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Tubulocystic Renal Cell Carcinoma With Pure Morphology and Confirmed "Wild Type" FH/2SC Immunophenotype:
Lara R Harik1,2, Cristina Magi-Galluzzi3, Varsha Manusha4
1Department of Pathology and Laboratory Medicine, Emory University School of Medicine.
Abstract:
Tubulocystic renal cell carcinoma is a rare neoplasm, first adopted into the WHO classification of kidney tumors in 2016. The diagnostic criteria were refined in the 2022 WHO classification, requiring "pure morphology" and exclusion of other renal cell carcinoma subtypes with overlapping features. We identified 31 tubulocystic renal cell carcinomas from 30 patients. Median age was 60 years (30 to 77 y) with male:female ratio of 13.5:1. Race was known for 26 patients, and the majority were African American (n = 16/26,62%), followed by white/Caucasian (10/26, 38%). Eleven patients (37%) had a history of chronic or end-stage renal disease. Median tumor size was 2.3 cm (range: 0.4 to 6.3 cm). All tumors were characterized by cysts and tubules, surrounded by fibrotic stroma. Lining epithelial cells had eosinophilic cytoplasm, ranging from flattened to cuboidal to hobnail in arrangement. By definition, solid epithelial nodules and destructive invasion were absent. In addition, all tumors had a normal pattern of FH and 2SC expression by immunohistochemistry. AJCC stage was pT1 for all 31 tumors: 30 pT1a and 1 pT1b. All patients had no evidence of disease at last follow-up (median: 35 mo; range: 1 to 294 mo). We report a large series of tubulocystic renal cell carcinomas with pure morphology and confirmed normal/"wild type" FH/2SC immunophenotype. When these strict definitions are applied, our findings confirm an indolent clinical behavior.
Insights
Tubulocystic renal cell carcinoma, a rare kidney tumor, exhibits a predominantly indolent clinical behavior when diagnosed with pure morphology and a normal FH/2SC immunophenotype. This study confirms its favorable prognosis in a significant patient cohort.
Area of Science:
- Oncology
- Pathology
- Urology
Background:
- Tubulocystic renal cell carcinoma (TRCC) is a rare kidney neoplasm, with diagnostic criteria updated in the 2022 WHO classification.
- Refined criteria emphasize "pure morphology" and exclusion of overlapping subtypes.
- Understanding TRCC's characteristics and behavior is crucial for accurate diagnosis and patient management.
Purpose of the Study:
- To report a large series of TRCC cases adhering to strict diagnostic criteria.
- To evaluate the clinical behavior and immunophenotype of TRCC with pure morphology.
- To confirm the indolent nature of TRCC when defined by specific pathological features.
Main Methods:
- Retrospective analysis of 31 TRCC cases from 30 patients.
- Review of histopathological features, including morphology and absence of solid nodules or invasion.
- Immunohistochemical assessment of FH (fumarate hydratase) and 2SC (2-succinocyclinase) expression.
- Correlation of pathological findings with clinical outcomes and AJCC staging.
Main Results:
- 31 TRCCs were identified, predominantly in older males, with a significant proportion in African American patients.
- A notable percentage of patients had a history of end-stage renal disease.
- All tumors showed characteristic tubulocystic patterns, lacked solid nodules/invasion, and displayed normal FH/2SC expression.
- All tumors were staged as pT1, and patients showed no evidence of disease at follow-up, indicating an indolent course.
Conclusions:
- Strict adherence to the "pure morphology" definition and normal FH/2SC immunophenotype in TRCC is associated with an indolent clinical behavior.
- TRCC, when meeting these precise diagnostic criteria, demonstrates a favorable prognosis.
- These findings reinforce the importance of refined diagnostic criteria for accurate TRCC classification and outcome prediction.
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