Multiple Gastric Carcinomas Associated with Potter Type III Cystic Disease.

Abstract

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.

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Page 1

Case Rep Gastroenterol 2011;5:590–596
DOI: 10.1159/000329179
Published online:
October 7, 2011
© 2011 S. Karger AG, Basel
ISSN 1662–0631
www.karger.com/crg

This is an Open Access article licensed under the terms of the Creative Commons Attribution-
NonCommercial-NoDerivs 3.0 License (www.karger.com/OA-license), applicable to the online
version of the article only. Distribution for non-commercial purposes only.


Kenji Mimatsu, MD, PhD

Department of Surgery, Social Insurance Yokohama Central Hospital
268 Yamashita-cho, Naka-ku, Yokohama, Kanagawa 231-8553 (Japan)
Tel. +81 45 641 1921, E-Mail mimtatsu.kenji @ yokochu.jp

590


Multiple Gastric Carcinomas
Associated with Potter Type III
Cystic Disease
Kenji Mimatsua Takatsugu Oidaa Atsushi Kawasakia
Hisao Kanoa Kazutoshi Kidaa Nobutada Fukinoa
Youichi Kuboia Sadao Amanob
aDepartment of Surgery, Social Insurance Yokohama Central Hospital, Yokahama,
and bDepartment of Surgery, Nihon University School of Medicine, Tokyo, Japan


Key Words
Gastric carcinoma · Polycystic disease · Polycystic liver and kidney disease
Abstract
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.

Page 2

Case Rep Gastroenterol 2011;5:590–596
DOI: 10.1159/000329179
Published online:
October 7, 2011
© 2011 S. Karger AG, Basel
ISSN 1662–0631
www.karger.com/crg




591
Introduction
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, Osathanondh and Potter [1] classified polycystic kidney into three types. Potter
type III cystic disease corresponds to the adult type of polycystic kidney. 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 [2–8]; 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.
Case Report
A 65-year-old man was admitted to our hospital because of anemia. 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. Laboratory examination revealed
hemoglobinemia (8.5 g/dl). The result of the diagnostic tests for hepatitis B and C was negative. 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. 1). These findings suggested
that he suffered from polycystic disease of the liver and kidney. 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. Operative examination
confirmed the presence of multiple cysts in the liver (fig. 2a) and on both sides in the kidney and
mesenterium (fig. 2b).
Macroscopic examination showed that two tumors of type II were present in the middle and lower
portions of the stomach (fig. 3a). On the basis of a histopathological examination, 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. 3c). Normal gastric mucosa was
observed between the tumors. Lymph node metastases had occurred around the right gastroepiploic
artery and right gastric artery. Helicobacter pylori infection was not detected in the stomach mucosa.
Discussion
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 [9]. Many classifications for cystic diseases of the kidney have been
proposed. The most accepted among them is the Osathanondh and Potter system that was

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Case Rep Gastroenterol 2011;5:590–596
DOI: 10.1159/000329179
Published online:
October 7, 2011
© 2011 S. Karger AG, Basel
ISSN 1662–0631
www.karger.com/crg



established in 1964, according to which polycystic kidneys are classified into three types
[1]. Potter type III cystic disease involves one or more of several abnormalities. 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, Hatfield and Pfister pathologically examined 399 patients with
multiple cysts in both kidneys [10]. 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 [10], 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 [9]. In these instances, molecular genetic analysis is required. A
family history, however, may be absent in 20–40% 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 [11]. 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.

592
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) [2–8]. 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. [12] 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.
ADPKD is caused by defects in PKD-1 or PKD-2 [13]. 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
[14]. 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 [14]. Moreover, PKD-1 inhibits cancer cell
migration and invasion and may be considered a new member of the tumor suppressor
family of genes [15]. These molecular studies suggested that PKD genes inhibit cancer
progression. 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,

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Case Rep Gastroenterol 2011;5:590–596
DOI: 10.1159/000329179
Published online:
October 7, 2011
© 2011 S. Karger AG, Basel
ISSN 1662–0631
www.karger.com/crg



as mutations in these genes have not been found in all families, undiscovered genes
responsible for causing APLD probably still exist [13].

593
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.
Disclosure Statement
None of the authors has any conflict of interest.



Table 1. Malignant neoplasms associated with polycystic disease of the liver and kidney
Case Refer-
ence
First
author
Year Age Sex Family
history
Location of cyst Hemo-
dialysis
Neoplasms Treatment for
neoplasms
1
2
3
4
5
6
7
8
9
2
3
3
4
5
6
7
8
o.c.
Cryer
Landais
Landais
Niv
Sakurai
Sasaki
Naitou
Sato
Mimatsu
1977
1984
1984
1997
2001
2002
2005
2009
2011
66
52
56
68
63
60
43
63
65
F
F
M
M
M
M
M
M
M
PKD
ND
PKD
PKD
PKD/PLD kidney/liver
PKD
PKD
none
ND
kidney/liver
kidney/liver
kidney/liver
kidney/liver
ND
done
done
not done panc. cystadenocarcinoma
not done panc. ductal adenocarcinoma
done IHCC
ND IPMT
not done IPMN
multiple gastric carcinomas
panc. adenocarcinoma
chorangiocarcinoma
chorangiocarcinoma
operation
none
none
none
none
none
operation (PD)
operation (PD)
operation (G)
kidney/liver
kidney/liver/panc.
kidney/liver/panc.
kidney/liver/mesen. done
G = Gastrectomy; IHCC = intrahepatic cholangiocarcinoma; IPMN = intraductal papillary mucinous neoplasm; IPMT = intraductal papillary
mucinous tumor; mesen. = mesenterium; ND = not described; o.c. = our case; panc. = pancreas; PD = pancreaticoduodenectomy; PKD = polycystic
kidney disease; PLD = polycystic liver disease.

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Case Rep Gastroenterol 2011;5:590–596
DOI: 10.1159/000329179
Published online:
October 7, 2011
© 2011 S. Karger AG, Basel
ISSN 1662–0631
www.karger.com/crg




594

Fig. 1. Abdominal computed tomography scan showed multiple cysts in the liver (a) and cystic lesions
on both sides in the kidney (b).



Fig. 2. Operative findings showed the presence of multiple cysts in the liver (a) and mesenterium (b).