Abstract
Background: Tubulocystic renal cell carcinoma (TC-RCC) is a rare and distinct subtype of renal cell carcinoma with characteristic histopathological and immunohistochemical features. Due to its rarity, limited data exist regarding its clinicopathological profile, biological behavior, and prognosis. This review aims to analyze reported TC-RCC cases to improve understanding of tumor characteristics, outcomes, and key diagnostic markers. Methods: Scoping review of 30 published TC-RCC cases was conducted. Data collected included tumor laterality, site, size, focality, stage, associated malignancies, local extension, metastatic spread, lymph node involvement, vascular invasion, and immunohistochemical findings relevant to diagnosis and prognosis. Results: TC-RCC demonstrated a left-sided predominance (63.3%) and was most often unifocal. The upper renal pole was the most commonly involved site (36.7%). Tumor size ranged from 0.8 to 37 cm. Concurrent malignancies were identified in 26.7% of cases, most frequently papillary renal cell carcinoma. Extra-renal extension and distant metastases occurred in 16.7% of cases, while lymphovascular invasion was uncommon. Immunohistochemistry showed frequent positivity for AMACR (66.7%), CD10 (46.7%), and Vimentin (43.3%). Conclusion: TC-RCC exhibits variable clinical behavior and a notable association with concurrent malignancies. Immunohistochemical markers, particularly AMACR and CD10, are diagnostically valuable. Further studies are needed to establish prognostic markers and standardized management strategies.
