J Korean Surg Soc.  2011 Jun;80(Suppl 1):S43-S46. 10.4174/jkss.2011.80.Suppl1.S43.

A giant retroperitoneal lymphangioma in a patient with neurofibromatosis type 1

• Kim JH
• Kim MO
• Choi YJ
• Han HY
• Park KS
• Cho BS
• Kang DW

• KMID: 2096649
• DOI: http://doi.org/10.4174/jkss.2011.80.Suppl1.S43

Abstract

Neurofibromatosis type 1 (NF-1) is a genetically inherited disorder that may cause skin abnormalities and tumors that form on nerve tissues. These tumors can be small or large and can occur anywhere in the body, including the brain, spinal cord, or other peripheral nerves. Retroperitoneal lymphangiomas are very rare benign malformations of the lymphatic system. About 95% lymphangiomas occur in the skin and the subcutaneous tissues of the head, neck and axillary region and the remaining 5% appear in other parts of the body such as lungs, pleura, pericardium, liver, gallbladder, kidney, and the mesentery. Herein, we report the case of a giant retroperitoneal lymphangioma in a patient with NF-1 with a review of the literature.

Keyword

Lymphangioma; Retroperitoneal neoplasms; Neurofibromatosis 1

MeSH Terms

Brain
Gallbladder
Head
Humans
Kidney
Liver
Lung
Lymphangioma
Lymphatic System
Mesentery
Neck
Nerve Tissue
Neurofibromatoses
Neurofibromatosis 1
Pericardium
Peripheral Nerves
Pleura
Retroperitoneal Neoplasms
Skin
Skin Abnormalities
Spinal Cord
Subcutaneous Tissue

Figure

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  Fig. 1 (A) Chest computed tomography (CT) shows multifocal soft tissue masses with calcification in the bilateral axillae, right pectoralis muscle, intermuscular layer of the left posterolateral chest wall. (B) Abdominal CT findings. It shows retroperitoneal giant cystic mass.

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  Fig. 2 Gross findings shows lymphangioma.

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  Fig. 3 Microscopic findings show lymphangioma. Proliferation of anastomosing blood vessels and smooth muscle (H&E, ×400).

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