SUMMARY Intramedullary Osteosarcomas are malignant, aggressive, osteogenic bone tumors most commonly found in the distal femur or proximal tibia. Patients are typically children or young adults that present with rapidly progressive pain and swelling. Diagnosis is made with a biopsy showing tumor cells with significant atypia and the presence of lacey osteoid. Treatment is usually neo-adjuvant chemotherapy, limb salvage surgical resection, followed by adjuvant chemotherapy. Epidemiology Demographics usually occurs in children and young adults bimodal distribution of occurrence majority occur in the second decade of life second peak in occurrence is in elderly patients with Paget's disease Anatomic location most common site is the distal femur and proximal tibia other common sites include proximal humerus, proximal femur, and pelvis EIOLOGY Malignancy most commonly diagnosed as Stage IIB (high grade, extra-compartmental, no metastases) 10-20% of patients present with pulmonary metastases (obtain CT of chest) lung is most common site of metastasis bone is second most common site Genetics tumor suppressor genes patients with mutations in the Retinoblastoma tumor suppressor gene (Rb) are predisposed to osteosarcoma patients with mutations in the P53 tumor suppressor gene are at risk for Li-Fraumeni syndrome which is associated with a high incidence of breast cancer and osteosarcoma risk increased in Rothmund Thomson syndrome AR inheritance, mutations in RECQL4 gene, chr 8q24.3 sun-sensitive facial poikiloderma rash (pigmentation, thinned skin, prominent blood vessels) absent eyelashes, eyebrows, hair juvenile cataracts, teeth abnormalities osteosarcoma, fibrosarcoma, gastric adenocarcinoma, cutaneous BCC and SCC Symptoms Symptoms rapidly progressive pain, fever, and swelling Physical exam may feel mass on examination Imaging Radiographs characteristic blastic and destructive lesion sun-burst or hair on end pattern of matrix mineralization periosteal reaction (Codman's triangle) large soft tissue mass with maintenance of bone cortices MRI must include entire involved bone used to determine: soft tissue involvement neurovascular involvement presence skip metastases if skip metastases are found, this is equivalent to metastatic (stage III) disease Bone scan very hot in osteosarcoma useful to evaluate extent of local disease and presence of bone metastases CT chest CT is required at presentation to evaluate for pulmonary metastases STUDIES Serum Labs Elevated alkaline phosphatase may be 2-3 times normal value Histology Characteristics tumor cells show significant atypia, and produce "lacey" osteoid stroma cells show malignant characteristics with atypia, high nuclear to cytoplasmic ratio, and abnormal mitotic figures May have mixed histology with different combinations of chondroblastic,osteoblastic, or fibroblastic looking cells depends on the subtype of osteosarcoma Giant cells may be present in giant cell rich osteosarcoma often confused with giant cell tumor of bone Diagnosis biopsy is required to obtain tissue diagnosis and institute therapy improper biopsy techniques are associated with increased rates of complications biopsy should be performed by the surgeon responsible for definitive treatment of the sarcoma, or after discussion with this surgeon DifferentiaL Osteosarcoma Differential Destructive bone lesion in young patients Treatment is Wide Resection & Chemotherapy Osteosarcoma (intramed. & periosteal) • • Ewing's sarcoma • • Leukemia • Lymphoma • Eosinophilic granuloma • Osteomyelitis • Desmoplastic fibroma • MFH / fibrosarcoma • Dedifferentiated chondrosarcoma • Secondary sarcoma • Rhabdomyosarcoma (soft tissue) • ASSUMPTIONS: (1) Younger patient is < 40 yrs; (2) assuming no impending fracture Treatment Operative neoadjuvant chemotherapy, limb salvage resection, followed by adjuvant chemotherapy indications high grade osteosarcoma chemotherapy preoperative chemotherapy given for 8-12 weeks followed by maintenance chemotherapy for 6-12 months after surgical resection 98% necrosis after neo-adjuvant chemotherapy is good prognostic sign expression of multi-drug resistance (MDR) gene tends to have a poor prognosis tumor cells can pump chemotherapy out of cell with MDR expression present in 25% of primary lesions and 50% of metastatic lesions surgical technique trend towards limb salvage whenever possible overall survival in osteosarcoma is equal after limb salvage vs. amputation to deal with local extent of disease rotationplasty is a great surgical option which optimizes the patient's function, and most commonly done in a pediatric population wide surgical resection indications indicated in low grade osteosarcoma such as parosteal osteosarcoma amputation indications pathologic fracture encasing neurovascular bundle enlarging during preop chemo AND adjacent to neurovascular bundle Prognosis 76% long-term survival with modern treatment Poor prognostic factors include advanced stage of disease (most predictive of survival) response to chemotherapy (as judged by percent tumor necrosis of resected specimen) Greater than 90% is a good prognostic indicator tumor site and size expression of P-glycoprotein high serum alkaline phosphatase high lactic dehydrogenase vascular involvement surgical margins type of chemotherapy regimen
