Surgical Pathology Criteria

Clear Cell Tubulopapillary Renal Cell Carcinoma

Differential Diagnosis

Papillary Renal Cell Carcinoma Clear Cell Tubulopapillary Renal Cell Carcinoma
Clear cell cytoplasm, if present, is focal Widespread clear cell cytoplasm, frequently subnuclear/basal
Frequently has foamy macrophages, psammoma bodies, stromal hemosiderin Lacks foamy macrophages, psammoma bodies, stromal hemosiderin
May have high grade nuclear features and necrosis Low grade nuclear features, no necrosis
CD10 pos, Racemase/AMACR pos CD10 neg, Racemase/AMACR neg

Conventional Clear Cell Renal Cell Carcinoma Clear Cell Tubulopapillary Renal Cell Carcinoma
Sheets and clusters of cells with extensive vascular network Nearly all have branched tubules and anastomosing ribbons
Solid areas have clusters more than two cells wide Solid areas usually formed by compressed tubules, each only two cells wide
May have high grade nuclear features, vascular invasion and necrosis Low grade nuclear features, no invasion or necrosis
Cysts frequently due to degeneration, lined in areas by multiple layers of cells Uniformly lined by single layer of cells
Clear cytoplasm apical or diffuse, non-localized Frequent regular basal clearing, resembling interval endometrium
Usually CK7 neg, CD10 pos CK7 pos, CD10 neg to focal

Xp11 Translocation Renal Cell Carcinoma Clear Cell Tubulopapillary Renal Cell Carcinoma
Frequently occurs under age 20 Mean age 60
Typically high grade nuclear features Low grade nuclear features
Voluminous cytoplasm Moderate amount of clear cytoplasm
TPE3 immunostain or FISH positive in >80% Lacks Xp11 translocations
CK7 and other keratins negative to weak, CD10 pos, Racemase/AMACR pos CK7 strong pos, CD10 neg, Racemase/AMACR neg

Multilocular Cystic Renal Cell Carcinoma Clear Cell Tubulopapillary Renal Cell Carcinoma
No tubules, ribbons or papillae Nearly all have branched tubules, anastomosing ribbons and papillae
Clear cytoplasm apical or diffuse, non-localized Frequent regular basal clearing, resembling interval endometrium
Nests of clear cells in septa Nests of clear cells if present are formed by compressed tubules, each of which is only two cells wide
CK7 neg, CD10 pos CK7 pos, CD10 neg to focal

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