polycystic liver disease is a condition characterized by the presence of multiple cysts in the liver , which may be inherited or sporadic , and most cases of adult polycystic liver disease ( apld ) are related to autosomal dominant polycystic kidney disease ( adpkd ) . in 1964 ,
there have been some reports , till date , that describe the association of malignant neoplasms with potter type iii cystic disease of the liver and kidney [ 2 , 3 , 4 , 5 , 6 , 7 , 8 ] ; however , most of these describe pancreatic and hepatobiliary neoplasms arising in patients with polycystic liver and kidney diseases including adpkd , and no report has described the association of gastric carcinoma with potter type iii cystic disease of the liver and kidney .
this is the first case report of multiple gastric carcinomas associated with potter type iii cystic disease of the liver , mesenterium and kidney .
although it is unclear whether the development of multiple gastric carcinomas is directly associated with the pathogenesis of multiple cysts in the liver , mesenterium and kidney , this case provides a perspective for etiology of gastric carcinoma with polycystic disease .
he had been receiving hemodialysis treatment 3 times a week for 2 years because of chronic renal failure due to polycystic kidneys .
he stated that his sister had suffered from polycystic kidney disease . physical examination on admission revealed distended abdomen due to polycystic disease .
the patient had high serum levels of carcinoembryonic antigen ( 7.9 ng / ml ) and carbohydrate antigen 19 - 9 ( 202 mg / dl ) .
an abdominal computed tomography scan showed multiple cysts in the liver and cystic lesions on both sides in the kidney and mesenterium ( fig .
gastrointestinal fiberscopy showed the presence of two ulcerated lesions in the lesser curvature in the middle and lower portions of the stomach .
histopathological analysis of the gastric tumor biopsy specimens revealed papillary adenocarcinoma in one tumor and poorly differentiated adenocarcinoma in another .
distal gastrectomy with lymph node dissection was performed , and the gastric tumors were surgically diagnosed to be t2n1m0 , stage ii , according to the tnm classification for gastric carcinoma .
macroscopic examination showed that two tumors of type ii were present in the middle and lower portions of the stomach ( fig .
the distal lesion was diagnosed as a poorly differentiated adenocarcinoma ( 38  39  6 mm ) within the muscularis propria , with marked lymphatic invasion ( ly2 ) and vascular invasion ( v2 ) ( fig .
3b ) ; the lesion on the proximal side was diagnosed as a papillary adenocarcinoma ( 58  47  6 mm ) within the submucosal layer , with marked lymphatic invasion ( ly2 ) and vascular invasion ( v1 ) ( fig .
adpkd is the most common of all inherited renal cystic diseases and has various external manifestations , including cysts in the pancreas , seminal vesicles , arachnoid membrane , and liver .
the most accepted among them is the osathanondh and potter system that was established in 1964 , according to which polycystic kidneys are classified into three types .
the cysts may be present in any portion of the nephron or tubule , but they are most commonly found in bowman 's space and the angle of the loop of henle . in the older classification ,
potter type iii cystic disease was considered to represent adult - type polycystic disease of the kidney . in 1972 ,
they also observed cysts in the liver and/or pancreas in 58 of these patients ; hence , they described this condition as potter type iii polycystic disease . in the present case , computed tomography and ultrasonography scans showed multiple renal and hepatic cysts in addition to multiple cysts in the mesenterium .
therefore , according to hatfield and pfister 's criteria , we considered that this case of association of apld with multiple renal cysts could be classified as potter type iii cystic disease .
the patient stated that his sister had suffered from polycystic kidney disease ; however , we could not confirm the presence of a family history of polycystic kidney disease because his sister had been out of contact for long . in the absence of a family history of adpkd , bilateral multiple renal cysts with hepatic cysts , together with the absence of other manifestations suggesting a different renal cystic disease , provide evidence for diagnosis . in these instances ,
a family history , however , may be absent in 2040% of new patients in whom the diagnosis of adpkd first is suspected from imaging studies , which can be due to a de novo mutation of pkd-1 or pkd-2 genes . although we could not confirm the mutation of pkd-1 or pkd-2 in this case , family history , clinical findings and imaging studies suggested that the patient suffered from adpkd .
reports on the association of malignant neoplasm with apld and adpkd ( potter type iii cystic disease ) are extremely rare .
to our knowledge , only 8 such cases have been reported in the english language literature ( table 1 ) [ 2 , 3 , 4 , 5 , 6 , 7 , 8 ] . till date , most cases of neoplasms associated with polycystic disease of the liver and kidney have been reported as either pancreatic or hepatobiliary neoplasms , namely 5 cases of pancreatic neoplasms and 3 cases of cholangiocarcinoma . since 5 of these cases were pancreatic neoplasms , we think that adpkd has some genetic influence on the development of pancreatic neoplasms .
aziz et al .   reported 2 patients from the same family ( a brother and a sister ) with adpkd who developed adenocarcinoma of the gastroesophageal junction ; however , these patients did not have cysts in the liver or other organs except for the kidney . in the present case , although a detailed family history could not be obtained , multiple cysts were observed in the liver , both kidneys , and mesenterium .
these genes encode a member of the polycystin protein family , which is an integral membrane protein that functions as a regulator of calcium - permeable cation channels and intracellular calcium homoeostasis .
some studies have described an association between the pkd genes and cancer cells ; polycystin-1 , encoded by pkd-1 , was reported to induce apoptosis and cell cycle arrest in the g0/g1 phase in cancer cells . moreover
, pkd-1 inhibits cancer cell migration and invasion and may be considered a new member of the tumor suppressor family of genes .
therefore , adpkd , which is caused by defective pkd-1 and pkd-2 , may be a carcinogenic condition .
in addition , mutations in two genes , namely prksch ( which codes for hepatocystin ) and sec63 , have been found to cause apld . however , as mutations in these genes have not been found in all families , undiscovered genes responsible for causing apld probably still exist .
although it is unclear whether the development of multiple gastric carcinomas is directly associated with the pathogenesis of cysts in the liver , mesenterium and kidney , the present case demonstrates that gastric carcinoma can be an external complication in patients with polycystic disease .
 we report a case of multiple gastric carcinomas associated with potter type iii cystic disease of the liver , mesenterium and kidney . 
 a 65-year - old man with chronic renal failure due to polycystic kidneys and under hemodialysis treatment 3 times a week for 2 years was admitted to our hospital because of anemia . 
 he stated that his sister had suffered from polycystic kidney disease . 
 gastrointestinal fiberscopy showed two lesions in the lesser curvature in the lower portion of the stomach , and histopathological analysis of the gastric tumor biopsies revealed that one of the tumors was a papillary adenocarcinoma and the other a poorly differentiated adenocarcinoma . 
 helicobacter pylori infection was not detected in the stomach mucosa . 
 abdominal computed tomography scan revealed polycystic lesions in the liver , mesenterium and both kidneys . 
 these imaging findings and family history suggested that the patient suffered from multiple gastric carcinomas associated with potter type iii cystic disease of the liver , mesenterium and kidney . 
 reports on the association of malignant neoplasm with potter type iii cystic disease are extremely rare . 
 especially , no case of the association of gastric carcinoma with potter type iii cystic disease of the liver and kidney has been described previously . 
 this is a first report of the association of gastric carcinoma with potter type iii cystic disease . 
 we also review reports of other malignant neoplasms associated with polycystic disease . 
Introduction
Case Report
Discussion
Disclosure Statement