Experience of CT diagnosis and management of primary renal Ewing’s sarcoma: A retrospective analysis of 6 cases and a literature review

Abstract

To present the clinical experience of primary renal Ewing's sarcoma/primitive neuroectodermal tumors (rEWs/PNET) admitted to our hospital and systematically review the published literature. A retrospective analysis was performed on patients with pathologically confirmed renal EWs/PNET (rEWs) in our hospital, and the literature on rEWs published in PubMed and Embase databases before March 1, 2022 was searched for analysis. A total of 337 rEWs were included in the statistical analysis, including 6 cases of our patients and 331 cases published in the literature. The common clinical symptoms of rEWs are abdominal pain, hematuria, abdominal mass and so on. computed tomography (CT) plays an important role in the diagnosis of rEWs, and the typical manifestation is a large heterogeneous soft tissue density mass, with a specific "septum-like" enhancement in contrast-enhanced scan. The 2-year overall survival rate of rEWs was 48%, with a median survival time of 18 months. "Septum-like" enhancement on CT can be used as a relatively specific sign for the differential diagnosis of rEWs from Wilms tumor and neuroblastoma. The maximum diameter of the rEWs was usually greater than 10 cm, the clinical symptoms of weight loss, metastasis at diagnosis, tumor thrombogenesis of renal vein or/and inferior vena cava tumor, and the failure to undergo radical nephrectomy were the factors of poor prognosis. The incidence of primary rEWs is low and the prognosis is poor. Early diagnosis and radical nephrectomy combined with chemotherapy is the key to improve the prognosis of patients, and CT plays an important role in early diagnosis.

Primary Ewing sarcoma of the kidney mimicking cystic papillary renal cell carcinoma in an older patient: A case report

“…The mean overall survival (OS) was 18.3 months, and the median OS was 16 months. This was shorter than the median OS of primary renal ES treated with radical nephrectomy of 27.4 months in a previous study[ 8 ]. This suggests that primary renal ES in older people has a worse prognosis, and that age is a significant prognostic factor for primary renal ES.…”

“…Tumors are common in young children and adolescents. The average age at diagnosis was 30.7 years with a male to female ratio of 6:4[ 8 ]. Limited number of cases of primary renal ES with an aggressive clinical course has been reported in the English literature.…”

“…Among the tumors, 95.9%, 32.7%, and 11.6% showed necrosis, intratumoral hemorrhage, and parenchymal calcification, respectively. In contrast, mild and progressive enhancement with invasion of the perirenal fascia (47.2%) and renal sinuses (67.9%) has been noted[ 8 ]. The radiological findings of PNET are similar to those of ES.…”

“…ES lacks specific radiological findings; however, common features include large necrotic masses and intratumoral hemorrhage. Contrast-enhanced imaging reveal a mild and progressive enhancement pattern frequently associated with invasion of the perirenal fascia and renal sinuses[ 8 ]. Histological characteristics that confirm positive CD99 expression and the existence of a reciprocal translocation between the ES gene on chromosome 22 and members of the ETS family, most commonly friend leukemia integration 1 transcription factor (FLI1) or ERG, are typically used to confirm tissue diagnosis[ 6 ].…”

Fluoro-18-fluorodeoxyglucose positron emission tomography/computed tomography detects Ewing’s sarcoma of the larynx with multiple distant bone metastases: a case report and literature review

“…According to the World Health Organization's 5th classification of bone and soft-tissue tumors in 2020, EWS of bone, primitive neuroectodermal tumors, extraskeletal EWS, and Askin's tumors are referred to collectively as the EWS family; extraskeletal EWS are rare, accounting for only 15%-20% of the EWS family (2). The most common extraskeletal EWS affect the paravertebral soft tissue, lung, stomach, kidney, and bladder; the larynx is a rare site of origin (2)(3)(4)(5). The clinical features of laryngeal EWS are associated with tumor progression, but no clinical manifestation is obvious when the tumor is small.…”