Huge Retroperitoneal Lymphangioma Presenting With Duodenal Obstruction: a case report.

lymphangiomas are benign tumors caused by the congenital proliferation of lymphatic tissue, causing an obstruction in the lymphatic ducts, producing fluid filled cysts (1). Histologically they are polycystic, with cysts divided by thin septas lined with endothelial cells. Most common presentation of cystic lymphangiomas are in the neck (75%) and axillary region (20%) and only 5% are intra-abdominal, while retroperitoneal lymphangiomas account for only 1% of cases (2). It is more common in children with males affected more than women, while women have shown to encounter pancreatic lymphangiomas more than men (3). Often retroperitoneal lypmhangiomas are asymptomatic but due to their large size they might cause a wide variety of symptoms from mild abdominal distention/discomfort and back pain to duodenal obstruction, sepsis and DIC (4). Diagnostic techniques are ultrasound, CT scan and MRI but often it is found accidentally and definitive diagnosis is made post-operatively. Treatment of choice is complete surgical resection with cystectomy

A rare case of retroperitoneal lymphangioma in a 74-year-old Chinese male

Retroperitoneal lymphangioma is an uncommon and benign mesodermal tumour that arises from the retroperitoneal lymphatics. Notably, it is a rare occurrence in adults, where <200 adult retroperitoneal lymphangioma cases have been published in the literature. Additionally, retroperitoneal lymphangioma is often difficult to diagnose preoperatively and formal diagnosis is frequently determined following surgical exploration. Here, we describe a rare case of retroperitoneal lymphangioma in a 74-year-old man who presented with a 6-month history of intermittent fresh per rectal bleeding with an incidental non-tender left iliac fossa firm mass on examination. Computed tomography scan established a retroperitoneal cystic lesion abutting the aorta and left common iliac vessels. Surgical exploration revealed a large cystic mass and a clean plane of dissection was performed, where the mass was completely excised with all the key structures preserved. Histology was consistent with a retroperitoneal lymphangioma.

A case of giant retroperitoneal lymphangioma and IgG4-positive fibrosis: Causality or coincidence?

Several chronic inflammatory diseases have been found to be a subtype of IgG4-related disease, all of which have a typical clinical and histological change, which is based in particular on an overexpression of IgG4 and subsequent fibrosis. At least a part of the retroperitoneal fibrosis, which was originally classified as idiopathic, seems to be assigned to IgG4-related disease. Lymphangiomas are benign, cystic tumors that rarely occur in adults. However, there is no firm association with IgG4-related disease described in the literature to date. This report is about a patient suffering from acute renal failure due to a giant retroperitoneal cyst. Surgical resection remains incomplete in the iliac vessel area due to severe fibrosis and histology revealed features of both lymphangioma and IgG4+ fibrosis. The case description is followed by a brief overview of IgG4-related disease and a consideration of whether lymphangiomas might be assigned to this topic.