Technique Guide Technique guides are not considered high yield topics for orthopaedic standardized exams including ABOS, EBOT and RC. Endoprosthetic Reconstruction of Proximal Femur Malignant Lesion Orthobullets Team Pathology - Intramedullary Osteosarcoma Technique Guide Technique guides are not considered high yield topics for orthopaedic standardized exams including ABOS, EBOT and RC. Endoprosthetic Reconstruction of Proximal Humerus Orthobullets Team Pathology - Intramedullary Osteosarcoma Technique Guide Technique guides are not considered high yield topics for orthopaedic standardized exams including ABOS, EBOT and RC. Distal Femur Resection with Endoprosthetic Replacement Ahmed Siddiqi Pathology - Intramedullary Osteosarcoma
QUESTIONS 1 of 31 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 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 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 Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ13.109) A 14-year-old immigrant from a developing country had complained of pain playing soccer 4 months prior. Radiographs at that time were read as normal and he was given a prescription for NSAIDs. More recently, his parents noticed he is limping and complaining of pain after playing games. He has had some intermittent fevers and swelling around his thigh. A complete blood workup is performed. Current radiographs and MRI are seen in Figures A and B respectively. What is the next step in treatment QID: 4744 FIGURES: A B Type & Select Correct Answer 1 Biopsy 95% (3390/3586) 2 Genetic screening for EXT1 and EXT2 gene mutations 1% (48/3586) 3 NSAIDS and single-dose radiation therapy 1% (20/3586) 4 Intravenous antibiotics 3% (93/3586) 5 Observation 0% (17/3586) L 1 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review Tested Concept Review Full Topic (OBQ13.86) An 11-year-old male presents with left forearm swelling and pain after getting kicked while playing soccer. Figures A through E are the radiographs, bone scan and MRI scan of the forearm and biopsy results. What is the most likely diagnosis? QID: 4721 FIGURES: A B C D E Type & Select Correct Answer 1 Chondroblastoma 4% (152/3442) 2 Intramedullary osteosarcoma 50% (1719/3442) 3 Parosteal osteosarcoma 37% (1281/3442) 4 Giant cell tumor 4% (124/3442) 5 Osteoblastoma 4% (141/3442) L 5 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (OBQ13.70) An 11-year-old boy presents with knee pain, warmth and swelling after a fall. Radiographs, MRI, bone scan and biopsy of the knee are seen in Figures A through E. What is the most appropriate treatment plan? QID: 4705 FIGURES: A B C D E Type & Select Correct Answer 1 Curettage and targeted intravenous antibiotics for 6 weeks. 4% (136/3819) 2 Curettage, cancellous bone grafting, non-weightbearing until visible callus is seen on follow-up radiographs. 8% (287/3819) 3 Curettage, placement of antibiotic beads, intravenous antibiotics for 6 weeks, removal of beads and placement of bone graft substitute at 6 weeks. 4% (144/3819) 4 Neoadjuvant chemotherapy, physeal-sparing resection, intercalary bone allograft, locked plate fixation, postoperative adjuvant chemotherapy. 5% (196/3819) 5 Neoadjuvant chemotherapy, wide resection of the distal one-third of the femur, reconstruction with a custom implant, postoperative adjuvant chemotherapy. 79% (3027/3819) L 2 Question Complexity A Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic (OBQ12.228) A 13-year-old presents with persistent leg pain. Representative radiographs are shown in Figures A and B. A biopsy is subsequently obtained and is shown in Figure C. What is the most likely diagnosis? QID: 4588 FIGURES: A B C Type & Select Correct Answer 1 Ewing's Sarcoma 8% (338/4251) 2 Eosinophilic granuloma 14% (581/4251) 3 Lymphoma 1% (52/4251) 4 Osteosarcoma 61% (2581/4251) 5 Osteoblastoma 15% (645/4251) L 3 Question Complexity A Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic (OBQ12.175) A 13-year-old boy presents with left leg pain for 10 weeks. He reports a remote history of trauma while falling off of a trampoline 12 weeks ago. He reports constitutional symptoms for the past 4 weeks. The patient's serum LDH and alkaline phosphatase are elevated. A radiograph of the left knee is shown in Figures A and B. A bone scan is displayed in Figure C. Magnetic resonance imaging (MRI) is shown in Figure D and E. A biopsy