Diffuse Xanthogranulomatous Cholecystitis: Master of Disguise

Abstract

A 67-year-old woman presented with clinical symptoms, radiological findings, and preoperative work-up highly suggestive of advanced stage IV carcinoma of the gallbladder (CG). An extended cholecystectomy with the excision of adjacent liver segments and loco-regional lymphadenectomy was performed. Final pathology results revealed diffuse xanthogranulomatous cholecystitis (XG) with ruptured Rokitansky-Aschoff sinuses with tumor-resembling adenomyosis without atypical or malignant cells. There was a reactive inflammatory and fatty degeneration of the adjacent hepatic tissue and a nonspecific inflammatory reaction of the enlarged periportal lymph nodes. The main concern in the management of patients with mass-forming XG is that this benign condition shares strikingly similar clinical, imaging, biochemical, and intraoperative features with advanced CG, which has one of the poorest overall survival rates. Misdiagnosis is not uncommon, which causes significant distress for patients and their families and, in some cases, may result in erroneous treatment. Although the presence of some preoperative imaging findings and/or intraoperative frozen section biopsies may be helpful in suspecting XG, definitive diagnosis is usually delayed until the final pathology result that may come as a surprise. Increasing awareness of this rare, insidious disease will contribute to a better understanding of its biology and natural history and, eventually, help improve management.

Xanthogranulomatous Cholecystitis: Our Clinical Experience

“…Rupture of Rokitansky-Aschoff sinuses, small mucosal ulcerations, gallstones, and presence of bacteria in the bile eliminated by macrophages may play a role in this serious inflammation. Furthermore, XGC has benign histological features but a severe clinical picture; in addition, it is clinically important due to difficulty in its differentiation from gallbladder cancer (6).…”

A unique case of xanthogranulomatous cholecystitis complicated by multiple liver abscesses and portal vein and hepatic artery thrombosis and occlusion

“…XGC is a poorly understood and rare gallbladder disease that can mimic gallbladder cancer. There are several theories about pathophysiology, but the most widely cited one hypothesizes that increased intraluminal pressure in the gallbladder causes extravasation of bile into the gallbladder wall which is then taken up by activated histiocytes and fibroblasts causing a granulomatous reaction followed by cellular immune response and fibrosis [4, 5].…”

“…Establishing the correct diagnosis of XGC remains challenging, especially when the inflammatory process involves the surrounding tissues, the diagnoses is frequently delayed until the final pathology results [4]. Waskin et al [6] studied multi detector CT as an imaging modality to detect XGC and showed a moderate sensitivity but poor specificity for differentiation of XGC from gallbladder cancer.…”

“…Although it is found in 1–6% of cholecystectomies worldwide, the etiology of the disease is not well-known [3]. In the most severe cases, inflammation and fibrosis may extend into the adjacent tissues mimicking advanced gallbladder cancer which makes diagnosis and management of this disease challenging [4]. We present a unique case of XGC which was characterized by inflammatory involvement of surrounding organs causing portal vein involvement and occlusion, occlusion of secondary biliary radicles and multiple liver abscess concerning for extrahepatic cholangiocarcinoma versus gallbladder cancer.…”

Xanthogranulomatous Cholecystitis Mimicking Carcinoma Gallbladder

“…Although XGC is not an exceptional fnding, direct involvement of extra-gallbladder organs/multivisceral structures like the colon, duodenum, and right hepatic artery is very rare, with only a few cases reported in the literature [11][12][13][14].…”