Introduction
- Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common hereditary renal disorders characterized by varied manifestations including renal cysts, extrarenal cysts, intracranial aneurysms and dolichoectasias, aortic root dilatation and aneurysms, mitral valve prolapse, and abdominal wall hernias
- Approximately one per 800-1000 population carries a mutation for this condition.
- There is established association between ADPKD patients with ESRD and Renal cell carcinoma
- The association of liver, colon and renal cancer with polycystic kidney disease without end-stage renal disease has also been reported
- But only a few cases of gastric carcinoma happening in ADPKD patients have been reported.
Clinical Presentation
- The patient was a 55-year- old male whose chief complaints were abdominal pain, vomiting, jaundice and weight loss.
- He was a known case of Autosomal dominant polycystic kidney disease.
- His father also had Kidney problems and elder brother also is a k/c/o ADPKD.
Investigations
- Abdominal imaging studies showed bilateral polycystic kidneys, an irregular asymmetrical circumferential wall thickening of pylorus for a length of 5.6 cm with obstruction of the proximal bile duct and dilated intrahepatic biliary radicles.
Intraoperative Findings
- A mass arising from pylorus was seen infiltrating into the lesser omentum along with involvement of porta hepatis lymph node with fibrosis and stricture at the confluence of hepatic ducts.
- However no compression or involvement of distal CBD was observed.
- Hence, the malignant stricture and fibrosis at the hepatic duct confluence was inferred as the cause for obstructive jaundice
Surgical Management
- Roux en y hepaticojejunostomy, Subtotal gastrectomy, gastrojejunostomy, cholecystectomy and jejunojunostomy were done.
Histopathological Report
- HPE revealed a well differentiated adenocarcinoma of the stomach. The duodenal stump and resected margins were free of tumour. Samples of fibrotic tissue from the site of obstruction at the confluence of hepatic ducts were negative for tumour infiltration as well.
Postoperative Period
- Patient recovered uneventfully from surgery and the serum bilirubin reduced after which he was referred to medical oncology for chemotherapy.
ADPKD and Adenocarcinoma of Stomach
- 4 cases of Carcinoma stomach were found in literature review which were reported in association with ADPKD.
- One of the case reports reported siblings with ADPKD, both developing adenocarcinoma of stomach.
References
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Autosomal Dominant Polycystic Kidney Disease in the Same Family: KAYA, AKDAĞ, COŞKUNOĞLU, TÜRKELİ, YILMAZ. Turkiye Klinikleri J Med Sci 2012;32(6):1796-800
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