specimen is shown in Figure F. What is the most appropriate next step in management? QID: 4535 FIGURES: A B C D E F Type & Select Correct Answer 1 Neoadjuvant chemotherapy, surgical excision, adjuvant radiation therapy 11% (465/4256) 2 Surgical irrigation and debridridement followed by 6 weeks of culture directed intravenous antibiotics 5% (199/4256) 3 Surgical excision followed by adjuvant chemotherapy and radiation therapy 7% (314/4256) 4 Neoadjuvant chemotherapy, surgical excision, adjuvant chemotherapy 74% (3157/4256) 5 Surgical irrigation and debridridement with antibiotic cement spacer placement followed by 6 weeks of culture directed intravenous antibiotics 2% (92/4256) L 3 Question Complexity A Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic (OBQ11.72) A 8-year-old boy presents with knee pain and an effusion. Biopsy and staging studies show a distal femoral osteosarcoma with contamination of the knee joint. Which of the following treatment options will provide this child with the best chance of local control and the highest level of function? QID: 3495 Type & Select Correct Answer 1 Through knee amputation 1% (34/2468) 2 Above knee amputation 17% (414/2468) 3 Rotationplasty 63% (1545/2468) 4 Extra-articular resection, endoprosthetic reconstruction, and free flap coverage 14% (353/2468) 5 Extra-articular resection, allograft prosthetic composite, and free flap coverage 4% (110/2468) L 3 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ10.32) A 16-year-old boy presents with a 6-month history of increasing knee pain and swelling. He is otherwise healthy, but the child has a known retinoblastoma gene mutation. A mass is found on the posterior aspect of his knee, and a biopsy is taken. A radiograph is shown in Figure A. Which of the following biopsy specimens in Figures B through F most likely fits with this patient's history, exam, and radiograph? QID: 3120 FIGURES: A B C D E F Type & Select Correct Answer 1 Figure B 11% (320/3009) 2 Figure C 15% (445/3009) 3 Figure D 11% (335/3009) 4 Figure E 26% (773/3009) 5 Figure F 37% (1104/3009) L 4 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic (OBQ09.193) Which of the following most accurately lists the most common location of osteosarcoma in decreasing frequency? QID: 3006 Type & Select Correct Answer 1 axial skeleton > distal femur > proximal tibia > proximal humerus 1% (19/1912) 2 distal femur > proximal tibia > proximal humerus > axial skeleton 88% (1676/1912) 3 distal femur > axial skeleton > proximal tibia > proximal humerus 1% (27/1912) 4 distal femur > proximal tibia > axial skeleton > proximal humerus 8% (162/1912) 5 proximal tibia > distal femur > axial skeleton > proximal humerus 1% (14/1912) L 2 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 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 Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ08.159) You are asked to consult on a 8-year-old child in the emergency department with the pathologic fracture seen in Figure A. In preparation for transfer to a sarcoma center for treatment, all of the following are correct steps in the child's management EXCEPT? QID: 545 FIGURES: A Type & Select Correct Answer 1 Pain management 1% (16/2442) 2 Hanging arm sling 3% (74/2442) 3 Tumor staging, including bone scan, CT of chest, MRI of the entire bone 4% (94/2442) 4 Consultation with pediatric oncology 1% (23/2442) 5 Tumor biopsy performed by interventional radiology 91% (2217/2442) L 1 Question Complexity A Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic (OBQ08.46) A 19-year-old woman presents with dull right thigh pain for the past 2 months. Radiographs and a biopsy specimen are shown in Figures A-C. What is the most appropriate definitive treatment? QID: 432 FIGURES: A B C Type & Select Correct Answer 1 Nonsteroidal anti-inflammatory drugs with follow-up x-ray in 6 months 2% (41/1697) 2 Intramedullary nailing 1% (16/1697) 3 Intravenous antibiotics 1% (16/1697) 4 Radiation therapy 2% (42/1697) 5 Preoperative chemotherapy, wide resection, and postoperative chemotherapy 93% (1573/1697) L 1 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 (OBQ07.63) In patients with an extremity-based osteosarcoma without metastasis, all of the following are risk factors for disease progression and poor outcomes EXCEPT? QID: 724 Type & Select Correct Answer 1 High histologic grade 0% (3/1201) 2 Low serum level of alkaline phosphatase at diagnosis 80% (965/1201) 3 Large tumor volume 2% (19/1201) 4 Inadequate surgical margins following resection 0% (6/1201) 5 70% histologic tumor necrosis after pre-operative chemotherapy 17% (203/1201) L 2 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (OBQ06.197) A 13-year-old girl presents with right thigh pain and constitutional symptoms for 8 weeks. The patient's serum LDH and alkaline phosphatase are