Malignant human renin producing paraganglionoma–localization with ¹²³I‐MIBG and treatment with ¹³¹I‐MIBG

Abstract

A 22-year-old woman presenting severe hypertension and hypokalaemia is described. Initial evaluation showed a large tumour localized at the position of the left adrenal gland. Subsequent surgery temporarily relieved all signs and symptoms caused by the tumour. The symptoms relapsed after a 2-year disease-free interval. At re-evaluation, the tumour was shown to produce an uncontrolled secretion of renin, thus triggering aldosterone-dependent hypertension. This report describes the diagnosis, treatment and clinical course of this unique patient with a malignant paraganglionoma of adrenal origin.

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

“…ERRPTs are exceptionally rare, with only 36 cases reported. [8][9][10][11][12][13][14][15][16][17] The majority of the reported cases of extrarenal renin-production have been caused by malignant neoplasms of various origins (Table 1). 8,9,15,16 As renin producing tumours are so rare, more common causes of high renin levels such as renovascular disease and medication effects upon RAAS 18 should be excluded first.…”

“…Reninomas are most commonly localised to the kidneys. ERRPTs are exceptionally rare, with only 36 cases reported 8–17 . The majority of the reported cases of extrarenal renin‐production have been caused by malignant neoplasms of various origins (Table 1).…”

Complete recovery after the removal of an ectopic testicle in a case of primary reninism and retroperitoneal hemangioma

“…In the majority of the approximately 50 cases reported in the published literature, autonomous renin production was caused by intrarenal juxtaglomerular tumors, of which only six were malignant [1]. The extrarenal entities, some of which were described several times, included a liver cell carcinoma [2], a hepatoblastoma [3], a pelvic ter- atoma [4], a serous cystadenocarcinoma of the ovary [5], a leiomyosarcoma of the uterus [6], a leiomyosarcoma of the lung [7], a pancreas carcinoma [8], an adrenocortical carcinoma [9], a malignant paraganglionoma [10], a pheochromocytoma [11], a tumor of the fallopian tube [12] and a nephroblastoma [13].…”

Adrenal Mass in Carney Triad

“…A mild I-MIBG uptake can be observed in up to 25% of normal adrenal medulla 31 . Others have described a case of recurrent reninproducing paraganglioma which was first misdiagnosed as an aldosterone-producing cortical tumor on routine histopathological examination 32 . In our patient, the left adrenal nodule detected by the CT scan did not show any pathological MIBG uptake.…”