Renin-secreting Tumor Research Articles

Hypertension Due to a Renin-Secreting Tumour Localised by Segmental Renal Vein Sampling

An 18-year-old female was found to be hypertensive on routine medical examination. Further investigation disclosed persistent hypokalaemia and elevated plasma renin activity in peripheral venous blood. Segmental renal vein sampling with assay of blood samples located the source of excess renin secretion in the lower mid-zone of the left kidney. This localization was not confirmed by either angiography or by palpation of the exposed kidney before nephrectomy but macroscopic examination of the freshly sectioned kidney revealed a small tumour in the region suggested by renal vein sampling. The tumour had the morphologic pattern fo an haemangiopericytoma with abundant ultrastructural specific granules and very high renin activity by tissue assay. Plasma renin activity fell precipitously after nephrectomy and remained very low for the first week. Although the immediate post-operative blood pressure fell to normal, hypertension recurred temporarily and was associated with elevated plasma aldosteron, producing a syndrome similar to primary aldosteronism. All variables returned to normal without specific therapy and hypertension has not subsequently recurred.

Renin-Secreting Clear Cell Carcinoma of the Kidney

A young woman with mild to moderate hypertension and normal PRA, serum potassium levels, and urinary aldosterone excretion rate was found to have a renal tumor by selective renal arteriogram. Renal vein renin activity indicated an increased production of renin from the kidney containing the tumor. At surgery, a clear cell carcinoma of the kidney was found that contained renin activity in excess of the surrounding kidney tissue. The renin activity appeared identical to human kidney renin. Previous renin-producing tumors have been associated with severe hypertension, elevated plasma renin activity, hypokalemia, and elevated urinary aldosterone excretion. This case should call attention to the renin-secreting tumor as a cause of even mild hypertension.

Renin-secreting tumor associated with hypertension.

Hypertension in a 6-year-old girl was cured following nephrectomy for a reninsecreting renal tumor. Preoperative diagnosis allowed optimal tissue preservation for morphological studies, which demonstrated similarities of the tumor cells both to normal juxtaglomerular cells and to those of the few reninsecreting tumors previously described.

Selective renal-vein renin sampling in hypertensive patients with segmental renal lesions.

Abstract Selective intrarenal catheterization was used to obtain venous blood draining a lesion affecting only a segment of one kidney in nine hypertensive patients (four segmental infarcts, four renal-artery branch stenoses and one renin-secreting tumor). The main renal-vein renin ratio between the affected and contralateral kidneys (Ra/Rc) was > 1.5 in only four of nine patients (mean ratio ± S.E. 2.1 ± 0.5), whereas the ratio when the segmental sample (Rseg/Rc) was used was significantly higher: > 1.5 in all (4.5 ± 1.1). By contrast, neither plasma renin activity in segmental samples from normal areas of the affected kidney nor that in intrarenal samples of two patients with main renal-artery stenosis exceeded plasma renin activity of the affected kidney. Selective renal-vein renin sampling can specifically identify a localized source of renin that may be overlooked by main renal-vein sampling and facilitate the detection of patients with surgically remediable lesions. (N Engl J Med 290:1153–1157, 1974)

The relationship between the plasma concentrations of renin and angiotensin with total exchangeable sodium in hypertension.

That the renin-angiotensin system is involved in the pathogenesis of clinical hypertension, at least in some cases, is suggested particularly by studies of the type illustrated in Fig. 1. The data shown in this Figure were obtained during the investigation of an 8 year old girl whose severe hypertension was cured by the excision of a renin-secreting tumour from the left kidney.

Primary reninism: hypertension, hyperreninaemia and secondary aldosteronism due to juxtaglomerular renal cell tumours.

1. A further case of renin-secreting renal juxtaglomerular cell tumour is reported. 2. Hypertension, aldosteronism and hypokalaemia were associated with high peripheral levels of renin. 3. Renin activity was twice as high in right as in left renal vein plasma. 4. A renal juxtaglomerular cell tumour was found in the right kidney and removed, after which the biochemical abnormalities and blood pressure were promptly corrected. 5. Explants of tumour tissue in culture produced large amounts of renin.

Role of renin and aldosterone in hypertension due to a renin-secreting tumor

A renin-secreting renal tumor is a rare cause of curable hypertension characterized clinically by hypertension, hypokalemia, hyperaldosteronism and increased plasma renin activity (PRA). Prior subtotal adrenalectomy in such a patient allowed examination of the role of hyperreninemia in the absence of excessive aldosterone secretion. Hypertension was severe, but serum potassium and aldosterone production were normal. PRA was markedly increased under all conditions studied, with a persistent increase noted with changes in posture. Renal vein renin studies were critical in localizing the renal tumor, and unilateral nephrectomy resulted in a prompt fall in PRA to subnormal levels and dramatic cure of the hypertension. Renin content of the renal tumor was more than 500 times higher than in the adjacent kidney, and histologic studies confirmed that the tumor was of juxtaglomerular cell origin. This case demonstrates that severe hypertension can occur in this syndrome despite subtotal adrenalectomy and further illustrates an association of renin overproduction with a specific type of hypertension.