elevated. A radiograph of the right femur is shown in Figure A. Computed tomography (CT) of the chest reveals no pulmonary metastases. Magnetic resonance imaging (MRI) of the right femur shows erosion of the tumor through the cortex into the adjacent soft tissue, but no skip lesions are seen. A biopsy specimen is shown in Figures B and C. What stage does this patient's case most appropriately represent using the Enneking Staging System for Musculoskeletal Tumors? QID: 383 FIGURES: A B C Type & Select Correct Answer 1 IA 1% (15/2965) 2 IB 4% (104/2965) 3 IIA 14% (428/2965) 4 IIB 78% (2298/2965) 5 IIIA 4% (105/2965) L 2 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic (OBQ06.243) What is the most common site of metastasis for osteosarcoma? QID: 254 Type & Select Correct Answer 1 Bone 7% (132/1861) 2 Liver 2% (30/1861) 3 Lung 89% (1657/1861) 4 Kidney 0% (5/1861) 5 Lymph nodes 2% (33/1861) L 1 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic (OBQ06.78) A 17-year-old male presents with increasing right shoulder pain and swelling. There is no history of trauma and he is otherwise healthy. Representative clinical photograph, radiograph, bone scan, MRI, and histology are shown in Figures A through E. What is the most appropriate treatment for this patient? QID: 189 FIGURES: A B C D E Type & Select Correct Answer 1 Observation 1% (17/2502) 2 Chemotherapy and radiation therapy 2% (54/2502) 3 Surgery alone 13% (333/2502) 4 Surgery and chemotherapy 75% (1876/2502) 5 Surgery and radiation therapy 8% (210/2502) L 2 Question Complexity A Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic (OBQ05.120) A 13-year-old boy is diagnosed with osteosarcoma of his distal femur. The treating surgeon samples the bone marrow from the resection margin of the femur during definitive limb salvage surgery and finds microscopic evidence of tumor. Assuming no change in the resection level, what type of resection would this be considered? QID: 1006 Type & Select Correct Answer 1 Intra-lesional 57% (1071/1873) 2 Inter-lesional 4% (68/1873) 3 Wide 7% (128/1873) 4 Marginal 30% (556/1873) 5 Radical 2% (43/1873) L 3 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review Tested Concept Review Full Topic (OBQ04.39) A 14-year-old child presents to your office with 6 months of worsening knee pain. Figures A through D show the respective radiographs, bone scan, MRI, and histology. What treatment protocol has been shown to maximize survival in patients with this diagnosis? QID: 100 FIGURES: A B C D Type & Select Correct Answer 1 Neoadjuvant chemotherapy followed by surgical excision 2% (28/1344) 2 Neoadjuvant chemotherapy, surgical excision, adjuvant chemotherapy 85% (1137/1344) 3 Surgical excision followed by radiation therapy 2% (32/1344) 4 Neoadjuvant chemotherapy, surgical excision, radiation therapy 8% (114/1344) 5 Surgical excision followed by adjuvant chemotherapy 2% (31/1344) L 1 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (OBQ04.164) A 10-year-old boy has 2 months of right knee pain that started at summer camp. The patient denies constitutional symptoms. There is no lymphadenopathy present. CT of the chest shows no signs of metastatic disease. Imaging studies and biopsy results are shown in Figures A-E. What is the most likely diagnosis? QID: 1269 FIGURES: A B C D E Type & Select Correct Answer 1 Synovial sarcoma 1% (14/1835) 2 Malignant fibrous histiocytoma 5% (98/1835) 3 Chondrosarcoma 6% (110/1835) 4 Ewing's sarcoma 9% (172/1835) 5 Osteosarcoma 78% (1434/1835) L 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic (OBQ04.7) A 30-year-old man has had leg pain for 6 months. A lesion is identified in the proximal femur and biopsy it taken. Histology is shown in Figure A and is consistent with a low-grade intramedullary osteogenic sarcoma. Additional imaging studies confirm that this is an isolated lesion with no metastasis. What is the standard treatment for this type of lesion? QID: 118 FIGURES: A Type & Select Correct Answer 1 chemotherapy and surgery 57% (457/800) 2 chemotherapy only 1% (5/800) 3 surgery only 31% (248/800) 4 radiation and surgery 10% (79/800) 5 radiation only 0% (4/800) L 5 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic
All Videos (2) Podcasts (1) Login to View Community Videos Login to View Community Videos oncology Orthopedic Oncology Course - Malignant Bone Forming Tumors (Osteosarcoma) - Lecture 4 James C. Wittig Pathology - Intramedullary Osteosarcoma B 12/22/2016 504 views 4.0 (2) Login to View Community Videos Login to View Community Videos Histopathology of Osteosarcoma - Pathology Rounds General - Intramedullary Osteosarcoma C 10/3/2012 674 views 3.3 (10) Pathology | Intramedullary Osteosarcoma Pathology - Intramedullary Osteosarcoma Listen Now 12:56 min 10/16/2019 491 plays 4.7 (6)
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