ABSTRACT
BACKGROUND: Primary diffuse large B-cell lymphoma of the cervix is a very rare disease, with non-specific clinical presentation. Its prognosis depends on accurate and timely diagnosis and therapy. Moreover, the management of this tumour has never been standardized. CASE REPORT: Herein we present a rare case of primary diffuse large B-cell lymphoma of the cervix misdiagnosed as cervical myoma. Our systematic review of the English literature identified 143 cases of primary diffuse large B-cell lymphoma of the uterus. Patients' characteristics and oncological, surgical, and safety data were recorded and analyzed. CONCLUSION: Although rare, primary diffuse large B-cell lymphoma of the cervix should never be ruled-out. Given its non-specific clinical symptoms, a multidisciplinary approach is required to perform a timely diagnosis and administer appropriate therapy. Immunochemotherapy (Rituximab + CHOP or CHOP-like regimen) with/without radiotherapy is the most common and most effective treatment; surgery should be avoided.