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Paraneoplastic hepatic dysfunction with jaundice in a case of primary renal synovial sarcoma: A very rare scenario.

ABSTRACT: Nonmetastatic hepatic dysfunction known as Stauffer's syndrome is a rare entity. It is commonly associated with clear cell carcinoma of kidney. Rarely it has been reported in cases of prostatic carcinoma, bronchogenic tumor and lymphoproliferative diseases. Stauffer s syndrome presents as elevated alkaline phosphatase, aminotransferases, and prolonged prothrombin time without jaundice. However a very rare variant of Stauffer's syndrome presenting with jaundice has been reported in few case reports of clear cell carcinoma of kidney. But such a presentation in cases of renal sarcomas has not been reported so far. Here we report a unique case of primary synovial sarcoma of kidney presenting as nonmetastatic cholestatic jaundice.

SUBMITTER: Dewana SK

PROVIDER: S-EPMC6562268 | biostudies-literature | 2019 May

REPOSITORIES: biostudies-literature

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Paraneoplastic hepatic dysfunction with jaundice in a case of primary renal synovial sarcoma: A very rare scenario.

Dewana Sudheer Kumar SK Parmar Kalpesh Mahesh KM Sharma Gopal G Bansal Amit A Panwar Pankaj P Mavuduru Ravimohan S RS

Urology case reports 20190211

Nonmetastatic hepatic dysfunction known as Stauffer's syndrome is a rare entity. It is commonly associated with clear cell carcinoma of kidney. Rarely it has been reported in cases of prostatic carcinoma, bronchogenic tumor and lymphoproliferative diseases. Stauffer s syndrome presents as elevated alkaline phosphatase, aminotransferases, and prolonged prothrombin time without jaundice. However a very rare variant of Stauffer's syndrome presenting with jaundice has been reported in few case repor ...[more]

PMID: 31211052

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Project description:Postoperative acute renal failure in patients with obstructive jaundice is still a serious clinically complication, yet the mechanisms remain unclear. Renin-angiotensin-aldosterone system (RAAS) plays a central role in renal disease progression. Several lines of evidence shows that angiotensin-converting-enzyme-2 (ACE2), a main effector of RAAS acts as a local regulator for renal protection. This study aims to investigate the role of ACE2 and the effect of spironolactone treatment in obstructive jaundice(OJ) rats with renal injury. The rats with obstructive jaundice were established by bile duct ligation. Total bilirubin (TBil), serum creatinine (Scr) and the expression of ACE2 in kidney tissue of obstructive jaundice rats were detected. Comparatively, the expression of ACE2, renin, angiotensin II (AngII), angiotensin-(1-7)[Ang-(1-7)], aldosterone and intercellular adhesion molecule 1 (ICAM-1) in kidney tissues after spironolactone administration were measured by ELISA. Renal necrosis, inflammation and fibrosis induced by OJ were also measured by HE staining and Masson staining. The correlation between the expression of ACE2 and TBil, also the Scr level were investigated. With the time of common bile duct ligation prolonged, the TBil and Scr concentration increased while the expression of ACE2 in OJ rats' kidney tissues decreased. However, after spironolactone intervention, the expressions of ACE2, renin, AngII, Ang-(1-7), aldosterone and ICAM-1 in kidney tissue were changed, moreover, necrotic, inflammatory and fibrotic condition was also decreased. The relationship between the mRNA expression of ACE2 and TBil/Scr was observed to be moderately negatively correlated (r?=?-0.516, R2?=?0.292, P?<?0.01), (r?=?-0.576, R2?=?0.332, P?<?0.01), respectively. RAAS exerted an important effect in the renal damage caused by OJ. Spironolactone intervention not only improved the degree of renal fibrosis induced by OJ, but also upregulated the ACE2 expression in the kidney of OJ rats and rescued the renal function.

Project description:Pancreatic cancer frequently causes diabetes. We recently proposed adrenomedullin as a candidate mediator of pancreatic ?-cell dysfunction in pancreatic cancer. How pancreatic cancer-derived adrenomedullin reaches ? cells remote from the cancer to induce ?-cell dysfunction is unknown. We tested a novel hypothesis that pancreatic cancer sheds adrenomedullin-containing exosomes into circulation, which are transported to ? cells and impair insulin secretion.We characterized exosomes from conditioned media of pancreatic cancer cell lines (n = 5) and portal/peripheral venous blood of patients with pancreatic cancer (n = 20). Western blot analysis showed the presence of adrenomedullin in pancreatic cancer-exosomes. We determined the effect of adrenomedullin-containing pancreatic cancer exosomes on insulin secretion from INS-1 ? cells and human islets, and demonstrated the mechanism of exosome internalization into ? cells. We studied the interaction between ?-cell adrenomedullin receptors and adrenomedullin present in pancreatic cancer-exosomes. In addition, the effect of adrenomedullin on endoplasmic reticulum (ER) stress response genes and reactive oxygen/nitrogen species generation in ? cells was shown.Exosomes were found to be the predominant extracellular vesicles secreted by pancreatic cancer into culture media and patient plasma. Pancreatic cancer-exosomes contained adrenomedullin and CA19-9, readily entered ? cells through caveolin-mediated endocytosis or macropinocytosis, and inhibited insulin secretion. Adrenomedullin in pancreatic cancer exosomes interacted with its receptor on ? cells. Adrenomedullin receptor blockade abrogated the inhibitory effect of exosomes on insulin secretion. ? cells exposed to adrenomedullin or pancreatic cancer exosomes showed upregulation of ER stress genes and increased reactive oxygen/nitrogen species.Pancreatic cancer causes paraneoplastic ?-cell dysfunction by shedding adrenomedullin(+)/CA19-9(+) exosomes into circulation that inhibit insulin secretion, likely through adrenomedullin-induced ER stress and failure of the unfolded protein response.

Dataset Information

Paraneoplastic hepatic dysfunction with jaundice in a case of primary renal synovial sarcoma: A very rare scenario.

Publications

Paraneoplastic hepatic dysfunction with jaundice in a case of primary renal synovial sarcoma: A very rare scenario.

Similar Datasets

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