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Abdominal (retroperitoneal) cystic lymphangioma

Case contributed by: Dr Abdel-Rahman Abdel-Halim

Presentation:

Right sided abdominal vague pain

Patient Data:

Age: 50
Gender: Female
Race: Middle eastern

A huge lobular retroperitoneal cystic soft tissue lesion is seen in the right lumbar region, it shows no contrast enhancement and has imperceptible wall. It extends superiorly to the hepatorenal pouch and inferiorly to the right broad ligament, right mesorectal fat planes and presacral space. This mass lesion is displacing the right ureter laterally, the rectum to the left side and the small bowel loops and pancreas anteromedially and it is seen encasing the ureter and renal vessels. 

Case Discussion:

Retroperitoneal cystic lymphangioma 

A lymphangioma is a benign proliferation of lymph vessels, producing fluid-filled cysts that result from a blockage of the lymphatic system. The incidence of abdominal lymphangiomas is unknown.

Retroperitoneal lymphangiomas account for nearly 1% of all lymphangiomas, and are uncommon incidental findings usually at surgery, autopsy or lymphography. These may be capillary, cystic or cavernous, with a uniseptate or multiseptate appearance. Although retroperitoneal lymphangiomas may sometimes be asymptomatic, they usually present as a palpable abdominal mass, and are easily confused with other retroperitoneal cystic tumors including those arising from the liver, kidney and pancreas. They may become symptomatic if they become large enough to impose on surrounding structures.

These rare tumors have an excellent prognosis, with symptomatic relief and cure achieved with complete surgical excision.

cystic retroperitoneal lesion can carry a relatively broad differenital which includes 

  • cystic lymphangioma
  • mucinous cystadenoma
  • cystic teratoma
  • cystic mesothelioma
  • pseudomyxoma retroperitonei with cystic change
  • perianal mucinous carcinoma with cystic change
  • retroperitoneal hematoma with liquifaction.
  • retroperitoneal hydatid cyst.
  • retroperitoneal bronchogenic cyst.

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