Introduction A malignant osteogenic tumor similar to classic osteosarcoma in epidemiology and genetics similar in ABC in presentation (must differentiate) Epidemiology (similar to classic osteosarcoma) incidence rare (4% of osteosarcomas) demographics male > female location proximal humerus, proximal femur, distal femur, proximal tibia occur in same location as ABC risk factors history of prior radiation Genetics associated with the following mutations tumor suppressor genes Rb-1 p53 oncogenes HER2/neu c-myc c-fos Prognosis prognostic variables poor advanced tumor stage is most important indicator increased lactate dehydrogenase (LDH) and alkaline phosphatase (ALP) expression of multi-drug resistance (MDR) gene pathologic fractures increased risk of recurrence favorable 98% necrosis with chemo is good prognostic sign survival more chemosensitive but same survival as intramedullary osteosarcoma 5 year survival with tumor localized to an extremity is ~70% 5 year survival with metastases is ~20% Presentation Symptoms pain 25% present with pathologic fracture Physical exam soft tissue swelling local tenderness Imaging Radiographs lytic, destructive, and expansile lesion; entire cortex may be compromised Bone scan shows increased uptake MRI indications indicated in all cases to determine soft tissue involvement findings fluid-fluid levels extensive edema in surrounding tissue Studies Histology characterized by high grade sarcoma with mitotic figures is seen in intervening cellular areas lakes of blood mixed with malignant cells (not in ABC) not as much osteoid as intramedullary osteosarcoma histology is critical to differentiate from ABC Gross pathology will show "bag of blood" Differential ABC ABC differentiating ABC from telangietic osteosarcoma is difficult and critical both have similar radiographic appearance both have large blood filled spaces both have similar location (proximal humerus, proximal femur, distal femur, proximal tibia) diagnosis needs to be confirmed by an experienced musculoskeletal pathologist ABC vs Telangietic Osteosarcoma Aneurysmal Bone Cyst Telangietic Osteosarcoma Epidemiology • 75% of patient < 20 years.• Locations (proximal humerus, proximal femur, distal femur, proximal tibia) • Locations (proximal humerus, proximal femur, distal femur, proximal tibia) Radiographs • Expansive, eccentric and lytic lesion with bony septae • Expansive, eccentric and lytic lesion with bony septae MRI • Clear fluid levels• May extend into soft tissue. • Fluid levels less defined• Edema in surrounding tissue Histology • Blood-filled spaces, spindle cells, benign giant cells, no evidence of malignant cells • Lakes of blood mixed with malignant cells with mitotic bodies Treatment Operative multi-agent (neoadjuvant) chemotherapy and limb salvage resection indications standard of care in most patients surgical resection trend towards limb salvage whenever possible can be performed in close to 90% of cases chemotherapy preoperative chemotherapy given for 8-12 weeks followed by maintenance chemotherapy for 6-12 months after surgical resection doxorubicin/cisplatin/methotrexate/ifosamide 98% necrosis with chemo is good prognostic sign expression of multi-drug resistance (MDR) gene portends very poor prognosis cells can pump chemo out of cell present in 25% of primary lesions and 50% of metastatic lesions outcomes risk of recurrence is decreased with good cellular response/wide surgical margins Complications Local recurrence uncommon (~5%) associated with poor prognosis increased risk of recurrence in patients with displaced, pathologic fracture Ibank Location Xray Xray CT Bone scan MRI MRI Case A prox. humerus Case B distal femur Case C distal femur
QUESTIONS 1 of 1 1 Previous Next You have 100% on this question. Just skip this one for now. Take This Question Anyway (OBQ10.124) a 21-year-old male has 6 months of increasing knee pain and has recently noticed a mass at his knee. Radiographs are shown in Figures A and B. A biopsy specimen of the proximal tibia mass is shown in Figure C. What is the most appropriate first step in management? Review Topic | Tested Concept QID: 3218 FIGURES: A B C Type & Select Correct Answer 1 Neoadjuvant chemotherapy 36% (783/2166) 2 Wide surgical excision 11% (247/2166) 3 Intralesional curettage and bone grafting 51% (1103/2166) 4 Radiation therapy 1% (28/2166) 5 Hip disarticulation 0% (1/2166) L 5 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1
Evidence Telangiectatic osteosarcoma: a review of 87 cases Pathology - Telangiectatic Osteosarcoma 0 views
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