Abstract
INTRODUCTION AND IMPORTANCE: Renal cell carcinoma (RCC) in pregnancy is extremely rare, with an estimated incidence of 13 per million pregnancies. Papillary RCC, a less common subtype, poses unique diagnostic and therapeutic challenges during gestation. CASE PRESENTATION: A 38-year-old woman at 26 weeks' gestation presented with persistent right flank pain unresponsive to initial treatment. Imaging via ultrasound and magnetic resonance imaging (MRI) revealed a large right renal mass. Following multidisciplinary evaluation, an open radical nephrectomy was performed. Histopathology confirmed papillary RCC, pT2, Grade III. Both maternal and fetal outcomes were favorable, with no recurrence at 7 months' follow-up. CLINICAL DISCUSSION: Diagnosing RCC during pregnancy is challenging due to symptom overlap with common gestational disorders such as urinary tract infections or preeclampsia. Ultrasound and MRI, which avoid ionizing radiation, are the preferred imaging modalities, but can be limited by anatomical changes during pregnancy. Surgical resection remains the definitive treatment, with the second trimester offering a balance between minimizing fetal risk and preventing tumor progression. Decisions on timing and surgical approach must be individualized, considering tumor size, location, and gestational age. A multidisciplinary team involving urology, obstetrics, anesthesiology, and neonatology is essential for optimal maternal-fetal outcomes. Despite the predominance of clear cell RCC in pregnancy, this case highlights the need for awareness of rarer subtypes like papillary RCC. The absence of standardized management guidelines underscores the importance of case-by-case assessment. CONCLUSION: Timely diagnosis, individualized surgical planning, and coordinated multidisciplinary care are crucial in managing RCC during pregnancy, particularly for rare histological variants.