An angiocentric orbital lesion with an immature natural killer cell immunophenotype.
In this report, a unique case of a large unilateral orbital tumor occurring in a 5-year-old white girl is described. The lesion, which was associated with no systemic clinical symptoms, waxed and waned in size over 12 months and eventually spontaneously resolved. Multiple biopsies were performed, which showed an angiocentric and angioinvasive infiltrate composed of a monotonous population of atypical, immature-appearing, large lymphocytes. Extensive molecular and immunophenotypic studies led to the diagnosis of an Epstein-Barr virus-negative angiocentric lymphoproliferative disorder with an immature natural killer (NK) cell immunophenotype (CD16+CD56-), specifically an immunophenotype expressed by normal cord blood NK cells. HUM PATHOL 32:339-342.
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Department of Pathology, Weill Medical College of Cornell University, New York, NY 10021, USA.
Werner
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Chadburn
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D001706:Biopsy; D001808:Blood Vessels / Q000473:pathology*; D019002:CD56 Antigen / Q000032:analysis; D002675:Child, Preschool; D003228:Conjunctiva / Q000473:pathology; D004848:Epithelium / Q000473:pathology; D005143:Eyelids / Q000473:pathology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000276:immunology / Q000473:pathology*; D009915:Orbit / Q000098:blood supply*; D009918:Orbital Neoplasms / Q000276:immunology / Q000473:pathology*; D017452:Receptors, IgG / Q000032:analysis; D012075:Remission, Spontaneous
D019002:CD56 Antigen; D017452:Receptors, IgG