Atipical immunophenotype in a littoral cell angioma

Background. Littoral-cell angioma (LCA) is a recently described benign vascular tumor of the spleen, whose imaging and pathologic characteristics have been discussed only by a few authors. The tumor is characterized by a mixture of papillary and cystic areas lined by neoplastic cells deriving from n...

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Main Authors: Čolović Radoje, Suvajdžić Nada, Grubor Nikica, Čolović Nataša, Terzić Tatjana
Format: Article
Language:English
Published: Ministry of Defence of the Republic of Serbia, University of Defence, Belgrade 2009-01-01
Series:Vojnosanitetski Pregled
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Online Access:http://www.doiserbia.nb.rs/img/doi/0042-8450/2009/0042-84500901063C.pdf
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author Čolović Radoje
Suvajdžić Nada
Grubor Nikica
Čolović Nataša
Terzić Tatjana
author_facet Čolović Radoje
Suvajdžić Nada
Grubor Nikica
Čolović Nataša
Terzić Tatjana
author_sort Čolović Radoje
collection DOAJ
description Background. Littoral-cell angioma (LCA) is a recently described benign vascular tumor of the spleen, whose imaging and pathologic characteristics have been discussed only by a few authors. The tumor is characterized by a mixture of papillary and cystic areas lined by neoplastic cells deriving from normal splenic lining - littoral cells. The neoplastic LCA cells express both endothelial and histiocytic antigens associated with CD8 negativity, compared with the normal endothelium of the venous sinuses of the spleen red pulp that only expresses endothelial antigens and CD8 positivity. Therefore, the typical and characteristic immunohistochemical pattern of the LCA is as follows: CD31, CD68, CD163, CD21, FVIII antigen positive; CD34, CD8 negative. Case report. We reported a 60-year-old male with moderate nodular splenomegaly with one large hypoechogenic solid lesion and mild thrombocytopenia in whom the diagnosis of LCA was made after the elective splenectomy. Namely, histopathological and immunohistochemical data allowed a final diagnosis of classical LCA in spite of CD21 negativity. As far as we know this is the first reported CD21-negative LCA patient. Histological specimens were presented and differential diagnoses discussed. Conclusion. Littoral-cell angioma is a very rare benign splenic neoplasm that should be considered in the differential diagnosis of multinodular splenomegaly, particularly if the patient has the signs of hypersplenism.
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spelling doaj-art-bdceddd32b934c508a40c5affbe993342025-08-20T03:04:45ZengMinistry of Defence of the Republic of Serbia, University of Defence, BelgradeVojnosanitetski Pregled0042-84502009-01-01661636510.2298/VSP0901063CAtipical immunophenotype in a littoral cell angiomaČolović RadojeSuvajdžić NadaGrubor NikicaČolović NatašaTerzić TatjanaBackground. Littoral-cell angioma (LCA) is a recently described benign vascular tumor of the spleen, whose imaging and pathologic characteristics have been discussed only by a few authors. The tumor is characterized by a mixture of papillary and cystic areas lined by neoplastic cells deriving from normal splenic lining - littoral cells. The neoplastic LCA cells express both endothelial and histiocytic antigens associated with CD8 negativity, compared with the normal endothelium of the venous sinuses of the spleen red pulp that only expresses endothelial antigens and CD8 positivity. Therefore, the typical and characteristic immunohistochemical pattern of the LCA is as follows: CD31, CD68, CD163, CD21, FVIII antigen positive; CD34, CD8 negative. Case report. We reported a 60-year-old male with moderate nodular splenomegaly with one large hypoechogenic solid lesion and mild thrombocytopenia in whom the diagnosis of LCA was made after the elective splenectomy. Namely, histopathological and immunohistochemical data allowed a final diagnosis of classical LCA in spite of CD21 negativity. As far as we know this is the first reported CD21-negative LCA patient. Histological specimens were presented and differential diagnoses discussed. Conclusion. Littoral-cell angioma is a very rare benign splenic neoplasm that should be considered in the differential diagnosis of multinodular splenomegaly, particularly if the patient has the signs of hypersplenism.http://www.doiserbia.nb.rs/img/doi/0042-8450/2009/0042-84500901063C.pdfsplenic neoplasmsimmunohistochemistryhistologyantigens, CDsplenectomy
spellingShingle Čolović Radoje
Suvajdžić Nada
Grubor Nikica
Čolović Nataša
Terzić Tatjana
Atipical immunophenotype in a littoral cell angioma
Vojnosanitetski Pregled
splenic neoplasms
immunohistochemistry
histology
antigens, CD
splenectomy
title Atipical immunophenotype in a littoral cell angioma
title_full Atipical immunophenotype in a littoral cell angioma
title_fullStr Atipical immunophenotype in a littoral cell angioma
title_full_unstemmed Atipical immunophenotype in a littoral cell angioma
title_short Atipical immunophenotype in a littoral cell angioma
title_sort atipical immunophenotype in a littoral cell angioma
topic splenic neoplasms
immunohistochemistry
histology
antigens, CD
splenectomy
url http://www.doiserbia.nb.rs/img/doi/0042-8450/2009/0042-84500901063C.pdf
work_keys_str_mv AT colovicradoje atipicalimmunophenotypeinalittoralcellangioma
AT suvajdzicnada atipicalimmunophenotypeinalittoralcellangioma
AT grubornikica atipicalimmunophenotypeinalittoralcellangioma
AT colovicnatasa atipicalimmunophenotypeinalittoralcellangioma
AT terzictatjana atipicalimmunophenotypeinalittoralcellangioma