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Table of Contents
Year : 2019  |  Volume : 6  |  Issue : 3  |  Page : 128-131

Juxtaglomerular cell tumor with lung metastases in a young male patient

1 Division of Hematology-Oncology, Department of Internal Medicine, Chang Gung Memorial Hospital at Linkou and Chang Gung University College of Medicine, Taoyuan, Taiwan
2 Department of Pathology, Chang Gung Memorial Hospital at Linkou and Chang Gung University College of Medicine, Taoyuan, Taiwan
3 Department of Medical Imaging and Intervention, Chang Gung Memorial Hospital at Linkou and Chang Gung University College of Medicine, Taoyuan, Taiwan

Date of Submission24-Jul-2018
Date of Decision23-Nov-2018
Date of Acceptance30-Nov-2018
Date of Web Publication20-Aug-2019

Correspondence Address:
Dr. Po-Jung Su
Division of Hematology-Oncology, Department of Internal Medicine, Chang Gung Memorial Hospital, No. 5 Fuxing Street, Guishan Township, Linkou, Taoyuan County 333
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JCRP.JCRP_19_18

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Juxtaglomerular cell tumor (JGCT) is a rare pathologically benign renal neoplasm mostly reported as localized disease, which can secrete renin and induce hypertension, hypokalemia, and hyperaldosteronism. In this report, a young male was diagnosed with JGCT with lung metastases. In general, the diagnosis of JGCT is made by a pathological examination, and the standard treatment of localized disease is to resect the tumor completely. However, there is currently no standard treatment for metastatic or unresectable JGCT, and chemotherapy plays a debatable role.

Keywords: Chemotherapy, juxtaglomerular cell tumor, metastasis

How to cite this article:
Huang PW, Lin YC, Wu KF, Sheng TW, Su PJ. Juxtaglomerular cell tumor with lung metastases in a young male patient. J Cancer Res Pract 2019;6:128-31

How to cite this URL:
Huang PW, Lin YC, Wu KF, Sheng TW, Su PJ. Juxtaglomerular cell tumor with lung metastases in a young male patient. J Cancer Res Pract [serial online] 2019 [cited 2020 Dec 3];6:128-31. Available from: https://www.ejcrp.org/text.asp?2019/6/3/128/264840

  Introduction Top

Juxtaglomerular cell tumor (JGCT) is an extremely rare renal neoplasm arising from the juxtaglomerular apparatus. The first case was described by Robertson et al. in 1967, and it was first named by Kihara et al. in 1968.[1],[2] This disease more frequently affects younger individuals.[3],[4] Patients usually have hypertension, hyperaldosteronism, high plasma renin level, and hypokalemia secondary to tumor renin secretion.[5] JGCT is pathologically benign and is considered to be curable by resection of the tumor. Only two cases of metastatic disease have been reported.[3],[6] Herein, we report a case of JGCT with lung metastases at initial diagnosis.

  Case Report Top

A 37-year-old male patient was generally in good health. However, hematospermia occurred in February 2018, and he received antibiotic treatment. He had a health examination, and left renal tumor was found by kidney sonography at a local hospital. Elevated blood pressure was also noted, and he received bisoprolol and valsartan/amlodipine as primary control. His blood pressure remained around 130–150 mmHg after the initiation of treatment. A computed tomography (CT) urogram revealed a heterogenous left lower pole kidney tumor with a small (about 0.4 cm) left renal vein thrombosis and multiple nodules in the visible lower lung field [Figure 1]. He was transferred to our urology outpatient department for surgical evaluation. His laboratory data were mostly within normal ranges, except that his creatinine level was 1.32 mg/dL. After discussion, surgical removal was suggested and he underwent left radical nephrectomy in March 2018. A 5 cm × 5 cm left kidney tumor was found adhering to the renal hilum; however, no apparent regional lymph node enlargement was noted. Under gross examination by a pathologist, the tumor was lobulated, tan-brown, soft and partially encapsulated with foci of cystic change, and hemorrhage, but renal veins had no evidence of thrombus [Figure 2]a. Microscopically, the renal tumor was composed of sheets of rather uniform tumor cells with round nuclei, inconspicuous nucleoli, and eosinophilic cytoplasm with prominent dilated thin-walled vessels and focal clusters of thick-walled vessels and fibromyxoid stroma [Figure 2]b and [Figure 2]c. Nuclear atypia was mild, and mitotic activity was 0-1/10 HPF. Focal hemorrhage and necrosis were seen. Immunohistochemically, the tumor cells were positive for CD34, vimentin, and smooth muscle actin and negative for PAX-8, CD10, AE1/AE3, HMB45, Melan-A, desmin, GATA3, CD117, CK7, S-100 protein, and STAT6. These findings suggested that it was a JGCT. Due to benign nature of JGCT, a lung biopsy and positron emission tomography (PET) were arranged, which revealed that the lung tumor was morphologically and immunochemically identical to JGCT. PET only revealed mildly increasing fluorodeoxyglucose uptake in the multiple lung tumors. We also checked the renin, aldosterone, and angiotensin-converting enzyme blood levels, which were 64.4 ng/L (lying down status, reference range, 3.6–20.1 ng/L), 34.4 ng/dL (lying down status, reference range, 4.93–17.5 ng/dL), and 16.5 IU/L (reference range, <22.5 IU/L), respectively. After a diagnosis of JGCT with lung metastases had been made, he received palliative chemotherapy with 5-FU and cisplatin in June 2018. He is currently receiving further chemotherapy and being followed up.
Figure 1: Computed tomography urogram. (a) Early phase, (b) delayed phase, and (c) lung metastases

