Typical histology | Expected immunoprofile | Recommendation | |
---|---|---|---|
Papillary | Nuclear pseudostratification, voluminous eosinophilic cytoplasm, high nucleolar grade | CK7 and AMARC variable | - rule out FH deficient |
Fumarate hydratase deficient | Prominent cherry-like nucleoli (may be focal); Mixed patterns including tubulocystic, papillary intracystic, tubulopapillary. | 2SC overexpression, FH negative | - recommend genetic testing for FH mutations (if 2SC/FH immunostain not available) in eosinophilic unclassified, papillary type 2, collecting duct carcinoma and tubulopapillary with solid foci |
Xp11 translocation | Large epitheloid clear and eosinophilic cells, psammoma bodies | TFE3+, Cathepsin K + | - refer to TFE3 break-apart FISH testing (if TFE3 immunostain not available) if suggestive morphology or presentation < 50 year or with lymph node metastasis |
Collecting duct | Infiltrative growth, desmoplastic stromal reaction | PAX8+ CK7 + SMARCB1/INI-1 + HMWCK + OCT3/4 – GATA3 – P63 - | - diagnosis of exclusion after ruling out FH deficient, medullary, urothelial and metastatic carcinoma |
Medullary | Infiltrative growth; desmoplastic stromal reaction; adenoid cystic, reticular and microcystic patterns | PAX8+ CK7 + SMARCB1/INI-1 - HMWCK - OCT3/4 + | - only diagnose it if proved sickle cell disease or sickle cell trait |