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) 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 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
QUESTIONS 1 of 5 1 2 3 4 5 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (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? Tested Concept QID: 3295 FIGURES: A B C D Type & Select Correct Answer 1 Above knee amputation 3% (86/2880) 2 Hip disarticulation 1% (37/2880) 3 Intramedullary nail placement with adjuvant multiagent chemotherapy and possible irradiation 44% (1268/2880) 4 Limb salvage with wide resection and tumor prosthesis placement 14% (405/2880) 5 Neoadjuvant chemotherapy followed by surgical resection followed by adjuvant chemotherapy 37% (1070/2880) L 4 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review tested concept (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: Tested Concept QID: 1184 Type & Select Correct Answer 1 Closure of the wound and chemotherapy 2% (54/2423) 2 Radical resection of the proximal femur and megaprothesis hemiarthroplasty 4% (91/2423) 3 Hemiarthroplasty with postoperative staging and chemo-radiotherapy as indicated 93% (2259/2423) 4 Bone marrow transplantation 0% (5/2423) 5 Hip disarticulation 0% (7/2423) L 1 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review tested concept
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