PDF(Pubmed)
Abstract：
Primary rhabdomyosarcoma (RMS) of the kidney in an adult is rare, with only a few cases published in the literature. It is a mesenchymal tumor associated with an aggressive and rapid clinical progression course. We present a case of primary renal RMS in a 58-year-old female who presented with intermittent abdominal pain in the past year. The computed tomography (CT) scan revealed a 20×25×8 cm heterogeneous solid mass in the middle pole extended to the lower pole of the right kidney. Therefore, the patient underwent a right radical nephroureterectomy. Histopathology examination and immunohistochemistry studies confirmed the diagnosis of RMS with pleomorphic components. Postoperatively, the patient was discharged without any complications and was referred to an oncologist for chemotherapy. However, a follow-up CT scan in 2 months showed widespread liver metastasis and local recurrence. The patient received Gemcitabine and Docetaxel, but her condition worsened, and she passed away 5 months later. Primary renal RMS is rare in adults. In addition, liver metastasis is uncommon and poorly understood. Hence, we describe the clinicopathologic characteristics, including clinical follow-up of our case, focusing on the disease progression, treatment, and outcome.
摘要：
成人肾脏原发性横纹肌肉瘤（RMS）很少见，只有少数案例发表在文献中。它是与侵袭性和快速临床进展过程相关的间充质肿瘤。我们介绍了一名58岁女性的原发性肾RMS病例，该女性在过去一年中出现间歇性腹痛。计算机断层扫描（CT）扫描显示，中极有20×25×8cm的异质固体块延伸到右肾的下极。因此,患者接受了右肾输尿管根治性切除术。组织病理学检查和免疫组织化学研究证实了具有多形性成分的RMS的诊断。术后,患者出院，无任何并发症，并转诊至肿瘤科医生接受化疗。然而,随访2个月的CT扫描显示广泛的肝转移和局部复发.患者接受吉西他滨和多西他赛，但她的病情恶化了,5个月后她就去世了.原发性肾RMS在成人中很少见。此外,肝转移并不常见，也知之甚少。因此，我们描述了临床病理特征，包括我们病例的临床随访,关注疾病进展，治疗,和结果。
