234. RCC

Question 1

RCC Epidemiology

• prevalence in M and F
• how do most present
• Main RF, other RFs

Answer 1

M: 6th most common Ca
F: 8th most common Ca

Present localized, most lethal of urologic cancers

RF: SMOKING MAIN (obesity, ESRD, chemical exposure)

Question 2

RCC: Presenting signs

• classic triad
• main way they are dx
• what is the mechanism of Stauffer’s syndrome?

Answer 2

1. Hematuria
2. Flank Pain
3. Palpable mass

> 70% dx incidentally

Stauffers syndrome (non-metastatic hepatic dysfx due to high IL6)

Question 3

RCC: Dx

• what is the workup of hematuria
• how are solid kidney lesions classified?
• how are cystic kidney lesions classified?

Answer 3

Hematuria: Multipase Cross-Sectional Imaging, Urine Culture, Cystoscopy

Solid Lesions: Malignant (80%) - RCC, medullary RCC, CD Carcinoma, UCa
Benign (20%) - Oncocytoma, Angiomyolipoma

Cystic Lesions
I - simple homogenous, no malignancy risk
II - thin septa, low risk
III - thick irregular septa, mild heterogeneity, high risk
IV - thick walls, nodular, heterogeneity, ENHANCEMENT - highest malignancy risk

Question 4

Sporadic RCC

• demographic, presentation
• subtypes: CCRCC (mLc bio), PRCC, ChRCC (origin cell)
• prognosis for subtypes

Answer 4

95% unilateral
Late Age >50

Clear Cell RCC: from proximal tubule, due to abnormal HIF regulation = high VEGF = vascular growth = development of CCRCC

Papillary RCC: from proximal tubule

Chromophobe RCC: from intercalated cell of CD

Prognosis: Clear Cell worst, Chromophobe Best

Question 5

Hereditary RCCs
- presentation, genetics, age

Explain the mutation and type of RCC and other findings of the following

• von Hippel Lindau
• Hereditary Papillary RCC
• Hereditary Leiomyomatosis RCC
• Birt-Hogg-Dube

Answer 5

Presents Bilaterally/multifocal, AD mutations, younger age (30s-50s)

vHL: vHL gene, CCRCC, retinal/CNS hemangioblastoma, PCC, pancreatic cyst, neuroendocrine tumors

HPRCC: MET gene, Pap RCC type 1 (solid multiple bilateral)

HLRCC: FH mutation, Pap RCC type 2 (higher grade), CD Ca, solitary and aggressive, uterine leiomyoma, uterine leiomyosarcoma, cutaneous nodules

BHD: BHD mutation, hybrid RCC (oncocytic, chromophobe, clear cell), cutaneous papules, lung cysts, colon polyps

Question 6

RCC staging

Answer 6

Size of tumor and invasiveness
T1: local to kidney, <7cm (1a <4cm; 1b 4-7cm)
T2: local to kidney, >7cm (2a 7-10cm, 2b >10cm)
T3: extends into major veins or perinephric tissues (not mets if continuous)
T4: invades beyond Gerota’s fascia (adjacent organs or distant mets)

Higher stage = worse prognosis

Question 7

What are the 3 ways to manage organ-confined disease

Answer 7

1. Surveillance - if small (<3cm), elderly/comorbid pt, CKD (prevent GFR loss), Hereditary syndromes (wait til >3cm)
2. In Situ Ablation
3. Surgical Excision: STANDARD OF CARE (most common)
   - radical nephrectomy
   - partial only if smaller to preserve GFR (bilateral/solitary kidney, low GFR, polar tumor)

Question 8

How to manage IVC tumor thrombosis? Grading of IVC tumor thrombosis

Answer 8

1. renal vein
2. further into renal vein
3. into IVC
4. IVC above hepatic veins, may be into RA

Need to remove thrombus with mass

• IVC thrombectomy
  (RA thrombus survival rate comparative to low stage disease)

Question 9

Mgmt of Metastatic Disease

• prognosis
• tx (5)

Answer 9

Prog: median 11 mo (poor) 11% 5 year survival

Tx

1. Cytotoxic Chemo (poor results)
2. Antiangiogenesis Agents (block VEGF/HIF - tyrosine kinase inhibitors -inib)
3. mTOR inhibitors (evrolimus, temsirolimus)
4. Immunotherapy (Anti-CTLA4, PD1, PDL1)
5. Cytoreductive surgery/metastatectomy