Unilateral cervical carcinoma in a septate uterus with double cervix: A case report and literature review

Background The exact incidence of cervical cancer occurring in patients with Müllerian anomalies is unknown as both conditions are relatively rare and the literature is compromised principally of individual case reports or small series. Despite the rarity of this coexistence, the literature consistently highlights the significant diagnostic and therapeutic challenges it presents. This is particularly true when cervical duplication is present as the altered anatomy can hinder adequate cytologic sampling and delay the detection of malignant lesions. Case presentation We report a case of unilateral invasive endocervical adenocarcinoma in a 43-year-old patient with complete septate uterus and longitudinal vaginal septum. The tumor was confined to the left cervix, staged as FIGO IB2 and underwent a type C1 radical hysterectomy with bilateral salpingo-oophorectomy and pelvic and para -aortic lymphadenectomy. Following the histologic confirmation of a well-differentiated adenocarcinoma with lymphovascular invasion and pelvic nodal metastasis the patient underwent chemoradiation and remains disease-free at five years. Conclusion This case highlights how cervical duplication, particularly in the presence of a longitudinal vaginal septum may compromise routine screening and underscores the importance of careful anatomic assessment to ensure accurate diagnosis and individualized management in patients with uncommon Müllerian variants.