The Case for Treating Severe Hypertension; Two Reversible Complications of Malignant Phase Hypertension; Renin-Secreting Tumours Another Reversible Cause of Secondary Aldosteronism in Hypertension

The Case for Treating Severe Hypertension; Two Reversible Complications of Malignant Phase Hypertension; Renin-Secreting Tumours Another Reversible Cause of Secondary Aldosteronism in Hypertension

A renin-secreting renal tumour associated with hypertension.

Summary: The case history of a 13-year-old boy with severe hypertension is described. On the basis of renal arteriography and estimations of plasma renin, a pre-operative diagnosis of a renin-secreting tumour was made. After the affected kidney was removed, the hypertension and the elevated plasma renin fell to normal.

Fine structure of juxtaglomerular cells and mast cells in a renin-secreting kidney tumour

This paper presented the fine structure of a renal tumour which was associated with hypersecretion of renin and hypertension and discussed the possible significance of the cell types observed. There were 2 major cell types. The first contained cytoplasmic granules similar to those seen in juxtaglomerular cells. Some of these granules were rhomboidal or polygonal and were frequently seen to have a crystalline substructure. Other granules were round and were either amorphous or contained remnants of rhomboidal crystalline granules. The second cell type contained cytoplasmic granules characteristic of human mast cells. Spiral and cylindrical lamellae having transverse periodicities with 6nm. centre-to-centre spacing and granular material of the 'mulberry' type were seen. Rhomboids of the type seen in the first cell type did not occur in the mast cells, and granules containing lamellae did not occur in the juxtaglomerular cell type. There is much evidence to suggest that at least one important site of renin production in man is the juxtaglomerular apparatus, and the rhomboidal crystalline granules have been suggested as being renincontaining. Their occurrence in this tumour is therefore significant. Mast cells are not known to produce renin. It is possible that mast cells could have a homeostatic role in this particular tumour in view of the known in-vivo inhibition by heparin of the renin-angiotensin-aldosterone system. The role of mast cells in renal disease and turnours and in disturbances of the renin-angiotensin-aldostcrone system needs clarification.

A juxtaglomerular cell tumour: light and electron microscopic studies of a renin-secreting kidney tumour containing both juxtaglomerular cells and mast cells

The fine structure of a renal tumour which was associated with hypersecretion of renin with resulting hypertension has been studied. There were two main cell types in the tumour. One contained cytoplasmic granules similar to those seen in juxtaglomerular cells. Some of these granules were rhomboidal or polygonal and some were round and many were shown to have a crystalline substructure. These granules are similar to specific juxtaglomerular cell granules believed to be renin-containing. The other type contained numerous cytoplasmic granules of different size, shape and electron density, characteristic of those seen in the human mast cell. The tumour is best regarded as a growth of juxtaglomerular cells with secondary mast cell infiltration. The term juxtaglomerular cell tumour' is recommended for renin secreting tumours in which specific juxtaglomerular cell granules are identified. The fine structure of a renal tumour which was associated with hypersecretion of renin with resulting hypertension has been studied. There were two main cell types in the tumour. One contained cytoplasmic granules similar to those seen in juxtaglomerular cells. Some of these granules were rhomboidal or polygonal and some were round and many were shown to have a crystalline substructure. These granules are similar to specific juxtaglomerular cell granules believed to be renin-containing. The other type contained numerous cytoplasmic granules of different size, shape and electron density, characteristic of those seen in the human mast cell. The tumour is best regarded as a growth of juxtaglomerular cells with secondary mast cell infiltration. The term juxtaglomerular cell tumour' is recommended for renin secreting tumours in which specific juxtaglomerular cell granules are identified.

Renin Levels in Nephroblastoma (Wilms' Tumour): Report of a Renin Secreting Tumour

The case history is reported of a child aged 1 year 10 months with malignant hypertension in association with Wilms9 tumour. In addition there was a marked electrolyte disturbance, with hyponatraemia and hypokalaemia, and a blood picture of microangiopathic haemolytic anaemia. All these features resolved after removal of the kidney and tumour. Pre-operative plasma renin concentration was extremely high and had returned to normal 3 months after operation. The renal tumour showed a marked degree of differentiation, with glomerulus-like structures attached to tubules, and significant quantities of renin were found on assay. No renin was detected in the renal cortex of the affected kidney. In two other normotensive patients with Wilms9 tumour no renin could be detected in the tumour tissue. We consider that the evidence suggests that this patient had a renin-secreting nephroblastoma.