Eosinophilic Granuloma

 

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.

 

1.  Eosinophilic Granuloma (70%)

 

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