The female genital tract can be involved as a secondary manifestation of disseminated lymphomas or leukaemia but can rarely be the primary site of so-called extranodal lymphomas. Primary lymphomas of the female genital tract can affect the uterine corpus, uterine cervix, vulva, vagina, or adnexa. Only about 0.008% of all cervical tumours are primary malignant lymphomas. The most common clinical presentation of primary cervical lymphomas is a history of prolonged minor abnormal uterine bleeding, while unstoppable bleeding at presentation is rarely reported in the literature. “B” symptoms related to nodal lymphomas are usually absent. Since vaginal bleeding is a nonspecific symptom, the first diagnostic hypothesis is usually of one of the more common female genital conditions such as cervical or endometrial carcinoma or sarcoma, fibroids, adenomyosis, or endometriosis. Cervical cytology is usually negative. Preoperative diagnosis requires deep cervical biopsy. No guidelines regarding optimal treatment exists; radiotherapy, chemotherapy, and surgery are used in different combinations. Conservative treatment with the combination of surgery and chemotherapy or surgery and radiotherapy has been reported in a few cases with apparent success. With this review, we aim to understand what the best therapeutic approaches for this rare pathology in young and elderly women are. Moreover, we find favorable pregnancy outcome in patients treated with a fertility sparing approach.

The female genital tract can be involved as a secondary manifestation of disseminated lymphomas or leukemia but can rarely be the primary site of so-called extranodal lymphomas. Primary lymphomas of the female genital tract can affect the uterine corpus, uterine cervix, vulva, vagina or adnexa. Only about 0.008% of all cervical tumors are primary malignant lymphomas. The presenting symptoms are unspecific and also refer to much more common diseases of the female genital tract. Cervical cytology is usually negative. Preoperative diagnosis requires deep cervical biopsy. To date there is no consensus regarding optimal treatment. Radiotherapy, chemotherapy and surgery are used in different association. We report the first case reported in literature managed with an urgent hysteroscopic resection of a primary cervical diffuse large B-cell lymphoma, followed by adjuvant radiotherapy. Relevant literature was reviewed. Our conservative approach needs to be validated in the future, especially for women with pregnancy desire and for those with low performance status. To date, after 24 months from diagnosis, our patient is still disease-free.