summary Ewing's Sarcoma is a malignant, distinctive small round cell sarcoma associated with a t(11:22) translocation and most commonly occurs in the diaphysis of long bones. Patients typically present at age < 25 with insidious onset of regional pain, swelling, and fevers. Diagnosis is made with a biopsy showing sheets of monotonous small round blue cells with prominent nuclei and minimal cytoplasm. Immunostaining will show positive CD99. Treatment is usually neo-adjuvant chemotherapy, limb salvage surgical resection, followed by adjuvant chemotherapy with or without radiation. Epidemiology Demographics typically found in patients from 5-25 years of age second most common malignant bone tumor in children uncommon in African Americans and Chinese Anatomic location ~50% are found in the diaphysis of long bones most common locations pelvis, distal femur, proximal tibia, femoral diaphysis, and proximal humerus 5% metaphysis Etiology Genetics t(11:22) translocation found in 95% of cases leads to the formation of a fusion protein (EWS-FLI1) can be identified with PCR and useful to differentiate Ewing sarcoma from other round cell lesions Staging almost all tumors are MSTS stage IIB or III Presentation Symptoms pain often accompanied by fever often mimics an infection Physical exam swelling and local tenderness Imaging Radiographs required images AP and lateral of affected area findings large destructive lesion in the diaphysis or metaphysis with a permeative moth-eaten appearance lesion may be purely lytic or have variable amounts of reactive new bone formation periosteal reaction may give an "onion skin" or "sunburst" appearance Bone scan indications required as initial staging workup findings will show very "hot" lesion MRI indications required as initial staging workup to identify soft-tissue extension and marrow involvement findings often shows a large soft tissue component CT chest indications required as initial staging workup to look for pulmonary metastasis Studies Labs ESR is elevated WBC is elevated anemia is common lactic dehydrogenase Bone marrow biopsy required as part of workup for Ewing's to rule out metastasis to the marrow Histology Gross appearance may have liquid consistency mimicking pus Characteristic findings sheets of monotonous small round blue cells prominent nuclei and minimal cytoplasm may have pseudo-rosettes (circle of cells with necrosis in center) Immunostaining positive CD99 (in 95%) MIC2 vimentin PAS positive (intracellular glycogen) neuron specific enolase (NSE) S100 Leu7 negative cytokeratin reticulin (positive in lymphoma) neurofilament (positive in neuroblastoma) see complete immonostaining chart Differential Small-round-cell tumor differential (by age) < 5 yrs: neuroblastoma or leukemia 5-10 yrs: eosinophilic granuloma 5-30 yrs: Ewing's sarcoma >30 yrs: lymphoma > 50 yrs: myeloma Osteosarcoma Osteomyelitis Differential of Ewing's Sarcoma Destructive lesion in young patients Small round cell tumors Treatment is Wide Resection & Chemotherapy Ewing's Sarcoma o o o Osteosarcoma o o Lymphoma o o Leukemia o o Eosinophilic Granuloma o o Osteomyelitis o Desmoplastic fibroma o Metastatic disease Neuroblastoma (soft tissue) o Rhabdomyosarcoma (soft tissue) Secondary Sarcoma Dediff. Chondrosarcoma o MFH/Fibrosarcoma Multiple Myeloma o Treatment Nonoperative chemotherapy + radiation therapy indications non-resectable tumors (eg. large spinal tumors) sites where functional deficit is unacceptable trend is towards surgical resection/away from RT because of morbidity associated with radiation and risk of secondary malignancies Operative chemotherapy + limb salvage resection ± adjuvant radiation indications standard of care in most patients where primary tumor can be completely removed (expendable and surgically reconstructible sites) chemotherapy vincristine, doxorubicin, cyclophosphamide, and dactinomycin preoperative chemotherapy (neoadjuvant) given for 8-12 weeks followed by surgical resection and maintenance (adjuvant) chemotherapy for 6-12 months adjuvant radiation not necessary if margins are adequate and there is good response to chemotherapy indications positive post-resection surgical margins patients who present with widely metastatic disease where chemotherapeutic response has been poor Complications Secondary neoplasms bone sarcoma (from radiation therapy) risk is 10-20% at 20y arises in prior RT treatment field dose dependant ≥ 60Gy of RT confers 20% risk 50-60Gy confers 5% risk <48Gy has no risk treatment related