Deyrup Andrea T, Montgomery Elizabeth, Fisher Cyril
Department of Pathology, University of Chicago, Chicago, IL, USA.
Am J Surg Pathol. 2004 Feb;28(2):178-82. doi: 10.1097/00000478-200402000-00004.
Leiomyosarcoma of kidney is a rare lesion for which limited data are available. Cases coded as leiomyosarcoma of the kidney from three institutions were reviewed. These cases comprised 3 men and 7 women, 40 to 75 years of age. Immunohistochemistry was performed where possible and clinical follow-up information was obtained. On immunohistochemical staining, 6 of 6 tumors expressed smooth muscle actin, desmin, calponin, and h-caldesmon, and epithelial membrane antigen was positive in 1 of 5. Tumors were negative for cytokeratin and S-100 protein. Follow-up information was available for 9 patients. Two patients had metastases at diagnosis, four developed metastases, and two had recurrent disease. Five of 9 died of disease. Two patients were alive with no evidence of disease after 19 and 60 months, and 2 patients were alive with disease after 48 months and 56 months. Comparing outcome with tumor grade, the one patient with grade 1 tumor was alive with no evidence of disease; of 5 patients with grade 2 tumor, 2 died of disease, 1 was alive with no evidence of disease, 1 was alive with disease, and 1 was alive with extensive disease; all 3 grade 3 patients died of disease. In summary, the majority of renal leiomyosarcomas are intermediate or high grade with correspondingly poor prognosis.
肾平滑肌肉瘤是一种罕见的病变，相关数据有限。对来自三个机构编码为肾平滑肌肉瘤的病例进行了回顾。这些病例包括3名男性和7名女性，年龄在40至75岁之间。尽可能进行了免疫组织化学检查，并获取了临床随访信息。免疫组织化学染色显示，6例肿瘤中有6例表达平滑肌肌动蛋白、结蛋白、钙调蛋白和h-钙黏蛋白，5例中有1例上皮膜抗原呈阳性。肿瘤细胞角蛋白和S-100蛋白呈阴性。9例患者有随访信息。2例患者在诊断时已有转移，4例发生转移，2例有复发性疾病。9例中有5例死于该病。2例患者分别在19个月和60个月后无疾病证据存活，2例患者分别在48个月和56个月后带瘤存活。将结果与肿瘤分级进行比较，1例1级肿瘤患者无疾病证据存活；5例2级肿瘤患者中，2例死于该病，1例无疾病证据存活，1例带瘤存活，1例广泛疾病存活；3例3级患者均死于该病。总之，大多数肾平滑肌肉瘤为中级或高级别，预后相应较差。
