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Title: [Clinicopathologic features of glomus tumor of the kidney].
Author: Zhao M, Wang AX, Zhu X, Yu JJ, Wang W, Zhang DH, He XL, He HY, Teng XD.
Journal: Zhonghua Bing Li Xue Za Zhi; 2018 Aug 08; 47(8):580-584. PubMed ID: 30107661.
Abstract:
Objective: To investigate the clinicopathologic and differential diagnostic features of glomus tumor of the kidney. Methods: Four cases of glomus tumor of the kidney were collected from the archives of Peking University Third Hospital, the Second Hospital of Tianjin Medical University, Ningbo Yinzhou Second Hospital and Zhejiang Provincial People's Hospital between January 2012 to June 2017; the clinical and radiologic features, histomorphology, immunohistochemistry, ultrastucture and prognosis were analyzed and the relevant literature was reviewed. Results: Patients consisted of 2 men and 2 women with ages ranging from 37 years to 66 years (mean 55 years). Three patients had history of hypertensive disease (grade Ⅱ, 3 to 10 years). The tumors measured in maximum diameter from 3.0 cm to 4.0 cm (mean 3.6 cm) and showed gray-white to yellow and tan on cut surface. Macroscopical examinations showed all tumors were circumscribed but non-encapsulated. Histologically, 1 tumor presented as glomus tumor with extensive myxoid change, 1 as cellular and solid pattern glomus tumor, 1 as glomangioma with focal myopericytoma-like pattern and 1 as symplastic glomus tumor with areas resembling myopericytoma. The tumor cells in two cases showed scant cytoplasm and uniform, bland-appearing nuclei without mitoses. In one case, the tumor cells were epithelioid with abundant eosinophilic cytoplasm and relatively well-defined cell borders. There was an increased mitosis of 4/50 HPF; however, no evidence of atypical mitosis or nuclear atypia was noted. In the symplastic glomus tumor the tumor cells showed frequently nuclear pleomorphism without mitoses. By immunohistochemistry, all tumors showed strong and diffuse reactivities to at least 3 of the 4 muscle-associated markers (SMA, h-Caldesmon, MSA and Calponin), 3 tumors strongly and diffusely expressed collagen Ⅳ, 2 expressed CD34 and 1 focally expressed desmin; whereas markers including epithelial, neuroendocrine, nephrogenic, melanoma-associated, STAT6, S-100 protein, CD117 and β-catenin all were negative in all the 4 tumors. Ultrastuctural analysis was done in 2 cases and showed prominent cytoplasmic actin bundles and pericellular basement membrane, and lacking of rhomboid renin crystals in both tumors. The hypertension persisted after surgical resection for all the 3 patients with this medical history. Follow-up information (range: 6-64 months, mean: 44 months)showed that no evidence of local recurrence or distant metastasis was identified in all 4 patients. Conclusions: Glomus tumor rarely occurs in the kidney and usually has a good prognosis. Careful attention to its morphology with the judicious use of immunohistochemistry and ultrastuctural analysis can be helpful for its diagnosis and differential diagnosis. 目的： 探讨肾脏血管球瘤的临床病理学特征和鉴别诊断要点。 方法： 收集北京大学第三医院、天津医科大学第二医院、宁波市鄞州第二医院以及浙江省人民医院2012年1月至2017年6月间诊治的4例肾脏血管球瘤，观察其临床及影像学特点、组织病理学、免疫组织化学、超微结构特征以及预后，并结合相关文献进行复习。 结果： 2例男性，2例女性。发病年龄37～66岁(平均55岁)。3例患有高血压病(Ⅱ级，3～10年)。肿瘤最大直径3.0～4.0 cm(平均3.6 cm)，切面灰白至灰黄灰褐。镜下，肿瘤均与周围肾实质分界清楚，无包膜。1例组织学上表现为血管球瘤伴广泛的黏液变性，1例为富于细胞的实体性血管球瘤，1例为球血管瘤伴局灶的肌周细胞瘤，1例为共质体性血管球瘤伴部分肌周细胞瘤。2例瘤细胞胞质稀少，核形态温和，大小一致，未见核分裂象；1例瘤细胞胞质丰富呈上皮样，胞膜相对清楚，核形态温和，核分裂象约4/50 HPF，未见不典型核分裂象。1例瘤细胞部分可见多形性核，未见核分裂象。免疫组织化学染色，4例肿瘤均表达至少3种肌样分化标志物(平滑肌肌动蛋白、h－Caldesmon、肌特异性蛋白、Calponin)，3例弥漫强表达Ⅳ型胶原，2例弥漫强表达CD34，1例局灶表达结蛋白。4例均不表达上皮、神经内分泌、肾源性、黑色素性、STAT6、S－100蛋白、CD117以及β－catenin等标志物。2例行超微结构分析均显示胞质内丰富的肌丝束和细胞间基底膜物质，未见菱形肾素小体。术后3例高血压患者血压均未见明显变化，4例随访6～64个月(平均44个月)均未见肿瘤复发或转移证据。 结论： 血管球瘤罕见的可发生于肾脏，预后较好，组织学观察联合免疫组织化学染色和超微结构分析可助于其诊断和鉴别诊断。.

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