GIANT PANCREATIC LYMPHANGIOMA HERNIATING ABOVE THE DIAPHRAGM

Objectives: Lymphangiomas of pancreatic origin are uncommon and often do not require resection. Many of these tumors are found incidentally, while some present with abdominal symptoms such as pain and vomiting. Here, we report a case of a pancreatic lymphangioma that herniated through the esophageal hiatus.
A 46-year-old female presented to our emergency department with one day of exquisite epigastric abdominal pain and vomiting. She also endorsed loose stools and recent history of chills, night sweats, and twenty-pound weight loss in the last three months. Her bloodwork was significant for leukocytosis. Computed tomography of the abdomen showed a cystic septated mass of the distal pancreas with a small enhancing nodule measuring 10.6 x 11.4 x 25.4 cm occupying a large space in retroperitoneum and extending superiorly into the mediastinum via a hiatal hernia. Gastroenterology was consulted and performed an esophagogastroduodenoscopy (EGD) with endoscopic ultrasonography (EUS) to determine the extent of involvement of the posterior wall of the stomach and esophagus, since the cystic mass was bordering those organs. On EUS, the mass could be seen abutting and completely surrounding the esophagus, but did not demonstrate any invasion into the esophagus or stomach.
In light of her recent weight loss prior to presentation, the decision was made to initiate tube feeding for several days to improve her nutritional status prior to surgery. Given the extent of the mass and her acute presentation, she was taken to the operating room for an exploratory laparotomy with retroperitoneal mass excision, distal pancreatectomy, splenectomy, and hiatal hernia repair with Dor fundoplication. The pathology report showed a cystic lymphangioma encasing the distal pancreas, measuring 15.0 x 14.5 x 7.5 centimeters. The size and volume of the specimen was likely decreased due to entry into the cyst during the case. Histologic analysis revealed that the stains for SMA, CD31, and D2-40 were positive. Immunohistochemical stains for keratin, calretinin, HMB45, and CD117 were all negative. She received the standard post-splenectomy vaccinations and after tolerating a pureed diet, she was discharged on postoperative day 3. She has since followed up in our clinic, tolerating a regular diet and with no further episodes of abdominal pain.
This illustrates the first case, to our knowledge, of pancreatic lymphangioma extending beyond the abdominal cavity. These tumors can grow significantly in size and even herniate through available defects; it is important to recognize that this can affect the clinical presentation.

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