DX: Xanthogranulomatous Cholecystitis

This was a biopsy proven case that wasn't on my first diagnosis arsenal as a differential.

US gave me the impression  that a bile stone was "impacted" in gallbladder.

CT findings were: focal gallbladder wall thickening with pseudo-impingement at fundus, with no adjacent stones only near the neck. It had IV contrast enhancement. No liver lesions.

Imaging characteristics were not as conclussive as wanted but there was a neoplasic component for sure. I reccomended to correlate with tumor markers especially Carbohydrate Antigen 19-9 (CA 19-9). Results were abnormal so, Gallbladder Carcinoma was the clinical-radiological diagnosis.

She went to surgery and a Cholecytectomy was performed. Biopsy of the specimen was reported as Xanthogranulomatous Cholecystitis (XGC).

XGC is a rare inflammatory disease of the gallbladder characterized by a focal or diffuse destructive inflammatory process, with accumulation of lipid laden macrophages, fibrous tissue, and acute and chronic inflammatory cells. In 1970, it was known by the descriptive term fibroxanthogranulomatous cholecystitis, but in 1981 the name xanthogranulomatous cholecystitis was proposed in a review of 40 cases from the Armed Forces Institute of Pathology. Its importance lies in the fact that it is a benign condition that may be confused with carcinoma of the gallbladder, which is associated with a poor prognosis.

XGC was initially described as a variant of chronic cholecystitis. However, while the latter is usually regarded as a benign condition with questionable clinical significance, xanthogranulomatous cholecystitis is an active and destructive process that can lead to significant morbidity as the inflammatory process usually extends into the gallbladder wall and adjacent structures. Thus, it should be considered a distinct clinical entity.

The pathogenesis of XGC is thought to be related to extravasation of bile into the gallbladder wall from rupture of Rokitansky-Aschoff sinuses or by mucosal ulceration. This event incites an inflammatory reaction in the interstitial tissue, whereby fibroblasts and macrophages phagocytose the biliary lipids in bile, such as cholesterol and phospholipids leading to the formation of xanthoma cells.

Gallstones may have an important role in the pathogenesis, since they appear to be present in all patients. It has been suggested that xanthogranulomatous cholecystitis is analogous to xanthogranulomatous pyelonephritis, which results from obstruction and stasis due to renal calculi.

 A few recent reports have shown a possible association of this disease with carcinoma of the gallbladder.
The inflammatory process often extends into neighboring organs, such as the liver, omentum, duodenum, and colon. The clinical importance of XGC lies in the fact that it can be confused radiologically with a gallbladder carcinoma.

Several reports demonstrated the radiological features of XGC. However, as some are nonspecific, it is often difficult to distinguish XGC from gallbladder carcinoma by the conventional imaging techniques of  ultrasonography, CT and MRI. Moreover, the fact that XGC can infrequently be associated with gallbladder carcinoma makes the differentiation more difficult.

http://www.uptodate.com/contents/xanthogranulomatous-cholecystitis
http://www.ajronline.org/content/148/4/727.long
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2721248/pdf/WJG-15-3691.pdf
http://www.surgpath4u.com/caseviewer.php?case_no=660&view=yes&h=&w=&fs=



Hope it was helpful as it was to me..until next time...