Figure 1
Axial post-contrast portovenous abdominal CT
Diffuse tissue thickening is demonstrated around the liver (white arrow) containing calcification (red arrow).
Abdominal and thoracic lymphangioma with unusual imaging
SectionAbdominal imaging
Case TypeClinical Cases
AuthorsManal Al-kaiem, Zahia Zaitout.
Connected authors
39 years, female
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.
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.
Discussion
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.
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.
References
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[2] Levy AD, Cantisani V, Miettinen M. (2004) Abdominal lymphangiomas: imaging features with pathological correlation. AJR Am J Roentgenol 182 (6): 1485-91 (PMID: 15149994)
[3] Scanlan DB. (1959) Primary retro-peritoneal tumours. J Urol 81:740. Medline
[4] Davidson AJ, Hartman DS. (1990) Primary retro-peritoneal tumours. Lymphangioma of the retroperitoneum: CT and sonographic characteristic. Radiology 175:507-510 (PMID: 2183287)
[5] Surlin V, Georgescu E, Dumitrescu C, Râmboiu S, Comănescu MV, Ghiluşi M. (2011) Retropancreatic cystic lymphangioma - considerations upon a case. Rom J Morphol Embryol 52(Suppl):493–496 (PMID: 21424100)
[6] Makni A, Chebbi F, Fetirich F, Ksantini R, Bedioui H, Jouini M, Kacem M, Ben Safta Z. (2012) Surgical management of intra-abdominal cystic lymphangioma. World J Surg 36:1037–1043. doi: 10.1007/s00268-012-1515-2 (PMID: 22358782)
[7] Gray G, Fried K, Iraci J. (1998) Cystic lymphangioma of the pancreas: CT and pathological findings. Abdom Imaging 23:78–80. doi: 10.1007/s002619900289 (PMID: 9437068)
[8] Schneider G, Seidel R, Altmeyer K, Remberger K, Pistorius G, Kramann B, Uder M. (2001) Lymphangioma of the pancreas and the duodenal wall: MR imaging findings. Eur Radiol 11:2232–2235. doi: 10.1007/s003300100846 (PMID: 11702164)
[9] Tapan Bhavsar, Daryoush Saeed-Vafa, Sean Harbison, and Susan Inniss. (2010) Retroperitoneal cystic lymphangioma in an adult: A case report and review of the literature. World J Gastrointest Pathophysiol 1(5):171-6 doi: 10.4291/wjgp.v1.i5.171 (PMID: 21607159)
[10] Yang DM, Jung DH, Kim H, Kang JH, Kim SH, Kim JH, Hwang HY (2004) Retroperitoneal cystic masses: CT, clinical, and pathologic findings and literature review. Radiographics 24:1353–1365 (PMID: 15371613)
[11] Roisman I, Manny J, Fields S, Shiloni E. (1989) Intra-abdominal lymphangioma. Br J Surg 76:485–489 (PMID: 2660949)
[12] Pia F, Aluffi P, Olina M. (1999) Cystic lymphangioma in the head and neck region. Acta Otorhinolaryngol Ital 19:87–90 (PMID: 10434440)
[13] Chen C.W., Hsu S.D., Lin C.H., et al: (2005) Cystic lymphangioma of the jejunal mesentery in an adult: a case report. World J Gastroenterol 11: pp. 5084-5086 [Cross Ref] (PMID: 16124074)
[14] Takiff H, Calabria R, Yin L, Stabile BE. (1985) Mesenteric cysts and intra-abdominal cystic lymphangiomas. Arch Surg 120(11):1266–1269 (PMID: 4051731)
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[16] Wani I. (2009) Mesenteric lymphangioma in adult: A case series with a review of the literature. Digestive Diseases and Sciences 54(12):2758-62 (PMID: 19142726)
Case information
| URL: | https://www.eurorad.org/case/14475 |
| DOI: | 10.1594/EURORAD/CASE.14475 |
| ISSN: | 1563-4086 |
License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Figure 4
Axial non-contrast abdominal CT
This shows the density of the surrounding soft tissue (40 HU).
Figure 5
Axial CT chest with contrast in the arterial phase
Axial CT post-contrast porto-venous phase shows diffuse tissue thickening in the right paracardiac region (arrow).
Figure 6
Coronal abdominal CT post contrast
Coronal CT post-contrast porto-venous phase demonstrates the abnormal tissue thickening around the liver and the hepatorenal recess.
Axial post-contrast portovenous abdominal CT
Diffuse tissue thickening is demonstrated around the liver (white arrow) containing calcification (red arrow).
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MKUH
Axial non-contrast abdominal CT
This shows the density of the surrounding soft tissue (40 HU).
MKUH
MKUH
Axial CT chest with contrast in the arterial phase
Axial CT post-contrast porto-venous phase shows diffuse tissue thickening in the right paracardiac region (arrow).
MKUH
MKUH
Coronal abdominal CT post contrast
Coronal CT post-contrast porto-venous phase demonstrates the abnormal tissue thickening around the liver and the hepatorenal recess.
MKUH
MKUH