Juxtaglomerular cell tumor of the kidney in an 11-year-old child: case report

Background. The juxtaglomerular cell tumor (JCT) of the kidney, or reninoma, is a rare benign renal neoplasm that occurs exceptionally rarely in pediatric patients. In contrast to other renal tumors, JCT pathogenesis is driven by renin-angiotensin system activation, clinically manifesting as refractory hypertension due to excessive tumor-derived renin secretion. We present a clinical case of an 11-year-old patient with JCT.

Materials and methods. From January 2012 to January 2025 in the Pathology Department of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology only one case of JCT was identified.

Results. An 11-year-old girl had headaches and developed arterial hypertension (blood pressure 170/90 mmHg) during 3 months. Abdominal ultrasound revealed a tumor in the left kidney.

Further evaluation at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology showed contrast-enhanced abdominal computed tomography demonstrated a solid, heterogeneous mass (3.2 × 2.9 × 3.0 cm, V = 14.5 cm3) in the midsegment of the left kidney. There were no metastatic lung lesions on contrast-enhanced chest computed tomography.

According to the clinical-radiological findings, patient's age (11 years), and tumor characteristics (size < 200 cm3), nephron-sparing surgery was performed. The patient underwent initial laparoscopic left kidney resection with lymph node sampling.

Histopathological examination confirmed the diagnosis of JCT and radical tumor resection. Arterial hypertension was relieved in the postoperative period.

Conclusions. The diagnosis and management of JCT require a multidisciplinary approach integrating clinical presentation, laboratory parameters, and expertise from pediatric oncologists, radiologists, surgeons, and pathologists. Given the tumor's typically small dimensions and benign biological behavior nephron-sparing surgery should be discussed with curative intent. 

1. Shao L., Manalang M., Cooley L. Juxtaglomerular cell tumor in an 8-year-old girl. Pediatr Blood Cancer. 2008;50(2):406–9. doi: 10.1002/pbc.21048.

2. Duan X., Bruneval P., Hammadeh R., Fresco R., Eble J.N., Clark J.I., Vigneswaran W.T., Flanigan R.C., Picken M.M. Metastatic juxtaglomerular cell tumor in a 52-year-old man. Am J Surg Pathol. 2004;28(8):1098–102. doi: 10.1097/01.pas.0000126722.29212.a7.

3. Chen W.S., Chang J.W. Extrarenal juxtaglomerular cell tumor in bone: report of a case with review of the literature. Chin Med J (Engl). 1987;100(1):78–82. PMID: 3109831.

4. Shera A.H., Baba A.A., Bakshi I.H., Lone I.A. Recurrent malignant juxtaglomerular cell tumor: A rare cause of malignant hypertension in a child. J Indian Assoc Pediatr Surg. 2011;16(4):152–4. doi: 10.4103/0971-9261.86876.

5. Robertson P.W., Klidjian A., Harding L.K., Walters G., Lee M.R., Robb-Smith A.H. Hypertension due to a renin-secreting renal tumour. Am J Med. 1967;43(6):963–76. doi: 10.1016/0002-9343(67)90256-2.

6. Wang F., Shi C., Cui Y., Li C., Tong A. Juxtaglomerular cell tumor: clinical and immunohistochemical features. J Clin Hypertens (Greenwich). 2017;19(8):807–12. doi: 10.1111/jch.12997.

7. Brandal P., Busund L.-T., Heim S. Chromosome abnormalities in juxtaglomerular cell tumors. Cancer. 2005;104(3):504–10. doi: 10.1002/cncr.21205.

8. Méndez G.P., Klock C., Nosé V. Juxtaglomerular cell tumor of the kidney: case report and differential diagnosis with emphasis on pathologic and cytopathologic features. Int J Surg Pathol. 2011;19(1):93–8. doi: 10.1177/1066896908329413.

9. Sirohi D., Werneck Cunha I., Gupta S. Juxtaglomerular cell tumor. In: WHO classification of tumors editorial board. WHO Classification of Tumours: Urinary and Male Genital Tumours. 5th ed. Vol. 8. Lyon: IARC; 2022. [Electronic resource]: https://tumourclassification.iarc.who.int/chapters/36/34.

10. Quach P., Hamza A. Juxtaglomerular cell tumor: report of a case with unusual presentation. Autops Case Rep. 2022;12:e2021406. doi: 10.4322/acr.2021.406.

11. Hagiya A., Zhou M., Hung A., Aron M. Juxtaglomerular cell tumor with atypical pathological features: report of a case and review of literature. Int J Surg Pathol. 2020;28(1):87–91. doi: 10.1177/1066896919868773.

12. Robertson P.W., Klidjian A., Harding L.K, Walters G., Lee M.R., Robb-Smith A.H. Hypertension due to a renin‐secreting renal tumour. Am J Med. 1967;43(6):963–76. doi: 10.1016/0002-9343(67)90256-2.

13. Kim J.H., Kim J.H., Cho M.H., Park E., Hyun H.S., Ahn Y.H., Kang H.G., Moon K.C., Ha I., Cheong H.I. Reninoma: a rare cause of curable hypertension. Korean J Pediatr. 2019;62(4):144–7. doi: 10.3345/kjp.2018.06926.

14. Minnullin M.M., Kachanov D.Yu., Grachev N.S. Laparoscopic nephrectomies and kidney resections in children. Khirurgiya. Zhurnal im. N.I. Pirogova = Pirogov Russian Journal of Surgery. 2024;(7):61–72. (In Russ.).

