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

## TL;DR

Xanthogranulomatous pancreatitis (XGP) is a rare benign pancreatic condition often mistaken for cancer, requiring careful diagnosis to avoid unnecessary surgery.

## Contribution

This case report highlights the diagnostic challenges of XGP and advocates for echoendoscopy with biopsy as a more accurate alternative to major surgery.

## Key findings

- XGP is frequently misdiagnosed as pancreatic neoplasm due to non-specific imaging findings.
- Echoendoscopy with biopsy can improve diagnostic accuracy and avoid unnecessary Whipple procedures.
- XGP is associated with obstruction of the pancreatic duct by mucin, leading to chronic inflammation.

## Abstract

Xanthogranulomatous pancreatitis (XGP) is a rare, benign, and idiopathic disease that often presents with non-specific symptoms and can mimic or coexist with other pancreatic diseases. Despite its infrequency, XGP is frequently misdiagnosed as a pancreatic neoplasm, with only 15 reported cases in the literature. The pathogenesis of XGP remains unclear.

We present the case of a 34-year-old woman with no pathological history who experienced continuous abdominal pain and oral intolerance, without signs of cholestasis. An abdominal CT scan initially suggested a cystic neoplasm of the pancreas, leading to a laparotomic cephalic duodenopancreatectomy. The anatomopathological study and immunohistochemistry revealed XGP in association with a mucinous cystic neoplasm with mild to moderate atypia. The patient remained hospitalized for six days post-surgery without any complications.

XGP may be induced by the inflammatory reaction secondary to the obstruction of the pancreatic duct by mucin. The etiology is unknown, but it is attributed to a combination of obstruction, hemorrhage, or ductal infection. Abdominal pain is the most common symptom. Differentiating XGP from malignant processes of the pancreatic gland is challenging. Surgical treatment typically involves the Whipple procedure; however, echoendoscopy with biopsy is now available for a more accurate and early differential diagnosis.

XGP is a rare and challenging differential diagnosis for pancreatic neoplasms. Due to its potential to mimic malignant lesions, a high index of suspicion is necessary. Echoendoscopy with fine-needle aspiration biopsy should be considered a routine diagnostic tool before major surgery, such as the Whipple procedure.

•Xanthogranulomatous pancreatitis (XGP) is a rare form of chronic inflammation of the pancreatic duct resulting from its obstruction by mucin.•Abdominal pain is the most common symptom. Other less frequent symptoms include weight loss, acute pancreatitis, or jaundice.•The diagnosis is usually challenging because imaging studies can confuse this benign entity with a malignant process due to the absence of pathognomonic characteristics•XGP is documented in deferred anatomopathological and immunohistochemistry studies.•Given its potential to mimic malignant lesions, a high index of suspicion is necessary.•Surgical treatment typically involves the Whipple procedure.

Xanthogranulomatous pancreatitis (XGP) is a rare form of chronic inflammation of the pancreatic duct resulting from its obstruction by mucin.

Abdominal pain is the most common symptom. Other less frequent symptoms include weight loss, acute pancreatitis, or jaundice.

The diagnosis is usually challenging because imaging studies can confuse this benign entity with a malignant process due to the absence of pathognomonic characteristics

XGP is documented in deferred anatomopathological and immunohistochemistry studies.

Given its potential to mimic malignant lesions, a high index of suspicion is necessary.

Surgical treatment typically involves the Whipple procedure.