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JUXTAGLOMERULAR CELL TUMORS: A CASE SERIES

Objective: Juxtaglomerular cell tumors (JCTs) are a rare but potentially curable cause of hypertension. Their diagnosis is challenging because these tumors may not be detected or mistaken for a cyst on CT-scan. Design and method: Between 1986 and 2017, 10 patients with JCTs were retrospectively identified in our Hypertension Unit. Clinical, biological and radiological features were extracted from our clinical data warehouse. Results: Among the 10 patients with JCT, 8 were women, 2 were men. Median age was 24.5 [15–49] years. 8 of 10 patients had grade III hypertension. Severe hypokalemia related to a marked secondary hyperaldosteronism was a constant feature. Medians of plasma renin and aldosterone concentrations were 392 [70.5–4800] mUI/L and 1490 [671–2492] pmol/L, respectively. Median plasma pro-renin concentration was 835.5 [133–6546] mUI/L. Median tumor size was 17.5 [8–33] mm. On CT-scan, JCTs were spontaneously isodense with little enhancement after contrast media injection. On MRI, JCTs were iso (7/10) or hypointense (3/10) on T1-weighted images (WI). On T2-WI, JCTs were hypointense (2/10), isointense (4/10, with 3/4 heterogeneous) or heterogeneously hyperintense (4/10). Six of 10 JCTs had a thin peripheral “pseudo-capsule” (hypointense on T2-WI). Contrast enhancement was low, slightly heterogeneous and delayed. On diffusion-WI, tumors were hyperintense with a restricted apparent diffusion coefficient. Surgical resection allowed hormonal recovery in all cases and hypertension cure in all but one patient because of a mild zone of renal infarction in the tumorectomy area. After 43 [0–228] months follow-up, one patient had a recurrence of hypertension 4 years after surgery. Conclusions: This is the largest study describing clinical, biological and radiological characteristics of JCTs, including detailed description of MRI features. In hypertensive patients with secondary hyperaldosteronism, imaging (CT or ultrasound) should be performed to analyze renal arteries and renal cortex. In case of unexplained secondary hyperaldosteronism, renal MRI should be performed if a renal lesion without cystic specific characteristics is detected. MRI should also be discussed in patients with grade III hypertension, severe secondary hyperaldosteronism and normal CT-scan, particularly in women. Non-invasive imaging technics, especially renal MRI, may improve diagnosis of JCTs.