title: Littoral cell angioma of the spleen: a study of 25 cases with confirmation of frequent association with visceral malignancies.
abstract: OBJECTIVE
  Littoral cell angioma (LCA) is a rare primary splenic tumour that is frequently associated with internal malignancies. Immunohistochemistry can demonstrate a distinct hybrid endothelial-histiocytic phenotype of littoral cells, and is a helpful adjunct for making the correct diagnosis. The aims of this study were to present a series of 25 LCAs, with an emphasis on the frequent association of the neoplasm with visceral malignancies, and to provide a detailed immunohistochemical analysis by employing new markers.
  
  
  RESULTS
  All 25 cases with available tissue blocks were immunohistochemically stained for endothelial and histiocytic markers. Clinical and follow-up data were retrieved from the respective institutions. The tumours were obtained from 16 males and nine females, whose age ranged from 32 to 86 years (mean 56.2 years). Clinical information was available for 24 of 25 patients, and follow-up for 11 of 25 patients (range 2-19 years; mean 11.6 years). Immunohistochemically, all cases were positive for LYVE-1, factor VIII, FLI-1, vascular endothelial growth factor receptor (VEGFR)-2, VEGFR-3, claudin-5, ERG, LMO2, CD31, CD163, lysozyme, and CD4, but negative for D2-40, CD8, and factor XIIIa. Fifteen of 25 cases were associated with various malignancies, including epithelial, mesenchymal and haematological tumours.
  
  
  CONCLUSIONS
  The cohort of 25 patients is the largest series of LCAs published to date. By using antibodies against recently introduced endothelial markers, we have expanded the immunoprofile of LCA. We have further highlighted the clinical significance of LCA, as more than half of the patients in this study also harboured a coexisting visceral malignancy. Therefore, we conclude that the finding of splenic LCA mandates a thorough clinical evaluation for a concomitant malignancy.
authors.affiliation: Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Pilsen, Czech Republic. peckova.kveta@gmail.com.
authors.forename: Kvetoslava
authors.initials: K
authors.lastname: Peckova
authors.affiliation: Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Pilsen, Czech Republic.|Biomedical Centre, Faculty of Medicine in Plzen and Charles University Hospital Plzen, Pilsen, Czech Republic.
authors.forename: Michael
authors.initials: M
authors.lastname: Michal
authors.affiliation: Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Pilsen, Czech Republic.
authors.forename: Ladislav
authors.initials: L
authors.lastname: Hadravsky
authors.affiliation: Department of Pathology and MCW Cancer Center, Medical College of Wisconsin, Milwaukee, WI, USA.
authors.forename: Saul
authors.initials: S
authors.lastname: Suster
authors.affiliation: Department of Pathology and Laboratory Medicine, The University of Kansas School of Medicine, Kansas City, KS, USA.
authors.forename: Ivan
authors.initials: I
authors.lastname: Damjanov
authors.affiliation: Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Pilsen, Czech Republic.
authors.forename: Marketa
authors.initials: M
authors.lastname: Miesbauerova
authors.affiliation: Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Pilsen, Czech Republic.
authors.forename: Dmitry V
authors.initials: DV
authors.lastname: Kazakov
authors.affiliation: Department of Pathology, Charles University, Third Medical Faculty and Charles University Hospital Kralovske Vinohrady, Prague, Czech Republic.
authors.forename: Zdenka
authors.initials: Z
authors.lastname: Vernerova
authors.affiliation: Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Pilsen, Czech Republic.
authors.forename: Michal
authors.initials: M
authors.lastname: Michal
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis*; D005260:Female; D006391:Hemangioma / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D013160:Splenic Neoplasms / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor
keywords: immunohistochemistry; littoral cell angioma; spleen; vascular tumours; visceral malignancies