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Figure 2: Gross tumor and pathology of lung metastasis (H and E). (a) Gross tumor, (b) original magnification × 200, and (c) original magnification × 400

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  Discussion Top

JGCT, also known as a juxtaglomerular cell apparatus tumor or reninoma, is a very rare pathologically benign renal tumor which mostly affects children or young adults in their 20s or 30s (mean age, 27 years; male: female ratio, 1:2).[3],[4],[7] It originates from the modified smooth muscle cells that compose the vascular component of the juxtaglomerular apparatus.[4] To date, approximately 100 cases have been described in the English literature, only two of whom had metastatic disease. One was reported by Duan et al. in 2004, who described a patient with lung metastasis after radical nephrectomy 6 years later, and the other was reported by Cucchiari et al. in 2013, who described a patient with synchronous metastases in the spleen and liver at initial diagnosis [Table 1].[3],[6] To the best of our knowledge, our patient is the third reported case with metastatic disease and the first to have unresectable lung metastases at initial diagnosis.
Table 1: Clinical features of three reported cases of metastatic juxtaglomerular cell tumor

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Clinically, JGCT is subdivided into three categories, including the typical variant, atypical variant, and nonfunctioning variant.[8] Typical variant and atypical variant tumors secrete renin and thus can induce severe secondary hypertension and hyperaldosteronism, while the potassium level is normal in the atypical variant. Symptoms of hypertension, such as headaches, retinopathy, dizziness, and proteinuria, are common as the initial presentation.[7] The nonfunctional variant is very rare, and only two cases have ever been reported.[9] In previous reports, JGCT mostly shows a hypoechoic mass lesion in sonography,[10],[11],[12] while dynamic CT scan and magnetic resonance imaging provide a better detection rate of nearly 100%.[13],[14] JGCT usually appears to be isodense or hypodense to the renal medulla without enhancement and shows a relatively decreased density in the early phase compared to the late phase after contrast enhancement.[15] In our case, the tumor had heterogeneous content and was partially enhanced during the early phase. This is compatible with the results of gross examination that the tumor was lobulated with cystic change. Other typical gross findings have been reported to include a well-circumscribed mass confined to the kidney and a yellow-to-tan-gray cut surface of the tumor with focal hemorrhage.[4],[12] Microscopically, the JGCT in our patient was consistent with the literature, in that the tumor was composed of a solid cell sheet with uniformly round, oval to spindled nuclei cells, variable amounts of lightly eosinophilic cytoplasm, and that it contained numerous small, thin-walled vessels. Some reports have reported that tumor cells have nuclear atypia but mitotic activity, and that Ki-67 is generally low or absent.[3],[4],[8],[12] Immunochemistry staining showed positive staining for CD34 and vimentin, but not for cytokeratin, PAX-8, desmin, S100 protein, Melan-A, or HMB-45. This clearly differentiates JGCT from the other renal tumors such as renal cell carcinoma (RCC) (positive cytokeratin, PAX-8 staining), angiomyolipoma (positive HMB-45 staining), and hemangiopericytoma (negative CD34 staining).[4],[12] Renin and CD117 can help confirm the diagnosis, while renin positivity may be observed in some cases of Wilms' tumor, RCC, or renal oncocytoma, and CD117 varies from case to case.[4],[12],[16]

The prognosis of JGCT in patients with localized disease is very good, and complete tumor resection by radical or partial nephrectomy is curative and the plasma renin level is usually normalized after resection. However, hypertension has been reported to continue because of hypertensive angiopathy in approximately 10% of cases.[12] One reported case died because of a massive brain hemorrhage secondary to severe hypertension. Therefore, hypertension should be well-managed. Although this tumor is usually considered to be a benign tumor, previous data suggest that it can have a malignant behavior.[3],[6] In the report of Cucchiari et al., the disease kept progressing despite using doxorubicin and ifosfamide as adjuvant therapy after surgical resection, followed by vinorelbine, paclitaxel, or dacarbazine. These failed attempts indicate that chemotherapy plays debatable role in metastatic JGCT.

