Squires J P, Ulbright T M, DeSchryver-Kecskemeti K, Engleman W
Cancer. 1984 Feb 1;53(3):516-23. doi: 10.1002/1097-0142(19840201)53:3<516::aid-cncr2820530325>3.0.co;2-3.
The authors describe a juxtaglomerular cell tumor (JGCT) which caused severe hypertension in a 58-year-old man. Light microscopy showed a circumscribed tumor composed of interlacing cords and occasional nodules of relatively uniform cells with no mitotic activity. Rhomboid crystals characteristic of "prerenin" were present within the cytoplasm of tumor cells, and there was a close relationship between the tumor and unmyelinated nerve axons. Intracytoplasmic renin was demonstrated by immunofluorescence, and tumor granules were shown to contain zinc by electron-beam microanalysis. Review of 14 prior cases, with additional follow-up of 9, showed that no patient had developed recurrence, metastasis, or another tumor. Four patients, however, are hypertensive but probably because of secondary tumor effects rather than recurrent hyperreninism. The distinction of JGCT from hemangiopericytoma with renal involvement is important because of the high mortality associated with the latter. The authors conclude that JGCT is benign, but patients with JGCT may remain hypertensive postnephrectomy because of hypertensive angiopathy.
作者描述了一例导致一名58岁男性严重高血压的肾球旁细胞瘤（JGCT）。光镜检查显示肿瘤界限清楚，由交错的条索状结构和偶尔的结节组成，细胞相对均匀，无有丝分裂活性。肿瘤细胞胞质内可见特征性的“前肾素”菱形晶体，且肿瘤与无髓神经轴突关系密切。免疫荧光法证实胞质内有肾素，电子束微量分析显示肿瘤颗粒含锌。回顾14例既往病例，并对其中9例进行额外随访，结果显示无患者出现复发、转移或其他肿瘤。然而，4例患者患有高血压，但可能是由于肿瘤的继发效应而非复发性高肾素血症。区分JGCT与累及肾脏的血管外皮细胞瘤很重要，因为后者相关死亡率很高。作者得出结论，JGCT是良性的，但JGCT患者肾切除术后可能因高血压性血管病变而仍患有高血压。
