Primary lymphomas of the cervix and uterus: The University of Pennsylvania's experience and a review of the literature

Primary lymphomas of the cervix and uterus are rare with ∼50 cases reported in the world literature to date. Appropriate diagnosis is often delayed until the post-operative setting as clinical and radiographic presentations are non-specific. Several sub-types of lymphoma arising primarily in the cervix or uterus have been reported with diffuse large B-Cell lymphoma (DLBCL) being the most frequent. Due to the low incidence of this disease, no randomized clinical trials exist to help guide treatment. This study reports the experience of four patients with primary lymphomas of the uterus and cervix that reflect the heterogeneity of cases reported in the literature to date. The patients had a mean age at diagnosis of 46 (range 35-56) and presented with abnormal uterine bleeding. One patient was diagnosed by total abdominal hysterectomy (TAH), two patients were diagnosed by cervical biopsy and one patient was diagnosed by endometrial biopsy. Three patients had DLBCL and one patient had marginal zone lymphoma (MZL). All patients had stage IIE disease. Of the patients with DLBCL, one received chemotherapy followed by TAH and two received TAH followed by chemotherapy. Two of these three patients remain disease-free post-initial therapy with the third now disease-free post-salvage therapy and autologous stem cell transplant. The patient with MZL was treated with TAH alone and remains disease-free. Based on the case series and a review of available literature, primary lymphomas of the uterus or cervix are rare and require an individualized approach to treatment. In general, patients with limited stage disease should be treated with localized and systemic therapy to optimize chances of cure.