Fact-sheet: Retroperitoneal cystic lymphangioma
Updated on 04/18/2017 at 7:00 AM
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Definition
Rare benign dysembryoplastic tumor of lymphatic origin, with slow growth.
Etiopathogenesis likely related to abnormal connections of the lymphatic spaces.
Ubiquitous location, with a predominance in the cervicofacial region.
6% of benign tumors in children, 1/12,000 births, developing before age 2 in 90% of cases.
Secondary forms exist in adults, related to lymphatic obstruction
Clinical features
Symptomatology related to volume and location.
Laboratory findings
Normal unless superinfected.
Ultrasound
Cystic mass with a thin wall, multiloculated, separated by thin septations, anechoic fluid content, without vascularity.
Septal thickening, hyperemia, and speckled hyperechoic content if superinfected.
CT
Hypodense mass with non-enhancing fluid content, well-defined margins, septa +/- visible +/- enhanced.
Possible calcification.
Negative fluid content (
hyperdense content and fluid-fluid level if hemorrhagic)
MRI
Fluid content hypointense on T1, hyperintense on T2.
Signal drop on opposite-phase imaging if warm content.
Septa hypointense on T1 and T2.
Minimal enhancement
Management
Diagnosis established by pathology.
Surgical treatment is the reference standard.
However, alternatives exist (percutaneous drainage, sclerotherapy, laser)