Hodgkin Lymphoma Presenting as a Vulvar Mass in a Patient With Crohn Disease: A Case Report and Literature Review

Objective. A case of Hodgkin lymphoma of the vulva and perineum is presented along with a review of the literature. Materials and Methods. Medical chart and clinical images were reviewed. Results. A 45-year-old female patient with a long-standing history of Crohn disease presented with a large vulvar and perineal mass. Physical examination revealed a mass measuring approximately 20 x 20 cm involving primarily the labia majora, the labia minora, and the clitoris as well as the perineum. Incisional biopsy of the vulvar mass revealed histologic diagnosis and immunohistochemistry typical of classic Hodgkin lymphoma. Imaging revealed involvement of multiple lymph nodes as well as the liver. The patient was designated as having stage IV disseminated Hodgkin lymphoma, and chemotherapy with doxorubicin, bleomycin, vinblastine, and dacarbazine was instituted. A significant reduction of the size of the vulvar mass was observed following 8 cycles of chemotherapy. Conclusions. Lymphoma of the vulva is rare with the majority being of the non-Hodgkin lymphoma type. The most common subtypes of vulvar lymphoma reported are diffuse large B-cell lymphoma and follicular lymphoma. Perianal Hodgkin lymphoma is also very rare but has been reported in association with human immunodeficiency virus infection, Epstein-Barr virus infection, and Crohn disease. This is only the second reported case of Hodgkin lymphoma of the vulva and the second case of Hodgkin lymphoma involving the perianal area in a female patient. There is currently no evidence that Crohn disease is associated with an increased risk of Hodgkin lymphoma.