Langerhans cell histiocytosis (skeletal manifestations)
The skeleton is the most commonly involved organ system in Langerhans cell histiocytosis (LCH) and is by far the most common location for single-lesion LCH, 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).
The skeletal system is the commonest site of involvement of Langerhans cell histiocytosis, and in 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.
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.
There is proliferation of Langerhans cells with an abundance of eosinophils, lymphocytes and neutrophils. These cells produce prostaglandins which result in medullary bone resorption: this is what causes the symptoms.
Patients may have one or, less commonly, 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%
- 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 vs. the outer table (hole within a hole) sign
- button sequestrum representing residual bone
- geographic skull
- irregular radiolucent areas mostly involving superficial alveolar bone
- floating tooth: loss of lamina dura
- vertebra plana: most common cause of vertebra plana in children; more often in thoracic spine
- depends on phase of disease which is imaged
- permeative and aggressive appearing lesion
- mainly involves diaphysis or metadiaphysis and respects 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
Similar to plain film findings with better demonstration of cortical erosion and soft tissue involvement. Excellent for biopsy and surgical planning.
Signal characteristics include
- T1: typically low signal
- T2: isointense to hyperintense
- STIR: hyperintense
- T1 C+ (Gd): often shows contrast enhancement
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
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
- radiofrequency ablation 4
- radiotherapy for spinal lesion
History and etymology
The term eosinpophilic granuloma was coined by Lichtenstein and Jaffe in 1940 2.
General imaging differential considerations include:
- 1. David R, Oria RA, Kumar R et-al. Radiologic features of eosinophilic granuloma of bone. AJR Am J Roentgenol. 1989;153 (5): 1021-6. AJR Am J Roentgenol (citation) - Pubmed citation
- 2. Lichtenstein L. Histiocytosis X; integration of eosinophilic granuloma of bone, Letterer-Siwe disease, and Schüller-Christian disease as related manifestations of a single nosologic entity. AMA Arch Pathol. 1953;56 (1): 84-102. Pubmed citation
- 3. Pizzo PA, Poplack DG. Principles and practice of pediatric oncology. Lippincott Williams & Wilkins. (2006) ISBN:0781754925. Read it at Google Books - Find it at Amazon
- 4. Corby RR, Stacy GS, Peabody TD et-al. Radiofrequency ablation of solitary eosinophilic granuloma of bone. AJR Am J Roentgenol. 2008;190 (6): 1492-4. doi:10.2214/AJR.07.3415 - Pubmed citation
- 5. World Health Organization, Pathology IA. Pathology and genetics of tumours of soft tissue and bone. World Health Organization. (2002) ISBN:9283224132. Read it at Google Books - Find it at Amazon
- 6. Lanzkowsky P. Manual of Pediatric Hematology and Oncology. Academic Press. (2010) ISBN:0123751543. Read it at Google Books - Find it at Amazon