Giant retroperitoneal lymphangioma

Case contributed by Dr Mostafa El-Feky

Presentation

Abdominal distension.

Patient Data

Age: 40 years
Gender: Female
CT

CT abdomen

A large left-sided retroperitoneal multispatial multicystic mass is noted measuring 17 × 24 x 33 cm in AP, SS and CC dimensions respectively. It is involving the retroperitoneal regions of the lower half and the left side of the abdomen. It shows homogenous density with soft tissue density (mean 45 HU). No evidence of post-contrast enhancement with preserved mean density.

The lesion extends cephalocaudally from the undersurface of the left diaphragmatic cupola to the paravesical space and Douglas pouch. It is completely encasing the left kidney, left renal pedicle and the left adrenal gland. It is abutting the spleen, pancreas, uterus, urinary bladder, bowel loops, both ureters, right psoas, aorta and iliac vessels. The mass is pushing the bowel loops towards the right side and superiorly. It pushes the uterus to the right side and is displacing the left kidney and adrenal gland anteriorly. No vascular compromise.

Case Discussion

This case was diagnosed since 2 and half years of having a retroperitoneal abdominopelvic cystic lesion that was considered mostly lymphangioma based on imaging features and performed aspiration that partially decreased lesion size. Now, the size of the lesion progressed with preserved same radiological features.

The diagnosis of retroperitoneal lymphangioma was made upon the creeping behavior of the lesion that appears multicystic of homogenous density with no post-contrast enhancement. It smoothly wraps the left kidney and adrenal gland gently displaces bowel loops and other abdominal structures with a line of cleavage from all surroundings. It is abutting vascular structures with no vascular compromise. All these features are representative of a benign slowly growing retroperitoneal cystic mass lesion which makes lymphangioma the most likely diagnosis.

Retroperitoneal lymphangioma is a rare slowly growing benign proliferation of lymph vessels. It accounts for nearly 1% of all lymphangiomas, the majority are at the head and neck (>95%). It is uncommon incidental findings usually at surgery, autopsy or lymphography. They may sometimes be asymptomatic or 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. Complete surgical excision is the treatment of choice. Definitive diagnosis is only by pathological examination.

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Case information

rID: 57863
Published: 19th Jan 2018
Last edited: 14th Aug 2019
System: Vascular
Inclusion in quiz mode: Included
Institution: Dar El Ashaa Center

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