Ewing's Sarcoma: A Rarity in Sinonasal Region

Cruz, Sara; Azevedo, Paula S.; Trigueiros, Nuno; Rodrigues, Manuel Rodrigues e

doi:10.1016/j.otoeng.2014.02.015

Cited by 4 publications

(1 citation statement)

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“…It should be noted that primary lung neoplasms are infrequent in children, they are usually secondary lesions or metastases of malignant solid tumors typical of childhood, and their clinical manifestations are initially confused with an infectious process. [2,5] There are few case reports of this tumor variant in early life. (Izaguirre,et al [3]) reported a 13-year-old patient with Ewing's sarcoma of the left fibula, but at such a young age as in our case it is extremely rare.…”

Section: Discussionmentioning

confidence: 99%

Untitled

2022

BJSTR

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Ewing's sarcoma is a malignant mesenchymal tumor, extremely rare in patients under 10 years of age, being more frequent from the second decade of life. Its annual incidence is low, 1 to 3 cases per year per million children under 15 years of age. We present a case of a patient of only two years of age with stage IV disease at the time of diagnosis, highlighting the value of multidisciplinary teamwork where imaging studies and pathology immunohistochemical techniques play a fundamental role in the diagnosis of the entity.

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Section: Discussionmentioning

confidence: 99%

Untitled

2022

BJSTR

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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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