cell angioma is a rare vascular tumor of the splenic red pulp, and is
typically an incidental finding on abdominal imaging. The splenic lesion
can only truly be differentiated from other splenic masses by histologic
Top ten interesting facts about the tumour:
(1) Littoral-cell angioma a rare primary tumour of the spleen that was first described by Falk et al in 1991.
(2) It is a new subtype of benign vascular tumour that involves the cells that line the splenic sinus channels. The tumour expresses both vascular and histiocytic antigens.
(3) This splenic tumour can occur at any age. It usually occurs in middle-aged men and women and has equal sex distribution. (1-77 years; median age, 50 years).
(4) Clinically, patients with Littoral cell angioma present with splenomegaly, thrombocytopenia, anemia, or with constitutional symptoms, such as fever of unknown origin.
(5 ) Littoral cell angioma may appear as single or multiple nodular lesions in the spleen. Splenic lesions may range from 0.2 to 6.0 cm in maximal dimension.
(6) This unique tumour has characteristic morphologic and immunohistochemical characteristics that differentiate it from other vascular splenic tumours.
Morphological diagnosis is based on the presence of anastomosing narrow sinus-like vascular channels lined with tall endothelial cells, focal papillary fronds, and normal splenic sinuses at the periphery of the lesion. Combination of morphological and immunohistochemical analyses show a hybrid endothelial-histiocitic phenotype which helps in establishing the diagnosis.
(7) Immunohistochemistry: The neoplastic cells express histiocytic antibody CD68 as well as VIII whereas usual endothelial splenic cells only express VIII.
(8) A malignant subtype, littoral cell angiosarcoma has also been described and possesses cytologically atypical cells and malignant morphologic features, such as invasion of surrounding organs.
(9) The treatment for Littoral cell angioma is splenectomy, which is both diagnostic and therapeutic.
(10) There is a strong association between this neoplasm and a group of immunologic or oncologic entities, including Crohn disease, tumours of visceral organs, including colorectal, renal, hepatocellular, lung and pancreatic adenocarcinomas, malignant lymphoma], myelodysplastic syndrome, Wiskott Aldrich syndrom, ankylosing spondylitis, aplastic anaemia and Gaucher’s disease.
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