</h4><p>The lesions may be asymptomatic and discovered as an incidental radiographic finding.</p><p>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.</p><h4>Pathology</h4><p>There is proliferation of <a href="/articles/langerhans-cells">Langerhans cells</a> 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.</p><h5>Location/distribution</h5><p>Patients may have one or many lesions. The most common locations are the skull and long bones <sup>5, 6</sup>:</p><ul>- +<p>The <strong>skeleton </strong>is the most commonly involved organs system in<strong> Langerhans cell histiocytosis (LCH) </strong>and is by far the most common location for single lesion, often referred to as<strong> eosinophilic granuloma (EG)</strong> (the terms are used interchangeably in this article). For a general discusion of this disease please refer to the article on <a href="/articles/langerhans-cell-histiocytosis">Langerhans cell histiocytosis (LCH)</a>.</p><h4>Epidemiology</h4><p>The skeletal system is the commonest site of involvement of <a href="/articles/langerhans-cell-histiocytosis">Langerhans cell histiocytosis</a>, 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 to female ratio of 2:1.</p><h4>Clinical presentation</h4><p>The lesions may be asymptomatic and discovered as an incidental radiographic finding.</p><p>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.</p><h4>Pathology</h4><p>There is proliferation of <a href="/articles/langerhans-cells">Langerhans cells</a> 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.</p><h5>Location/distribution</h5><p>Patients may have one or many lesions. The most common locations are the skull and long bones <sup>5, 6</sup>:</p><ul>
-</ul><h5>CT</h5><p>Similar to plain film findings with better demonstration of cortical erosion and soft tissue involvement. Excellent for biopsy and surgical planning.</p><h5>MRI</h5><p>Signal characteristics include </p><ul>- +</ul><h5>CT</h5><p>Similar to plain film findings with better demonstration of cortical erosion and soft tissue involvement. Excellent for biopsy and surgical planning.</p><h5>MRI</h5><p>Signal characteristics include</p><ul>
-<strong>T1:</strong> typically low signal </li>- +<strong>T1:</strong> typically low signal</li>
-<strong>T2:</strong> isointense to hyperintense </li>- +<strong>T2:</strong> isointense to hyperintense</li>
-<li>radiotherapy for spinal lesion </li>- +<li>radiotherapy for spinal lesion</li>
Image 19 MRI (T1) ( create )
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