Painless Vulvar Masses as Initial Manifestation of Marginal Zone Lymphoma

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Vulvar non-Hodgkin lymphoma (NHL) is quite rare, but female genital tract involvement may be a manifestation of systemic disease.1 One review of 186 cases of NHL in the female genital tract found that only 4% involved the vulva.2,3 The majority are of the diffuse large B-cell subtype.1 Vulvar NHL typically presents as a localized, painless mass in the labia majora.4 The literature suggests that vulvar NHL is often an aggressive disease, with death occurring in less than 5 years for many.5 Treatment may involve a combination of surgery, chemotherapy, and radiation achieving favorable outcomes.6 Here, we present a case of a rare subtype of vulvar marginal zone lymphoma (MZL).

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