Xanthogranulomatous pancreatitis -a rare case defying clinical, radiological and tumor marker diagnostics with a review of literature

Abstract

Xanthogranulomatous inflammation of the pancreas is an extremely rare entity mimicking pancreatic neoplasia. Elevation of tumor markers has seldom been reported in xanthogranulomatous inflammation of the pancreas, rendering the diagnosis difficult and highlighting the importance of a meticulous histopathological examination. A 58-year-old male presented with severe abdominal pain and anorexia for six months. A heterogeneously enhancing solid cystic mass was detected in the pancreatic head on contrast enhanced computed tomography. Endoscopic ultrasound guided aspiration yielded fluid showing elevated levels of CA19-9 (12000 IU/ml) and CEA (221ng/ml) with a concordant increase in the serum tumour marker levels (CA 19-9:407 IU/ml; CEA: 70 ng /ml). Clinical, radiological and biochemical evidence favouring malignancy prompted a whipple's procedure. A thorough histopathological examination of the specimen failed to reveal malignancy. Sheets of macrophages (CD 68 +, hAM 56 +), chronic inflammatory cells, fibroblasts and proliferating capillaries replacing most of the pancreatic parenchyma were seen instead, nailing the diagnosis of xanthogranulomatous inflammation of the pancreas. To the best of our knowledge this is the first report of xanthogranulomatous inflammation of the pancreas without associated malignancy displaying an elevation of serum and fluid tumour markers. After seven months of follow up, the patient is asymptomatic and is doing well.

Xanthogranulomatous pancreatitis: A rare entity in the spectrum of pancreatic lesions, a case report

“…It is characterized by the deposition of foamy histiocytes in the pancreatic parenchyma, along with the proliferation of fibroblasts and other inflammatory cells, and may also involve necrosis and hemorrhage [ 2 ]. XGP typically affects men more frequently and presents around the age of 60; however, in our case, the patient was a young adult woman [ 1 ]. Abdominal pain is the most common symptom, as reported in this case and in other studies [ [1] , [2] , [3] ].…”

“…Xanthogranulomatous pancreatitis (XGP) is a rare form of chronic pancreatitis characterized by the deposition of numerous foamy histiocytes in the pancreatic parenchyma, along with other inflammatory cells, cholesterol, and fibroblastic proliferation [ 1 ]. The etiology of XGP is currently unknown, but it is suspected to involve a combination of ductal obstruction, infection, and repeated intraductal bleeding [ 2 ].…”

“…To date, in our knowledge there are 15 cases that have been reported in the literature up to 2022 ( Table 1 ). In all of these cases, lesions that mimicked malignancy in imaging studies such as CT scans or magnetic resonance imaging (MRI) were discovered, leading to subsequent surgical treatment [ 1 , [3] , [4] , [5] ]. Differential diagnoses include pancreatic pseudocyst, cystic mucinous neoplasm, and solid pseudopapillary neoplasm [ 1 , 2 ].…”

Xanthogranulomatous Pancreatitis Accompanied by Xanthogranulomatous Cholecystitis

“…In total, 56 articles were obtained, of which 17 articles reported the 31 cases of XGP (Table 1). 1,3,[8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] To the best of our knowledge, this is the first report of XGP accompanied with xanthogranulomatous cholecystitis and the fourth case of XGP with IPMN. Xanthogranulomatous inflammation can extend into the adjacent surrounding structure; therefore, there are a lot of reports of XGI directly infiltrating multiple close organs.…”

Xanthogranulomatous pancreatitis combined with multifocal necrosis: A case report and literature review

“…Kwon [20] 60/M Pancreas MASS Korea Body − CEA + NA Low-attenuated ↓T1, ↑T2 uncertain Solid DP 1.6 × 1.5 Navarro et al [10] 86/F Epigastric pain and weight loss Becker et al [21] 56/M Prostate adenocarcinoma American Tail − CEA + Heterogeneously hypoechoic Hypoattenuating ↓T1, ↑T2 IPMN Cystic DP + S 2 . 8× 2.5 Gaur et al [22] 58/M Abdominal pain and weight loss not experience any recurrence. This case is reported in accordance with the SCARE 2018 standard [1] .…”