Retroperitoneal mixed cystic lymphatic malformation

Case contributed by Ahmad Allababidi

Presentation

Unclear cystic mass in the left paravertebral region.

Patient Data

Age: 1 year
Gender: Female

Cystic mass in the left paravertebral region containing fine septa without duplex signal.

5.9 cm cystic, fine septated formation prevertebral left, predominantly T2 hyperintense without restricted diffusion or contrast enhancement. Close contact with the distal aorta with questionable contact with the thoracic duct. 

Case Discussion

The first differential diagnosis is a mixed cystic lymphatic malformation. Other differentials could be a rare, congenital, purely cystic neuroblastoma or ganglioneuroblastoma possible.

In such a case of mixed cystic lymphatic malformation, an ultrasound could help to differentiate by changing the position of the patient. A connection of the formation to the thoracic duct could lead to a size reduction.

Mixed cystic lymphatic malformations are well-defined multi-locular cystic masses with typically homogeneous fluid content. Post-contrast enhancement of the cyst wall and septa may be seen. It is important to remember that they can cross fascial planes and involve multiple compartments.

These malformations were formerly called lymphangiomas. This expression is out-of-date according to the 2018 ISSVA classification. 

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