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Lymphoma

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Topic updated on 03/03/14 9:56pm
Introduction
  • Malignant hematopoietic tumor uncommonly found primarily in bone that occurs in three forms
    • primary lymphoma of bone (solitary site)
      • most primary lymphomas of bone are Non-Hodgkin's B-cell lymphomas rather than T-cell variants
      • diagnosed when there is only a single node of disease for six months
    • multiple bony sites (no visceral sites)
    • bone and soft tissue lymphoma
  • Epidemiology
    • incidence
      • 10-35% of non-Hodgkin's lymphoma patients have extranodal disease
      • primary lymphoma of bone is very rare
    • demographics
      • males > females
      • can occur in all age groups
      • most common in patients aged 35 to 55
    • location
      • bones with persistent red marrow
      • most common sites are pelvis, spine, and ribs
      • other common sites include knee (distal femur and proximal tibia), proximal femur, and shoulder girdle
    • risk factors
      • immunodeficiency (HIV, hepatitis)
      • viral or bacterial infections
  • Prognosis
    • primary lymphoma of bone has a better prognosis than secondary involvement of bone in lymphoma
Presentation
  • Symptoms
    • pain unrelieved by rest
    • 25% present with a pathologic fracture
    • neurologic symptoms from spinal compression
    • fever, nightsweats, weight loss (B-cell symptoms)
  • Physical Exam
    • inspection
      • warm and swollen large soft tissue masses are common
Imaging
  • Radiographs
    • large ill-defined diffuse lytic lesions with a subtle mottled appearance   
    • more common in the diaphysis of long bones 
    • 25% show cortical thickening 
    • "ivory" vertebrae
    • multiple sites of disease is common
    • differential diagnosis
      • metastatic disease
      • multiple myeloma
      • osteomyelitis
  • CT
    • CT of chest, abdomen, and pelvis required for staging
  • MRI
    • extensive marrow involvement with large soft-tissue mass
  • Bone scan
    • intensely positive
  • PET
    • useful to stage and follow the disease
Studies
  • Biopsy
    • bone marrow aspiration and biopsy are required for staging 
  • Histology 
    • diagnosis difficult to make with needle biopsy alone because tissue is often crushed
    • mixed small round blue cell infiltrate (different sizes and shapes)   
    • diffuse infiltration of trabeculae (as opposed to nodular)
    • immunohistochemical stains positive
      • CD20 positive
      • CD45 positive
      • lymphocyte common antigen positive 
Treatment
  • Nonoperative
    • multi-agent chemotherapy +/- local irradiation  
      • indications
        • mainstay of treatment
        • radiation may be added to obtain local control in persistent disease
      • techniques
        • cyclophosphamide, doxorubicin, prednisone and vincristine
      • outcomes
        • chemotherapy alone is effective for most lesions
        • 70% 5-year survival in disseminated disease
  • Operative
    • fracture stabilization
      • indications
        • stabilization of pathologic fractures or prophylactic fracture management 
Differentials & Groups
 
Destructive lesion in young patients(1)
 
Malignant lesion in older patient(2)
 
Malignant small cells tumor
 
Treatment is chemotherapy alone(3)
Lymphoma
 
 
 
Leukemia
     
   
Osteosarcoma
           
Ewing's sarcoma
     
   
Eosinophilic granuloma
           
Osteomyelitis
           
Desmoplastic fibroma
           
Metastatic disease    
 
   
Myeloma    
     
Chondrosarcoma    
       
MFH    
       
Secondary sarcoma    
       
Rhabdomyosarcoma        
   
Neuroblastoma        
   
ASSUMPTIONS: (1) Younger patient is < 40 yrs; (2) older patient > 40 (3) assuming no impending fracture
 
Image Bank
 
Location
Xray
Xray
CT
B.Scan
MRI
MRI
Histo(1)
Case A femur
         
Case B humerus
         
Case C pelvis
 
     
Case D femur
   
(1) - histology does not always correspond to clinical case 


 

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Qbank (2 Questions)

TAG
(OBQ10.202) A 45-year-old woman presents with severe thigh pain and constitutional symptoms for the past 4 months. A radiograph of the femur is shown in Figure A. Figure B displays a coronal T2 MRI and Figure C shows a sagittal T1 MRI with an arrow pointing to an adjacent soft tissue mass. Histology of a biopsy specimen is shown in Figure D. Immunohistochemistry stains are positive for CD20 and CD45. What is the most appropriate treatment for this tumor? Topic Review Topic
FIGURES: A   B   C   D    

1. Above knee amputation
2. Hip disarticulation
3. Intramedullary nail placement with adjuvant multiagent chemotherapy and possible irradiation
4. Limb salvage with wide resection and tumor prosthesis placement
5. Neoadjuvant chemotherapy followed by surgical resection followed by adjuvant chemotherapy

PREFERRED RESPONSE ▶
TAG
(OBQ04.79) A 75-year-old man presents with a displaced femoral neck fracture. During your surgical exposure for a hemiarthroplasty, the femoral neck has fractured through a pathologic lesion which is diagnosed as a lymphoma on frozen section. The lesion is located in the center of the femoral neck and the calcar femorale is not involved. Your treatment should include: Topic Review Topic

1. Closure of the wound and chemotherapy
2. Radical resection of the proximal femur and megaprothesis hemiarthroplasty
3. Hemiarthroplasty with postoperative staging and chemo-radiotherapy as indicated
4. Bone marrow transplantation
5. Hip disarticulation

PREFERRED RESPONSE ▶




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