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Open Access Highly Accessed Review

Review of juxtaglomerular cell tumor with focus on pathobiological aspect

Naoto Kuroda1*, Hiroko Gotoda2, Chisato Ohe3, Shuji Mikami4, Keiji Inoue5, Yoji Nagashima6, Fredrik Petersson7, Isabel Alvarado-Cabrero8, Chin-Chen Pan9, Ondrej Hes10, Michal Michal10 and Zoran Gatalica11

Author affiliations

1 Department of Diagnostic Pathology, Kochi Red Cross Hospital, Shin-honmachi 2-13-51, Kochi City, Kochi 780-8562, Japan

2 Department of Pathology, Sapporo Kosei General Hospital, 8-5 Kita 3-jo Higashi, Sapporo, Hokkaido 060-0033, Japan

3 Department of Pathology, Kansai Medical University, Hirakata Hospital, 2-3-1 Shinmachi, Hirakata, Osaka 573-1191, Japan

4 Division of Diagnostic Pathology, Keio University Hospital, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8252, Japan

5 Department of Urology, Kochi Medical School, Kochi University, Kohasu, Oko-cho, Nankoku City, Kochi 783-8505, Japan

6 Department of Molecular Pathology, Yokohama City University Graduate School of Medicine, B252, 3-9 Fukuura, Kanazawa-ku, Yokohama City, Kanagawa 236-0004, Japan

7 Department of Pathology, National University Hospital System, 119 074 Singapore, Singapore

8 Department of Pathology, Mexican Oncology Hospital, Instututo Mexicano del Seguro Social, Mexico City, Mexico

9 Taipei Veterans General Hospital, No.201, Shi-Pai Rd. Sec2, Taipei 11217, Taiwan

10 Sikl's Department of Pathology, Charles University Hospital Plzen, Alej Svobody 80, 30460 Plzen, Czech Republic

11 Depertment of Pathology, Creighton University School, Omaha, NE 68131, USA

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Citation and License

Diagnostic Pathology 2011, 6:80  doi:10.1186/1746-1596-6-80

Published: 26 August 2011

Abstract

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.