Article

Retroperitoneal cystic lymphangioma

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Abstract

Cystic lymphangiomas (cystic hygromas) are comparatively rare tumors. Seventy-five percent of these lesions are in the neck, 20% are in the axillary region, and 5% are in the mediastinum, retroperitoneal region, pelvis, or groin.

Rarest are retroperitoneal cystic lymphangiomas. Beahrs et al1 reported nine cases of such tumors diagnosed at the Mayo Clinic from 1911 to 1947. Rauch2 found 22 cases of retroperitoneal lymphangiomas reported before 1959. Kafka and Novak3 added 10 cases and reported one of their own. Recently, Singh et al4 described another case, bringing the total number of reported cases of retroperitoneal cystic lymphangiomas to 34.

We report two cases with unusual features: one patient had preoperative angiograms, the second patient had an acute condition within the abdomen.

Pathology

It is believed that cystic lymphangiomas develop from portions of the sacs sequestered from the primary lymph sacs during embryonic life. Different theories regarding the pathogenesis of these lesions have included infiltrating neoplasm, areas of localized lymphatic stasis due to congenital blockage of regional lymphatic drainage, lymph node degeneration, obstruction secondary to inflammation, or fibrosis of existing lymph channels. When a lymphangioma develops, the increase in size is probably due to partial or complete lack of lymphatic communication with adjacent channels.

Grossly these lesions are multilocular masses of cysts varying from 1 mm to 5 cm or more in diameter. They are thin-walled and often communicate with each other. The cysts are filled with clear or straw-colored serous fluid. Microscopically, cyst walls appear as a single layer of . . .


 

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