Retroperitoneal cystic lymphangioma in an adult: A case report and review of the literature

Abstract

Lymphangiomas are rare benign cystic tumors of the lymphatic system. Retroperitoneal lymphangiomas account for 1% of all lymphangiomas, and approximately 186 cases have been reported. They may clinically present as a palpable abdominal mass and can cause diagnostic dilemmas with other retroperitoneal cystic tumors, including those arising from the liver, kidney and pancreas. This report describes the rare case of a cystic retroperitoneal lymphangioma in a 54-year-old male patient. The lymphangioma had progressed to the point of inducing clinical symptoms of abdominal distention, abdominal pain, anorexia, fever, nausea and diarrhea. Radiological imaging revealed a large multiloculated cystic abdominal mass with enhancing septations involving the upper retroperitoneum and extending into the pelvis. Surgical removal of the cyst was accomplished without incident. A benign cystic retroperitoneal lymphangioma was diagnosed on histology and confirmed with immunohistochemical stains.

Keywords: Cystic; Lymphangioma; Retroperitoneum.

Figure 1

Abdominal computed tomography scan showing a large multilocular mass in the retroperitoneum (white arrows).

Figure 2

Photomicrograph of cystic lymphangioma. A: Hematoxylin & Eosin staining demonstrating multiple dilated cystic spaces filled with pale pink proteinaceous material, fascicles of smooth muscles and inflammatory cells in stroma (× 100); B: CD31 immunostain showing positive staining of cystic lining endothelial cells (× 100); C: D2-40 antibody immunostain showing positive staining of cystic lining endothelial cells (× 100); D: Calretinin immunostain showing negative staining (× 100); E: HMB-45 immunostain showing negative staining (× 100); F: EMA immunostain showing negative staining (× 100).