RESUMO
CONTEXT: Cystic-cavernous lymphangioma is a rare cystic tumor especially for adults and pancreas. CASE REPORT: We reported a case of a 33-year-old woman who presented with a visible and palpable abdominal mass found to be a huge lymphangioma of the pancreas. An abdominal magnetic resonance imaging (MRI) showed a multiloculated, lobulated T1 hypo/hyper, T2 hyperintense cystic mass extending from right subhepatic space to the pelvis measuring 155x167x100 mm. A pancreaticoduodenectomy was performed encompassing the distal stomach and a segment of the transverse colon, because of their close, inseparable relationship to the mass. The cystic mass was histopathologically diagnosed as partly cavernous and partly cystic lymphangioma. CONCLUSION: To our knowledge this is the first case of pancreatic lymphangioma requiring additional organ resection besides a standard pancreaticoduodenectomy. To reduce recurrences, we recommend a complete resection for this pathology, even though its benign nature.