Tubulocystic Renal Cell Carcinoma
Epidemiology and Etiology:
- < 1% of all renal carcinomas
- Male predominance
- Mean 58.4 y (30-94 y)
Common sites:
- Left kidney (70% of cases)
Gross features:
- Renal cortex or corticomedullary
junction
- Solitary
- Well-circumscribed
- Multicystic
- White, spongy cut surface
- Multiple small to intermediate-sized cysts
- 0.7 to 17 cm (mean 4.2 cm)
Histologic features:
- Classic histologic features required
- Small to intermediate-sized tubules
- admixed with larger cysts
- single layer of flattened, cuboidal/columnar
and hobnail epithelium
- nuclei enlarged and irregular
- ISUP grade 3 nucleoli (intermediate to large)
- Abundant eosinophilic oncocytoma-like
aspects
- Fibrotic stroma
- Components identical to papillary RCC may exist
- Poorly differentiated or sarcomatoid
areas maybe
Immunophenotype:
Marker:
|
Sensitivity:
|
Specificity:
|
|
|
|
Molecular features:
- (Similar to papillary RCC)
- Gains:
- Losses:
Other features:
- Incidental finding usually (60%)
- Indolent behavior usually
- Only 1/70 cases recurred and 4/70 metastasized
References:
- Moch
et al. (eds.) WHO Classification of Tumours of
the Urinary System and Male Genital Organs, 4th ed. (2016)