An angiocentric orbital lesion with an immature natural killer cell immunophenotype.

TitleAn angiocentric orbital lesion with an immature natural killer cell immunophenotype.
Publication TypeJournal Article
Year of Publication2001
AuthorsEly SA, Werner A, Chadburn A
JournalHum Pathol
Volume32
Issue3
Pagination339-42
Date Published2001 Mar
ISSN0046-8177
KeywordsBiopsy, Blood Vessels, CD56 Antigen, Child, Preschool, Conjunctiva, Epithelium, Eyelids, Female, Humans, Immunohistochemistry, Immunophenotyping, Killer Cells, Natural, Orbit, Orbital Neoplasms, Receptors, IgG, Remission, Spontaneous
Abstract

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.

DOI10.1053/hupa.2001.22744
Alternate JournalHum Pathol
PubMed ID11274645
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