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Sarcomatoid renal cell carcinoma: a case report and literature review

机译:肉瘤样肾细胞癌1例并文献复习

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The poorly differentiated renal cell carcinoma (RCC) with rhabdomyosarcomatous sarcomatoid differentiation shows a severely aggressive biological behavior characterized by rapid disease progression. Preoperative identification of the subtype with the prognostic factors and imaging features of sarcomatoid renal cell carcinoma (SRCC) would be of great clinical significance. A 45-year-old male patient presented a nine day history of gross hematuria without any other symptoms. A computed tomography (CT) and a full-body fluorine-18 fluoro-2-deoxyglucose (FDG) positron emission tomography (PET) - computed tomography (CT) scan urogram were performed. An initial diagnosis identified a space-occupying lesion of the right kidney, retroperitoneal and right renal hulum lymph node metastases, as well as a space-occupying lesion of the third thoracic vertebra (T3). A right radical nephrectomy was performed. Pathologic analysis revealed poorly differentiated RCC with rhabdomyosarcomatous sarcomatoid differentiation that extends into the renal sinus and the ureteral (T3N1M1). Five days later, the Magnetic Resonance imaging (MRI) evidenced a diffused osseous metastatic disease in the thoracic and lumbar vertebra and multiple retroperitoneal lymph node metastases. The disease progressed quickly to multiple organ dysfunction syndrome (MODS) in half a month and the patient died of respiratory failure two days later. The patient refused any chemoradiotherapy in the hospital. Our case presents a SRCC with severe, aggressive, and rapid disease progression. Classifying SRCC imaging features by CT, MRI as well as PET-CT techniques could potentially be helpful for preoperative identification of the subtype. The prognostic factors of SRCC would be of great clinical interest.
机译:具有横纹肌肉瘤肉瘤样分化的低分化肾细胞癌(RCC)显示出以疾病快速发展为特征的严重侵袭性生物学行为。术前确定亚型的肉瘤样肾细胞癌(SRCC)的预后因素和影像学特征将具有重要的临床意义。一名45岁的男性患者有9天的肉眼血尿病史,没有任何其他症状。进行了计算机断层扫描(CT)和全身氟18氟-2-脱氧葡萄糖(FDG)正电子发射断层扫描(PET)-计算机断层扫描(CT)扫描尿管造影。初步诊断确定了右肾的占位性病变,腹膜后和右肾的hulum淋巴结转移以及第三胸椎(T3)的占位性病变。进行了右根肾切除术。病理分析显示,RCC分化较差,横纹肌肉瘤样肉瘤样样分化延伸到肾窦和输尿管(T3N1M1)。五天后,磁共振成像(MRI)证实了在胸椎和腰椎中存在弥漫性骨转移性疾病,并发生了多个腹膜后淋巴结转移。该病在半个月内迅速发展为多器官功能障碍综合症(MODS),患者在两天后死于呼吸衰竭。病人拒绝在医院进行任何放化疗。我们的病例显示了具有严重,侵袭性和快速疾病进展的SRCC。通过CT,MRI和PET-CT技术对SRCC影像学特征进行分类可能会有助于术前识别亚型。 SRCC的预后因素将具有重大的临床意义。

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