Nasrollahi Hamid, Eslahi Ali, Ariafar Ali, Ahmed Faisal, Monabati Ahmad
Radiation Oncology Department, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.
Department of Urology, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.
J Kidney Cancer VHL. 2022 Apr 14;9(1):55-58. doi: 10.15586/jkcvhl.v9i1.218. eCollection 2022.
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.
成人原发性肾横纹肌肉瘤(RMS)罕见,文献中仅报道过少数病例。它是一种间叶性肿瘤,临床进展快速且侵袭性强。我们报告一例58岁女性原发性肾RMS病例,该患者在过去一年中出现间歇性腹痛。计算机断层扫描(CT)显示右肾中极有一个20×25×8 cm的异质性实性肿块,并延伸至下极。因此,患者接受了右侧根治性肾输尿管切除术。组织病理学检查和免疫组化研究确诊为伴有多形性成分的RMS。术后患者无并发症出院,并转诊至肿瘤内科进行化疗。然而,2个月后的随访CT扫描显示广泛肝转移和局部复发。患者接受了吉西他滨和多西他赛治疗,但病情恶化,5个月后去世。原发性肾RMS在成人中罕见。此外,肝转移不常见且了解甚少。因此,我们描述了该病例的临床病理特征,包括临床随访情况,重点关注疾病进展、治疗及转归。