Primary Ewing's sarcoma of the sinonasal tract in adults: A challenging disease

Lombardi, Davide; Mattavelli, Davide; Zinis, Luca Oscar Redaelli de; Accorona, Remo; Morassi, Maria Laura; Facchetti, Fabio; Ferrari, Vittorio; Farina, Davide; Bertulli, Rossella; Nicolai, Piero

doi:10.1002/hed.24649

Cited by 9 publications

(6 citation statements)

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“…Stavrakas [ 2 ] also noted higher 60-month survival rates for patients treated with adjuvant therapy vs. surgery alone. In their study of 5 patients with sinonasal Ewing’s sarcoma Lombardi et al [ 185 ] noted only one death, due to distant metastases, with all of the 5 patients being treated with multimodality treatment.…”

Section: Discussionmentioning

confidence: 99%

Treatment, outcomes, and demographics in sinonasal sarcoma: a systematic review of the literature

Gore

2018

BMC Ear Nose Throat Disord

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BackgroundSarcomas comprise a diverse group of soft tissue mesenchymal malignancies. The sinuses and nasal region are a relatively rare site of sarcomas.MethodsRetrospective review of the literature on sinonasal sarcomas from 1987-2017. Data were analyzed for demographics, treatment type, stage, and histopathologic type. Kaplan-Meier analysis was used to assess and compare survival.ResultsA total of 198 cases of sinonasal sarcoma were identified and analyzed. The median age at diagnosis was 39 years. Overall 5-, 10-, and 20-year survival was 61.3%, 58.9%, and 49.1%, respectively, and disease-free 5-, 10-, and 20-year survival was 53.2%, 49.1%, and 38.3%, respectively. Lymph node metastasis was present at diagnosis in 3.0% of cases, and distant metastasis was present in 3.5% of cases. On univariate analysis T stage, overall stage, treatment type, histopathologic subtype, and presence of distant metastasis significantly affected survival. On multivariate analysis overall stage alone significantly predicted overall survival. Open vs. endoscopic surgery, total radiation dose, and presence of neck metastasis did not significantly affect survival. Combined modality treatment was associated with higher survival rates than single modality therapy.ConclusionsSinonasal sarcoma is a relatively rare malignancy. Lower T and overall stage, lack of distant metastasis, and multimodality therapy were associated with improved survival. Certain histopathologic subtypes were associated with poorer survival.Electronic supplementary materialThe online version of this article (10.1186/s12901-018-0052-5) contains supplementary material, which is available to authorized users.

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Section: Discussionmentioning

confidence: 99%

Treatment, outcomes, and demographics in sinonasal sarcoma: a systematic review of the literature

Gore

2018

BMC Ear Nose Throat Disord

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“…The majority of sinonasal malignancies, vastly 3% of all head and neck cancers, are of epithelial origin . Only a handful of investigations provide data concerning the epidemiology and the prognosis of malignant neoplasms of mesenchymal origin, mainly owing to the overall rarity of this entity of disease . In addition to that, the majority of retrospective single‐institution cohort studies focus on soft tissue sarcomas of the sinonasal tract .…”

Section: Discussionmentioning

confidence: 99%

“…A vast heterogeneity is innate to this entity of disease, rendering sinonasal sarcomas a therapeutic and diagnostic challenge . In this context, sinonasal sarcomas can be subclassified in accordance with their differential biological, clinical, and prognostic behavior, as well as their response to specific treatment modalities . Importantly, patient age plays a crucial role in therapeutic decision making, as there are biologically distinct features between sarcomas in infants and adults .…”

Section: Introductionmentioning

confidence: 99%

Sarcomas of the sinonasal tract

et al. 2018

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“…Chemotherapy is considered to be ineffective for most sinonasal non-RMS. However, Ewing sarcoma/PNET is sensitive to chemotherapy and systemic chemotherapy plays a pivotal role in its treatment [ 7 ]. In our study, objective responses were observed in 2 patients with Ewing sarcoma/PNET treated by chemotherapy with the VAC regimen.…”

Section: Discussionmentioning

confidence: 99%

“…Sinonasal teratocarcinosarcoma is an extremely rare and highly aggressive malignant neoplasm, with complex components of mesenchymal, epithelial, and neuroectodermal elements; it is not easy to forecast its trend of development and prognosis owing to its uncertain clinical behavior [ 6 ]. Other histological tumor types reported in the sinonasal tract include Ewing’s sarcoma/PNET, malignant fibrous histiocytoma, chondrosarcoma and osteosarcoma, fibrosarcomas, leiomyosarcomas, and synovial sarcoma [ 7 – 11 ].…”

Section: Introductionmentioning

confidence: 99%

Treatment Outcomes and Prognostic Factors of Adult Sinonasal Sarcomas: A Single-Institution Case Series

et al. 2018

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BackgroundThe purpose of this study was to investigate the treatment outcomes and evaluate the prognostic factors of adult sinonasal sarcomas.Material/MethodsA retrospective review was performed on consecutive patients with adult sinonasal sarcomas treated in our institution from 2005 to 2016. The Kaplan-Meier method was used to evaluate local recurrence-free survival (LRFS), distant metastases-free survival (DMFS), and overall survival (OS). Univariate and multivariate analyses using Cox proportional hazard models were performed to determine the prognostic factors associated with survival outcomes.ResultsA total of 49 patients were followed up for 6–122 months, with a median time of 36 months. The 5-year LRFS, DMFS, and OS rates of all patients were 68.3%, 62.8%, and 43.2%, respectively. The results of univariate analysis revealed that patients with an advanced stage of primary tumor and those who received incomplete surgical resection had worse LRFS (p=0.013; p=0.026). Patients with the histological type rhabdomyosarcoma (RMS) and existing regional lymph node metastasis had worse DMFS (p=0.000; p=0.001). The histological type RMS, advanced stage of primary tumor, existing regional lymph node metastasis, and receiving incomplete surgical resection had an unfavorable effect on OS (p=0.001; p=0.002; p=0.008; p=0.011). The results of multivariate analysis showed that histological type and degree of surgical resection were the independent prognostic factors for OS.ConclusionsOur results suggest that the histological type RMS and receiving incomplete surgical resection are independent prognostic factors for worse OS.

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Primary Ewing's sarcoma of the sinonasal tract in adults: A challenging disease

Cited by 9 publications

References 40 publications

Treatment, outcomes, and demographics in sinonasal sarcoma: a systematic review of the literature

Treatment, outcomes, and demographics in sinonasal sarcoma: a systematic review of the literature

Sarcomas of the sinonasal tract

Treatment Outcomes and Prognostic Factors of Adult Sinonasal Sarcomas: A Single-Institution Case Series

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