Updated: 4/2/2019

Eosinophilic Granuloma

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Introduction
  • Histiocytosis X or Langerhans cell histiocytosis is a spectrum of diseases of the reticuloendothelial system with one of three general presentations
    • Eosinophilic granuloma (EG)
      • usually a single self-limited lesion found in younger patients 
    • Hand-Schuller-Christian disease (HSC)
      • chronic, disseminated form with bone and visceral lesions
      • also known as Langerhans cell histiocytosis with visceral involvement
    • Letterer-Siwe disease (LSD)
      • fatal form that occurs in young children
  • Epidemiology
    • demographics
      • most commonly occurs in children (80% of afflicted < 20 years of age)
      • HSC disease presents in children > 3 years of age
      • LSD occurs in children < 3 years of age
      • Male to female ratio of 2:1
    • location
      • eosinophilic granuloma
        • commonly presents in the skull, ribs, clavicle, scapula, mandible
        • isolated lesions of the spine (thoracic most common)
        • can also occur in diaphyseal regions of long bones and the pelvis
      • HSC
        • multiple bony sites
        • multiple lytic skull lesions
        • visceral involvement of the lungs, spleen, liver, skin, lymph nodes
  • Genetics
    • no clear genetic pattern of inheritance or locus has been determined
  • Prognosis
    • EG
      • isolated involvement generally treatable with local management
      • spine lesions can spontaneously resolve
    • HSC
      • prognosis depends on response to chemotherapy
      • worsening prognosis with increasing extraskeletal involvement
    • LSD
      • generally fatal in children < 3 years of age
Presentation
  • Symptoms
    • skeletal involvement
      • pain and swelling at the region of involvement
      • limping can be seen with pelvic or lower extremity involvement
    • vertebral involvement
      • localized or diffuse back pain
      • increasingly kyphotic posture
      • radiculopathy can occur with more aggressive lesions
    • HSC  
      • classic triad of 
        • multiple lytic skull lesions
        • diabetes insipidus
          • increased thirst and water intake
        • exopthalmos 
      • visceral involvement
        • diffuse or nonspecific abdominal or chest pain
Imaging
  • Radiographs
    • general
      • known as "the great mimicker" as it appears similar to many lesions
      • radiographic differential includes osteomyelitis, leukemia, lymphoma, fibrous dysplasia, or Ewing's sarcoma
    • diaphyseal lesions
      • well defined intramedullary lytic or "punched-out" lesion 
      • cortex may be thinned, expanded, or destroyed
      • may have periosteal reaction
    • metaphyseal lesions
      • extend up to but not through the physis
      • less central location than diaphyseal lesions 
    • spinal lesions
      • vertebra plana (flattened vertebrae) in spine 
      • increased kyphosis
    • cranial involvement
      • multiple "punched-out" lytic lesions 
  • MRI
    • may show a soft tissue mass adjacent to boney lesions
  • Bone scan
    • generally shows increased uptake in the region of boney lesion
Studies
  • Histology
    • Langerhan's cells
      • mononuclear histiocyte-like cells with oval nuclei with well-defined round or oval cytoplasm.    
      • a prominent nuclear groove (coffee bean nuclei) can be seen in most of the nuclei
      • eosinophilic cytoplasm (pink generally) 
      • stain with CD1A  
      • electronmicroscopy
        • birbeck granules seen inside Langerhan's cells 
    • mixture of inflammatory cells also present 
    • giant cells are present
    • lack of nuclear atypia and atypical mitoses
      • differentiates this condition from malignant conditions such as Ewings sarcoma, lymphoma of bone, and metastatic neuroblastoma, which may look similar based on the round cells alone
Treatment
  • Nonoperative
    • observation alone
      • indications
        • a self-limited process and it is reasonable to treat with observation alone
    • bracing
      • indications
        • to prevent progressive kyphosis of the spine
      • outcomes
        • will correct deformity in 90% of patients
        • vertebral lesions generally regain 50% of their height
    • low dose irradiation (600-800 cGy)
      • indications
        • indicated for lesions in the spine that compromise stability, neurologic status
        • lesions not amenable to injection or open treatment
      • outcomes
        • effective for most lesions
    • chemotherapy
      •  indications
        •  diffuse HSC
      • outcomes
        • prognosis is improved with less severe extraskeletal involvement
    • corticosteroid injection
      • indications
        • isolated lesions 
        • can be performed after curettage as well
  • Operative
    • curettage and bone grafting
      • indications
        • for lesions that endanger the articular surface or are a risk for impending fractures
    • spinal deformity correction
      • indications
        • progressive spine deformity refractory to bracing
          • approximately 10% of patients with spine lesion will need operative intervention for deformity correction
Differentials & Groups
 
