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      Juxtaglomerular Cell Tumor: A Distinct Mesenchymal Tumor of Kidney

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          Abstract

          Juxtaglomerular cell tumor (JGCT) is an unusual mesenchymal entity of the kidney. It is a benign renin-secreting tumor causing hypertension and hypokalemia due to secondary hyperaldosteronism. It is curable if it is discovered early and surgically removed, but may cause a fatal outcome usually due to complications of associated hypertension.

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          Review of juxtaglomerular cell tumor with focus on pathobiological aspect

          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.
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            Metastatic juxtaglomerular cell tumor in a 52-year-old man.

            Juxtaglomerular cell tumor is a rare renal neoplasm arising from the juxtaglomerular apparatus. Approximately 70 cases have been reported in the English literature since it was first described by Robertson et al in 1967. This tumor has been considered benign and resection has so far been curative. In this paper, we report the first metastatic juxtaglomerular cell tumor. The 15-cm tumor occurred in the right kidney of a 46-year-old man. It invaded the renal vein, and was treated by radical nephrectomy in 1995. The diagnosis at that time was renal cell carcinoma. The patient was well for 6 years and then developed bilateral lung masses, which were resected. Microscopically, the tumors from the kidney and the lungs were similar, consisting of solid sheets of uniformly round-to-polygonal cells intermixed with abundant delicate vasculature. Both renal and pulmonary tumors were positive for vimentin, renin, and only focally to CD34. Electron microscopic studies performed on the paraffin-embedded renal tumor and formalin-fixed lung tumor revealed the typical rhomboid crystals of proto-renin. In consideration of the characteristic morphologic features, immunohistochemistry, and the presence of rhomboid crystals of proto-renin, the diagnosis was modified to malignant juxtaglomerular cell tumor.
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              Hypertension due to a renin-secreting juxtaglomerular cell tumor.

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                Author and article information

                Journal
                J Clin Imaging Sci
                J Clin Imaging Sci
                JCIS
                Journal of Clinical Imaging Science
                Medknow Publications & Media Pvt Ltd (India )
                2156-7514
                2156-5597
                2014
                30 June 2014
                : 4
                Affiliations
                Department of Pathology, Ibn Sina Hospital, Rabat, Morocco
                Author notes
                Address for correspondence: Dr. Hafsa Elouazzani, Department of Pathology, Ibn Sina Hospital, Rabat, Morocco. E-mail: hafoussa02@ 123456hotmail.fr
                Article
                JCIS-4-33
                10.4103/2156-7514.135482
                4142465
                Copyright: © 2014 Elouazzani H.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                Categories
                Radiologic Pathologic Correlation

                Radiology & Imaging

                renin, kidney, juxtaglomerular cell

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