Littoralzellangiom der Milz

Littoralzellangiome

der Milz in der Magnetresonanztomografie: Links T2 und T1, rechts Kontrastmitteldynamik.

Littoral cell

angioma of the spleen in a patient with previous pulmonary sarcoidosis: a TNF-α related pathogenesis?. Computed tomography showing multiple hypoattenuating lesions (arrows).

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Incidental

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

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Incidental

littoral cell angioma of the spleen. 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.

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Incidental

littoral cell angioma of the spleen. 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.

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Littoral cell

angioma, a rare cause of long standing anaemia: a case report. Ultrasound of the spleen demonstrates enlarged spleen with well-defined hypoechoic, and hyperechoic lesions.

casesjournal.biomedcentral.com • CC-by-2.0

Littoral cell

angioma, a rare cause of long standing anaemia: a case report. CT unenhanced (a) and contrast enhanced during the early portal venous phase (b) images show an inhomogeneous hypodense well defined mass with some internal enhancement in the spleen.

casesjournal.biomedcentral.com • CC-by-2.0

Computed

tomography of the spleen: how to interpret the hypodense lesion. a, b Transverse contrast-enhanced CT images acquired during the portal-venous phase at two different levels in a 55-year-old man with littoral cell angioma, which presents as multiple hypodense, partially contrast-enhancing, lesions of different size (arrows)

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Radiopaedia • CC-by-nc-sa 3.0 • de

Littoral cell angioma of the spleen is a rare, benign primary vascular tumor of the spleen.

Epidemiology

Littoral cell angiomas may occur at any age and have no gender predilection.

Associations

Littoral cell angiomas have been diagnosed in association with various malignancies outside the spleen . The condition has also been linked to Crohn disease .

Clinical presentation

The lesion is often detected incidentally in asymptomatic patients. Symptomatic patients may present with abdominal pain, splenomegaly on physical examination, and/or laboratory evidence of hypersplenism including anemia and thrombocytopenia.

Pathology

Littoral cell tumors arise from the littoral cells lining the sinusoids of splenic red pulp. Littoral cell angiomas are the benign type, as opposed to the less common, low-grade malignant littoral cell hemangioendothelioma and the rare and aggressive littoral cell angiosarcoma .

Macroscopic appearance

The spleen is enlarged with multiple nodules, the color of which varies by age of blood products .

Microscopic appearance

Histopathology demonstrates a proliferation of anastomosing vascular channels congested with blood and lined with tall endothelial cells that show hemophagocytosis .

Immunophenotype

The tumor cells express both vascular endothelial markers (such as CD31 and factor VIII) and histiocytic markers (such as CD68) .

Radiographic features

Imaging usually demonstrates splenomegaly and multiple mass lesions.

CT

The masses are hypoattenuating compared to normal spleen . They demonstrate progressive homogeneous contrast enhancement, later than normal splenic parenchyma .

MRI

The masses are often low in T1 and T2 signal intensity due to hemosiderin .

Treatment and prognosis

As many patients are symptomatic and imaging findings are non-specific, splenectomy is usually performed for definitive histological diagnosis and treatment. Littoral cell angiomas are considered benign.