Introduction/Background Primary lymphoma of the gynecologic tract is very rare and often misdiagnosed due to its rarity. Extra-nodal presentation of non-Hodgkin’s lymphoma (NHL) accounts for only 0.008% of all cervical malignancies and 1.5% of all ovarian malignancies. Since the prognosis and the treatment are completely different, thus, differential diagnosis should be considered.

Methodology We present two cases of a 45-year-old woman with a primary diffuse large B-cell lymphoma (DLBCL) of the cervix (FIGO IIIB; Ann Arbor IV) and a 67-year-old woman with a primary DLBCL of the ovary (FIGO IA; Ann Arbor IV) synchronous cervical cancer (squamous cell carcinoma; FIGO IB2). Diagnosis NHL of the cervix and ovary was based on immunohistochemical study. Systemic therapy using conventional regimens of R-CHOP and R-Mini-CHOP were used, respectively. However, patient with cervical lymphoma died after first cycle of chemotherapy due to suspicion of B Symptoms, while patient with ovarian lymphoma still within second cycle of chemotherapy with a worsening condition.

Results The diagnosis of primary lymphoma of the gynecologic tract was a challenge for clinicians and pathologists since misdiagnosis and histologic confirmation using IHC may lead to a longer time to make a final diagnosis and treatment plan. Systemic therapy with a four-drug regimen (cyclophosphamide, doxorubicin, vincristine, and prednisone, with/without rituximab) has demonstrated effectiveness in lymphoma carcinoma. It yields a cure rate of ~60–70% and a wide range of adverse effects (cardiac, hematologic, and neurologic toxicities). However, our two cases of gynaecologic lymphoma were treated with RCHOP with unsatisfied outcomes. The question arises whether RCHOP is effective in treating patients with primary gynecological NHL or following clinical guidelines for gynecologic cancers.

Conclusion A prompt diagnosis should be highlighted considering that the prognosis and treatment of primary gynecological lymphoma are completely different. Further study is needed to evaluate the best treatment of primary gynecological NHL.

Disclosures This study have no conflict of interest.