Kidney Cancer

Breaking down RCC

Types of RCC

Tumor Type % all RCC Cellular origin Nuggets
(mainly prognosis)
Clear cell 70-80% Proximal tubule Aggressive behavior
Responds to targeted therapy
Papillary Type I 5-10% Good prognosis
Papillary Type II Worse prognosis than Type I
Chromophobe 3-5% Intercalated cells of collecting duct Sarcomatoid has worse prognosis
Clear cell papillary 5% Good prognosis with indolent behavior
Seen in VHL and ESRD
Collecting duct (Bellini) < 1% Collecting duct Poor prognosis
May respond to chemo
Renal medullary Collecting duct Poor prognosis
Usually seen in sickle cell patients
HLRCC-associated - Early metastasis
Poor prognosis
SDH-associated Occur in young adults
MiT Family (Xp11 and t(6;11) 40% pediatric RCC
Acquired cystic-disease-associated Seen in ESRD and ACD patients
Good prognosis
Multilocular cystic clear cell Mass with multiple cysts
Almost uniformly benign
Tubulocystic Favorable prognosis
Mucinous tubular and spindle cell Favorable prognosis
Hybrid oncocytic chromophobe Generally good prognosis
Seen in Birt-Hogg-Dube
Sarcomatoid differentiation 1-5% Variant seen in most RCC Indicates worse prognosis
Sarcomatoid differentiation
Unclassifed 1-5% Unclear Poor prognosis
Aggressive behavior

Familial RCC Syndromes

Syndrome Gene/Chromosome Type of RCC Associated Findings Management
Von-Hippel Lindau (VHL) VHL (3p25) Clear cell (may be multifocal) Renal cysts
Hemangioblastomas
Retinal angiomas
Pheochromocytoma
Epididymal cystadenomas
If < 3cm - active surveillance
If > 3cm - nephron sparing surgery
Hereditary Papillary Renal Carcinoma MET (7q31) Type I Papillary (multiple, bilateral) None
Birt Hogg Dube (BHR) FLCN (17p11) Chromophobe Oncocytoma
Renal cysts
Cutaneous fibrofolliculomas
Lung cysts
Spontaneous pneumothorax
Cowden Syndrome PTEN (10q23) Clear cell
Chromophobe
Type I papillary
Mucocutaneous lesions
Facial trichilemmomas
Breast tumors
Epithelial thyroid cancer
Hereditary Leiomyomatosis RCC (HLRCC) FH (1q42-43) Type II Papillary
Collecting Duct
Cutaneous leiomyomas
Uterine leiomyomas
Surgical excision with wide margins
Succinate dehydrogenase deficiency RCC SDHB (qp36)
SDHC (1q23)
SDHD (11q23)
Clear cell
Chromophobe
Type II papillary
Oncocytoma
Paragangliomas
Papillary thyroid cancer
Tuberous Sclerosis TSC1 (9q34)
TSC2 (16p13)
Clear cell Angiomyolipoma
Oncocytoma
Polycystic kidneys
Cardiac rhabdomyomas
Cutaneous angiofibromas
Lymphangiomyomatosis
Seizures
Autism
AML: surveillance (< 3cm), everolimus (3-5cm), surgery/embolization (> 5cm)
RCC: surgery (> 3cm)

Diagnosis

Presentation

Imaging findings concerning for malignancy

Complete the Workup

When to Biopsy

Treatment Options

Active Surveillance

Focal Ablation

Surgical Considerations

Partial Nephrectomy

Radical Nephrectomy

Management of complicated RCC situations

Locally Invasive (T4)

Clinical Node Disease (cN1)

Adrenal involvement

IVC Thrombus

Local Recurrence

Adjuvant therapy

Metastatic RCC Management

Current targeted therapies

Current recommendations

MSK model for predicting outcomes with chemotherapy or cytokine therapy

IMDC model for predicting outcomes with VEGF-targeted therapy

Cytoreductive nephrectomy (CRN)

Prior therapies (mainly historical interest)

Non-RCC Tumors

Sarcoma

Leukemia/Lymphoma

Metastatic disease

Benign renal tumors

Algorithm for AML management, from Campbell's

Renal Cysts

Bosniak Renal Cyst Classification

Bosniak Class Findings % Malignancy Plan
I Water density
Homogenous wall
No septa, calcifications, or enhancement
0-2% No follow-up required
II Few septations with possible enhancement
Fine calcifications
No obvious enhancement
0-18% No follow-up
IIF Multiple septations
Perceived wall/septal enhancmenets
Calcifications without enhancement
No obvious enhancement
3-18% Repeat imaging to assess stability
III Thickened/irregular walls/septations with measurable enhancement 33-50% Observation, excision, ablation
IV Complex cystic mass
Enhancing nodular components
"clearly malignant"
75-93% Surgery

Oncocytoma

Angiomyolipoma

Other masses

References