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 o o o o Leukemia o o Osteosarcoma o Ewing's sarcoma o o Eosinophilic granuloma o Osteomyelitis o Desmoplastic fibroma o Metastatic disease o o Myeloma o o Chondrosarcoma o MFH o Secondary sarcoma o Rhabdomyosarcoma o Neuroblastoma o 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
QUESTIONS 1 of 6 1 2 3 4 5 6 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (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: A B C D Type & Select Correct Answer 1 Observation, NSAIDs, continued physical therapy 3% (42/1638) 2 Chemotherapy + limb salvage resection ± adjuvant radiation 42% (680/1638) 3 Joint aspiration 8% (127/1638) 4 IV antibiotics 6% (96/1638) 5 Multi-agent chemotherapy ± local irradiation 42% (683/1638) L 4 Question Complexity A Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic 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? QID: 3295 FIGURES: A B C D Type & Select Correct Answer 1 Above knee amputation 3% (99/3117) 2 Hip disarticulation 1% (41/3117) 3 Intramedullary nail placement with adjuvant multiagent chemotherapy and possible irradiation 44% (1360/3117) 4 Limb salvage with wide resection and tumor prosthesis placement 15% (452/3117) 5 Neoadjuvant chemotherapy followed by surgical resection followed by adjuvant chemotherapy 37% (1150/3117) L 4 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic (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 Type & Select Correct Answer 1 Closure of the wound and chemotherapy 2% (56/2629) 2 Radical resection of the proximal femur and megaprothesis hemiarthroplasty 4% (103/2629) 3 Hemiarthroplasty with postoperative staging and chemo-radiotherapy as indicated 93% (2448/2629) 4 Bone marrow transplantation 0% (6/2629) 5 Hip disarticulation 0% (8/2629) L 1 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic
All Videos (0) Podcasts (1) Pathology âŞLymphoma Orthobullets Team Pathology - Lymphoma Listen Now 13:30 min 6/4/2020 133 plays 0.0 (0)