Splenic lymphangioma
Updates to Article Attributes
Splenic lymphangiomas are relatively rare benign tumours that correspond to malformations of the lymphatic system.
Epidemiology
Most occur in children, were rarely reported in adults2.
Clinical presentation
Presentation ranges from asymptomatic incidental finding to a large multicentric, symptomatic mass requiring surgical intervention. May occur alone on spleen or as part of a systemic lymphangiomatosis. 2
Pathology
The cysts are thought to be formed by abnormal dilatation of lymphatic channels; this can be congenital or acquired.
Cysts tend to be subcapsular and multilocular but can be unilocular.
The cyst wall and septa are composed of thin bands of fibrous tissue lined by bland endothelium, the nature of which can be confirmed on immunohistochemistry using endothelial markers (CD31, podoplanin); this may be necessary to rule out a cystic epithelial lesion.
The fibrous cyst walls/septa can show focal calcification.
Radiographic features
Ultrasound
Appear as well defined round hypoechoic lesions and may show occasional internal septation and intralocular echogenic debris. Tiny echogenic calcifications may be present.
CT
They appear as single or multiple thin-walled low attenuation masses with sharp margins that are typical subcapsular in location. They are hypodense with no enhancement. The presence of curvilinear peripheral mural calcifications suggests the diagnosis of cystic lymphangioma1-2.
MRI
On T1W they appear hypointense relative the surrounding viscera. High T1 signal intensity may occur with internal bleeding or large amounts of intracystic proteinaceous content. T2W images demonstrated multiloculated hyperintense areas that correspond to the dilated lymphatic spaces. The intervening septa appear as hypointense bands, corresponding to the presence of fibrous tissue.
See also
-<p><strong>Splenic lymphangiomas </strong>are relatively rare benign tumours that correspond to malformations of the lymphatic system. </p><h4>Epidemiology</h4><p>Most occur in children, were rarely reported in adults<sup>2</sup>. </p><h4>Clinical presentation </h4><p>Presentation ranges from asymptomatic incidental finding to a large multicentric, symptomatic mass requiring surgical intervention. May occur alone on spleen or as part of a <a href="/articles/lymphangioma">systemic lymphangiomatosis</a>.<sup> 2</sup></p><h4>Radiographic features</h4><h5>Ultrasound</h5><p>Appear as well defined round hypoechoic lesions and may show occasional internal septation and intralocular echogenic debris. Tiny echogenic calcifications may be present.</p><h5>CT</h5><p>They appear as single or multiple thin-walled low attenuation masses with sharp margins that are typical subcapsular in location. They are hypodense with no enhancement. The presence of curvilinear peripheral mural calcifications suggests the diagnosis of cystic lymphangioma<sup>1-2</sup>.</p><h5>MRI</h5><p>On T1W they appear hypointense relative the surrounding viscera. High T1 signal intensity may occur with internal bleeding or large amounts of intracystic proteinaceous content. T2W images demonstrated multiloculated hyperintense areas that correspond to the dilated lymphatic spaces. The intervening septa appear as hypointense bands, corresponding to the presence of fibrous tissue.</p><h4>See also</h4><ul>- +<p><strong>Splenic lymphangiomas </strong>are relatively rare benign tumours that correspond to malformations of the lymphatic system. </p><h4>Epidemiology</h4><p>Most occur in children, were rarely reported in adults<sup>2</sup>. </p><h4>Clinical presentation </h4><p>Presentation ranges from asymptomatic incidental finding to a large multicentric, symptomatic mass requiring surgical intervention. May occur alone on spleen or as part of a <a href="/articles/lymphangioma">systemic lymphangiomatosis</a>.<sup> 2</sup></p><h4>Pathology</h4><p>The cysts are thought to be formed by abnormal dilatation of lymphatic channels; this can be congenital or acquired.</p><p>Cysts tend to be subcapsular and multilocular but can be unilocular.</p><p>The cyst wall and septa are composed of thin bands of fibrous tissue lined by bland endothelium, the nature of which can be confirmed on immunohistochemistry using endothelial markers (CD31, podoplanin); this may be necessary to rule out a cystic epithelial lesion.</p><p>The fibrous cyst walls/septa can show focal calcification.</p><h4>Radiographic features</h4><h5>Ultrasound</h5><p>Appear as well defined round hypoechoic lesions and may show occasional internal septation and intralocular echogenic debris. Tiny echogenic calcifications may be present.</p><h5>CT</h5><p>They appear as single or multiple thin-walled low attenuation masses with sharp margins that are typical subcapsular in location. They are hypodense with no enhancement. The presence of curvilinear peripheral mural calcifications suggests the diagnosis of cystic lymphangioma<sup>1-2</sup>.</p><h5>MRI</h5><p>On T1W they appear hypointense relative the surrounding viscera. High T1 signal intensity may occur with internal bleeding or large amounts of intracystic proteinaceous content. T2W images demonstrated multiloculated hyperintense areas that correspond to the dilated lymphatic spaces. The intervening septa appear as hypointense bands, corresponding to the presence of fibrous tissue.</p><h4>See also</h4><ul>
Image 2 Pathology (Gross pathology) ( create )
Image 3 Pathology (H&E) ( create )
