A case of primary mediastinal Ewing′s sarcoma /primitive neuroectodermal tumor presenting with initial compression of superior vena cava

Reali, Alessia; Mortellaro, Gianluca; Allis, Simona; Trevisiol, Edoardo; Anglesio, S.; Bartoncini, Sara; Redda, Maria Grazia Ruo

doi:10.4103/1817-1737.109834

Cited by 18 publications

(7 citation statements)

References 10 publications

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“…As stated above for chondro-osseous tumours, an origin from bony structures such as vertebrae and chest wall must be excluded in order to qualify as primary mediastinal eES/PNET, leading to the exclusion of several reported cases where tumours extended to these structures or where insufficient details were given [ 112 , 231 ]. Less than 20 convincing cases of eES/PNET have been reported in the mediastinum [ 3 , 50 , 74 , 75 , 100 , 102 , 119 , 153 , 164 , 169 , 178 , 231 ]. The cases mostly occurred in young individuals (median age 28 years, range 5–66 years) with equal gender distribution and in all compartments of the mediastinum.…”

Section: Chondro-osseous Tumoursmentioning

confidence: 99%

Mesenchymal tumours of the mediastinum—part II

et al. 2015

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Section: Chondro-osseous Tumoursmentioning

confidence: 99%

Mesenchymal tumours of the mediastinum—part II

et al. 2015

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“…The thoracic sites of EES include the chest wall, trachea, spinal epidural space, paraspinal area and mediastinum ( 7 – 11 ). Primary mediastinal EES/PNET is extremely rare ( 1 , 12 ); to the best of our knowledge, only five such cases have previously been reported ( 1 , 11 – 14 ). The present case will therefore aid in expanding our understanding of this distinct neoplasm.…”

Section: Discussionmentioning

confidence: 86%

“…However, complete resection plus radiotherapy is known to have an advantage over radiotherapy. For unresectable cases or tumors that cannot be removed completely, radiotherapy with a radical dose of 50–60 Gy should be performed ( 1 ).…”

Section: Discussionmentioning

confidence: 99%

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Extraskeletal Ewing’s sarcoma/primitive neuroectodermal tumor of the mediastinum: Significant response to chemoradiotherapy

Liu

Dong

et al. 2014

Oncology Letters

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Primary mediastinal extraskeletal Ewing’s sarcoma (EES) is quite rare. To the best of our knowledge, only five cases have been reported. Given the paucity of data, there is consequently no optimal treatment strategy available. The current study presents the case of a 51-year-old female with unresectable EES of the posterior mediastinum. Chemoradiotherapy achieved near complete remission without severe side-effects. The literature associated with EES is also reviewed. The present case highlights the possibility of the diagnosis of EES for a mediastinal mass. Chemoradiotherapy may be a good option for unresectable cases. In the future, large-scale collaborative clinical trials should be initiated to provide an improved understanding of the characteristics of EES and the best treatment strategy.

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“…among the EES are those of posterior mediastinum [2]. These are aggressive tumors with a high incidence of distant metastasis and recurrence after treatment [3]. We present a case of metastatic EES of posterior mediastinum in a 20-year-old boy presenting with sudden onset gradually progressive sensorimotor paraplegia.…”

mentioning

confidence: 96%

Posterior mediastinal Ewing sarcoma with multiple metastases on FDG PET/CT: a rare entity

Singh

Verma

Thakur

et al. 2023

Egypt J Radiol Nucl Med

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Background Ewing sarcoma and peripheral primary neuroendocrine tumors are aggressive neoplasms which consist of small, round, blue cells of neuroectodermal origin. They usually arise from the skeleton and consist of genetic mutations EWSR1 in chromosome 22 and FL1 gene on chromosome 11. Extraskeletal Ewing sarcomas (EES) are rare entities with most common sites of EES being extremities, head and neck region and retroperitoneum. Posterior mediastinal Ewing sarcoma is rare. For its evaluation, 18F-fluorodeoxyglucose positron emission tomography (18F FDG PET/CT) plays significant role in staging, management planning and prognostication. Case presentation We describe a rare case of EES of posterior mediastinum in a 20-year-old boy who presented with signs of upper motor neuron lesion below D10 level. Contrast-enhanced magnetic resonance imaging (CEMRI) showed a heterogeneously enhancing posterior mediastinal mass in pre- and paravertebral region with intraspinal extension in D2-D4 levels. Fluorodeoxyglucose PET/CT showed a metabolically active mass occupying the superior, middle and posterior mediastinum on the left, displacing the trachea and esophagus toward the right side and causing complete collapse of the left lung. Posteriorly the mass was seen destroying the D2-D5 vertebrae with intraspinal extension at D2-D4 level. Metabolically active metastatic disease was seen in pleura, skull, D12 vertebra, right iliac bone and bilateral proximal femorae. Biopsy obtained from lung and adjacent pleura showed features of a round cell tumor positive for NKX 2.2, weak positive for FLI 1 and negative for PAN CK, LCA, Vimentin and TLE-1, suggestive of Ewing sarcoma. Based on these investigations, a diagnosis of EES of posterior mediastinum was made. Conclusion Extraskeletal Ewing sarcoma of posterior mediastinum is a rare and aggressive entity. Management of metastatic EES comprises radiotherapy and systemic chemotherapy which reduces tumor burden and micrometastasis. However, response to treatment in metastatic EES is poorer than in localized disease with overall 5-year survival rates of less than 30%. Fluorodeoxyglucose PET/CT can be a useful tool to accurately detect the extent of local disease in the presence of atelectasic lung for radiotherapy planning as well as evaluating response to therapy.

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A case of primary mediastinal Ewing′s sarcoma /primitive neuroectodermal tumor presenting with initial compression of superior vena cava

Cited by 18 publications

References 10 publications

Mesenchymal tumours of the mediastinum—part II

Mesenchymal tumours of the mediastinum—part II

Extraskeletal Ewing’s sarcoma/primitive neuroectodermal tumor of the mediastinum: Significant response to chemoradiotherapy

Posterior mediastinal Ewing sarcoma with multiple metastases on FDG PET/CT: a rare entity

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