Retroperitoneal cystic lymphangioma: Report of two cases and review of the literature

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 al 1 reported nine cases of such tumors diagnosed at the Mayo Clinic from 1911 to 1947. Rauch 2 found 22 cases of retroperitoneal lymphangiomas reported before 1959. Kafka and Novak 3 added 10 cases and reported one of their own. Recently, Singh et al 4 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.

Retroperitoneal cystic lymphangioma

“…Many authors characterize CL as an abnormality of infancy and childhood [3,1 I], whereas the results of other studies [2, indicate that the CL, when located in the retroperitoneum, may occur in patients of all ages, and of both sexes [4].…”

“…Others favor a congenital origin, due to proliferation of remnants of the embryonic lymph sac, for unknown reasons [8]. The most widely accepted theory is that of early developmental sequestration of lymphatic vessels that fail to establish connection with normal draining lymphatics and become markedly dilated under the pressure of accumulating lymph [4]. This hypothesis does not account, however, for the often invasive character of CL; in addition, an active growth of endothelium may exist, resulting in slow dissection through normal tissues and pressure atrophy of adjacent structures [9]: It is also possible that the nature of tissue surrounding a lymphangioma may determine its type.…”

“…2). Cyst walls appear as a single layer of flattened endothelium [4] and contain a variable amount of fibrous and lymphoid tissue [6]. Endothelial proliferation is inconspicuous and the process is histologically benign [7].…”

“…When clinically significant, they generally present as a large, palpable mass causing displacement of abdominal organs; more rarely they can cause bowel or ureteral obstruction [3]. About 90% of the CL are diagnosed during the second year of life [4]; they rarely occur in adults. We report two cases of retroperitoneal CL, found in adult patients, successfully treated by surgical excision.…”

Giant cystic lymphangioma in childhood: a rare differential for the acute abdomen

“…A recent study series showed that they usually affect boys, with a male-to-female ratio of 5:2 and a mean age of presentation of 2.2 years 2. These lesions can be associated with specific genetic abnormalities, most notably Turner syndrome 3. Intra-abdominal cystic lymphangioma is particularly rare, accounting for 2–8% of all cases and the symptoms are variable 4.…”