INTRODUCTION
Non-Hodgkin’s lymphomas (NHL) comprises 70–80% of all lymphoma cases.1 They are generally found in lymph nodes or other lymphoid tissue like the spleen and bone marrow.2 However, around 20–24% of NHL cases are extranodal and mostly originate from areas like the gastrointestinal tract, lungs, central nervous system, skin, thyroid, and salivary glands.
Although the secondary involvement of the female genital tract, especially the ovaries is common, primary genital tract lymphomas are exceptionally rare, accounting for less than 0.5% of genital cancers and 1.5% of all NHL cases. These tumors are anatomically reported in the following order of prevalence: ovary (49%), uterus (29%), fallopian tube (11%), vagina (7%), and vulva (4%).2,3
Diffuse large B cell lymphoma (DLBCL) is a subtype of non-Hodgkin lymphoma that comprises around 30% of total NHL cases. DLBCL can be further classified into three subtypes; germinal center B-cell, activated B-cell, and unclassified subtype.
So far only a couple of dozen cases of DLBCL of vulva are reported in the medical literature and is hence a very rare entity.4
We present a rare case of DLBCL of the vulva in a 78-year-old elderly patient.