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Lymphangiomas are rare congenital benign tumors arising from the lymphatic system. The incidence of this disease in the pancreas is extremely rare, accounting for less than 1% of these tumors. Before introducing the review we reported a case of a 67-year-old woman with cystic lymphangioma of the pancreas. We reported the radiological investigations carried out preoperatively and the treatment performed. The review tries to identify the features described in literature of the pancreatic lymphangioma. We have performed a PubMed research of the world literature between January 1 st 2000, to November 31 st 2017, using the keywords [Lymphangioma pancreas], [diagnosis], [CT lymphangioma] and [MRI lymphangioma]. We have found 158 articles, of which about 100 were case reports. Based on our search criteria, we have identified 31 pancreatic lymphangioma in literature reporting their imaging characteristics. According to our report and to several authors in literature the diagnosis of cystic pancreatic lymphangioma should be considered as a differential diagnosis of pancreatic cystic lesions (PCLs). The role of imaging exams (CT and MRI) can help to identify and suspect this possibility of diagnosis. The endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) can have a potential role to reach the correct diagnosis.

Cystic lymphangioma of the pancreas: a hard diagnostic challenge between pancreatic cystic lesions—review of recent literature