Wilms Tumor/RAS

Question 1

What is a Wilms tumor?

Answer 1

Cancerous tumor in the cells of the kidney

Question 2

What age group are Wilms tumors most prevalent in?

Answer 2

Children, especially <10 y.o.

Question 3

What is the most common etiology of Wilms tumors?

Answer 3

Abnormal renal development: proliferation of metanephric blastemal w/o normal tubular and glomerular differentiation

Question 4

What 4 gene mutations are associated with Wilms tumors?

Answer 4

(1) WAGR syndrome- Wilms tumor, Aniridia, Genitourinary anomalies + intellectual disability (mental retardation)
(2) chromosomal deletion- WT1 gene
(3) the Denys-Drash syndrome
(4) Beckwith-Wiedemann syndrome

Question 5

What is the main sign of a Wilms tumor?

Answer 5

Sudden appearance of a solid mass in the abdomen or swelling of the abdomen

Question 6

What 4 signs do pts with a subscapular hemorrhage have?

Answer 6

(1) rapid abdominal enlargement
(2) anemia
(3) HTN
(4) sometimes fever

Question 7

What is the most common metastatic site of a Wilms tumor?

Answer 7

Lungs + lymph nodes

Question 8

What is found on PE of a Wilms tumor?

Answer 8

A firm, nontender, smooth mass that is eccentrically located and rarely crosses the midline

Question 9

What 3 dx tests do you order when suspecting a Wilms tumor?

Answer 9

(1) abdominal ultrasonography
(2) abdominal CT or MRI

(3) chest imaging x-ray or CT

Question 10

What 5 labs do you order when suspecting a Wilms tumor?

Answer 10

(1) UA
(2) CBC
(3) CMP (+ LFTs, renal functions)
(4) serum Ca
(5) coag studies

Question 11

What is a Wilms tumor typically surrounded by?

Answer 11

A pseudocapsule

Question 12

What are the 3 favorable cell types of Wilms tumor and the 1 unfavorable cell type?

Answer 12

Favorable:

(1) blasternal
(2) stromal
(3) epithelial

Unfavorable:
(1) anaplasia

Question 13

What is the National Wilms Tumor Study (NWTS) based upon?

Answer 13

Surgical evaluation prior to admin of chemo

Question 14

What is the mainstay of Wilms tumor tx?

Answer 14

Surgery

Question 15

What are the 4 indications for doing an upfront biopsy + pre-nephrectomy chemo?

Answer 15

(1) tumor thrombus above the level of the hepatic vein
(2) pulmonary compromise from massive tumor or extensive pulmonary metastases
(3) resection requiring removal of contiguous structures (not inc. adrenal glands)
(4) surgeon judges that attempting nephrectomy would result in significant morbidity, tumor spill, or residual tumor

Question 16

What are 3 high risk pt groups requiring serial abdominal US?

Answer 16

(1) Beckwith-Wiedemann syndrome or isolated hemihyperplasia
(2) WAGR and WT1 related syndromes
(3) siblings of an individual w/familial Wilms tumor and offspring of survivors of bilat Wilms tumor

Question 17

What is renal artery stenosis (RAS)?

Answer 17

Narrowing or complete occlusion of one or both renal arteries

Question 18

What is the main cause of RAS and what are 3 additional causes?

Answer 18

(1) atherosclerosis-main cause
(2) arterial embolism
(3) vasculitis
(4) fibromuscular dysplasia (FMD)

Question 19

What is a classic finding of FMD?

Answer 19

Beads on a string

Question 20

What are 4 RAS associated conditions?

Answer 20

(1) atherosclerotic disease (CAD, PAD)
(2) new onset of HTN
(3) extrinsic compression on a renal artery (mass/tumor)
(4) embolic disease (Afib, MI)

Question 21

What are 3 common clinical manifestations of RAS?

Answer 21

(1) onset of stage II HTN after 55 yrs
(2) mod-severe HTN in a pt w/any of the following that are unexplained: diffuse atherosclerosis, unilateral small kidney, asymmetry in renal size of more than 1.5 cm, or moderate to severe HTN and recurrent episodes of flash pulmonary edema
(3) acute elevation in Cr→ 30% elevation after starting ACE or ARB

Question 22

What are 4 findings on PE of RAS?

Answer 22

(1) HTN
(2) abdominal bruit
(3) flank bruit
(4) hypertensive retinopathy

Question 23

What are 4 labs/tests for RAS work up?

Answer 23

(1) repeat BP
(2) UA: protein, blood
(3) BMP
(4) elevated plasma renin

Question 24

What is the gold standard dx test for RAS?

Answer 24

Renal arteriography (invasive)

Question 25

What is a less invasive dx test for RAS?

Answer 25

Renal ultrasound w/doppler

Question 26

When should you suspect bilateral RAS?

Answer 26

Anytime there is an acute dec in renal function after giving an ACE inhibitor

Question 27

What are 3 components of RAS management?

Answer 27

(1) medical management: control HTN, manage lipids, aspirin
(2) weight loss
(3) smoking cessation

Question 28

What 4 types of pts have a high likelihood of benefitting from invasive intervention?

Answer 28

(1) a short duration of BP elevation prior to dx
(2) failure of optimal medical therapy to control the BP
(3) intolerance to optimal medical therapy (4) recurrent flash pulmonary edema +/or refractory heart failure

Question 29

What are the 3 tx of unilateral RAS?

Answer 29

(1) medical therapy
(2) percutaneous angioplasty (PCTA) with or w/o stent placement
(3) surgical revascularization

Question 30

What are 3 tx of bilateral RAS?

Answer 30

(1) medical therapy alone
(2) percutaneous angioplasty (PCTA) usually w/stent placement + medical therapy
(3) surgical revascularization + medical therapy

Question 31

What are 5 RAS tx pearls?

Answer 31

(1) ACE is no longer drug of choice
(2) PCTA is primary tx for FMD
(3) stenting is necessary for atherosclerotic RAS
(4) surgical revascularization is reserved for pts who cannot be stented
(5) pharmacotherapy is necessary even w/surgical tx

Question 32

What do you want to make sure to check in RAS monitoring?

Answer 32

Check BMP 3-4 weeks after starting ACE inhibitor