Xanthogranulomatous cholecystitis


Xanthogranulomatous cholecystitis

Description, Causes and Risk Factors:

Abbreviation: XGC.

Xanthogranulomatous cholecystitis is a rare variant of chronic cholecystitis characterized by severe proliferative fibrosis and accumulation of lipid-laden macrophages in areas of destructive inflammation. The macroscopic appearance generally mimics a gallbladder carcinoma.

The pathogenesis of XGC is obscure, although it has been stated, that acute inflammation and obstruction with stasis, is an important etiologic factor. Its pathogenesis consists of an interplay between obstruction to bile flow with infection and subsequent inflammation associated with leakage of bile to tissues where histiocytes accumulate and phagocytose the bile pigment, hemosiderin and cholesterol, resulting in formation of xanthoma cells.

Another possible cause is chronic infection and gallstones with bile stasis. Some authors have attributed the process to delayed type hypersensitivity reaction. Immunohistochemical studies have demonstrated a closer correlation with bacterial infection in the sub-acute form than in the chronic one.

Normally it presents at 45-60 years of age group with equal incidence in both the sexes with more malignant potential in females.

Symptoms:

Xanthogranulomatous cholecystitis has unusual presentation. It presents mainly with complications such as empyema, pericholecystic abscess, perforation and cholecystoduodenal fistula. Other symptoms such as the recurrent right upper abdominal pain, obstructive jaundice, fever, acute cholecystitis attack, right upper quadrant mass may also present.

Xanthogranulomatous cholecystitis

Diagnosis:

The pre- or intraoperative differential diagnosis of XGC from other gallbladderdiseases remains a challenge in medicalpractice. The presence of firm adhesionsof the gallbladder to neighboring organsand tissues, thickened gallbladder wall,together with gallstone in a patient withchronic disease, is highly suggestive ofXGC.

The common imaging techniques, for example ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI), are able to detect abnormalities of the gallbladder, but they are not always able to differentiate XGC from GB (gallbladder) cancer, and the final diagnosis is usually made by histological examination of the resected specimen.

On? histologic? examination? too,? diagnostic? difficulty can? arise? due? to? presence? of? deep? Rokitansky-Aschoff sinuses and? inflammatory? atypia? in? the? epithelium.? In such? cases,? combined? immunohistochemical? staining with? p53,? PCNA? and? beta-catenin? can? be? of? some? help. In patient with no nuclear beta-catenin staining, or low p53 positivity and low PCNA (proliferating cell nuclear antigen) index score may help clear the ambiguity in diagnosis XGC. This can prevent a patient from excessive surgery.

Treatment:

The treatment of choice would be surgery. An accurate preoperative diagnosis with a careful surgical planning is very important in the management of XGC. The severe inflammatory process with dense adhesions and edematous tissues distort the anatomy. Firm adhesions of the gallbladder to neighboring organs and tissues are common and lead to difficulty in surgical treatments. The mode of operation depends on specific conditions in varying cases, and since frozen-section examination plays an important role in determining the nature of the lesions, intraoperative frozen-section examination should be carried out to differentiate XGC from carcinoma of the gallbladder.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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