CASE 14475 Published on 25.04.2017

Abdominal and thoracic lymphangioma with unusual imaging


Abdominal imaging

Case Type

Clinical Cases


Manal Al-kaiem, Zahia Zaitout.

Milton Keynes University Hospital, NHS;

39 years, female

Area of Interest Abdomen ; Imaging Technique CT, MR
Clinical History
A 39-year-old female patient presented with a 10 year history of worsening right side abdominal pain. The pain was aggravated by lying on the right side. A CT examination was subsequently performed.
Imaging Findings
The CT examination demonstrated an extensive nodular thickening (HU 40-49) around the liver (Fig. 1, 2) and the right hepatorenal recess (Fig. 3, 4). It had multiple well-defined round calcification foci (Fig. 1) with phlebolith-like configuration.

This soft tissue extended along the right diaphragm, through the oesophageal hiatus (Fig. 4) and the thorax including the right para-cardiac area (Fig. 5).

The initial diagnosis was vascular or lymphatic malformations based on the presence of phleboliths.

The appearances were not typical for malignancy due to the long clinical history, the lack of ascites, omental disease (Fig. 6) and lymphadenopathy. There were no other significant findings.

Further evaluation with MRI demonstrated the same abnormal soft tissue. It has a hypo-intense signal on T1WI (Fig. 6a), hyper-intense signal on T2WI (Fig. 7a) with calcification (Fig. 7b) and mild patchy post-contrast enhancement (Fig. 6b). Subsequent trans-abdominal biopsy following MDT discussion confirmed the diagnosis of lymphangioma.
Lymphangiomas are unusual benign tumours of the lymphatic system. Approximately one-fifth occur in adults whilst the majority are diagnosed in patients under the age of 2 [13, 15]. They originate in different sites; of these, only 5% could be found in the retroperitoneum, mesentery, abdominal viscera, lung, greater omentum, and mediastinum. 95% occur in the neck and axillary regions [1, 2, 10].

The aetiology of lymphangioma is not completely understood. According to the most acceptable theory, it originates from a congenital malformation of the lymphatic channels which are dilated, forming uni or multi-cystic masses. These malformations are postulated to arise from a sequestrated lymphatic tissue that did not communicate with the main lymphatic flow [12] or from an abnormal budding of the lymphatic endothelium during prenatal life [8, 9].

The small bowel mesentery is the most common site for abdominal lymphangiomas followed by the retroperitoneum where they constitute 1% of all retroperitoneal neoplasms [1, 3].

Clinically, most adult patients present with an incidental finding of an asymptomatic mass [4]. However, when large enough, they can have mass effect on the surrounding structures [11]. Rarely, complications can occur due to their size, including intra-cystic bleeding, infection, and compression of adjacent organs [5].

The classic ultrasound appearance is a well-circumscribed cystic lesion containing multiple thin septa with some solid echogenicity resulting in a honeycomb or cobweb pattern. On contrast-enhanced abdominal CT examination, lymphangiomas appear as unilocular or multilocular masses with wall and septal contrast enhancement [4, 14].

In our case report, the density of the abnormal structures was not typical of the usual low/fluid density commonly seen in cystic lymphangiomas. This is most likely because it has been reported that some cases appear inhomogeneous because of the presence of proteinaceous, blood or fat components within the lesion.

Calcification and ossification have also been described [16] and this can be seen in our case.

Radical surgical resection is the treatment of choice. However, this may be difficult due to its infiltrative pattern [3]. Recurrence rate is relatively high in case of incomplete resection (50% compared with 7% for complete resection) [6, 7]. Conservative management can be used in asymptomatic disease.

Teachings points:

The diagnosis of lymphangioma can be challenging on imaging examinations alone and histopathological confirmation is essential. The lymphangioma can demonstrate high density on CT scan (unlike the usual cystic density) and this is most likely caused by high proteinaceous or blood content.
Differential Diagnosis List
Diffuse abdominal and thoracic lymphangioma.
Metastatic or primary peritoneal disease
Peritoneal mesothelioma
Final Diagnosis
Diffuse abdominal and thoracic lymphangioma.
Case information
DOI: 10.1594/EURORAD/CASE.14475
ISSN: 1563-4086