Destructive lesion in young patients
 
Multiple lesions in young patients (1)
 
Treatment is Observation alone (2)
   
Eosinophilic granuloma
 
 
   
Osteomyelitis
           
Osteosarcoma
           
Ewing's sarcoma
           
Desmoplastic fibroma
           
Lymphoma
 
       
Leukemia
 
       
Fibrous dysplasia    
 
   
Enchondroma / Olliers / Marfucci's    
 
   
Osteochondroma / MHE    
 
   
Hemangioendothelioma    
       
NOF / Jaffe-Campanacci syndrome    
 
   
Paget's        
   
ASSUMPTIONS: (1) Younger patient is < 40 yrs; (2) assuming asymptomatic and no impending fracture
 
Ibank
  Location
Xray
Xray
CT
B. Scan
MRI
MRI
Histo(1)

Case A

spine
 

Case B

radius
 
 
 

Case C

humerus
 
 
 
Case D femur, 13 yo female
         

Case E

skull
 
 
 
 
 
(1) - histology does not always correspond to clinical case 
 

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Questions (6)

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(OBQ13.220) A 6-year-old boy presented to the emergency department with a suspected head injury after a fall. His parents attribute the injury to a loss of balance as they have noticed a worsening left sided limp over the past 2 months. Radiographs of the head and pelvis are seen in Figures A and B. Further questioning revealed the child has recently been worked up for increasing thirst and non-specific abdominal pain. A histology specimen can be seen in Figure C. What is the most likely diagnosis? Review Topic

QID: 4855
FIGURES:
1

Hand-Schuller-Christian disease

67%

(1723/2570)

2

McCune-Albright syndrome

16%

(416/2570)

3

Maffucci's syndrome

5%

(130/2570)

4

Neurofibromatosis

7%

(190/2570)

5

Ollier disease

4%

(92/2570)

L 3

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(OBQ13.47) A 2-year-old boy is brought in by his mother after an unwitnessed fall. She reports the child is constantly thirsty. His clinical appearance and radiographs are shown in Figures A through D. What is the most likely diagnosis? Review Topic

QID: 4682
FIGURES:
1

Child abuse

2%

(49/2303)

2

Neurofibromatosis

9%

(206/2303)

3

Metastatic neuroblastoma

16%

(365/2303)

4

Adamantinoma

2%

(55/2303)

5

Langerhans cell histiocytosis

70%

(1610/2303)

L 4

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(OBQ11.32) A 5-year-old child presents with skin lesions and a limp. He has met all developmental milestones accordingly but his parents have noticed he has recently stopped gaining weight. Clinical photograph of his skin lesion, radiograph of his left hip, and histology slide representative of his hip lesion are shown in Figures A, B, and C respectively. What is the most likely diagnosis? Review Topic

QID: 3455
FIGURES:
1

Neuroblastoma

5%

(100/1873)

2

Ewing's sarcoma

5%

(85/1873)

3

Eosinophilic granuloma

77%

(1442/1873)

4

Acute lymphoblastic leukemia

10%

(191/1873)

5

Fifth's disease

3%

(50/1873)

L 2

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(OBQ10.275) A 7-year-old boy complains of pain in the right thigh for 1 month with no history of traumatic injury. A radiograph is provided in Figure A. A histology specimen from incisional biopsy is provided in Figures B and C. Which of the following is the most appropriate management? Review Topic

QID: 3363
FIGURES:
1

Conservative treatment with crutches and repeat radiographs in 6 weeks

19%

(242/1260)

2

Wide excision

5%

(66/1260)

3

Wide excision and chemotherapy

62%

(775/1260)

4

Radiation therapy alone

6%

(80/1260)

5

Curettage and bone grafting

7%

(87/1260)

L 5

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