  Conclusion Top

We report a case of JGCT with lung metastases at initial diagnosis. The patient also had hypertension caused by an elevated renin level. The diagnosis of JGCT is based on pathology and immunohistochemistry. Surgical removal can cure localized disease, whereas appropriate treatment for metastatic or unresectable disease remains unclear.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initial will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Robertson PW, Klidjian A, Harding LK, Walters G, Lee MR, Robb-Smith AH. Hypertension due to a renin-secreting renal tumour. Am J Med 1967;43:963-76.  Back to cited text no. 1
Kihara I, Kitamura S, Hoshino T, Seida H, Watanabe T. A hitherto unreported vascular tumor of the kidney: A proposal of “juxtaglomerular cell tumor”. Acta Pathol Jpn 1968;18:197-206.  Back to cited text no. 2
Duan X, Bruneval P, Hammadeh R, Fresco R, Eble JN, Clark JI, et al. Metastatic juxtaglomerular cell tumor in a 52-year-old man. Am J Surg Pathol 2004;28:1098-102.  Back to cited text no. 3
Martin SA, Mynderse LA, Lager DJ, Cheville JC. Juxtaglomerular cell tumor: A clinicopathologic study of four cases and review of the literature. Am J Clin Pathol 2001;116:854-63.  Back to cited text no. 4
More IA, Jackson AM, MacSween RN. Renin-secreting tumor associated with hypertension. Cancer 1974;34:2093-102.  Back to cited text no. 5
Cucchiari D, Bertuzzi A, Colombo P, De Sanctis R, Faucher E, Fusco N, et al. Juxtaglomerular cell tumor: Multicentric synchronous disease associated with paraneoplastic syndrome. J Clin Oncol 2013;31:e240-2.  Back to cited text no. 6
McVicar M, Carman C, Chandra M, Abbi RJ, Teichberg S, Kahn E. Hypertension secondary to renin-secreting juxtaglomerular cell tumor: Case report and review of 38 cases. Pediatr Nephrol 1993;7:404-12.  Back to cited text no. 7
Dong D, Li H, Yan W, Xu W. Juxtaglomerular cell tumor of the kidney – A new classification scheme. Urol Oncol 2010;28:34-8.  Back to cited text no. 8
Sakata R, Shimoyamada H, Yanagisawa M, Murakami T, Makiyama K, Nakaigawa N, et al. Nonfunctioning juxtaglomerular cell tumor. Case Rep Pathol 2013;2013:973865.  Back to cited text no. 9
Daniele A, Sabbadin C, Costa G, Vezzaro R, Battistel M, Saraggi D, et al. A10-year history of secondary hypertension: A challenging case of renin-secreting juxtaglomerular cell tumor. J Hypertens 2018;36:1772-4.  Back to cited text no. 10
Gottardo F, Cesari M, Morra A, Gardiman M, Fassina A, Dal Bianco M. Akidney tumor in an adolescent with severe hypertension and hypokalemia: An uncommon case – Case report and review of the literature on reninoma. Urol Int 2010;85:121-4.  Back to cited text no. 11
Kuroda N, Gotoda H, Ohe C, Mikami S, Inoue K, Nagashima Y, et al. Review of juxtaglomerular cell tumor with focus on pathobiological aspect. Diagn Pathol 2011;6:80.  Back to cited text no. 12
Tanabe A, Naruse M, Ogawa T, Ito F, Takagi S, Takano K, et al. Dynamic computer tomography is useful in the differential diagnosis of juxtaglomerular cell tumor and renal cell carcinoma. Hypertens Res 2001;24:331-6.  Back to cited text no. 13
Tanabe A, Naruse K, Kono A, Hase M, Hashimoto Y, Nakazawa H, et al. Avery small juxtaglomerular cell tumor preoperatively identified by magnetic resonance imaging. Intern Med 1996;35:295-300.  Back to cited text no. 14
Katabathina VS, Vikram R, Nagar AM, Tamboli P, Menias CO, Prasad SR. Mesenchymal neoplasms of the kidney in adults: Imaging spectrum with radiologic-pathologic correlation. Radiographics 2010;30:1525-40.  Back to cited text no. 15
Kim HJ, Kim CH, Choi YJ, Ayala AG, Amirikachi M, Ro JY. Juxtaglomerular cell tumor of kidney with CD34 and CD117 immunoreactivity: Report of 5 cases. Arch Pathol Lab Med 2006;130:707-11.  Back to cited text no. 16


  [Figure 1], [Figure 2]

  [Table 1]


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