Consensus recommendations in the management of Ewing sarcoma from the National Ewing Sarcoma Tumor Board
Ajay GuptaRichard F. RiedelChirag ShahScott C. BorinsteinMichael S. IsakoffRashmi ChughJeremy RosenblumErin S. MurphyShauna R. CampbellCatherine M. AlbertStacey ZahlerStefanie M. ThomasMatteo Trucco
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Abstract Ewing sarcoma (ES) is a malignant tumor of bone and soft tissue that most often occurs in adolescents and young adults. Despite an international coordinated approach, several nuances, discrepancies, and debates remain in defining the standard of care for treating ES. In this review, the authors leverage the expertise assembled by formation of the National Ewing Sarcoma Tumor Board, a multi‐institution, multidisciplinary virtual tumor board that meets monthly to discuss complicated and challenging cases of ES. This report is focused on select topics that apply to the management of patients with newly diagnosed ES. The specific topics covered include indications for bone marrow aspirate and biopsy for initial evaluation compared with fluorodeoxyglucose‐positron emission tomography, the role of interval compressed chemotherapy in patients aged 18 years and older, the role of adding ifosfamide/etoposide to vincristine/doxorubicin/cyclophosphamide for patients with metastatic disease, the data on and role of high‐dose chemotherapy with autologous stem cell transplantation, maintenance therapy, and whole‐lung irradiation. The data referenced are often limited to subgroup analyses and/or compiled from multiple sources. Although not intended to replace the clinical judgement of treating physicians, the guidelines are intended to provide clarity and recommendations for the upfront management of patients with ES. Plain Language Summary Ewing sarcoma is a malignant tumor of bone and soft tissue that most often occurs in adolescents and young adults. For this review, the authors used the experience of the National Ewing Sarcoma Tumor Board, a multi‐institution, multidisciplinary virtual tumor board that meets monthly to discuss complicated and challenging cases of Ewing sarcoma. Although not intended to replace the clinical judgement of treating physicians, the guidelines will focus on the development of consensus statements for the upfront management of patients with Ewing sarcoma.Keywords:
Ewing's sarcoma
Bone Sarcoma
Ewing's sarcoma, a highly malignant bone tumor, typically affects the pelvis and long bones of the lower extremities in children and young adults; primary involvement of the skull is rare. Primary Ewing's sarcoma arising from the skull is very rare. In most cases, this disease is fatal, although the prognosis of Ewing sarcoma improves with radiation and chemotherapy after surgery.
Ewing's sarcoma
Primary bone
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Ewing Sarcoma is the second most common primary bone cancer. To date, only a few cases of primary cutaneous Ewing Sarcoma have been published. We present a unique case of primary cutaneous Ewing Sarcoma, which presented as a blue to violaceous mass on a young woman’s chest. The lesion clinically did not align with previous reports of primary cutaneous Ewing Sarcoma but pathology and immunohistochemical stains confirmed the diagnosis. Because primary cutaneous Ewing Sarcoma may masquerade as benign tumors, dermatologists must be suspicious of otherwise benign looking tumors.
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Ewing’s sarcoma principally arises in bone but can also present as a soft tissue tumour. Very few studies have assessed the outcomes of extra-skeletal Ewing’s sarcomas. This study compares the oncological outcomes of the two forms of Ewing’s sarcomas to see if there is any difference in prognostic factors. 198 patients with primary, non metastatic Ewing’s sarcoma diagnosed between 1980 and 2005 were identified from our database. There were 118 males and 80 females with a median age of 15 years. The three most common sites of diagnosis were the femur (24%), pelvis (15%) and tibia (13%). There were 169(85%) bony Ewing’s and 29 (15%) extra-skeletal Ewing’s sarcomas. All patients received chemotherapy. 86% of the patients had surgery for local control but 28(14%) patients had radiotherapy. The overall survival at five years was 89% and was related to the age of patient (92% This large series has shown that the oncological outcomes of Ewing’s sarcoma is related to tumour characteristics, patient age and treatment factors and not determined by the tissue component.
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Bone Sarcoma
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Ewing's sarcoma of bone and osteosarcoma are rare tumors. A combination of high-grade osteosarcoma and Ewing's sarcoma of bone in anatomically unrelated sites is unique, especially in the absence of previous radiation or retinoblastoma. We present a patient with a rare case of Ewing's sarcoma of the scapula that showed no evidence of recurrence (after 10 years of continued followup) and who subsequently presented with a primary osteosarcoma of the femur.
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Primary bone
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Paraffin sections from 6 cases of Ewing's sarcoma were immunostained by ABC method using BMP—McAb for investigating the existence of BMP in Ewing's sarcoma. All cases were positive. The result was coincided with human tumor transplants in athymic nude mouse by Bauer. It is shown that Ewing's sarcoma can express BMP. So we support the hypothesis that Ewing's sarcoma originates from primitive multipotential cells and can pluripotentially differentiate.
