Cystic lymphangioma of the pancreas Fig. 4. Gross finding of cystic lymphangioma of the pancreas. The surgical specimen is 15 × 10.5 × 7 cm in size, weighing 800 g with a multinodular external surface. The attached pancreas shows atrophy and inflammatory change. The spleen is unremarkable. were found inside the cavity after the procedure. The peri- toneal cavity was irrigated thoroughly with normal saline solution and Jackson-Pratt drains were placed in the left subdiaphragmatic area and in the pelvis. The removed mass had a clearly distinguishable outer membrane, a smooth, polished surface, and signs of swel- ling due to the liquid it contained (Fig. 4). The sectioned specimen contained mildly bloody serous liquids and a large swollen cyst. Small multiseptated cystic masses of various sizes were also seen situated along some of the thicker portions of the cystic wall. Pathologic findings showed that the mass was comprised of a number of mul- tiseptated cystic masses of varying sizes that were wrap- ped with flat lymphatic endothelial cells. Some lymphatic infiltration was found in the mass. However, no con- nection with the pancreatic main duct was observed de- spite the firm attachment between the mass and the pan- creas (Fig. 5). At immunohistochemistry, the endothelial cells that made up the cyst showed a strong positive reaction to CD31 and D2-40 and a mild positive response to CD34. Based on the results of the microscopic and im- munohistochemical findings, a diagnosis was made for a cystic lymphangioma consisting of lymphatic endothelial cells (Fig. 6). The patient recovered uneventfully and was discharged on the 9th postoperative day. At the follow-up examina- thesurgery.or.kr Fig. 5. Microscopic findings. Variably sized multilocular cysts lined by thin attenuated endothelial cells and fibrotic stroma. Attached pancreas shows mild atrophy (H&E, ×40). tion 3 months after surgery, she was doing well. DISCUSSION Cystic lymphangioma of the pancreas was first identi- fied in 1913 by Koch [4] as a form of benign cyst secondary to blocked regional lymphatic ducts. It is extremely rare, accounting for less than 1% of lymphangiomas [3]. Fre- quently occurring in the pancreatic tail, the disease is re- ported more often in females, with a similar incidence across all age groups. Several factors are assumed to cause cystic lymphan- gioma. These include lymphatic abnormalities resulting in children from sequestration of lymphatic tissues during prenatal development and in adults from blockage of lym- phatic ducts due to abdominal trauma, operation, radio- logical treatment, or infection. Cystic lymphangioma can occur in any location where normal lymphatic ducts are found. The most frequently reported sites are the head and neck (75%), the axilla (20%), and other organs (5%). Cystic lymphangioma inside the abdominal cavity is very rare, occurring most frequently in the mesenteric region, and less frequently in the mesocolic, greater omental and ret- roperitoneal regions, small intestinal and gastric walls, and peritoneum [1]. Cystic lymphangioma of the pancreas is particularly rare, with only 60 cases reported so far 143