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Cystic neoplasms of the kidney are rare, and present a unique diagnostic challenge. We report the case of an elderly male who presented with a large cystic neoplasm, which was a diagnostic dilemma clinically and radiologically. Histopathological examination showed a tumour composed of variably sized tubules lined by atypical cells having large round nuclei with prominent nucleoli. Hobnailing was seen at places. Tumour cells were immunopositive for pancytokeratin, vimentin, CD10, CK19 and AMACR, confirming a diagnosis of tubulocystic renal cell carcinoma (TC-RCC).

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Results: PostCA19-9 were measured within 3 months after surgery (20-88 days, median; 43 days). Group C patients showed significantly poorer survival (group A/B/C; 3-year OS 61.9% /53.4% /12.4%; median survival time 44.2m /37.3m/ 13.6m; p< 0.001). The early recurrence rate within 6 month (p< 0.001) and the frequency of liver metastasis (p=0.009) were significantly higher in Group C. Multivariate analysis revealed that without adjuvant chemotherapy (HR=3.29; p< 0.001), preoperative-CT tumor size>20mm (HR=2.76; p< 0.001) and sustained elevation of postCA19-9 (HR=2.49; p=0.0017) were the independent significant prognostic factors for poor survival. The ROC curve analysis revealed that the optimal cut-off value of preCA19-9 which predict postCA19-9 normalization was 116 U/ml. Conclusion: Sustained elevation of postCA19-9 is a strong prognostic factor for R-PDAC. Patients with preCA19-9>120 could be considered the existence of potential distant metastasis (especially liver metastasis).