Journal List > J Korean Soc Endocrinol > v.20(2) > 1063775

Choi, Hong, Cho, Park, Shin, Park, Kim, Cho, and Lee: A Case of Primary Reninism Manifested by Hypertension with Hypokalemia

Abstract

Primary reninism is a rare cause of hypertension manifesting along with hypokalemia. A high level of plasma renin activity and a high level of serum aldosterone are the whole markers of primary reninism. Upon making the diagnosis of primary reninism, other more common causes of aldosteronism must be differentiated, such as renovascular hypertension and primary aldosteronism. Primary reninism is commonly caused by juxtaglomerular cell tumor, which is one of the curable causes of hypertension, and this can be successfully treated by conservative surgery. We report here on a case of primary reninism that was caused by juxtaglomerular cell tumor that developed in a 22-year-old female patient. She was recently diagnosed with hypertension and hypokalemia. She had markedly elevated plasma renin activity and an increased serum aldosterone concentration. Computed tomography revealed a mass located in the right kidney and selective renal vein sampling suggested that the mass was secreting an excess of renin. Right nephrectomy was done and her hypertension with hypokalemia was successfully treated. We report here a case of primary reninism that presented with juxtaglomerular cell tumor along with a review of the literature.

Figures and Tables

Fig. 1A
Precontrast CT scan shows a round mass with heterogeneous attenuation in the lower pole of right kidney (arrows). Major part of the mass shows slightly high attenuation compared with renal parenchyma.
jkse-20-168-g001
Fig. 1B
On contrast-enhanced CT scan, this mass shows mild enhancement with a small, nonenhancing part in the anterior portion (arrow).
jkse-20-168-g002
Fig. 2
On contrast-enhanced, fat-saturated T1-weighted sagittal MR images, this mass (arrows) shows low-signal intensity with renal parenchyma, suggesting hypovascularity.
jkse-20-168-g003
Fig. 3
In microscopic examination, broad papillae covered by epithelioid cells was noted. And inside epitheloid cells. Islands of medium-sized round cells with pale cytoplasm and central nuclei was noted (H&E×200).
jkse-20-168-g004
Fig. 4
PAS staining revealed intracytoplasmic PAS-positive pink granules (PAS×400).
jkse-20-168-g005

References

1. Robertson PW, Klidjian A, Harding LK, Walters H, Lee MR, Robb-Smith AH. Hypertension due to a renin-secreting renal tumor. Am J Med. 1967. 43:963–976.
2. 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.
3. Corvol P, Pinet F, Plouin PF, Bruneval P, Menard J. Renin-secreting tumors. Lab Invest. 1977. 37:357–368.
4. Haag M, Selbach J, Keberle E. Juxtaglomerular cell tumor (reninoma) as the etiology of severe juvenile hypertension. Blood Press. 1997. 6:147–153.
5. White PC. Mechanisms of Disease: Disorders of Aldosterone Biosynthesis and Action. N Engl J Med. 1994. 331:250–258.
6. 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. 116:854–863.
7. Kim Chul H, Park Yong W, Ordonez Nelson G, Ayala Alberto G, Burroughs Judy F, Ro Jae Y. Juxtaglomerular Cell Tumor of the Kidney: Case Report with lmmunohistochemical and Electron Microscopic Investigations and Review of the Literature. International Journal of Surgical Pathology. 1999. 7:115–123.
8. Dunnick N Reed. 1999. In : The Radiological Society of North America 85th Scientific Assembly and Annual Meeting Image Interpretation Session; 206–261.
9. Haab F, Duclos JM, Guyenne T, Plouin PF, Corvol P. Renin Secreting tumors: Diagnosis, Conservative Surgical Approach and long-term results. The Journal of Urology. 1995. 153:1781–1784.
10. McVicar M, Carman C, Chandra M, Abbi RJ, Teichberg S, Kahn E. Hypertension secondary to reninsecreting juxtaglomerular cell tumor: case report and review of 38 cases. Pediatr Nephrol. 1993. 7:404–412.
11. Kashiwabara H, Inaba M, Itabashi A, Ishii J, Katayama S. A case of renin-producing juxtaglomerular tumor: effect of ACE inhibitor or angiotensin II receptor antagonist. Blood Press. 1997. 6:147–153.
12. Camilleri JP, Hinglais N, Bruneval P, Bariety J, Tricottet V, Rouchon M. Renin storage and cell differentiation in juxtaglomerular cell tumors: an immunohistochemical and ultrastructural study of three cases. Hum Pathol. 1984. 15:1069–1079.
13. Nielsen HO. Arterial hypertension due to a reninproducing renal carcinoma. Scand J Urol Nephrol. 1975. 9:293–296.
14. Pursell RN, Quinlan PM. Secondary hypertension due to a renin-producing teratoma. Am J Hypertens. 2003. 16:592–595.
15. Moritake H, Taketomi A, Kamimura S, Ikuno Y, Seo Y, Fukuda T, Iguchi H, Okamura J. Reninproducing hepatoblastoma. J Pediatr Hematol Oncol. 2000. 22:78–80.
16. Fromme M, Streicher E, Kraus B, Kruse-Jarres J. Arterial hypertension in renin-producing retroperitoneal leiomyosarcoma. Klin Wochenschr. 1985. 63:158–163.
17. Yokoyama H, Yamane Y, Takahara J, Yoshinouchi T, Ofuji T. A case of ectopic renin-secreting orbital hemangiopericytoma associated with juvenile hypertension and hypokalemia. Acta Med Okayama. 1979. 33:315–322.
18. Anderson PW, Macaulay L, Do YS, Sherrod A, d'Ablaing G, Koss M, Shinagawa T, Tran B, Montz FJ, Hsueh WA. Extrarenal renin-secreting tumors: insights into hypertension and ovarian renin production. Medicine (Baltimore). 1989. 68:257–268.
19. Chauveau D, Julien J, Pagny JY, Jeunemaitre X, Bruneval P, Guyenne TT, Le Chevalier T, Plouin PF, Corvol P. Epithelioid sarcoma of soft tissues: a case of extrarenal renin-secreting tumour. J Hum Hypertens. 1988. 2:261–264.
20. Atlas SA, Hesson TE, Sealey JE, Dharmgrongartama B, Laragh JH, Ruddy MC, Aurell M. Characterization of inactive renin("prorenin") from renin-secreting tumors of nonrenal origin. Similarity to inactive renin from kidney and normal plasma. J Clin Invest. 1984. 73:437–447.
21. Min Ki Ouk, Kwon Hi Jeong, Ahn Seok Joo, Chang Sang Ah, Chang Yoon Sik, Bang Byung Kee, Kim Jin, Park Moon Hyang, Jung Eun Sun, Choi Young Jin, Seo Eun Joo, Kim Byung Kee. Juxtaglomerular cell tumor of the Kidney: A Case Report. J Korean Med Sci. 2001. 16:233–236.
22. Bostwick David G. Urologic Surgical pathology. 1st ed. St. Louis: Missouri Mosby-year Book;113–115.
23. Corvol P, Renin F, Plouin PF, Bruneval P, Menard J. Renin-secreting tumors. Endocrinol Metab Clin North Am. 1994. 23:255–270.
TOOLS
Similar articles