A Case Report of Laparoscopic Distal Gastrectomy for Gastric Cancer with Autosomal Dominant Polycystic Kidney Disease and Multiple Liver Cysts

Abstract

Introduction

Here, we report a case of gastric cancer associated with autosomal dominant polycystic kidney disease (ADPKD).

Case Presentation

A 73-year-old woman with chronic renal failure due to polycystic kidneys, who was undergoing hemodialysis treatment three times a week for 1 month, was admitted to our hospital because of anemia. She reported that her mother had suffered from polycystic kidney disease. Gastrointestinal fiberscopy revealed a type 1 cancerous lesion in the lesser curvature of the antrum. Histopathological analysis of gastric tumor biopsy specimens revealed differentiated tubular adenocarcinoma. Abdominal computed tomography revealed multiple cysts in the liver, cystic lesions on both kidneys, and thickening of the gastric wall. A laparoscopic distal gastrectomy with lymph node dissection was performed.

Conclusion

Reports of the association between gastric cancer and ADPKD are exceptionally uncommon. Especially, no cases of laparoscopic distal gastrectomy for gastric cancer with ADPKD have been previously reported. To our knowledge, this is the first report of laparoscopic surgery for gastric cancer with ADPKD and multiple liver cysts.

Introduction

Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent hereditary renal cystic disease and has various external manifestations, including cysts in the liver, seminal vesicles, pancreas, and arachnoid membrane; intracranial aneurysms and dolichoectasias; aortic root dilatation and aneurysms; mitral valve prolapse; and abdominal wall hernias. Despite ongoing research, no pharmacological therapy has been proven to effectively delay disease progression, and approximately half of the patients develop end-stage renal disease by the age of 60 years and require renal replacement therapy. ADPKD is caused by genetic mutations in PKD1 (encoding polycystin-1; PC1, accounting for 85% of all cases) or PKD2 (encoding polycystin-2; PC2, accounting for the remaining 15%). To date, there have been some reports describing the association between gastric cancer and ADPKD [1–6]; however, most of them describe treatment with best supportive care and chemotherapy, and only four cases involved surgery. To date, there have been no reports of laparoscopic gastrectomy in patients with ADPKD and multiple hepatic cysts. To our knowledge, this is the first such report.

Case Report

A 73-year-old woman with anemia was admitted to our hospital. She was diagnosed with ADPKD at 41 years of age. She had been receiving hemodialysis treatment three times a week for 1 month because of chronic renal failure due to polycystic kidneys. She had hypertension, which was well controlled with oral medication, and a history of via open surgery for colonic carcinoids 13 years prior. She reported that her mother had suffered from polycystic kidney disease. Upon admission, her vital signs showed blood pressure of 148/88 mm Hg, heart rate 98 beats/min, respiratory rate 18 breaths/min, oxygen saturation 98%, and temperature 37.3°C. Her palpebral conjunctiva was slightly pale. Her abdomen was soft upon palpation; however, mid-abdominal swelling due to polycystic disease was detected. Laboratory examinations revealed hemoglobinemia (10.7 g/dL) and normal levels of serum total bilirubin (0.4 mg/dL), aspartate aminotransferase (21 IU/L), and alanine aminotransferase (14 IU/L). Renal function was impaired, with elevated blood urea nitrogen (40 mg/dL) and creatinine (4.19 mg/dL). The patient also had a high serum carbohydrate antigen 19-9 level (107 U/mL). Abdominal computed tomography showed multiple cysts on the liver, cystic lesions on both sides of the kidney (Fig. 1a), and thickening of the gastric wall (Fig. 1b). No collateral circulation due to portal hypertension was identified. Gastrointestinal fiberscopy revealed a type 1 cancerous lesion in the lesser curvature of the antrum (Fig. 1c). Histopathological analysis of gastric tumor biopsy specimens revealed differentiated and tubular adenocarcinoma. A laparoscopic distal gastrectomy with lymph node dissection was performed. The intra-abdominal space was limited by multiple liver cysts and large renal cysts (Fig. 2). Laparoscopic surgery can be safely performed by arranging the port closer to the midline, taking the patient’s position sufficiently. Additional intraoperative views and surgical steps are presented in online supplementary Figures S1–S3 (for all online suppl. material, see https://doi.org/10.1159/000547068). It seemed more effective to raise the liver with forceps than with a retractor. Special attention was paid to the presence of hepatic cysts, and gentle handling techniques were used to avoid cyst rupture. The gastric tumors were surgically diagnosed to be T1N0M0, stage IA according to the TNM classification for gastric carcinoma. The tumor in the resected gastric specimen measured 25 mm × 15 mm (Fig. 3a). On the basis of histopathological examination, the lesion was diagnosed as a differentiated tubular adenocarcinoma within the submucosa, with no lymphatic invasion (Ly0) or vascular invasion (V0) (Fig. 3b). No lymph node metastasis was observed. The postoperative course was favorable, including the absence of postoperative ascites collection.

Fig. 1.

Abdominal computed tomography scan and endoscopic image at the admission. a Coronal section of computed tomography scan showed multiple cysts in the liver and cystic lesions on both sides in the kidney. b Axial section of computed tomography scan revealed thickening of the gastric wall. c Endoscopic image showed the presence of type 1 cancer lesion in the lesser curvature in the antrum.

Fig. 2.

Laparoscopic operative findings showed the presence of multiple cysts in the liver.

Fig. 3.