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Human bone
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Advances in the treatment of Ewing's sarcoma have been dramatic. Present treatment protocols control local disease by radiotherapy, surgery, or both; systemic spread is limited by aggressive multi agent chemotherapy. In patients with localized osseous Ewing's sarcoma, five-year survival rates now range from 54% to an estimated 74%. With late relapse not uncommon, control of the primary lesion is critical to long-term survival. Several studies now show improved local control and possibly improved survival of patients with surgical treatment of primary osseous Ewing's sarcoma.
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Ewing's sarcoma is one of the most aggressive primary bone tumours. Ewing's sarcoma arising from the bones of the head and neck region is extremely rare; only 4-9% of all Ewing's sarcoma originate in this region. We report a case of Ewing's sarcoma localized in the mandible because of its unusual presentation.
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Mandible (arthropod mouthpart)
Presentation (obstetrics)
Primary bone
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Primary Ewing's sarcoma of the spine is sufficiently rare to justify the reporting of an additional case. The roentgenographic changes in that location obviously are not those typical of similar involvement of the long bones. A survey of the pertinent literature reveals 10 cases of primary Ewing's sarcoma of the spine. In a review of the material in the Bone Sarcoma Registry (1927) and a comprehensive discussion of sarcoma of the bone, Kolodny (1) states that Ewing's sarcoma constitutes 7.5 per cent of bone sarcomas. In his opinion, a Ewing's sarcoma usually affects more than one vertebra, but he believes that this represents multiple primary involvement and not metastatic growth. He reports a series of 650 cases of bone tumor with 40 instances of Ewing's sarcoma and 10 others of more or less doubtful nature. The spine is mentioned as being a favorite site of the tumor, but the incidence of such involvement is not given. Rix and Geschickter (2) report a series of 291 tumors of the spine, of which 21 were primary malignant neoplasms. They report no cases of Ewing's sarcoma but list 12 cases of sympathicoblastoma and discuss the possibility of it being confused with a Ewing's sarcoma. Geschickter and Copeland (3) report a series of 125 cases of Ewing's sarcoma and list one instance of primary involvement of the vertebra. Geschickter and Maseritz (4) discuss the clinical and microscopic similarity of neuroblastoma and Ewing's sarcoma and report a series of 135 cases of the latter tumor. No mention of primary involvement of the spine is made. Stewart Harrison (5) reports 8 cases of Ewing's bone sarcoma, none of which was primary in the spine. Lattman (6) published a review on Ewing's sarcoma but did not mention primary occurrence in the spine. He states that primary malignant disease of the bone is rare (1.5 to 2.0 per cent of all malignant neoplasms), with Ewing's sarcoma accounting for approximately 10 per cent of the bone sarcomas. Brav and Rechtman (7) reported a single case of a Ewing's sarcoma primary in the sacrum, with autopsy findings. Neely and Rogers (8) report two cases of primary bone neoplasm of the spine with a histopathological picture consistent with Ewing's sarcoma. These two cases showed no cell type compatible with a diagnosis of sympathicoblastoma. In a more recent study, Morton (9) reports 6 cases of Ewing's sarcoma of the spine as listed in the Bone Sarcoma Registry (covering the period up to the fall of 1936). A total of 184 cases of Ewing's sarcoma of bone were listed in the Registry for the same period. Case Report Clinical History: A white male, aged 21 years, was admitted to the Letterman General Hospital, March 21, 1944, for deep x-ray therapy to the 12th dorsal vertebra and the right 12th rib. The family history was irrelevant and the developmental history was normal. The patient had been well until September 1943, when he experienced a sudden sharp pain in his right hip.
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Primary bone
Bone Sarcoma
Vertebra
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Ewing's sarcoma is an aggressive bone and the surrounding soft tissue cancer primarily found in children and young adults. It is mostly observed in the trunk and long bones while unusually seen in the sacrum. Sacral Ewing sarcoma has a unique anatomic limitation due to neurological structures, vertebral column, and pelvic involvement. Hence, identifying sacral Ewing's sarcoma remains a challenge.This is a case series including three patients diagnosed with Sacral Ewing's sarcoma. These three cases have been diagnosed based on clinical and radiographic examination, MRI, histopathology, and immunohistochemistry examination.Sacral Ewing's sarcoma remains a challenge to diagnose due to its unique anatomy site. MRI examination is sensitive to detect lesions but nonspecific. Thus, require histopathological confirmation.Early diagnosis of Ewing's sarcoma is essential and we need to perform further examinations, such as immunohistochemistry examination, to confirm the diagnosis of Ewing's sarcoma.
Ewing's sarcoma
Histopathology
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Paraffin sections from'6 cases of Ewing's sarcoma were immunostained by ABC method using BMP -McAb for investigating the existence of BMP in Ewing's sarcoma. All cases were positive. The result was coincided with human tumor transplants in athymic nude mouse by Bauer. It is shown that Ewing's sarcoma can express BMP. So we support the hypothesis that Ewing's sarcoma originates from primitive multipotential cells and can pluripotentially differentiate.
Ewing's sarcoma
Human bone
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