acute myeloid leukemia / myelodysplasia (tMDS/AML)(from chemotherapy) in 2% of survivors of ES arises at ~5y after diagnosis Recurrence/progression extremely poor prognosis (<10% 5YS) Metastases 25-30% have macrometastases on presentation (lungs, bone, bone marrow) 10% have bone mets on presentation cure rates with chemotherapy 30% cure rate for lung mets alone 20% cure rate for bone mets alone 15% cure rate for combined bone and lung mets Radiation therapy complications limb length discrepancy (especially in skeletally immature) joint contracture muscle atrophy pathologic fracture secondary sarcoma Venous thromboembolism high rate of venous thromboembolic events in patients with sarcoma tumor activation of factor X to factor Xa Prognosis Survival 5 yr survival 65-80% for localized disease 25-40% for metastatic disease 10 yr survival 60% for localized disease 30% for metastatic disease Poor prognostic factors tumor size/location tumors greater than 100cm3 or >8cm in size spine and pelvic tumors (worst) > proximal extremities > distal extremities (best prognosis) age and sex older age (>14) worse prognosis male worse prognosis laboratory parameters elevated lactic dehydrogenase levels (>200IUml) indicates large tumors/metastatic disease anemia and elevated WBC indicates extensive disease molecular pathology p53 mutation in addition to t(11:22) translocation overexpression of cell proliferation antigen Ki-67 overexpression of HER-2/neu metastases (most important prognostic indicator) presence of mets has poor prognosis lung metastases better prognosis than bone/bone marrow mets skip metastases (same bone) better pronosis than metastases to another site chemotherapy response < 90% necrosis with chemotherapy
QUESTIONS 1 of 28 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 Previous Next (OBQ18.158) A 15-year old male without previous medical history has been complaining of right shoulder pain for 1 month. He also reports that he has lost 5 pounds within that same timeframe. He states that he has difficulty sleeping at night due to feeling warm and sweaty. Current imaging is shown in Figures A and B. A bone marrow biopsy is performed and is found to be positive for a EWS-FL1 fusion protein. Which of the following combines the correct diagnosis with the correct translocation? QID: 213054 FIGURES: A B Type & Select Correct Answer 1 Synovial sarcoma; t(x;18) 1% (18/1617) 2 Ewing's sarcoma; t(12;22) 5% (87/1617) 3 Osteosarcoma; t(9;22) 3% (45/1617) 4 Osteosarcoma; t(x;18) 1% (16/1617) 5 Ewing's sarcoma: t(11;22) 89% (1442/1617) N/A 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 (OBQ12.222) A 10-year-old male presents with ongoing complaints of left thigh pain after falling during a soccer game. A clinical photo is seen in Figure A. He has tenderness and general warmth over the lateral aspect of his left thigh. His ESR is 82 and his WBC is 15. A radiograph and histology slide are shown in Figures B and C. What is the most likely translocation and fusion protein associated with this condition? QID: 4582 FIGURES: A B C Type & Select Correct Answer 1 t (x:18), EWS-FL1 3% (126/4051) 2 t (2:13), PAK3-FKHR 2% (74/4051) 3 t (11: 22), EWS-FL1 82% (3335/4051) 4 t (11: 22), EWS-CHN 10% (396/4051) 5 t (9: 22), EWS-CHN 2% (89/4051) L 2 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 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 (OBQ10.194) A 15-year-old cross country runner complains of vague shin pain. A radiograph of the tibia is seen in Figure A. A biopsy is performed and is shown in Figure B. Which of the following chromosomal translocations is most likely associated with this condition? QID: 3286 FIGURES: A B Type & Select Correct Answer 1 t(10;20) 1% (33/4085) 2 t(11;22) 87% (3565/4085) 3 t(X;18) 4% (166/4085) 4 t(9;22) 6% (261/4085) 5 t(2;13) 1% (40/4085) L 1 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (OBQ10.171) Each of the following neoplasms demonstrate round cells when examined histologically EXCEPT QID: 3264 Type & Select Correct Answer 1 Embryonal rhabdomyosarcoma 10% (264/2625) 2 Primitive neuroectodermal tumors (PNET) 7% (183/2625) 3 Ewings sarcoma 2% (63/2625) 4 Neuroblastoma 5% (138/2625) 5 Leiomyosarcoma 75% (1967/2625) L 2 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 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 (OBQ08.53) For which of the following patients would a bone marrow biopsy be indicated during tumor staging? QID: 439 Type & Select Correct Answer 1 15-year-old girl with bone forming distal femoral lesion on radiographs 11% (235/2093) 2 7-year-old boy with lipomatous lesion in his proximal thigh 4% (93/2093) 3 3-year-old boy with pituitary intracranial mass 8% (165/2093) 4 5-year-old girl with lytic diaphyseal femur lesion positive for the EWS-FLI1 transgene 66% (1372/2093) 5 12-year-old boy with shepard's crook deformity of his proximal femur and deactivating mutation in the G-beta subunit of the G-protein coupled receptor. 