J Korean Surg Soc.  2011 Aug;81(2):141-145. 10.4174/jkss.2011.81.2.141.

Cystic lymphangioma of the pancreas

• Sohn BK
• Cho CH
• Chae HD

• KMID: 2096669
• DOI: http://doi.org/10.4174/jkss.2011.81.2.141

Abstract

Lymphangioma is a benign form of neoplasm arising from the lymphatic system. It occurs as a result of congenital malformations of the lymphatics leading to the obstruction of local lymph flow and the development of lymphangiectasia. Lymphangiomas are common in pediatric patients, in the soft tissues of the neck and the axillae, but lymphangioma of the pancreas is extremely rare, accounting for less than 1% of these tumors. It occurs more frequently in females and is often located in the distal pancreas. Although extremely rare, cystic lymphangioma of the pancreas should be taken into consideration as a differential diagnosis of pancreatic cystic or retroperitoneal lesions, especially in women. Herein, we report on a case of cystic lymphangioma of the distal pancreas in a 37-year-old woman who was treated with complete surgical resection with a review of the literature.

Keyword

Cystic lymphangioma; Pancreas; Retroperitoneal space

MeSH Terms

Accounting
Adult
Axilla
Diagnosis, Differential
Female
Humans
Lymphangioma
Lymphangioma, Cystic
Lymphatic System
Neck
Pancreas
Pancreatic Cyst
Retroperitoneal Space

Figure

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  Fig. 1 Simple abdominal X-ray. Simple abdomen shows no bowel gas in the left upper quadrant with displacement of bowel to the right abdomen by the left upper quadrant mass.

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  Fig. 2 Abdominal computed tomography (CT) scan. A contrast-enhanced helical CT scan of the abdomen at portal venous phase shows a huge, hypo-dense, well-capsulated and cystic lesion of unknown origin at the left upper quadrant of the abdomen (arrow).

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  Fig. 3 Operative findings shows a huge, well-defined and cystic mass densely attached to the tail of the pancreas (arrow).

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  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.

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  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).

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  Fig. 6 Immunohistochemical stain. The lining endothelial cells show strong immunopositivity for CD31 and D2-40 and weak positivity for CD34 (Immunohistochemistry, ×200).

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