Huge Cystic Lymphangioma of the Pancreas: Report of a Case

Abstract

We report herein the case of a 33-year-old woman who presented with palpable abdominal swelling found to be caused by a huge lymphangioma of the pancreas. An abdominal computed tomographic (CT) scan showed a large multilocular cystic mass with water-dense contents, which was derived from the pancreatic head. A pancreaticoduodenectomy (PD) was performed because the tumor had invaded the duodenum. The resected tumor, which was 23 x 12 x 23 cm in size with 21 of serous fluid, was pathologically diagnosed as a cystic lymphangioma. The endothelial cells lining the internal surface of the cystic spaces were immunohistochemically positive for factor VIII-R antigen and CD31. Our review of the literature revealed 45 reports of lymphangioma of the pancreas, including this one, but to the best of our knowledge this is only the fifth case that required a PD. Nevertheless, we recommend that a complete resection be performed to reduce the risk of recurrence.

Cystic lymphangioma of the pancreas

“…The final diagnosis is histological [1] , with the endothelial cells showing immunohistochemical reactivity to factor VIII/R antigen, CD 31 (+) positivity [6,8] and CD 34 (-) negativity [6] , as seen in our patient.…”

“…The tumour size may vary between 3 and 20 cm in diameter (average 12 cm) [6] . Patients usually present with abdominal pain [5] and an associated palpable abdominal mass [7][8][9] , although an acute abdomen has also been described [10] . Pancreatitis, weight loss, and laboratory abnormalities are not usual disease manifestations [1] .…”

“…A complete surgical excision is curative [6,8,15] , with incomplete excision being the only reason for recurrent disease [7] . Depending on the tumour location and size, complete excision may involve a simple marginal tumorectomy [10] or may require larger pancreatic resections with anastomoses.…”

Pancreatic cystic lymphangioma: Report of a case

“…Lymphangiomas are rare congenital benign tumors arising from the lymphatic system (1), and are mostly encountered in the neck and axillary regions of pediatric patients (95%) (2). Lymphangioma cases have also been reported in other organs, such as the liver, spleen, lungs, mediastinum, colon, retroperitoneum, pancreas, omentum, pericardium, pleura, kidneys, ureters, bone, scrotum, penis, and cervix, etc.…”

“…Lymphangioma cases have also been reported in other organs, such as the liver, spleen, lungs, mediastinum, colon, retroperitoneum, pancreas, omentum, pericardium, pleura, kidneys, ureters, bone, scrotum, penis, and cervix, etc. (2). Intraabdominal lymphangiomas are extremely rare, with a reported incidence of less than 1 in 20,000 to 1 in 250,000 hospital admissions (3).…”