The Small Size and Superficial Location Suggest That Laparoscopic Partial Nephrectomy Is the First Choice for the Treatment of Juxtaglomerular Cell Tumors

Abstract

BackgroundJuxtaglomerular cell tumor (JGCT) is a very rare disease, and surgical resection is the only possible way to cure this tumor. Open nephrectomy and partial nephrectomy have been reported to manage JGCTs with excellent results in the previous reviews. Laparoscopic surgery has been popularized in recent years, while critical issues associated with laparoscopic surgical management have been seldom reported. We summarized the JGCTs in our center to discover the optimal surgical management and its anatomic foundation.MethodsIn this retrospective study, we enrolled a total of 14 JGCT patients. All patients received surgeries and were followed up for up to 11 years. We mainly summarized the size and location of tumors, imaging features, and surgical strategies. A descriptive statistical analysis was performed.ResultsThe JGCTs in this study had a median size of 1.35 cm and all located superficially, mainly in the cortical or subcortical area of the kidney. All 14 patients had hypertension, ten had hypokalemia, and seven had elevated plasma renin activity. Pathologically, JGCT cells were polygonal or spindle shape, with positive CD34 and vimentin immunostaining. All patients received partial nephrectomy; nine were laparoscopic, and five were open. Laparoscopic partial nephrectomy (LPN) was performed in seven out of eight patients over the last nine years. Postoperative blood pressure, serum potassium, and plasma renin activity were normal in all patients. No recurrence occurred within a median follow-up of 60 months.ConclusionThe small size and superficial location are the characteristic anatomic features of JGCT; they suggest that LPN is the preferred surgical strategy. Laparoscopic ultrasound is helpful for the intraoperative detection of small JGCTs. Longer follow-up is required to examine the biological behavior of JGCTs and the effect of LPN.

Diagnostic challenges in patients with reninomas and extrarenal renin‐producing tumours

“…In our third case, the tumour size was 1.6 cm, which was significantly different from the previously reported sizes of ERRPTs. Instead, it resembled the size of reninomas localised in the kidneys, where the mean size is about 2–3 cm 2,19,21 . The hyperplasia of the adrenocortical zona glomerulosa was consistent with the effect of elevated renin levels.…”

“…Instead, it resembled the size of reninomas localised in the kidneys, where the mean size is about 2-3 cm. 2,19,21 The hyperplasia of the adrenocortical zona glomerulosa was consistent with the effect of elevated renin levels. To the best of our knowledge, this is the first reported case of a primary adrenal renin-producing tumour of juxtaglomerular cell type, with clinical, biochemical and histopathological findings and imaging, that were typical for a reninoma, with complete resolution of symptoms following adrenalectomy.…”

“…Reninomas can appear isodense compared to the renal cortex on non-enhanced CT images and are in other cases undetectable. 5,20,21 Even with the use of contrast enhanced CT scan, reninomas may be difficult to detect. Some reninomas display low enhancement compared to the renal parenchyma and can even be mistaken for a cyst, 22,23 possibly explained by local vasoconstrictor effect of angiotensin II and/or a lack of neovascular development within the tumour.…”

“…To identify the tumour, a contrast enhanced CT scan was used first in two of the cases and MRI in one. Reninomas can appear isodense compared to the renal cortex on non‐enhanced CT images and are in other cases undetectable 5,20,21 . Even with the use of contrast enhanced CT scan, reninomas may be difficult to detect.…”

“…Sampling during periods without active secretion may be an explanation in some cases 34 . In addition, reninomas are mostly located subcapsularly, 21 hence the venous blood from the tumour may be drained into the pericapsular veins instead of the renal vein 30,35 . Selective renal venous sampling from the segmental veins may help to further determine the localisation of the tumour within the kidney 5 …”