8-1: 10/4: Tumors Of Kidneys And Bladder

Front 1

What are the 2 most common renal tumors?

What tumor is most common in children, but rare in adults?

Back 1

RCC is most common (mostly in adults)

Urothelial carcinomas of renal pelvis are 2nd most common

Nephroblastoma (Wilms tumor) most common in children, rare in adults

Front 2

Subdivisions of RCC:
List them
Which is most common?
Which has best prognosis?

Back 2

Clear Cell: 70-80%; 45-70% survival
Papillary: 10-15%; 50-85% survival
Chromophobe: 5%, BEST PROGNOSIS: 80-95% survival
CD: 1% incidence

Front 3

RCC:
Triad (Presentation)
Mets Locations
What epithelium does it derive from?

Back 3

RCC:
Hematuria, costovertebral pain, paplable mass (but only in 10% of pts)
25% has mets at presentation, with half mets to lung, 1/3 mets to bone

Derived from renal TUBULAR epithelium (most commonly affects poles)

Front 4

_________ mutations in VHL, FH, BHD can result in _________.

Function of VHL?

Back 4

Loss of fn mutations in tumor suppressor genes (VHL, FH, BHD) can result in RCC.

VHL encodes ubiquitin for degradation of Hypoxia Inducible Factor (HIF); abnl VHL causes accumltn of HIF and subsequent release of GFs

Front 5

Activating mutations in MET can result in _______.

Back 5

RCC (MET = oncogene)

Front 6

Provide histogenesis (originating site in nephron) of renal cell neoplasms.

Back 6

PCT: Clear Cell
LOH: Papillary
CD: Chromophone, Oncocytoma

Front 7

This renal tumor invades adipose tissue and the renal vein.

Back 7

Clear Cell Carcinoma (YELLOW bc it likes fat)

Front 8

How do sporadic vs inherited kidney cancers differ in terms of age of onset?

Back 8

Inherited kidney cancers have much earlier onset (35-45 years)

Front 9

Von Hippel-Lindau disease is caused by mutations in _____.

Back 9

VHL gene! Encodes ubiquitin to degrade HIF.

Front 10

Loss of sequences on the short arm of chromosome 3p is associated with ______.

Back 10

Clear Cell RCC; 3p encodes VHL

Front 11

Type I vs Type II VHL Disease:
Associated Tumors

Back 11

Type I VHL: Clear Cell RCC

Type II VHL: Pheochromocytoma

Front 12

Patients who develop dialysis-associated cystic disease are likely to develop this type of RCC.

Back 12

Papillary RCC

Front 13

Mutations in MET proto-oncogene is associated with this RCC tumor.

Back 13

Hereditary Papillary RCC; late onset and multifocal

Front 14

This RCC is associated with mutations in fumarate hydratase.

Back 14

HLRCC (hereditary leiomyomatosis and RCC): leads to accumulation of hypoxia inducible factor just like clear cell carcinoma

Tends to be type II papillary RCC (aggressive!)
Skin symptoms!

Front 15

This RCC is associated with mutations in the BHD gene.

Back 15

Chromobode, oncotyoma (also presents with BENIGN SKIN TUMORS!!!)

Front 16

List associated mutations with RCC types.

Back 16

Clear: VHL
Pap 1: MET
Pap 2: Fumarate
Chromophobe: BHD
Oncocytoma: BHD

Front 17

This RCC is associated with sickle cell trait.

Back 17

Renal Medullary Carcinoma

Front 18

A renal papillary carcinoma that is under 0.5 cm is considered a _____.

Back 18

Renal Papillary Adenoma (benign!)

Front 19

Painless hematuris is the dominant clinical presentation of ______.

Back 19

Tumor of urinary bladder