Lin Wei-Ching, Chen Jeon-Hor, Westphalen Antonio, Chang Han, Chiang I-Ping, Chen Cheng-Hong, Wu Hsi-Chin, Lin Chien-Heng
From the Department of Radiology (W-CL, C-HC), China Medical University Hospital; School of Medicine (W-CL, H-CW), China Medical University; Department of Biomedical Imaging and Radiological Science (W-CL, C-HL), College of Health Care, China Medical University, Taichung; E-Da Hospital and I-Shou University (J-HC), Kaohsiung, Taiwan (ROC.); Center for Functional Onco-Imaging (J-HC), School of Medicine, University of California, Irvine, CA; Departments of Radiology and Biomedical Imaging, and Urology (AW), University of California, San Francisco, CA; Department of Pathology (HC, I-PC), China Medical University Hospital; Department of Urology (H-CW), China Medical University Hospital; and China Medical University Children's Hospital (C-HL), Taichung, Taiwan (ROC).
Medicine (Baltimore). 2016 May;95(21):e3771. doi: 10.1097/MD.0000000000003771.
Although the second peak of the age distribution of rhabdomyosarcoma (RMS) is at adolescence, renal RMS is extremely rare at this age group. This tumor is indistinguishable from other renal tumors based on clinical and imaging findings, and the diagnosis relies on histology and immunohistochemical staining. We report a unique case of adolescent renal RMS associated with tumor thrombus extending into the inferior vena cava (IVC) and right atrium.An 18-year-old female adolescent presented with shortness of breath and palpitations, associated with right flank discomfort, and hematuria. A pleomorphic-type renal RMS with Budd-Chiari syndrome and arrhythmia induced by IVC and RA thrombosis was diagnosed. Despite complete tumor resection, the patient developed multiple lung metastases a month after surgery. Chemotherapy was recommended, but the patient declined. She died within a year of the initial operation.Adolescent renal RMS is rare and associated with poor outcome. Early aggressive multimodal therapy seems to be appropriate, in particular, in the presence of tumor thrombosis.
尽管横纹肌肉瘤（RMS）的年龄分布第二个高峰在青春期，但肾RMS在该年龄组极为罕见。基于临床和影像学表现，这种肿瘤与其他肾肿瘤难以区分，诊断依赖于组织学和免疫组化染色。我们报告一例独特的青春期肾RMS病例，肿瘤血栓延伸至下腔静脉（IVC）和右心房。一名18岁女性青少年出现呼吸急促、心悸，伴有右侧胁腹不适和血尿。诊断为伴有布加综合征及由IVC和右心房血栓形成所致心律失常的多形型肾RMS。尽管肿瘤完整切除，但患者术后1个月出现多发肺转移。建议进行化疗，但患者拒绝。她在初次手术后1年内死亡。青春期肾RMS罕见且预后不良。早期积极的多模式治疗似乎是合适的，尤其是在存在肿瘤血栓的情况下。
