Updated: 8/10/2019

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Introduction
  • Histocytosis X is a spectrum of diseases of the reticuloendothelial system that includes
    • eosinophilic granuloma
      • usually a single self-limited lesion found in younger patients (first 3 decades of life)
    • Hand-Schuller-Christian disease
      • has both bone and visceral lesions
      • classic triad (< 25%) includes
        • exophthalmus
        • diabetes insipidus
        • lytic skull lesions
    • Letterer-Siwe disease
      • fatal form that occurs in young patients
Presentation
  • Symptoms
    • pain and swelling
Imaging
  • Radiographs
    • well defined intramedullay lytic or "punched-out" lesion 
    • cortex may be destroyed
    • may have periosteal reaction
    • vertebra plana in spine 
  • MRI
    • may show a soft tissue mass
    • bone scan is warm to hot
  • Electronmicroscopy
    • birbeck granules seen inside Langerhan's cells 
Histology
  • Characteristic histology shows
    • 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
      • pink cytoplasm (depends on stain)
    • lipid-laden foam cells
      • with nuclear debris a mixture of inflammatory cells 
    • 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
      • a self-limited process and it is reasonable to treat with observation alone
    • low dose irradiation (600-800 cGy)
      • effective for most lesions
      • indicated for lesions in the spine that compromise stability and neurologic status
  • Operative
    • curettage and bone grafting
      • indicated for lesions that endanger the articular surface or are a risk for impending fractures
Differentials and 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 aymptomatic 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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