Primary female genital lymphoma complicated by recurrent borderline mucinous ovarian tumor: a case report and review of the literature | BMC Women’s Health


PFGSL is a sporadic neoplastic disorder that accounts for 1.5% of all non-Hodgkin lymphomas. PFGSL usually occurs in women over the age of 60 [1, 2]. The most frequently affected genital locations are the ovaries and the uterus; the vagina and vulva are rarely affected. However, endometrial lymphoma is extremely rare. Its clinical manifestations consist of abnormal vaginal bleeding and discharge, abdominal pain and abdominal masses. The main histologic type of PFGSL is B cell lymphoma, of which diffusing large cell lymphoma (DLBCL) is the most common subtype. [3]. The diagnosis of PFGSL should meet the following criteria: (i) the disease process is clinically confined to the uterus at the time of initial diagnosis; (ii) a full investigation does not reveal any signs of disease elsewhere in the body; (iii) there is a lack of abnormal cells in the bone marrow or peripheral blood; and (iv) if other lymphomatous deposits occur at sites which are removed from the genital tract, a time interval of at least several months should elapse between the appearance of the primary and secondary tumors. [4, 5]. Therefore, combined with the characteristics of this case, the patient described in this report was diagnosed with DLBCL from PFGSL occurring in the endometrium.

Previous case series have reported that LDGCB is the most common histologic type of PFGTL [3, 6]. Its clinical symptoms are generally nonspecific and include vaginal bleeding (70%), perineal discomfort (40%), and persistent vaginal discharge (20%) [7]. In the case described in this report, genital symptoms and abnormal imaging findings were not observed. Therefore, the disease was difficult to diagnose by cytology, cervical biopsy, and endometrial curettage. We obtained malignant lymphoma tissue from the deep endometrial stroma and diagnosed the patient using a transcervical needle biopsy. [8].

There are currently no randomized clinical trials or specific guidelines for the treatment of PFLGT. Patients receive full treatment, which usually involves a combination of surgery, chemotherapy, and radiation therapy. Surgical treatment aims to define the pathology and stage of the tumor, reduce the tumor burden and improve the effectiveness of postoperative chemotherapy or radiotherapy. At present, comprehensive treatment based on chemotherapy and surgery can improve the survival rate of patients. However, chemotherapy is primarily given for lymphoma. Regarding treatment with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP), the combination of CHOP treatment, standard treatment, and rituximab, a chimeric anti-CD20 monoclonal antibody to immunoglobulin G, has been used successfully to treat CD20 positive. B cell non-Hodgkin lymphoma [9]. Currently, R-CHOP therapy is the first-line treatment for diffuse large B-cell lymphoma. R-CHOP therapy consists of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone. It has been reported that the effectiveness rate of R-CHOP therapy for DLBCL in the female reproductive system can reach 71.88% after surgery and chemotherapy [10]. However, there is no standard treatment for peripheral T cell lymphoma. Options include CHOP therapy and gemcitabine combined with L-asparaginase. The course of chemotherapy should be 4-6 cycles and 2-3 periods should occur after complete remission [11, 12]. Due to the unusual nature of the disease, the clinical manifestations are nonspecific, the diagnosis is difficult, and the method of treatment is entirely different from other tumors of the reproductive system. Early analysis of lymphoma as well as the pathologic type and stage of lymphoma can enable patients with PFGSL to receive timely and accurate treatment and improve patient survival rates. The clinical symptoms of PFGSL are not typical and the prognosis is poor. Therefore, clinicians should strengthen the analysis and understanding of the disease and attach great importance to pathological and immunohistochemical findings, early diagnosis of lymphoma, pathological type and stage of lymphoma so that patients with PFGSL can receive fast and accurate treatment and that the survival rate of patients can be improved.


About Author

Comments are closed.