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A 75 year old woman was referred to the gynaecology unit at her local hospital following two episodes of postmenopausal bleeding. Her last period had been 30 years previously. She was found to have a hard mass in the right vaginal fornix measuring 5 cm in diameter. The cervix was flush with the vaginal vault, and the parametrium felt thickened. An ultrasound scan showed a solid, adnexal mass, which was thought to be ovarian in origin. Laparoscopy showed a right-sided cyst, approximately 6 cm in diameter, densely adherent to the colon. The bowel, liver and diaphragm were normal. In view of these findings, the woman was referred to a gynaecological oncologist. Subsequent examination revealed the vaginal vault to be thickened as if infiltrated by a malignant process. The thickening extended to within 3 cm of the introitus and was confluent across both sides of the vault. The serum CA125 concentration was 6.0 u/L. Computed tomography revealed a partly cystic, partly solid mass in the pelvis, anterior to the uterus. Ascites was present, but there was no evidence of pelvic or para-aortic lymphadenopathy, and the liver, spleen, pancreas and left kidney all appeared normal. The right kidney was small with some scarring. The appearances were consistent with an ovarian tumour. Magnetic resonance imaging, however, suggested that the tumour was connected to the uterus, and that it had the appearance of a uterine fibroid; the paravaginal and paracervical tissues were thickened. There was no enlargement of the pelvic lymph nodes.
Examination under anaesthesia was performed. Biopsy of the bladder showed cystitis cystica. Biopsy of the vagina showed a fibrovascular connective tissue covered by normal stratified squamous epithelium. The stroma contained a dense infiltrate of lymphocytes, and immunohistochemistry showed a predominantly B-cell lymphoid population, suggesting a reactive or inflammatory process.
A biopsy of the paracervical tissue showed a mixed small and large cell follicular lymphoma. The disease was thought to arise in the cervix. Bone marrow aspiration showed that the bone marrow was infiltrated with tumour.
The woman was treated with chlorambucil. The tumour decreased in size after three months of treatment (Fig. 1). After six months, her disease is stable, and she has no symptoms.
Postmenopausal bleeding may be a symptom of carcinoma of the endometrium or cervix1, but lymphoma as a cause of postmenopausal bleeding is very unusual. Malignant extra nodal lymphoma occurring within the female genital tract is rare and usually arises in the cervix2. Primary lymphoma of the cervix is very rare, and lymphoma of the cervix is usually part of systemic spread. Lymphomas of the cervix are generally diffuse in nature3. In their case series, Harris et al.4 found that diffuse cervical lymphomas have usually not spread beyond the cervix and have a better prognosis than lymphomas in other sites.
The most common symptom of cervical lymphoma is abnormal vaginal bleeding. Other symptoms may include vaginal discharge, pain during sexual intercourse or pelvic pain. An abdominal or pelvic mass may be found on clinical examination. Fever, night sweats and weight loss are unusual2,4. Because the neoplasm originates in the submucosal cervical stroma and the overlying squamous epithelium is unaffected, cervical smears are usually normal5. Biopsy is required. The histological appearances of lymphoma are sometimes confused with benign inflammation6, and distinguishing between a poorly differentiated carcinoma and lymphoma may be difficult7. Diffuse enlargement and enhancement of the cervix and vagina on computed tomography or magnetic resonance imaging may suggest lymphomatous involvement, but the appearances are non-specific (Fig. 2) 8. Extensive lymphadenopathy suggests gynaecological involvement as part of disseminated disease, which is not infrequent in advanced lymphomas.
Treatment of follicular lymphomas depends on the stage and grade of the disease. A localised tumour may respond to radiation therapy. If there is invasion or dissemination, chemotherapy is the treatment of choice. Perren et al.9 reported five cases of lymphoma of the genital tract treated with chemotherapy or radiation therapy, and showed good results, without radical surgery.
The authors would like to thank Dr N. Wilkinson, Consultant Pathologist, for her assessment of the specimens submitted.