15. Martin S.A., Mynderse L.A., Lager D.J., Cheville J.C. Juxtaglomerular cell tumor: a clinicopathologic study of four cases and review of the literature. Am J Clin Pathol. 2001;116(6):854–63. doi: 10.1309/B10J-FKQ5-J7P8-WKU4.

16. Kim H.J., Kim C.H., Choi Y.J., Ayala A.G., Amirikachi M., Ro J.Y. Juxtaglomerular cell tumor of kidney with CD34 and CD117 immunoreactivity: report of 5 cases. Arch Pathol Lab Med. 2006;130(5):707–11. doi: 10.5858/2006-130-707-JCTOKW.

17. Kuroda N., Gotoda H., Ohe C., Mikami S., Inoue K., Nagashima Y., Petersson F., Alvarado-Cabrero I., Pan C.C., Hes O., Michal M., Gatalica Z. Review of juxtaglomerular cell tumor with focus on pathobiological aspect. Diagn Pathol. 2011;6:80. doi: 10.1186/1746-1596-6-80.

18. Treger T.D., Lawrence J.E.G., Anderson N.D., Coorens T.H.H., Letunovska A., Abby E., Lee-Six H., Oliver T.R.W., Al-Saadi R., Tullus K., Morcrette G., Ciaran Hutchinson J., Rampling D., Sebire N., Pritchard-Jones K., Young M.D., Mitchell T.J., Jones P.H., Tran M., Behjati S., Chowdhury T. Targetable NOTCH1 rearrangements in reninoma. Nat Commun. 2023;14(1):5826. doi: 10.1038/s41467-023-41118-8.

19. Ueda T., Morinaga Y., Inoue K., Hirano S., Matsubara H., Hongo F. Juxtaglomerular cell tumor diagnosed preoperatively by renal tumor biopsy. IJU Case Rep. 2021;4(4):207–9. doi: 10.1002/iju5.12283.

20. Kang S., Chen F., Zhong Y., Han B., Cheng G., Guo A., Tian Y., Tong X., Dou J., Ye H. Preoperative diagnosis of juxtaglomerular cell tumors in eight patients. J Clin Hypertens (Greenwich). 2016;18(10):982–90. doi: 10.1111/jch.12810.

21. Lin S.Y., Liu W.Y., Chen W.C., Chen R.H. Secondary hypertension due to a renin-secreting juxtaglomerular cell tumor. J Formos Med Assoc. 2010;109(3):237–40. doi: 10.1016/S0929-6646(10)60047-2.

22. Tanabe A., Naruse M., Ogawa T., Ito F., Takagi S., Takano K., Ohashi H., Tsuchiya K., Sone M., Nihei H., Toma H. Dynamic computer tomography is useful in the differential diagnosis of juxtaglomerular cell tumor and renal cell carcinoma. Hypertens Res. 2001;24(4):331–6. doi: 10.1291/hypres.24.331.

23. Lee H.J., Hyun J.S., Jang H.S., Sul H., Park S.G. Paraneoplastic secondary hypertension due to a renin-secreting desmoplastic small round cell tumor: A case report. Oncol Lett. 2014;8(5):1986–92. doi: 10.3892/ol.2014.2452.

24. Dong D., Li H., Yan W., Xu W. Juxtaglomerular cell tumor of the kidney – a new classification scheme. Urol Oncol. 2010;28(1):34–8. doi: 10.1016/j.urolonc.2009.08.003.

25. Sakata R., Shimoyamada H., Yanagisawa M., Murakami T., Makiyama K., Nakaigawa N., Inayama Y., Ohashi K., Nagashima Y., Yao M., Kubota Y. Nonfunctioning juxtaglomerular cell tumor. Case Rep Pathol. 2013:2013:973865. doi: 10.1155/2013/973865.

26. Moch H., Amin M.B., Berney D.M., Compérat E.M., Gill A.J., Hartmann A., Menon S., Raspollini M.R., Rubin M.A., Srigley J.R., Tan P.H., Tickoo S.K., Tsuzuki T., Turajlic S., Cree I., Netto G.J. The 2022 World Health Organization Classification of Tumours of the Urinary System and Male Genital Organs – Part A: Renal, Penile, and Testicular Tumours. Eur Urol. 2022;82(5):458–68. doi: 10.1016/j.eururo.2022.06.016.

27. Garfield K., LaGrange C.A. Renal cell cancer. StatPearls [Internet]. 2024. PMID: 29261992.

28. Xu B., Zhang Q., Jin J. Hypertension secondary to reninoma treated with laparoscopic nephron-sparing surgery in a child. Urology. 2012;80(1):210–3. doi: 10.1016/j.urology.2012.03.024.

29. Sakiyama H., Hamada S., Oshiro T., Hyakuna N., Kuda M., Hishiki T., Aoyama H., Kuroda N., Yorita K., Wada N., Yoshioka T., Koga Y., Nakanishi K. Juxtaglomerular cell tumor with pulmonary metastases: a case report and review of the literature. Pediatr Blood Cancer. 2023;70(4):e30068. doi: 10.1002/pbc.30068.

30. Rumyantsev A.G., Varfolomeeva S.R., Grachev N.S., Karachunsky A.I., Novichkova G.A. Principles and tools of evidencebased medicine in pediatric hematology/oncology Doktor.Ru = Doctor.Ru. 2015;10(111):6–13. (In Russ.).