10% (212/2093) L 3 Question Complexity A Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 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 (OBQ06.65) An 8-year-old girl comes to the office with increasing thigh pain mostly at night. Radiograph and biopsy specimen are shown in Figures A and B. The patients WBC and ESR are both elevated. What is the most likely diagnosis? QID: 176 FIGURES: A B Type & Select Correct Answer 1 Ewing's sarcoma 82% (1597/1949) 2 Osteosarcoma 8% (165/1949) 3 Osteomyelitis 8% (147/1949) 4 Giant cell tumor 0% (9/1949) 5 Chondrosarcoma 1% (11/1949) L 2 Question Complexity A Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review Tested Concept Review Full Topic (OBQ06.161) A 13-year old female complains of constant, dull right knee pain for the past several months. The pain is worse at night and with walking. She denies weight loss or fatigue. An AP and lateral radiograph are shown in Figures A and B, respectively. She was found to have a bony lesion in the distal femur which was biopsied at an outside institution which is shown in Figure C. What is the genetic translocation with this type of condition? QID: 347 FIGURES: A B C Type & Select Correct Answer 1 t (9; 22) 5% (86/1847) 2 t (11;22) 87% (1610/1847) 3 t (12;16) 3% (56/1847) 4 t (X;18) 3% (55/1847) 5 t (12;22) 2% (29/1847) L 1 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (OBQ06.214) After tumor staging, what is the most appropriate treatment for a 17-year-old male with stage IIb Ewing's sarcoma in his proximal tibia? QID: 225 Type & Select Correct Answer 1 Neoadjuvant radiotherapy, marginal surgical resection, adjuvant chemotherapy 3% (59/1892) 2 Neoadjuvant chemotherapy, marginal surgical resection, adjuvant radiotherapy 6% (113/1892) 3 Neoadjuvant chemotherapy, wide surgical resection, adjuvant chemotherapy 83% (1569/1892) 4 Wide surgical excision and reconstruction 5% (103/1892) 5 Radiotherapy and chemotherapy without surgery 2% (35/1892) L 1 Question Complexity A Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic (OBQ06.239) Which of the following tests is required for a standard work-up of Ewing's sarcoma that is not routinely obtained for staging of osteosarcoma? QID: 250 Type & Select Correct Answer 1 MRI 3% (79/2456) 2 CT scan 3% (71/2456) 3 Bone scan 7% (162/2456) 4 Protein electrophoresis 12% (292/2456) 5 Bone marrow biopsy 75% (1831/2456) L 2 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 (OBQ04.116) What is the appropriate treatment for a 10-year-old boy with Ewing's sarcoma isolated to the proximal femur? QID: 1221 Type & Select Correct Answer 1 Neoadjuvant chemotherapy and surgical excision 7% (46/658) 2 Neoadjuvant chemotherapy, surgical excision, and radiation therapy 18% (121/658) 3 Neoadjuvant chemotherapy, surgical excision, and adjuvant chemotherapy 69% (453/658) 4 Neoadjuvant radiation therapy and surgical excision 4% (24/658) 5 Surgical excision and hormonal therapy 0% (1/658) L 2 Question Complexity A Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic
FREE PDF Current Orthopaedic Practice: November/December 2016 - Volume 27 - Issue 6 - p 582–586 Bone sarcomas: an update of the recent literature Jones LB Barr JS Pathology - Ewing's Sarcoma Jones LB, 4 views 0.0
All Videos (6) Podcasts (1) Login to View Community Videos Login to View Community Videos Ewing’s sarcoma-Primitive Neuro-Ectodermal Tumor (PNET) Kemal Gokkus Pathology - Ewing's Sarcoma A 1/17/2021 120 views 5.0 (1) Login to View Community Videos Login to View Community Videos oncology Orthopedic Oncology Course- Small Round Blue Cell Tumors (Ewing Sarcoma, Lymphoma) - Lecture 8 James C. Wittig Pathology - Ewing's Sarcoma E 12/31/2016 662 views 5.0 (4) Login to View Community Videos Login to View Community Videos Total Scapula Prosthesis Ewings Sarcoma - Dr. Wittig - Part 4 Derek W. Moore Pathology - Ewing's Sarcoma E 8/18/2012 315 views 5.0 (3) Pathology | Ewing's Sarcoma Pathology - Ewing's Sarcoma Listen Now 17:46 min 10/16/2019 499 plays 5.0 (7) See More See Less
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