Sketchy Path: Renal - "Renal Cell Carcinoma & Nephroblastoma"

Question 1

RCC is most common in what demographic?

Answer 1

• Men
• 6th/7th decades

(Think of the older man driving the RenasCCar.)

Question 2

What are some risk factors for RCC?

Answer 2

• HTN (steam coming out of his ears)
• Smoking (he’s smoking a cigar)
• Obesity (his large belly)
• Petroleum products (oil slick)

Question 3

Why are clear cell carcinomas clear?

Answer 3

Glycogen and fat (like the donut on the car)

Question 4

RCC arises from what cell type?

Answer 4

PCT (like the Pro Cart Track banner)

Question 5

RCC is associated with a disorder on what chromosome?

Answer 5

3 (being von Hippel Lindau)

Think of the VHL and #3 on the first car.

Question 6

Explain the inheritance pattern of VHL.

Answer 6

It is a dominant-recessive mutation, meaning that you need to loss both copies to develop the phenotype, but this typically happens by inheriting one bad copy and having the other mutate during your lifetime.

Question 7

Other than RCC, those with VHL syndrome develop _______________.

Answer 7

hemangioblastomas of the retina (like the mom with curly red hair over her red sunglasses)

Question 8

How can the characteristics of RCC tumors clue you in to whether it is VHL or not?

Answer 8

VHL are more often bilateral, while sporadic is unilateral.

Think of the two kids with fatty helmets on each.

Question 9

RCC can cause what invasive symptoms?

Answer 9

RCC is aggressive. It can invade the renal vein and even the IVC, causing left varicocele and right-sided heart symptoms (congestive hepatopathy, peripheral edema).

(Think of the two drivers aggressively climbing the IVC pole and the audience member with the left dilated ber cap.)

Question 10

The triad of RCC symptoms is ______________.

Answer 10

flank mass, flank pain, and hematuria

(Think of the guy with his hands in his pockets –mass –getting hit with the bumper –flank pain – and the red stripe on the hood –hematuria.)

Question 11

List the four paraneoplastic syndromes associated with RCC.

Answer 11

• Erythrocytosis (bunch of red balloons with air PrO sign)
• Cushing’s (ACTion sign)
• Renin production (raining umbrella)
• PTHrp (PHd)

Question 12

RCC ______________ early.

Answer 12

metastasizes

Question 13

_______________ is also called nephroblastoma.

Answer 13

Wilms tumor

Think of the guys BLASTing against each other in the poster next to the WILl call.

Question 14

Which chromosome is Wilms tumor on?

Answer 14

11

Think of wi11 call.

Question 15

Describe the presentation of Wilms tumor.

Answer 15

• Diagnosed before age 10 (young kid in helmet)
• Unilateral, encapsulated (sleek helmet on one side)
• May cause HTN from excess renin production (guy with steam ears and rain umbrella)

Question 16

WAGR and ______________ are syndromic forms of Wilms tumor.

Answer 16

Denys-Drash (think of Dennis Dash, the driver in the poster)

Question 17

____________ is due to imprinting errors.

Answer 17

Beckwith-Wiedemann (like the fading ink – the improper printing –of Beck Will Win on the poster)

Question 18

What disorder often presents with benign tumors of the kidney?

Answer 18

Tuberous sclerosis, which presents with renal angiomyolipomas

(Think of the happy kid –meaning he’s not sick –with the TUBE of pringles eating a corn dog… The corn dog symbolizes the tumor because it has ketchup (angio), meat (myo), and greasy covering (lipoma).)