Info
Hereditary RCC and germline testing-of-renal-cell-carcinoma
- There are several RCC-assoc genetic CA syn incl.
- VHL
- HLRCC (FH gene),
- BAP1 tumor predisposition syn
- Birt-Hogg-Dubé (FLCN gene)
- among others
Consider any of the following for Hereditary RCC gene panel testing & CA Genetics referral
FHx
- Close relative w/ known pathogenic variant
- ≥1 1st-or 2nd-degree relative w/ RCC
Clinical Characteristics
- Dx’d at age ≤46 y
- Bilateral or multifocal tumors
- Angiomyolipomas of kidney + 1 other TSC-assoc criterion
- tuberous sclerosis
Histopathologic Characteristics
- Multifocal papillary histology
- Histologic features a/w HLRCC or FH deficiency found
- BHD syn-related histology (multiple chromophobe, oncocytoma, oncocytic hybrid)
- Succinate dehydrogenase (SDH) deficient histology
Siblings
- Epidemiology-of-renal-cell-carcinoma
- Histology and molecular biology-of-renal-cell-carcinoma
- Hereditary RCC and germline testing-of-renal-cell-carcinoma
- Clinical presentation-of-renal-cell-carcinoma
- Staging (TNM classification) and prognosis-of-renal-cell-carcinoma
- Treatment principles-of-renal-cell-carcinoma
- Systemic therapy for metastatic clear cell RCC
- Second-line therapies-of-renal-cell-carcinoma
- Combined-modality therapies-of-renal-cell-carcinoma
- Tyrosine kinase inhibitors-of-renal-cell-carcinoma
- Immunotherapies-of-renal-cell-carcinoma
- mTOR inhibitors-of-renal-cell-carcinoma
- Non-clear cell histology-of-renal-cell-carcinoma