Abstract
Pancreatic cystic lymphangiomas are rare benign lesions that arise from lymphatic vessels, accounting for less than 0.2% of all pancreatic cysts. Typically, these lesions are found incidentally on a study such as CT or MRI of the abdomen, and patients are usually asymptomatic. Herein, we report a case of pancreatic cystic lymphangioma diagnosed in a 43-year-old female. A 43-year-old female presented to the emergency department with intermittent abdominal pain. An MRI of the abdomen revealed a well-circumscribed 3.9 cm x 2.3 cm x 1.1 cm homogeneously T2 hyperintense exophytic multi-cystic mass arising from the uncinate process of the pancreas. The patient underwent an endoscopic ultrasounds (EUS), which revealed a large, irregular, cystic lesion that originated from the uncinate process. Subsequent aspiration of pancreatic cyst fluid demonstrated milky-white fluid with fluid amylase level of 152 U/L and CEA 0.8 ng/ml. Cytology predominately revealed lymphocytes, with rare macrophages and sheets of benign glandular cells. No malignant cells were identified. The diagnosis of pancreatic cystic lymphangioma was made. Based on the tumor size, a multidisciplinary decision was made against surgical resection and only follow-up surveillance was recommended. A 1-year follow-up with CT of the abdomen indicated no change in the size of the pancreatic cyst. Pancreatic cystic lymphangioma is a rare benign type of cystic pancreatic lesion, described first by Koch in 1913. It is more frequently found in females, affecting all age groups with the same incidence. Usually a patient is asymptomatic when the lesion is incidentally noted through an imaging study. EUS is an excellent modality to characterize the lesion and EUS-FNA allows cystic fluid analysis to increase diagnostic certainty. The characteristic appearance of a chylous, milky white aspirate fluid with a high triglyceride level is pathognomonic. Considering that lymphangiomas are benign lesions, a conservative approach with close follow-up is reasonable if definitive diagnosis is made by EUS. Complete surgical resection is curative in symptomatic patients or in patients with locally invasive cysts. In conclusion, lymphangiomas should be considered in the differential diagnosis of pancreatic cysts. They can be accurately diagnosed by EUS with FNA, allowing conservative management in selected patients.