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Volume 88 - 2025 - Fasc.1 - Case reports

Pancreatic cystic lymphangioma in an adult: a rare clinical entity

We report the case of a 24-year-old patient with abdominal discomfort and weight loss. On imaging, a large unilocular cystic lesion originating from the pancreatic body and tail was seen. Endoscopic fine needle aspiration revealed a slightly elevated lipase, suggesting a connection with the pancreatic duct. Furthermore, the liquid appeared chylous, and markedly elevated triglycerides were noted. A transgastric drainage and biopsies of the cyst wall were performed. The diagnosis of a pancreatic cystic lymphangioma was made. Our patient was successfully managed conservatively by an echoendoscopic transgastric drainage.

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An unusual hidden secret of a Meckel’s diverticulum: a rare case of small bowel adenocarcinoma

A Meckel’s diverticulum (MD) is the most prevalent congenital anomaly of the gastro-intestinal tract and the life-long risk for complications is estimated to be around 4%, mostly resulting in bleeding, obstruction, diverticulitis or intussusception. Although rare, about 3.1-5.1% of the complications are due to malignant evolution of the MD. Case: We present a 50-year old patient with progressive subobstructive symptoms leading the diagnosis of a complicated MD. Peroperative findings were suggestive for malignancy and the histopathological samples confirmed the presence of a metastatic adenocarcinoma, arising from the MD. Conclusion: This case illustrates a rare, malignant complication of a MD. This finding is of utmost clinical importance because the diagnosis may be challenging and early detection may improve the outcome of these patients. The clinician should be aware that malignant evolution of a MD exists and should be excluded in complicated cases.

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Unexplained portal hypertension and confusion in an elderly patient: a late presentation of congenital hepatic fibrosis

A 71-year old male patient with myelodysplastic syndrome presented with severe signs of portal hypertension. There was no underlying cirrhosis nor portal vein thrombosis. Despite liver stiffness being high, hepatic vein catheterisation failed to show an increased hepatic venous pressure gradient compatible with clinically significant portal hypertension. Finally, a liver biopsy showed enlarged fibrotic portal tracts with multiple dilated immature bile ducts compatible with congenital hepatic fibrosis – there was no macroscopic biliary disease. Mostly presenting in childhood or adulthood, congenital hepatic fibrosis is an uncommon cause of non-cirrhotic portal hypertension that can also have a late presentation. Treatment is supportive with management of portal hypertension (and its complications); liver transplantation is curative but is reserved for cases with liver failure or recurrent cholangitis (mostly in Caroli syndrome).

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An unusual case of acute cholestatic hepatitis: visceral leishmaniasis with secondary haemophagocytic lymphohistiocytosis

Visceral leishmaniasis (VL) is a severe vector-borne parasitic disease, endemic in various tropical countries but also in the Mediterranean Basin. We report the case of a 64-year-old immunocompetent male, who presented with jaundice, fever, splenomegaly, hyperferritinaemia and cytopenias after a recent trip to Portugal. Clinical and biochemical findings were consistent with the diagnosis of an acute cholestatic hepatitis and haemophagocytic lymphohistiocytosis (HLH), secondary to visceral Leishmania infantum which was later confirmed by bone marrow examination. Despite adequate therapy with liposomal amphotericin B, the disease and HLH relapsed. Retreatment with higher dosing was eventually successful. This case highlights the risk of acquiring VL in southern Europe and its possible unusual presentations, causing diagnostic delay, as well as its complex management that requires appropriate multidisciplinary communication.

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