Retroperitoneal haemorrhagic cystic lymphangioma

Clinical History

A 58-year-old female patient without medical history of interest was referred to our Emergency Department due to mesogastric colicky abdominal pain of 18 hours of evolution, radiating to the left upper abdominal quadrant and ipsilateral arm. The patient also presented with nausea and fever of 38ºC.

Imaging Findings

Abdominal ultrasound showed a polycystic mass in the left upper quadrant, adjacent to the spleen and kidney, consisting of several cysts rounded and elongated. The cysts had echogenic material inside, partly in the form of membranes, suspected to be bleeding based on clinical findings (Fig. 1).
Enhanced and non-enhanced abdominal CT showed the extension of the polycystic mass from subphrenic space to the iliac blade (length 22 cm), with lesser sac occupation and encompassing the vessels of the splenic hilum (Fig. 2).
Non-enhanced abdominal CT showed a liquid with densitometric values about 20 HU, and a denser material (bleeding) that was of 33 HU (Fig. 3). No enhancements were seen with intravenous contrast (Fig. 2).

Discussion

Background
Lymphangiomas are non-frequent and benign tumours of lymphatic origin that are typical of childhood [1, 2]. Most of them are located in the neck (75%) and axilla (20%). The remaining 5% are located in mesentery, retroperitoneum, abdominal viscera (spleen, colon, pancreas…) lung and mediastinum [2].

Clinical perspective
Lymphangiomas can be asymptomatic or compress adjacent structures [1]. Also, the cyst's haemorrhage or infection may lead to acute abdomen [3].

Imaging perspective
The diagnosis is usually made by ultrasound, CT or MRI [1-3]. Ultrasound shows multilocular cystic masses that can contain echogenic internal septa [3]. CT shows the size, extension and relationships with the adjacent structures. Lymphangiomas are usually homogeneous, although they can show wall or septa enhancement on contrast-enhanced CT or develop different complications such as haemorrhage or intestinal obstruction [3]. Usually, retroperitoneal lymphangiomas pass through different adjacent anatomical compartments [2]. On MRI, they have similar behaviour as fluid: low signal intensity on T1-weighted images and high signal intensity on T2-weighted images.
Final diagnosis is always histological [1, 2].

Outcome
The treatment is surgical [1-4] with good prognosis with early and complete resection. Our patient still had pain during a week so surgery was performed (Fig. 4). Histopathological study confirmed the diagnosis. The lesion was a cystic multiloculated mass with septa and haematic internal material (Fig. 5).

Differential Diagnosis List

Final Diagnosis

Retroperitoneal haemorrhagic cystic lymphangioma

URL:	https://www.eurorad.org/case/13757
DOI:	10.1594/EURORAD/CASE.13757
ISSN:	1563-4086