Introduction/Background*Non-Hodgkin lymphoma (NHL) is an hematologic malignancy and 25% of NHL patients present with a primary extranodal tumor. Primary female genital tract presentation is extremely rare (<0,1%) with only a few cases reported.
Result(s)*83-year-old, nullipara whom consulted for vaginal bleeding. She used a long-standing pessary as treatment for syntomatic pelvic prolapse. When pessary was removed an exophytic and violet tumor was observed. It involved the proximal vagina and part of cervix.
A transvaginal ultrasound confirmed a well-defined 35X40 mm lesion located in proximal vagina. Rectum was not involved.
During the diagnostic process the patient consulted for heavy genital bleeding, uncontrollable with conservative treatment such as packing of the vagina.
Computed tomography was performed. It revealed a solid homogeneous cervical mass of 6x7x6 cm with a 5 cm haematoma invading right parametria without observing active bleeding and uterine arteries embolization was performed by the Intervencional Radiology Unit achieving correct hemostasis.
Histopathological diagnosis was obtained after a punch vaginal biopsy and reported clonal plasmablastic large cell neoplasia, suggestive of intensely positive Epstein - Barr virus.
PET/CT showed the known mass in the cervico-uterine region with intense associated hypermetabolism, without visualizing any pelvic adenopathies or in other locations with abnormal metabolism.
Hematologists reviewed the patient and recommended chemotherapy. The patient is currently awaiting the treatment and coordinate follow-up care.
Conclusion*Although the primary cervicovaginal lymphoma has an extremely low prevalence, it should be included in the differential diagnosis of gynecologic malignancies.
Radical surgery does not play a role in the treatment of this malignancy since the mainstays of treatment are cytotoxic chemotherapy, monoclonal antibodies and/or radiation therapy. Fortunately, the vast majority of women are diagnosed as early stage disease and characterized by a very high sensitivity to chemotherapy and favorable prognosis.
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