Multiple Gastric Carcinomas Associated with Potter Type III Cystic Disease

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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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Most cited references 15

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Autosomal dominant polycystic kidney disease.

Vicente Torres, Peter Harris, Yves Pirson (2007)

Autosomal dominant polycystic kidney disease is the most prevalent, potentially lethal, monogenic disorder. It is associated with large interfamilial and intrafamilial variability, which can be explained to a large extent by its genetic heterogeneity and modifier genes. An increased understanding of the disorder's underlying genetic, molecular, and cellular mechanisms and a better appreciation of its progression and systemic manifestations have laid out the foundation for the development of clinical trials and potentially effective treatments.

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Diagnostic approach in autosomal dominant polycystic kidney disease.

York Pei (2006)

Autosomal dominant polycystic kidney disease (ADPKD) is the most common Mendelian disorder of the kidney and affects all racial groups worldwide. It is characterized by focal development of renal and extrarenal cysts in an age-dependent manner. Typically, only a few renal cysts are detected in most affected individuals before 30 yr of age. However, by the fifth decade of life, hundreds to thousands of renal cysts will be found in the majority of patients. ADPKD is genetically heterogeneous. Mutations of two genes, PKD1 and PKD2, account for approximately 85 and 15% of cases, respectively. Although the clinical manifestations of these two genotypes overlap completely, patients with PKD1 have much more severe renal disease compared with those with PKD2, as evidenced by their ESRD occurring approximately 15 yr earlier. Renal ultrasonography commonly is used for the assessment of ADPKD, and age-dependent ultrasound diagnostic criteria with high sensitivity and specificity have been established for individuals who are born with 50% risk for PKD1. Although these diagnostic criteria are used widely for genetic counseling and for the evaluation of at-risk individuals as living-related kidney donors to their affected relatives, their application to individuals who are at risk for PKD2 or have undefined genotype needs to be refined further. Molecular genetic testing is available for ADPKD and may be useful for evaluation of at-risk individuals with equivocal imaging results, younger at-risk individuals as a living-related kidney donor, and individuals with atypical or de novo renal cystic disease.

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PKD1 inhibits cancer cells migration and invasion via Wnt signaling pathway in vitro.

Ke-Ke Zhang, Yan-Chun Ye, Qin Zhou … (2015)

The approximately 14 kb mRNA of the polycystic kidney disease gene PKD1 encodes a large ( approximately 460 kDa) protein, termed polycystin-1 (PC-1), that is responsible for autosomal dominant polycystic kidney disease (ADPKD). The unique organization of its multiple adhesive domains (16 Ig-like domains/PKD domains) suggests that it may play an important role in cell-cell/cell-matrix interactions. Here we demonstrated that PKD1 promoted cell-cell and cell-matrix interactions in cancer cells, indicating that PC-1 is involved in the cell adhesion process. Furthermore in this study, we showed that PKD1 inhibited cancer cells migration and invasion. And we also showed that PC-1 regulated these processes in a process that may be at least partially through the Wnt pathway. Collectively, our data suggest that PKD1 may act as a novel member of the tumor suppressor family of genes. Copyright (c) 2007 John Wiley & Sons, Ltd.

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Author and article information

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Journal ID (nlm-ta): Case Rep Gastroenterol

Journal ID (publisher-id): CRG

Title: Case Reports in Gastroenterology

Publisher: S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.ch )

ISSN (Electronic): 1662-0631

Publication date Collection: Sep-Dec 2011

Publication date (Electronic): 7 October 2011

Publication date PMC-release: 7 October 2011

Volume: 5

Issue: 3

Pages: 590-596

Affiliations

[1] ^aDepartment of Surgery, Social Insurance Yokohama Central Hospital, Yokahama

[2] ^bDepartment of Surgery, Nihon University School of Medicine, Tokyo, Japan

Author notes

*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@ 123456yokochu.jp

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Publisher ID: crg0005-0590

DOI: 10.1159/000329179

PMC ID: 3219483

PubMed ID: 22110420

SO-VID: f67cb830-afad-4f3c-a374-a3756bd42e17

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This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial-No-Derivative-Works License ( http://creativecommons.org/licenses/by-nc-nd/3.0/). Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on http://www.karger.com and the terms of this license are included in any shared versions.

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Multiple Gastric Carcinomas Associated with Potter Type III Cystic Disease

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Karger: Gastroenterology

Most cited references 15

Autosomal dominant polycystic kidney disease.

Diagnostic approach in autosomal dominant polycystic kidney disease.

PKD1 inhibits cancer cells migration and invasion via Wnt signaling pathway in vitro.

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