Sarcoma de Ewing primário do rim com trombo na veia cava inferior com extensão à aurícula direita: caso clínico e revisão da literatura

Joana Alfarelos , Vanessa Figueiredo , Gustavo Gomes , Mário Matias , Amaral Canelas
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Abstract

The Ewing's family of tumours comprises a spectrum of malignancies of primitive neuroectodermal cells: embryonic cells that migrate from the neural crest. 1Primary kidney Ewing's sarcoma is a rare neoplastic disease representing less than 1% of renal tumors and is characterized by highly aggressive biological behavior.

We reported a case of an asymptomatic, 53 year old man with an incidental lesion in the right kidney found in a routine renal ultrasound. Computed tomography showed a solid nodule with 67 x 65 mm, occupying the lower pole of the right kidney with renal vein, inferior vena cava and right auricular invasion. The patient underwent a right radical nephrectomy and Inferior vena cava and atrial tumor thrombectomy with cardiopulmonary bypass and deep hypothermic circulatory arrest. Pathologic characteristics and immunohistochemical analysis confirmed the diagnosis of Ewing's Sarcoma. Surgery and early post‐operative were free of complications. The patient underwent chemotherapy and died 21 months after the surgery of relapse of the disease.

原发性肾尤文氏肉瘤伴下腔静脉血栓延伸至右心房:临床病例及文献综述
尤因氏肿瘤家族包括一系列原始神经外胚层细胞的恶性肿瘤:从神经嵴迁移的胚胎细胞。原发性肾脏尤文氏肉瘤是一种罕见的肿瘤疾病,占肾脏肿瘤的不到1%,其特点是具有高度侵袭性的生物学行为。我们报告一例无症状的53岁男性,在常规肾脏超声检查中发现右肾偶发病变。ct示实性结节,大小67 x 65 mm,侵占右肾下极,伴肾静脉、下腔静脉及右耳侵犯。患者行右侧根治性肾切除术、下腔静脉及心房肿瘤取栓术,同时行体外循环和深部低温循环停搏。病理特征和免疫组化分析证实了Ewing肉瘤的诊断。手术及术后早期无并发症。患者接受化疗,术后21个月因病情复发死亡。
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