Littoral cell angioma of the spleen
=Littoral Cell Angioma of the Spleen =
Littoral cell angioma (LCA) is a rare vascular tumor of the spleen, first described in 1991. It arises from the littoral cells lining the splenic sinuses. This condition is often discovered incidentally during imaging studies or splenectomy performed for other reasons.
Pathophysiology[edit | edit source]
Littoral cell angiomas are unique because they exhibit both endothelial and histiocytic features. The spleen is an organ involved in filtering blood, and the littoral cells are specialized cells that line the sinuses of the red pulp. These tumors are thought to originate from these cells, which have phagocytic capabilities.
Clinical Presentation[edit | edit source]
Most patients with littoral cell angioma are asymptomatic. However, some may present with symptoms related to splenomegaly, such as abdominal pain or discomfort. In rare cases, patients may experience anemia, thrombocytopenia, or other cytopenias due to hypersplenism.
Diagnosis[edit | edit source]
Diagnosis of littoral cell angioma is often made through imaging studies such as CT scan or MRI, which reveal splenic lesions. Definitive diagnosis is usually confirmed by histopathological examination after splenectomy. The tumors are typically well-circumscribed, spongy, and may vary in size.
Histology[edit | edit source]
Histologically, littoral cell angiomas show anastomosing vascular channels lined by cells with both endothelial and histiocytic markers. Immunohistochemical staining is positive for CD31, CD68, and factor VIII-related antigen, which helps differentiate it from other splenic lesions.
Treatment[edit | edit source]
The primary treatment for symptomatic littoral cell angioma is splenectomy. Asymptomatic cases may be monitored with regular follow-up imaging. There is no known risk of malignant transformation, but rare cases of associated malignancies have been reported.
Prognosis[edit | edit source]
The prognosis for patients with littoral cell angioma is generally excellent, especially when the condition is isolated to the spleen. Surgical removal of the spleen usually resolves any associated symptoms.
Related Conditions[edit | edit source]
- Hemangioma
- Splenic marginal zone lymphoma
- Angiosarcoma
- Falk, S., & Stutte, H. J. (1991). "Littoral cell angioma of the spleen: a novel splenic vascular lesion demonstrating histiocytic differentiation." *The American Journal of Surgical Pathology*, 15(11), 1023-1033.
- Rosai, J. (2011). "Rosai and Ackerman's Surgical Pathology." 10th Edition.
NIH genetic and rare disease info[edit source]
Littoral cell angioma of the spleen is a rare disease.
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Contributors: Prab R. Tumpati, MD