title: Primary uterine NK-cell lymphoma, nasal-type: a unique malignancy of a prominent cell type of the endometrium.
abstract: Natural killer (NK) cells host in the human endometrium with dedicated role in reproductive physiology. Interestingly, malignant transformation of these specialized cells has not been presented thus far. Here we report a primary endometrial NK-cell lymphoma of a 48 year-old patient presenting with irregular bleeding. The endometrial curetting showed a dense lymphomatous infiltrate demonstrating highly infiltrative aggressive features with characteristic angiocentric, partially angiodestructive growth pattern and accompanying focal necroses. The lymphoma cells displayed a CD3ε/CD56/TIA-1/granzyme-B-positive and CD5/CD4/CD8/TCRγδ-negative immunophenotype, proved to be positive for Epstein-Barr virus by EBER in situ hybridization, and revealed no clonal T-cell receptor gene rearrangement. The diagnosis of uterine extranodal NK-cell lymphoma, nasal-type was made. Clinically, the disease was limited to the uterus at diagnosis, but progressed rapidly, and the patient died within 5 months due disseminated lymphoma, irrespective of intensive chemotherapy. Genuine NK-cell lymphomas occurring in the uterus as primary site seem to be rare making the therapeutic decisions extremely complicated.
authors.affiliation: Department of Pathology, University of Debrecen, Nagyerdei krt. 98, 4032, Debrecen, Hungary. gabor.mehes@dote.hu
authors.forename: Gábor
authors.initials: G
authors.lastname: Méhes
authors.forename: Katalin
authors.initials: K
authors.lastname: Hegyi
authors.forename: Tamás
authors.initials: T
authors.lastname: Csonka
authors.forename: Ferenc
authors.initials: F
authors.lastname: Fazakas
authors.forename: Zsolt
authors.initials: Z
authors.lastname: Kocsis
authors.forename: Gáspár
authors.initials: G
authors.lastname: Radványi
authors.forename: István
authors.initials: I
authors.lastname: Vadnay
authors.forename: Enikő
authors.initials: E
authors.lastname: Bagdi
authors.forename: László
authors.initials: L
authors.lastname: Krenács
mesh_terms: D003937:Diagnosis, Differential; D016889:Endometrial Neoplasms / Q000175:diagnosis* / Q000188:drug therapy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000175:diagnosis* / Q000188:drug therapy; D008875:Middle Aged; D009669:Nose Neoplasms / Q000175:diagnosis* / Q000188:drug therapy; D002583:Uterine Cervical Neoplasms / Q000175:diagnosis* / Q000188:drug therapy