Peripheral T/natural killer-cell lymphoma involving the female genital tract: A clinicopathologic study of 5 cases

Malignant lymphoma of the female genital tract (FGT) is rare. In this study , 5 peripheral T/natural killer (NK)-cell lymphomas (PTCLs) involving the F GT are reported. They include 2 from the uterus and 1 each from ovary, uter us and ovary, and vagina, and were detected between 1996 and 2000. One of t he 2 ovarian tumors was bilateral. In all cases, the FGT was the initial si te of clinical presentation of disease. Age at presentation ranged from 21 to 52 years (median, 36 years). One case was stage I disease, 2 were stage II, and 2 were stage IV. All 5 tumors were positive for CD3 epsilon, and 3 harbored the Epstein-Barr virus, although the detailed immunophenotypic pro files varied. Three were diagnosed as nasal type T/NK-cell lymphoma, 1 as a naplastic large-cell lymphoma (anaplastic lymphoma kinase [ALK]-positive), and 1 as unspecified PTCL of cytotoxic phenotype, according to the forthcom ing World Health Organization classification. Four of 5 patients received l aparotomy and chemotherapy. Four patients tin stages II and IV) died of dis ease within 16 months of the initial diagnosis, whereas only 1 patient tin stage I) is alive without disease at 39 months of follow-up. Our experience in this series provided clinically relevant information on diagnosis, trea tment, and outcome for extremely rare tumors of the FGT. (C) 2001 The Japan ese Society of Hematology.