Lymphangiomas are benign tumors of the lymphatic system. They are classified as cavernous, lymphangioma simplex, or cystic lymphangioma [2
]. Cystic lymphangioma, first described in 1828 by Redenbacker, is a malformation of the lymphatic system. It can affect any site in the body but is seen more commonly in the head and neck region and the axilla. It is also reported to occur in the mediastinum, retroperitoneum, and other regions [2
]. Cystic lymphangiomas most commonly affect children. About 90% of these lymphangiomas manifest before 2 years of age and are very rarely encountered in adults [3
]. The reported patient was aged 70 years at presentation.
Lymphangiomas in children are considered to arise from sequestered lymphatic sacs that fail to communicate with the draining lymphatic channels. This is a widely accepted theory. However, the etiology in the adult population is controversial. Some authors believe that the adult manifestations are a result of delayed proliferation of congenital or acquired lymphoid nests after stimuli such as respiratory infection or local trauma [4
]. Others dispute the congenital origin and propose that adult cystic lymphangiomas arise as a result of trauma alone [5
]. There was no history of trauma in this patient.
Radiographic evaluation with magnetic resonance imaging or CT is invaluable for the diagnosis and determination of the extent of the lesion. In addition, it is essential in defining normal anatomical structures that need to be preserved when surgical excision is performed [4
]. Accurate pre-operative diagnosis of cystic lymphangioma is uncommon [6
] and was a problem faced in the reported case. The CT scan revealed a huge cystic lesion, but identification of the site of origin and diagnosis were not possible.
Lymphangiomas are treated by surgical excision. Complete excision of the mass with negative surgical margins is the optimal treatment, and the results are excellent [7
]. Intra-abdominal lymphangiomas have a 10% postoperative recurrence rate for incompletely excised lesions. We were able to achieve total excision of the cyst.
Effective immunohistochemical markers specific for lymphatic endothelial cells have been reported including lymphatic vessel endothelial receptor 1, vascular endothelial growth factor receptor 3, and Prox-1. However, the antibodies against these markers are available only for frozen section specimens. More recently, a new monoclonal antibody, D2-40, has become available; this is a specific marker of lymphatic endothelium, since it does not stain vascular endothelium [8
]. In this case, diagnosis was made only after immunostaining with the lymphatic marker D2-40.
We could only find one case of broad ligament cystic lymphangioma reported in the literature [1
]. The case presented here is probably only the second case of broad ligament lymphangioma to be reported. The lesion weighed 19 kg and is probably one of the largest to be reported. Malignant transformations of cysts are rare and have been reported only once [9
]. Such a transformation is an exception rather than a rule.