Retroperitoneal cystic lymphangioma in an adult: A case report.

Introduction

Lymphangiomas are rare cystic tumors of the lymphatic system, characterized by proliferating lymphatic vessels. It has a polymorphic clinical presentation. The evolution after surgical treatment is generally favorable.

Case report

A 68-year-old female presented with irritative lower urinary tract symptoms and left flank pain of few weeks duration. Clinically, there was not any palpable mass neither in the left hypochondrium nor in lumbar region. Her renal function test reports were normal. Computed tomographic scan of the abdomen showed a 4 cm-size retroperitoneal cystic mass (Fig. 1). The patient underwent an explorative lumbotomy. There was a plane of dissection between the kidney and the cyst, which was then excised entirely. The final histological examination concluded to a retroperitoneal cystic lymphangioma (Fig. 2). After 50 months of clinical and radiological check-up, there was no functional complaint or any sign of recurrence.

Fig. 1

Computed tomographic scan of the abdomen showed a 4 cm retroperitoneal cystic mass.

Fig. 2

Cystic lesion characterized by dilated lymphatic and vascular vessels. (HE X 20).

Discussion

Cystic lymphangioma is a rare lesion characterized by the proliferation of a benign vascular tumor of the lymphatic system. The existence of congenital malformations is the most probable of theories that have been postulated on the development of cystic lymphangiomas. The most frequently affected sites are the head and neck, followed by the axilla and abdomen. The retroperitoneal localization is very rare, described by Satwayin 1898. Cystic lymphangioma is most frequently seen in children, rarely in adults. The majority of the retroperitoneal lymphangiomas are asymptomatic and are discovered incidentally in later life during radiological procedures for other conditions, or during surgery or autopsy. The most common clinical manifestation is that of a slowly enlarging abdominal mass, left upper quadrant pain, loss of appetite, nausea and vomiting. They may be symptomatic if they become large or complicated., Ultrasound findings are not specific, the computed tomographic scan allows the initial diagnosis in adults. CT can also be used to evaluate the relationship of the tumor to neighboring organs and to distinguish retroperitoneal lymphangioma from intraperitoneal lymphangioma. The diagnosis of cystic lymphangioma can only be confirmed by histological examination. These include awell-circumscribed, cystic lesion, a stroma composed of a meshwork of collagen and fibrous tissue, and a wall containing focal aggregates of lymphoid tissue. The benign nature of the retroperitoneal lymphangioma warrants a conservative surgical approach. With cystic retroperitoneal lymphangioma, a simple total excision is usually the preferred treatment.

Conclusion

Cystic lymphangioma (CL) is a rare benign tumor that may arise in various sites. The retroperitoneal site of CL is less frequent than the mesenteric site. These rare tumors have an excellent prognosis.

Conflicts of interest

The authors declare that there are no conflicts of interest regarding the publication of this article.