title: Littoral cell angioma of the spleen in a patient with previous pulmonary sarcoidosis: a TNF-α related pathogenesis?
abstract: BACKGROUND
  Littoral cell angioma (LCA) is a rare vascular tumor of the spleen. Generally thought to be benign, additional cases of LCA with malignant features have been described. Thus, its malignant potential seems to vary and must be considered uncertain. The etiology remains unclear, but an immune dysregulation for the apparent association with malignancies of visceral organs or immune-mediated diseases has been proposed.
  
  
  METHODS
  We report a case of LCA in a 43-year old male patient who presented with a loss of appetite and intermittent upper abdominal pain. Computed tomography showed multiple hypoattenuating splenic lesions which were hyperechogenic on abdominal ultrasound. Lymphoma was presumed and splenectomy was performed. Pathological evaluation revealed LCA.
  
  
  CONCLUSIONS
  LCA is a rare, primary vascular neoplasm of the spleen that might etiologically be associated with immune dysregulation. In addition, it shows a striking association with synchronous or prior malignancies. With about one-third of the reported cases to date being co-existent with malignancies of visceral organs or immune-mediated diseases, this advocates for close follow-ups in all patients diagnosed with LCA. To our knowledge, this report is the first one of LCA associated with previous pulmonary sarcoidosis and hypothesizes a TNF-α related pathogenesis of this splenic tumor.
authors.affiliation: Department of Transplantation Medicine, University Hospital, Münster, Germany. s.cordesmeyer@ukmuenster.de
authors.forename: Stefanie
authors.initials: S
authors.lastname: Cordesmeyer
authors.forename: Manfred
authors.initials: M
authors.lastname: Pützler
authors.forename: Ulf
authors.initials: U
authors.lastname: Titze
authors.forename: Harald
authors.initials: H
authors.lastname: Paulus
authors.forename: Matthias W
authors.initials: MW
authors.lastname: Hoffmann
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000378:metabolism; D001706:Biopsy; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D006391:Hemangioma / Q000175:diagnosis / Q000276:immunology* / Q000378:metabolism; D006801:Humans; D007111:Immunity, Cellular; D007150:Immunohistochemistry; D008297:Male; D017565:Sarcoidosis, Pulmonary / Q000175:diagnosis / Q000276:immunology* / Q000378:metabolism; D013154:Spleen / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000175:diagnosis / Q000276:immunology* / Q000378:metabolism; D014057:Tomography, X-Ray Computed; D014409:Tumor Necrosis Factor-alpha / Q000276:immunology* / Q000378:metabolism
chemical_list: D014408:Biomarkers, Tumor; D014409:Tumor Necrosis Factor-alpha