Angioma de células litorales esplénico. Presentación de caso clínico / Littoral cell angioma of the spleen. Report on a clinical case / Angioma de células litoraneas do baço: apresentação de um caso clínico

El angioma de células litorales del bazo es una neoplasia vascular benigna poco frecuente, de presentación clínica, paraclínica e imagenológica inespecífica. Se realizó en nuestro paciente la esplenectomía, el tratamiento indicado y curativo, y se logró el diagnóstico a través de la histología e inmunohistoquímica de la pieza de anatomía patológica.

Littoral cell angioma of the spleen is rather an unusual benign vascular neoplasm with non-specific clinical, paraclinical and imaging presentation. Our patient underwent splenectomy, performed as the appropriate and remedial treatment. Diagnosis was made by means of histology and immunohistochemistry techniques of the pathologic anatomy of the surgical piece.

O angioma de células litoraneas do baço é uma neoplasia vascular benigna rara com apresentação clínica, laboratorial e de imagem inespecíficas. A esplenectomia foi realizada em nosso paciente, sendo esse o tratamento indicado e curativo, obtendo-se o diagnóstico através da histologia e imunohistoquímica da peça anatomopatológica.

13 cases of littoral cell angioma in spleens / 北京大学学报(医学版)

Objective: To investigate the clinicopathological features, morphological characteristics, immunophenotypes of littoral cell angioma (LCA) in spleen, and to provide new evidence for making diagnosis and avoiding misdiagnosis.Methods: Clinicopathological data, histological characteristics of 13 cases of LCA were retrospectively studied and immunohistochemical staining was imposed on the paraffi-nembedded specimens, and 5 cases of cavernous hemangioma, 4 cases of normal littoral cells of spleens were used as control groups, simultaneously.Results: All the 13 LCA patients included 7 males and 6 females, aged from 39 to 70 years with an average of 54.2 years and a median age of 55 years.Among these tumor patients, 6 cases were accompanied by malignances, benign tumors or inflammation states at abdominal cavities, and 7 cases were accidentally discovered by physical examinations.Grossly, spleens contained solitary or multiple gray red nodules ,which ranged from 0.5 to 6.2 cm in diameter.Histologically, tumors were composed by anastomosing vascular spaces which were lining by plump, rounded to cuboidal littoral cells that extended into vascular lumens.Usually, papillary frameworks that were covered by these cells were also seen extending into the lumens in some areas.Other types of histiocytoid cells were identified in lumens and the sizes were larger than the littoral cells.Both types of cells absented cytologic atypia.Immunohistochemical study demonstrated that the littoral cells in all cases were positive for vascular endothelial and histiocyte markers, such as CD21, CD31, CD68, polyclone FⅧRAg and ERG, while these cells were negative for CD8, CD34, and WT-1.These findings manifested that immunophenotype of littoral cell in LCA distinctive from that in controls.Conclusion: LCA is a benign lesion, which frequently occurs in the elderly.Its etiology remains confusion, however, immune dysregulation may associate with it because of the concomitance with other tumor or inflammation in some cases.The littoral cells in LCA show a hybrid endothelial-histiocytic phenotype on immunohistochemistry, therefore these cells may have features that intermediate between those of endotheliocytes and histiocytes.Emphasizing the histological findings and immunophenotypes is significant for diagnosis and differential diagnosis.

Splenic Littoral Cell Angioma with Hepatitis C Associated Liver Cirrhosis

Littoral cell angioma (LCA) of spleen is an uncommon vascular neoplasm of littoral cell origin. It is well established that LCA may be associated with other malignancy or autoimmune disorders. We describe a 34-year-old woman with viral hepatitis C associated liver cirrhosis who presented with the incidental finding of LCA. She showed ascites, esophageal varix and drowsy mentality. Abdominal computed tomography (CT) showed multiple benign looking nodules in both hepatic lobes, but no abnormality in spleen. Liver transplantation and splenectomy were performed. Microscopic findings revealed narrow anastomosing vascular channels lined with plump cells that exfoliated into the lumen. Immunohistochemically the lining cells were positive for CD31, CD68 and negative for CD34, consistent with LCA. Herein, a second case of an incidentally detected LCA with cirrhosis, viral hepatitis C associated in Korea is reported.

A Case of Bicytopenia Combined with Littoral Cell Angioma

Littoral cell angioma is a recently described vascular tumor of spleen with an unknown etiology. We present a case in a 74-year-old woman with severe anemia, thrombocytopenia and palpable splenomegaly. The computed tomography of abdomen showed well-defined multiple nodules in spleen. Splenectomy was performed and the histological and immunohistochemical features of splenic tumor were consistent with a littoral cell angioma. The patients had persistent anemia and thrombocytopenia after splenectomy. We suggest hepatic destruction or other autoimmune mechanism contributes persistent bicytopenia. This case illustrated the refractory bicytopenia combined with littoral cell angioma.