Wilms Tumor/RAS Flashcards
What is a Wilms tumor?
Cancerous tumor in the cells of the kidney
What age group are Wilms tumors most prevalent in?
Children, especially <10 y.o.
What is the most common etiology of Wilms tumors?
Abnormal renal development: proliferation of metanephric blastemal w/o normal tubular and glomerular differentiation
What 4 gene mutations are associated with Wilms tumors?
(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
What is the main sign of a Wilms tumor?
Sudden appearance of a solid mass in the abdomen or swelling of the abdomen
What 4 signs do pts with a subscapular hemorrhage have?
(1) rapid abdominal enlargement
(2) anemia
(3) HTN
(4) sometimes fever
What is the most common metastatic site of a Wilms tumor?
Lungs + lymph nodes
What is found on PE of a Wilms tumor?
A firm, nontender, smooth mass that is eccentrically located and rarely crosses the midline
What 3 dx tests do you order when suspecting a Wilms tumor?
(1) abdominal ultrasonography
(2) abdominal CT or MRI
(3) chest imaging x-ray or CT
What 5 labs do you order when suspecting a Wilms tumor?
(1) UA
(2) CBC
(3) CMP (+ LFTs, renal functions)
(4) serum Ca
(5) coag studies
What is a Wilms tumor typically surrounded by?
A pseudocapsule
What are the 3 favorable cell types of Wilms tumor and the 1 unfavorable cell type?
Favorable:
(1) blasternal
(2) stromal
(3) epithelial
Unfavorable:
(1) anaplasia
What is the National Wilms Tumor Study (NWTS) based upon?
Surgical evaluation prior to admin of chemo
What is the mainstay of Wilms tumor tx?
Surgery
What are the 4 indications for doing an upfront biopsy + pre-nephrectomy chemo?
(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
What are 3 high risk pt groups requiring serial abdominal US?
(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
What is renal artery stenosis (RAS)?
Narrowing or complete occlusion of one or both renal arteries
What is the main cause of RAS and what are 3 additional causes?
(1) atherosclerosis-main cause
(2) arterial embolism
(3) vasculitis
(4) fibromuscular dysplasia (FMD)
What is a classic finding of FMD?
Beads on a string
What are 4 RAS associated conditions?
(1) atherosclerotic disease (CAD, PAD)
(2) new onset of HTN
(3) extrinsic compression on a renal artery (mass/tumor)
(4) embolic disease (Afib, MI)
What are 3 common clinical manifestations of RAS?
(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
What are 4 findings on PE of RAS?
(1) HTN
(2) abdominal bruit
(3) flank bruit
(4) hypertensive retinopathy
What are 4 labs/tests for RAS work up?
(1) repeat BP
(2) UA: protein, blood
(3) BMP
(4) elevated plasma renin
What is the gold standard dx test for RAS?
Renal arteriography (invasive)
