Department of Diagnostic Pathology, Kochi Red Cross Hospital, Shin-honmachi 2-13-51, Kochi City, Kochi 780-8562, Japan.
Diagn Pathol. 2011 Aug 26;6:80. doi: 10.1186/1746-1596-6-80.
Juxtaglomerular cell tumor (JGCT) generally affects adolescents and young adults. The patients experience symptoms related to hypertension and hypokalemia due to renin-secretion by the tumor. Grossly, the tumor is well circumscribed with fibrous capsule and the cut surface shows yellow or gray-tan color with frequent hemorrhage. Histologically, the tumor is composed of monotonous polygonal cells with entrapped normal tubules. Immunohistochemically, tumor cells exhibit a positive reactivity for renin, vimentin and CD34. Ultrastructurally, neoplastic cells contain rhomboid-shaped renin protogranules. Genetically, losses of chromosomes 9 and 11 were frequently observed. Clinically, the majority of tumors showed a benign course, but rare tumors with vascular invasion or metastasis were reported. JGCT is a curable cause of hypertensive disease if it is discovered early and surgically removed, but may cause a fatal outcome usually by a cerebrovascular attack or may cause fetal demise in pregnancy. Additionally, pathologists and urologists need to recognize that this neoplasm in most cases pursues a benign course, but aggressive forms may develop in some cases.
肾小球旁细胞瘤（JGCT）通常影响青少年和年轻成年人。由于肿瘤分泌肾素，患者会出现与高血压和低钾血症相关的症状。大体上，肿瘤边界清楚，有纤维囊，切面呈黄色或灰白色，常有出血。组织学上，肿瘤由单调的多边形细胞组成，伴有被包裹的正常小管。免疫组织化学显示，肿瘤细胞对肾素、波形蛋白和 CD34 呈阳性反应。超微结构上，肿瘤细胞含有菱形的肾素原颗粒。遗传学上，常观察到染色体 9 和 11 的缺失。临床上，大多数肿瘤呈良性过程，但也有报道罕见的具有血管侵犯或转移的肿瘤。如果早期发现并手术切除，JGCT 是可治愈的高血压病因，但通常会因脑血管意外导致致命后果，或在妊娠中导致胎儿死亡。此外，病理学家和泌尿科医生需要认识到，这种肿瘤在大多数情况下呈良性过程，但在某些情况下可能会发展为侵袭性形式。
