Primary lymphoma of the uterine cervix: A systematic review and integrated analysis of case reports and series.

Evidence on the diagnosis and management of women with primary lymphoma of the uterine cervix (PLUC) is limited. The present study performed a systematic review of the literature and provided an overview of the reported cases of PLUC. A total of 213 reports were included, which comprised 339 patients with PLUC. The mean age of the patients was 48.5 years (median, 46 years; age range, 15-88 years). The most common presenting symptom was vaginal bleeding (189/318, 59.4%) and its duration ranged from 4.5 days to 24 months, with only a small fraction of patients developing 'B' symptoms including weight loss, night sweats and fever (28/318, 8.8%). Biopsy (either excisional or punch biopsy) was the most commonly used initial/primary diagnostic modality (78/278, 28.1%) followed by ultrasound (59/278, 21.2%). The most common management approach out of 309 patients was surgery (with or without adjuvant or neo-adjuvant treatment; 115/309), followed by chemotherapy alone (109/309), which was followed by chemo-radiotherapy alone (62/309). The follow-up period for survivors ranged from 4 weeks to 246 months, and the absolute 5-year and 10-year survival rates were 86.1 and 85.4%, respectively. The relatively low number of patients and high heterogeneity did not permit a robust comparative analysis of the survival outcomes. However, the longest median survival was reported for women who received neo-adjuvant chemotherapy followed by surgery (15 patients; 72 months). Although malignant PLUC is rare, early detection, optimal therapeutic management, and multidisciplinary involvement of gynecologic oncologists and lymphoma specialists may offer benefit to patients diagnosed with this rare disease.