L62 - Renal Tumors

Question 1

name 3 malignant neoplasms of the kidney

which is the most common

which is in peds patients

Answer 1

RRC – most common
Urothelial (transitional) carcinoma
Wilms (Nephroblastoma) – peds

Question 2

RCC –

how do these patients typically present ?

what is the “classic” triad of symptoms

Answer 2

“silent” tumors –typically present with paraneoplastic symptoms (EPO, PTH, ACTH)

If presenting with the tumor itself symptom triad: CVA tenderness, flank mass and hematuria

Question 3

RCC –
difference in presentaiton between hereditary and sporadic ?

what is the associated hereditary etiology

males or females more likely to get sporadic?

Answer 3

Hereditary – multiple and bilateral

Hereditary = VHL (loss of tumor suppressor 3p; leading to incresaed HIF and VEGF)

Sporadic: males > 60 yo; unilateral

Question 4

Most common morphology of RCC; what does this look like?

Answer 4

Clear Cell RCC - 75% of RCCs

Clear abundant cytoplasm

Question 5

what are the other different morphological subtypes of RCC besides Clear cell ?

what is a defining characteristic about each morphology?

which has the worst prognosis?

Answer 5

Papillary – fibrovascular stalks, Lipid laden foamy macrophages

Chromophobe RCC - “plant like”; well defined cell membranes

Sacromatoid: Worst prognosis

Question 6

how does RCC metastasize

what is the overall 5 year survival rate?

Answer 6

Hematogenous spread through the Renal vein and RCC

5 year survival - 70%

Question 7

RCC treatment options

Answer 7

Nephrectomy
Partial nephrectomy –
Ablation - cryotherapy
Chemo – mets

Question 8

from what part of the tubule do RCC arise?

Answer 8

PCT

Question 9

what part of the kidney do kidney urothelial carcinomas arise?

Answer 9

renal pelvis

Question 10

Urothelial carcinoma –

major risk factors

Answer 10

cigarette smoke
analine dyes
analgesic overuse

Question 11

Nephroblastoma (Wilms Tumor)

• who gets these?
• are the sporadic or congenital ?

histology

Answer 11

Kids

usually sporadic

Histology –triphasic morphology; kidney is immature and tries to recapitulate nephrogenesis –

(blastema, epithelial, stroma)

Question 12

what is a nehprogenic rest?

Answer 12

Precursor lesions to Wilm’s tumor

Question 13

of wilms tumor is congenital, what syndromes are they assocaited with?

associated genes; what chromosome

Answer 13

WAGR - wilms, aniridia, genital abnormalities, and mental/motor Retardation

WT1 and WT2 genes, Chr 11

Denys Drash Syndrome –

Beckwith Wiedemann Syndrome –

Question 14

Nephroblastoma –
where are the common mets?

treatment and prognosis

Answer 14

mets to the lungs

2 year survival rates ~90%

Treatment: Surgery + chemo

Question 15

4 benign renal tumors/growths

what is the most common

Answer 15

Papillary Adenoma – most common

Oncocytoma
Angiomyolipoma

cysts

Question 16

Oncocytoma –

defining histology

Answer 16

epithelial cell tumors;
Eosinophilic infiltrates
abundant mitochondrdia

Question 17

Angiomyolipoma

what three tissues are seen on hist?

a/w what tumor syndrome

Answer 17

vessels,
smooth muscle
Fat

Tuberous sclerosis