dr james c wittig, orthopedic oncologist, new york, new jersey
About Dr. Wittig
Lymphoma of Bone

General Information

  • Primary lymphoma of bone is defined as lymphoma arising within the medullary cavity of a bone in the absence of lymph node or organ involvement for at least 6 months after diagnosis
  • Primary lymphoma of bone is rare (3% of primary bone tumors) and most lymphomas that involve bone are metastatic from lymph node. If a lymphoma of bone is diagnosed one must look for another site.
  • Most primary lymphomas of bone are Non Hodgkin's, large cell lymphomas
  • In U.S. majority are B-cell proliferations
  • Must rule out presence of extraskeletal disease
  • May be misdiagnosed as chronic osteomyelitis

Clinical Presentation

Signs/Symptoms:

  • Localized dull or aching pain
  • May have palpable mass or swelling
  • Usually no general symptoms and appear healthy
  • Pathological fractures in 25% of cases
  • Prevalence: Male predilection (1.5:1)

Age:

  • Broad age range
  • Most occur after second decade with 50% occurring above 40 years
  • Rare in children

Sites:

  • Any bone can be involved
  • Lower extremities involved most often especially femur and pelvis
  • More common in appendicular than axial skeleton (opposite of metastatic lymphoma)

Radiographic Presentation

  • Permeative or moth eaten bone destruction (55%)
    • Geographic (11%); Blow out (1%); Blastic (2%); Normal XR (5%)
  • Metadiaphysis (75%)
  • Periosteal reaction—may look benign
    • Interrupted or solid single layer (66%)
    • Onion Skin 10%
    • Sunburst 2%
  • Soft tissue mass— by CT (80%); by MRI (100%)
  • Pathologic Fracture (22%)
  • Sequestra (16%)
  • Cross Joint (5%)
  • Diff Dx:
    • Metastatic Lymphoma
    • Ewings
    • Neuroblastoma
    • Rhabdomyosarcoma
    • Osteomyelitis
    • Eosinophilic Granuloma
Roll over the images for more information
Permeative or moth eaten  lytic lesion Permeative Lesion
Marrow Replacement Intrasseous Lesion, Soft Tissue Mass
Radiographic Presentation:<br />Primary Lymphoma of Bone TI Weighted MRI
MRI: Primary Lymphoma of Bone
X-ray: Primary Lymphoma of Distal Humerus, Permeative Lesion X-ray: Primary Lymphoma of Tibia
X-ray: Primary Lymphoma of Humerus X-Ray: Primary Lymphoma of Proximal Tibia, Permeative Lesion of Proximal Tibia with Pathological Fracture

Gross Pathology

  • Diffuse infiltrative growth pattern with soft tissue extension
  • Intraosseous component
    • Mixture of bone spicules and marrow fat as lesion permeates through the medullary canal and has an indistinct margin
  • Extraosseous tissue
    • Tan or white, resembles lymphomatous lymph nodes
  • Areas of necrosis, hemorrhage, and cystic degeneration are present

Microscopic Pathology

  • Diffuse growth pattern
  • Mixture of small lymphocytic cells and larger histiocytic components (Large Malignant B Cells in most cases)
  • Cells and no matrix
  • Nuclei
    • Vary in shape and size
    • Grooved vesicular nuclei
    • Prominant nucleoli
  • Cytoplasmic glycogen is absent
  • Complex reticulin framework
  • Prominent fibroblastic component
  • CD5 and Leukocyte Common Antigen Positive
  • CD3+ and CD45+ for B Cell Lymphoma; CD3+ for Rare T-Cell
Roll over the images for more information
Microscopic Pathology: Lymphoma Microscopic Pathology: Lymphoma
Microscopic Pathology: Lymphoma Microscopic Pathology: Lymphoma
Microscopic Pathology: Lymphoma Microscopic Pathology: Lymphoma
Microscopic Pathology: Lymphoma Microscopic Pathology: Lymphoma

Differential Diagnosis

  • Ewing Sarcoma
  • Chronic Osteomyelitis
  • Leukemia

Treatment

  • Chemotherapy and radiation
  • If truly an isolated primary lymphoma of bone, limb sparing surgery may be considered instead of or in conjunction with radiation
  • Surgical resections to treat residual or recurrent tumors after radiation
  • Amputation only recommended when lesions are unresponsive to radiation or recur and are not amenable to limb sparing surgery for various reasons

Prognosis

Patients with:

  • Monostotic disease & no soft tissue involvement
    • 58% 5-year and 53% 10-year survival
  • Multifocal bony disease
    • 42% 5 year and 35% 10 year survival
  • Osseous lymphoma with lymph node involvement
    • 22% 5-year and 12% 10-year survival
  • Chemotherapy increases survival rates (88% disease free survival at 7 years) of patients with primary lymphoma of bone
  • Has best prognosis of all osseous malignancies except for low grade intraosseous and parosteal osteosarcoma
  • Possible better prognosis for lymphomas with cleaved nuclei as compared to noncleaved, immunoblastic or pleomorphic variants
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