Renin producing neuroendocrine pancreatic carcinoma - a case report and review of the literature

Abstract

A 44 year old male patient presented with severe hypertension. The diagnostic work-up revealed elevated levels of plasma renin activity (about 10 times the upper limit of normal) in the presence of normal plasma aldosterone levels and serum potassium concentrations. Renovascular disease was excluded by angiography. Selective renal vein sampling did not show any renin gradient. CT-scans of the abdomen demonstrated normal morphology of the kidneys and adrenals but revealed a big mass in the pancreatic corpus and tail with infiltration of the splenic vein and the presence of enlarged local lymph nodes. The endocrine nature of the pancreatic mass was further supported by a positive octreotide scintigraphy scan. Surgical removal of the tumor by left sided pancreatectomy combined with splenectomy resulted in rapid normalization of elevated renin concentrations as well as blood pressure. Histological examination of the tumor tissue revealed the presence of a neuroendocrine pancreatic carcinoma. Highly (x 70) elevated renin levels were detected by radioimmunoassay in the tumor tissue. To our knowledge this is the first renin-producing neuroendocrine pancreatic carcinoma described in the literature. The present paper describes the case in detail and reviews the available literature on clinical symptomatology, diagnosis and treatment of renin-producing tumors.

An Anusual Cause of Secondary Hypertension in a Young Man

“…Blood pressure got normal after tumor removal. Hyperreninism has also been described with ovarian Sertoli cell tumor [2] and neuroendocrine pancreatic carcinoma [3]. Finally, this case underlines the importance of a complete examination and a biological assessment in front of unusual hypertension, especially if hypertension is resistant and/or on a young patient.…”

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.…”

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].…”