GIST With Nephrotic Syndrome as a Paraneoplastic Syndrome
Nephrotic syndrome can occur as malignancy-associated PNS, and previous studies have estimated that cancer occurs in 11% to 22% of patients with nephrotic syndrome. Therefore, if an adult presents with nephrotic syndrome, physicians should bear in mind the risk of underlying malignant disease. Gastric cancer, lung cancer, and malignant lymphoma are frequently associated with nephrotic syndrome of which the rates are 25, 15 and 10%, respectively. To the best of our knowledge, GIST has not been previously reported as an underlying disease of PNS, and this is the first such case to be documented.
In patients with PNS, surgical tumor removal can be followed by the regression of nephrotic syndrome. One study reported that nephrotic syndrome improved after operation in 78% of patients. Therefore, surgical resection should be performed even in the presence of poor general condition due to PNS, because cancer is a potential cause of nephrotic syndrome. The hypoalbuminemia and proteinuria in our patient did not preoperatively respond to symptomatic treatment. By contrast, clinical remission of nephrotic syndrome was obtained immediately after tumor removal. Considering that the patient did not take drugs perioperatively which cause nephrotic syndrome, such as non-steroidal anti-inflammatory drugs or tyrosine kinase inhibitors, the nephrotic syndrome was PNS associated with a GIST of the stomach.
Hypothetical explanations have been proposed to link minimal-change glomerulopathy to solid tumors. One potential mechanism involves the secretion of some tumoral factor that increases glomerular permeability without affecting glomerular capillary structure, resulting in massive proteinuria. Alternatively, tumor-specific antigens such as carcinoembryonic antigens may react with antibodies to form immune complexes, which accumulate as deposits in the glomerular basement membrane and cause renal dysfunction. The mechanism of this phenomenon can involve type III allergy.
In summary, we have documented a case of GIST of the stomach that was associated with nephrotic syndrome as PNS. Patients with refractory nephrotic syndrome caused by a malignant tumor should be treated aggressively to improve signs and symptoms associated with PNS. Otherwise, the opportunity for potentially curative surgery may be lost.
Discussion
Nephrotic syndrome can occur as malignancy-associated PNS, and previous studies have estimated that cancer occurs in 11% to 22% of patients with nephrotic syndrome. Therefore, if an adult presents with nephrotic syndrome, physicians should bear in mind the risk of underlying malignant disease. Gastric cancer, lung cancer, and malignant lymphoma are frequently associated with nephrotic syndrome of which the rates are 25, 15 and 10%, respectively. To the best of our knowledge, GIST has not been previously reported as an underlying disease of PNS, and this is the first such case to be documented.
In patients with PNS, surgical tumor removal can be followed by the regression of nephrotic syndrome. One study reported that nephrotic syndrome improved after operation in 78% of patients. Therefore, surgical resection should be performed even in the presence of poor general condition due to PNS, because cancer is a potential cause of nephrotic syndrome. The hypoalbuminemia and proteinuria in our patient did not preoperatively respond to symptomatic treatment. By contrast, clinical remission of nephrotic syndrome was obtained immediately after tumor removal. Considering that the patient did not take drugs perioperatively which cause nephrotic syndrome, such as non-steroidal anti-inflammatory drugs or tyrosine kinase inhibitors, the nephrotic syndrome was PNS associated with a GIST of the stomach.
Hypothetical explanations have been proposed to link minimal-change glomerulopathy to solid tumors. One potential mechanism involves the secretion of some tumoral factor that increases glomerular permeability without affecting glomerular capillary structure, resulting in massive proteinuria. Alternatively, tumor-specific antigens such as carcinoembryonic antigens may react with antibodies to form immune complexes, which accumulate as deposits in the glomerular basement membrane and cause renal dysfunction. The mechanism of this phenomenon can involve type III allergy.
In summary, we have documented a case of GIST of the stomach that was associated with nephrotic syndrome as PNS. Patients with refractory nephrotic syndrome caused by a malignant tumor should be treated aggressively to improve signs and symptoms associated with PNS. Otherwise, the opportunity for potentially curative surgery may be lost.
SHARE
