Littoral cell angioma of the spleen is a rare, benign primary vascular tumor of the spleen 1.
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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 10,12. The condition has also been linked to Crohn disease 13,14.
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. Rare cases of rupture and resultant hemoperitoneum are described.
Pathology
Littoral cell tumors arise from the littoral cells lining the sinusoids of the 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 9.
Macroscopic appearance
The spleen is enlarged with multiple nodules, the color of which varies by age of blood products 7.
Microscopic appearance
Histopathology demonstrates a proliferation of anastomosing vascular channels congested with blood and lined with tall endothelial cells that show hemophagocytosis 7,8.
Immunophenotype
The tumor cells express both vascular endothelial markers (such as CD31 and factor VIII) and histiocytic markers (such as CD68) 7,8.
Radiographic features
Imaging usually demonstrates splenomegaly and multiple mass lesions.
CT
There are multiple nodules or masses which are hypoattenuating compared to normal spleen 2-8. They demonstrate progressive homogeneous contrast enhancement, later than normal splenic parenchyma 2.
MRI
The masses are often low signal on T1 weighted images and the T2 signal characteristics are variable, occasionally being high signal due to cellularity or low signal due to hemosiderin 7,14. The lesions exhibit progressive enhancement 14.
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.
History and etymology
Littoral cell angioma of the spleen was first reported by the German pathologists Stephan Falk, Hans-Jochen Stutte and Glauco Frizzera in 1991 1,9.
Differential diagnosis
The imaging differential includes:
other primary splenic vascular tumors, such as hemangioma, hamartoma, hemangioendothelioma, angiosarcoma