Renal Ewing sarcoma with extensive neuroectodermal differentiation: Case report and literature review

Q4 Pharmacology, Toxicology and Pharmaceutics
Alexandra Daniela Sava, Tiberiu Bogdan Szekely, Cornelia Togănel, Adela Vacar, Catalina Bungardean, Simona Gurzu
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Abstract

Abstract Ewing sarcoma with renal localization is one of the rarest members of the Ewing sarcoma family with less than 200 cases reported in the Medline database. Considering the fact that the majority of data published on the Ewing sarcoma with neuroectodermal differentiation is obtained through a few case reports and case series, it becomes understandable why we currently have no universally accepted treatment regimens. Case summary : A 33-year-old patient presented to the Emergency Department with right lumbar pain following a mild trauma and an episode of macroscopic hematuria. Physical examination confirmed hematuria and flank pain and a palpable flank mass was identified. MRI showed a cystic lesion of the upper pole of the right kidney of 127/110/123 mm. After prior agreement of the multidisciplinary team, a 3D laparoscopic right radical nephrectomy was done. The histopathological diagnosis revealed an Ewing sarcoma with extensive neuroectodermal differentiation staged as pT3N1M0L1V2R0. Despite swift implementation of the chemotherapy protocol, the progression of the disease was quickly noted. Currently, one year after diagnosis, the disease is still progressing despite the chemotherapy treatment, the patient being a third line chemotherapy candidate. As renal localization of Ewing sarcoma with extensive neuroectodermal differentiation is extremely rare, multimodal treatment strategies must be established by a multidisciplinary team. Despite its aggressive biological behavior, a proper therapeutic management might increase patient life expectancy.
肾尤文氏肉瘤伴广泛神经外胚层分化:1例报告及文献复习
Ewing肉瘤伴肾脏定位是Ewing肉瘤家族中最罕见的成员之一,在Medline数据库中报道的病例不到200例。考虑到大多数发表的关于神经外胚层分化的尤文氏肉瘤的数据是通过少数病例报告和病例系列获得的,这就可以理解为什么我们目前没有普遍接受的治疗方案。病例总结:一名33岁的患者在轻度外伤和肉眼血尿发作后出现右腰椎疼痛,来到急诊科。体格检查证实有血尿、腹部疼痛和可触及的腹部肿块。MRI示右肾上极囊性病变127/110/123 mm。经多学科小组事先同意后,进行了三维腹腔镜右侧根治性肾切除术。组织病理学诊断为伴有广泛神经外胚层分化的尤文氏肉瘤,分期为pT3N1M0L1V2R0。尽管化疗方案迅速实施,但疾病的进展很快就被注意到了。目前,在诊断一年后,尽管进行了化疗,但疾病仍在进展,患者是三线化疗候选者。由于尤因肉瘤肾脏定位并广泛神经外胚层分化极为罕见,因此必须由多学科团队建立多模式治疗策略。尽管其具有侵略性的生物学行为,但适当的治疗管理可能会增加患者的预期寿命。
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来源期刊
Acta Marisiensis - Seria Medica
Acta Marisiensis - Seria Medica Pharmacology, Toxicology and Pharmaceutics-Pharmacology, Toxicology and Pharmaceutics (all)
CiteScore
0.40
自引率
0.00%
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0
审稿时长
24 weeks
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