Incidental littoral cell angioma of the spleen

Abstract

Background: Littoral cell angioma (LCA) is a recently described primary vascular neoplasm of the spleen that may be associated with other malignancies and may itself also have malignant potential.

Case presentation: We present a case of LCA that was discovered incidentally in a 52-year-old woman who presented with biliary colic at the time of consultation for cholecystectomy. This vascular neoplasm was evaluated by ultrasound, CT, MRI, Tc-99m labelled red blood cell scintigraphy, and core biopsy. A splenectomy revealed LCA by pathological evaluation. Post-operative outcome was favourable with no evidence of complication or recurrent disease. Following this case presentation, clinical, radiographic, and pathological features of LCA will be reviewed as well as recent advances in our understanding of this uncommon splenic lesion.

Conclusion: LCA is a rare, generally benign, primary vascular tumour of the spleen that typically is discovered incidentally. Individuals diagnosed with this tumour must be carefully evaluated to exclude primary, secondary, and synchronous malignancies.

Figure 1

Ultrasound of the spleen demonstrates a well-defined hyperechoic lesion.

Figure 2

CT scan after oral and iv-contrast in the arterial phase A and portal venous phase B demonstrates a hypodense well defined round lesion in the posterior portion of the spleen with some linear internal enhancement. C. The lesion is isodense compared to the normal spleen on the 5 minutes delayed image.

Figure 3

A. The lesion is hyperintense on the T2 weighted fast recovery fast spin echo (FRFSE) image. B. Internal linear enhancement is noted on the T1 weighted fat saturated image after iv-gadolinium in the portal venous phase.

Figure 4

Anastamosing vascular channels lined by plump cells with the appearance of sinus lining (arrow: 'littoral' cells) (H&E 100×).