Nephron 21: 325-333 (1978)
Juxtaglomerular Cell Tumor Grace Connor, Cleaves M. Bennett, Rabbe R. Lindstrom, Stanley A. Brosman, Luciano Barajas and David Edelbaum Departments of Medicine, Radiology, Surgery, and Pathology, Harbor General Hospital, UCLA School of Medicine, Torrance, Calif.
Key Words. Juxtaglomerular cell tumor • Renin producing tumor • Propranolol • Renal vein branch renins Abstract. A 15-year-old female with primary reninism presented with benign hypertension, normokalemia, normal aortagram and normal intravenous pyelogram. The diagnosis was sug gested only by a remarkably elevated plasma renin activity (PRA). Selective catheterization of renal vein branches was necessary to make the diagnosis of a tumor. A local resection of the tumor resulted in normalization of blood pressure and PRA. Prior to the definitive surgery, oral propranolol was effective in lowering PRA and blood pressure.
Primary reninism is the syndrome of hyper tension, hyperreninemia, hyperaldosteronism and hypokalemia, all secondary to a hemangi opericytoma of the juxtaglomerular cell ap paratus. To date, 10 cases have been reported (summarized in table I) [1—10]. 8 of these 10 cases were characterized by severe hyper tension; 7 by at least moderate hypokalemia and all had been symptomatic a variable length of time from 5 months to 15 years. The present report concerns a patient in whom mild symptomatology, near normal serum potas sium and mild hypertension were associated with a renin-secreting tumor. The incongruity of a markedly elevated plasma renin activity (PRA) led to an extensive evaluation for a
tumor. Localized excision resulted in a cure of hypertension and a return of PRA to normal.
Case Report T.R. a 15-year-old previously healthy Caucasian female, was noted to have mild hypertension (150/100) duringa routine physical examination. Initial diagnostic evaluation of her hypertension included a normal rapidsequence intravenous pyelogram, aortagram and urinary vanmandelic excretion. Serum electrolytes revealed a slightly low serum potassium of 3.4 mEq/I. PRA following a single dose of furosemide was inter preted to be slightly elevated at 14 ng/ml/h. Several months later, the patient was referred to this institution for further evaluation. Physical examination was within normal limits except for slight obesity, grade I KW retinopathy, hypertension (160-180/110-120) and tachycardia (pulse = 100 beats/min). The patient was
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Introduction
326
Connor/Bennett/Lindstrom/Brosman/Barajas/Edelbaum
Case No.
1
2
3
4
5
6
75
8
9
10
Reference
1967 Robertson et al. [1] Oxford, England 1968 Kihara et al. [2] Aizu, Wakamatsu, Japan 1971 Eddy and Sanchez [3] Temple, Tex. 1972 Bonnin et a!. [5] Adelaide, Australia 1972 Conn et al. [6] Ann Arbor, Mich. 1973 Schambelan et al. [7] San Francisco, Calif. 1973 Brown et a!. [8] Glasgow, Scotland 1974 Gherardi a!. [9] Framingham, Mass. 1974 Hirose et al. [4] Fuknoka, Japan 1975 Orjavik et al. [10] Oslo, Norway
Age
Sex
BP*1
PRA
Renal vein renlns234
16
M
250/150*
not done
not done
23
F
180/110
13.5 ¡xg/1 n < 2 ¡xg/1
not done
37
F
260/150
1207 ng/100ml 150 < n < 300 ng/100ml
not done
13
F
160/125
45 ng/ml/h n = 1.7 ± 0.5 ng/ml/h
positive
18
F
200/128
‘elevated’
positive
24
M
160/120
169 ng/ml/3 h 5 < n < 32 ng/ml/3 h
positive
7
F
210/150
78 units/1 4 < n < 20 units/1
positive
38
F
220/150
7,680 ng/100 ml n < 2,000 ng/100 ml
not done
10
M
180/130
216 ng/24 h n = 28.2 ±7.3 ng/24 h
positive
25
M
200/150
25 ng/ml/h n < 1.5 ng/ml/h
positive
M = Male; F = Female; n = normal; WNL = normal or within normal limits; N = nephrectomy; A = adrenalectomy. 1 Cases 1, 7 and 9, malignant hypertension; case 1, normotensive but addisonian. 2 Positive indicates lateralizing values for PRA. 3 Cases 2 and 9, aortograms; case 3, nephrotomography; remainder, angiograms. 4 24-hour urine excretion except case 7 which is serum. 5 Case 7 died from CNS bleed, renal tumor noted at autopsy.
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Tabic I. Summary of previous 10 juxtaglomerular cell tumors
327
Surgery
Radio graphy3
K Aldosterone'1 mEq/1
WNL
3.1
not done
N, A
? focal defect
2.5
416 (zg/24 h 80 < n < 200 p.g/24 h
N
focal defect
2.6
33 pg/24 h n < 20 pg/24 h
A, N
focal defect
2.6
not done
N
focal defect
3.5
44 p.g/24 h n < 20 ¡Jt-g/24 h
N
focal defect
3.9
13.6 ¡xg/24 h 4 < n < 17 (xg/24 h
A, N
focal defect
2.5
25 ng/100 ml n < 18 mg/I
N
WNL
‘1’
not done
A
WNL
3.5
370 (xg/24 h 50 < n < 150 jzg/24 h
N
225 |xg/ml n < 20 ¡xg/ml
N
WNL
2.9
receiving no medication during the subsequent evalu ation. Routine laboratory studies were within normal limits, including a serum potassium of 3.7 mEq/1 on several occasions. On two occasions, with the patient on a moderately low salt diet for 3 days, and after 1 h of upright activity, the PRA was 40.5 ng/ml/h with 24hour urinary sodium excretion of 141 mEq (fig. 1). Serum renin concentration was comparably elevated at 360 ng/ml (78 < normal < 138 ng/ml) and plasma aldosterone was 83.6 ng/100 ml (normal