Resected specimen and histological findings. a The tumor in the resected gastric specimen measured 25 mm × 15 mm. b HE staining of gastric cancer (×200).

Discussion

ADPKD is the most commonly inherited renal cystic disease and can present with extrarenal cysts in the liver, seminal vesicles, pancreas, and arachnoid membrane [7]. The frequency of occurrence is 1/1,000–1/400 [7] and is the result of genetic mutations in PKD1 (encoding PC1) or PKD2 (encoding PC2) [8]. PC1 is a complex protein that contains E-cadherin and β-catenin, and PC2 is a calcium ion channel-like protein, both of which play roles in organ development and maintenance through cell-cell and cell-extracellular matrix adhesion [9, 10].

Deficiency of these proteins leads to increased epithelial proliferation and apoptosis, especially in distal tubules, due to heightened sensitivity to proliferative stimuli [11]. PC1 suppresses cell invasion, metastasis, and proliferation in hepatocellular carcinoma, lung cancer, and colorectal cancer [12]. Conversely, E-cadherin mutations are associated with poorly differentiated gastric cancers, and scirrhous gastric cancer is more frequent in families with congenital cadherin gene mutations [13]. The present case involved a differentiated tubular adenocarcinoma, but the role of E-cadherin in this context remains unclear. Reports of the association between malignant neoplasms and ADPKD are exceptionally rare. In kidneys, tumor occurrence is associated more with age and pathology than with ADPKD itself [14]. In the liver, malignant cysts can occasionally lead to cholangiocellular carcinoma [15].

To our knowledge, only eight cases of ADPKD-associated gastric cancer have been reported in the available literature (Table 1). The data were obtained upon searching the PubMed and Ichushi-Web databases between 1975 and 2024 using the keywords “gastric cancer” and “polycystic kidney disease” [1–6]. Of these, only four were operable, and there are no previous reports of laparoscopic surgery for gastric cancer in patients with ADPKD and multiple liver cysts. This report is, to our knowledge, the first such case. Distal gastrectomy for gastric cancer with ADPKD is commonly performed via open surgery because of the presence of several bulky liver cysts and large renal cysts. In patients with chronic kidney disease, extensive incisions and impaired wound healing can prolong hospitalization and increase morbidity. However, laparoscopic surgery may be feasible in select cases. In this case, as expected from preoperative CT, the working space in the upper abdomen was narrow because of the large liver cyst, making securing the field of view difficult. Although gastric cancer surgeries are usually performed in the head-up position, the patient’s liver extended below the umbilicus, necessitating a head-down position to ensure visualization. Mild adhesions in the lower abdomen from previous colon carcinoid surgery prevented downward displacement of the small intestine, aiding in field preservation. Multiple positional adjustments were made to improve visibility. An additional port was placed near the cardiac fossa, and the liver was elevated with forceps rather than a retractor due to its sclerotic nature and numerous cysts. Ports were positioned more caudally and laterally than usual. Although the standard insufflation pressure of 10 mm Hg provided adequate space, it was temporarily increased to 12 mm Hg during the procedure, which did not significantly improve visualization. The CARE Checklist has been completed by the authors for this case report and is attached as online supplementary material.

Table 1.

Past case of gastric carcinoma associated with ADPKD (Search in PubMed and Icushi web, 1975–2024)

No.	Authors	Year	Age, years	Sex	Family history of PKD	Hemodialysis	Stage of gastric cancer	Treatment of gastric cancer	Histopathology	Outcome
1	Shekh et al. [1] (1998)	1998	40	M	Sister	Unknown	Unknown	Best supportive care	Intestinal type	Dead
2	Shekh et al. [1] (1998)	1998	50	F	Brother	Unknown	Unknown	Best supportive care	Intestinal type	Dead
3	Tsukayama and Yamamura [2] (2002)	2002	68	M	Mother, brother	Yes	Stage IA	Operation	Intestinal type	Alive
4	Yamanaka et al. [3] (2010)	2010	50	M	Unknown	No	Stage IV	Operation, chemotherapy	Diffuse type	Alive
5	Mimatsu et al. [4] (2011)	2011	65	M	Sister	Yes	Stage II	Operation	Diffuse type	Alive
6	Yasuda et al. [5] (2021)	2021	60	M	Mother, brother	No	Stage IVB	Chemotherapy	Diffuse type	Dead
7	Coco [6] (2023)	2023	70	M	None	Yes	Stage IV	Chemotherapy	Diffuse type	Alive
8	Our case	2024	73	F	Mother	Yes	Stage IA	Operation	Intestinal type	Alive

Statement of Ethics

Ethical approval is not required for this study in accordance with local or national guidelines at our institute. Written and verbal informed consent was obtained from the patient for publication of this case report and all accompanying images.

Conflict of Interest Statement

The authors of this manuscript do not have any conflicts of interest to declare.

Funding Sources

There was no external funding for this case report.

Author Contributions

Yukihiro Kato wrote the entire manuscript, with the co-authors (Hanae Matsuda, Takuya Kikukawa, Atsushi Isihara, Yasunori Matsuda, Seikan Miyashita, Masahiro Nishikawa, and Takahiro Uenishi) all involved in summarizing this case report and proofreading the final version of the manuscript.

Funding Statement

There was no external funding for this case report.

Data Availability Statement

All data generated or analyzed during this study are included in this article and its online supplementary material files. Further inquiries can be directed to the corresponding author.