Definition
Langerhan's Cell Histiocytosis
Benign tumor-like disorder
- abnormal proliferation of antigen-presenting cells of dendritic origin known as Langerhans cells
- infiltration by histiocytic cells (monocyte / macrophage lineage)
Etiology
Unknown
Epidemiology
Extremely rare
White children aged 5 - 10
Types
As a general rule, if named after someone, the prognosis is poor.
Monostotic (90%) - single bone lesion
Polyostotic (10%) - multiple bone lesions
2. Hand-Schuller-Christian Disease (20%)
Multiple, widespread EG's in bone / lymph nodes / liver / spleen / skin / lung
Classically collection of
- diabetes insipidus (parapituitary involvement)
- exophthalmos (orbital involvement)
- skull defects
- visceral lesions
Acronym = DEAL
- Diabetes insipidus
- Exophthalmos
- All over visceral lesions
- Lytic skill lesions
Less aggressive than LS - 10% die
3. Letterer-Siwe Disease (10%)
Infants < 1
- rare disseminated disease of infants with fulminant course
- major visceral involvement: RES, pulmonary, anaemia
- death from hepatic failure
Location
Skull
Vertebrae
Flat bones - pelvis, ribs
Clinical
Back pain from vertebral lesion
Pain, limp secondary to pathological fracture
X-ray
Vertebra Plana / flattened vertebrae
- compression of body with discs intact
- increased kyphosis
DDx
- Ewings
- multiple myeloma, metastasis, Lymphoma, Leukaemia
- ABC
- infection, tuberculosis
Skull
Most common site
- multiple punched out lucencies
- coalesce to give geographic skull
Long Bones
Punched out diaphyseal osteolytic lesion
DDx - HALFEE
MRI
Lesion is low signal on T1 & high signal on T2
Histology
Langerhan's cells - mononuclear histiocyte-like cells
Skeletal survey and CXR
Need to rule out HSCD or LS
Management
Solitary lesions including vertebroplanar
Typically self limiting
Will resolve
Treat kyphosis with brace
Polyostotic disease / Hand-Schuller-Christian Disease / Letterer-Siwe Disease
Chemotherapy
Surgical indications
Symptomatic
Progressive
Neurological deficit
Risk of pathological fracture
Options
1. Intralesional steroid injections
Rimondi et al Skeletal Radiol 2011
- CT guided intralesional steroid in 19 patients with spinal EG
- complete resolution of pain and the lesion in 17/19 (90%)
- 2 patients non responsive later diagnosed with polyostotic disease
Mavrogenis et al J Paediatri Orthop 2012
- 66 patients with symptomatic EG of the limbs treated with intralesional steroid
- complete resolution of symptoms at 7 days in all patients
- 60/66 (95%) had complete xray resolution at 2 years
2. Curettage and bone grafting
3. Spinal stabilization