Updated: 6/21/2021

Lymphoma

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https://upload.orthobullets.com/topic/8025/images/Case D - femur - xray a - Parsons_moved.png
https://upload.orthobullets.com/topic/8025/images/Case D - femur - MRI T2- Parsons_moved.png
https://upload.orthobullets.com/topic/8025/images/Case A - femur - xray - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8025/images/Case B -humerus - xray - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8025/images/Histology A - high power - Parsons_moved.png
  • summary
    • Lymphoma is a malignant hematopoietic tumor that occurs in three forms: primary lymphoma of bone, multiple bony sites, or bone and soft tissue lymphoma. Patients typically present between ages 35 and 55 with regional pain associated with fever, night sweats, and weight loss. 
    • Diagnosis is made with biopsy showing mixed small round blue cell infiltrate (different sizes and shapes) that are CD20 and CD45 positive on immunohistochemical stains.
    • Treatment is usually chemotherapy and radiation. Surgical management is indicated for associated fractures.
  • 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
    • Anatomic 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
  • Etiology
    • 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
  • 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)
        • differentiate from other small round blue cell tumors
        • CD99 negative and absent 11:22 chromosomal translocation
      • diffuse infiltration of trabeculae (as opposed to nodular)
      • immunohistochemical stains positive
        • CD20 positive
        • CD45 positive
        • lymphocyte common antigen positive
  • Differentials
    • Differential of Lymphoma

      Destructive lesion in young patients
      Malignant lesion in older patient
      Malignant small cells tumor
      Treatment is chemotherapy alone
      Lymphoma
      Leukemia
      Osteosarcoma
      Ewing's sarcoma
      Eosinophilic granuloma
      Osteomyelitis
      Desmoplastic fibroma
      Metastatic disease
      Myeloma
      Chondrosarcoma
      MFH
      Secondary sarcoma
      Rhabdomyosarcoma

      Neuroblastoma
  • 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
  • Prognosis
    • Primary lymphoma of bone has a better prognosis than secondary involvement of bone in lymphoma

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(OBQ17.159) A 42-year-old male presents to clinic with 1 year of atraumatic left knee pain. He also reports subjective fever and chills. His physical examination is remarkable for diffuse knee pain but there is no overlying erythema or induration. His current laboratory values reveal a white blood cell count of 9200/µL (reference range [rr], 5000-12000/µL), absolute neutrophil count of 1800/µL (rr, 2000-7000/µL) with 53.7% lymphocytes (rr, 20-40%), an erythrocyte sedimentation rate (ESR) of 21 mm/h (rr, 0-20 mm/h), and a C-reactive protein (CRP) level of 2.2 mg/dL (rr, < 2 mg/dL). He has attempted a trial of physical therapy as well as a trial of oral antibiotics with no improvement of symptoms. Imaging is shown in Figures A-C. A biopsy is performed and is shown in Figure D. Which of the following is the best next step in management?

QID: 210246
FIGURES:
1

Observation, NSAIDs, continued physical therapy

3%

(40/1597)

2

Chemotherapy + limb salvage resection ± adjuvant radiation

42%

(667/1597)

3

Joint aspiration

8%

(124/1597)

4

IV antibiotics

6%

(94/1597)

5

Multi-agent chemotherapy ± local irradiation

41%

(662/1597)

L 4 A

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(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?

QID: 3295
FIGURES:
1

Above knee amputation

3%

(99/3101)

2

Hip disarticulation

1%

(41/3101)

3

Intramedullary nail placement with adjuvant multiagent chemotherapy and possible irradiation

44%

(1356/3101)

4

Limb salvage with wide resection and tumor prosthesis placement

15%

(450/3101)

5

Neoadjuvant chemotherapy followed by surgical resection followed by adjuvant chemotherapy

37%

(1140/3101)

L 4 C

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(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:

QID: 1184
1

Closure of the wound and chemotherapy

2%

(55/2570)

2

Radical resection of the proximal femur and megaprothesis hemiarthroplasty

4%

(101/2570)

3

Hemiarthroplasty with postoperative staging and chemo-radiotherapy as indicated

93%

(2393/2570)

4

Bone marrow transplantation

0%

(5/2570)

5

Hip disarticulation

0%

(8/2570)

L 1 D

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