Renal tumors

Question 1

what is the inheritance of tuberous sclerosis? what are the symptoms?

Answer 1

• autosomal dominant
• epilepsy
• mental retardation
• adenoma sebaceum
• multiorgan hamartomas

Question 2

if there is fat within a renal mass, what does that point to? what can be excluded?

Answer 2

• angiomyolipoma

- RCC

Question 3

what is the treatment for angiomyolipoma under 4cm? over 4cm?

Answer 3

• under 4cm: observation

- over 4cm: selective embolization, partial nephrectomy, radical nephrectomy

Question 4

is an oncocytoma benign or malignant? from what cell type does it derive?

Answer 4

• benign

- distal tubules

Question 5

RCC typically arises from what cell type(s)?

Answer 5

proximal convoluted tubules (clear cell and papillary types)

Question 6

is RCC primarily sporadic or genetic?

Answer 6

sporadic

Question 7

when RCC is genetic-based, it is associated with what disease?

Answer 7

von hippel lindau

Question 8

what is the inheritance of von hippel lindau?

Answer 8

AD

Question 9

von hippel lindau is characterized by development of what cancers, and where?

Answer 9

• RCC
• pheochromocytomas
• retinal angiomas
• hemangioblastomas of brain stem, cerebellum, spinal cord

Question 10

von hippel lindau is associated with a mutation on what chromosome?

Answer 10

3

Question 11

what are the paraneoplastic syndromes found in RCC?

Answer 11

• hypercalcemia (PTRP)
• HTN (renin)
• polycythemia (EPO)
• nonmetastatic liver dysfunction (Stauffer’s)

Question 12

medullary type renal carcinoma is associated with what condition?

Answer 12

sickle cell trait

Question 13

papillary subtype renal carcinoma is associated with what oncogene mutation?

Answer 13

c-met

Question 14

chromophobe subtype renal carcinoma is derived from what cell type?

Answer 14

distal tubules

Question 15

what is the diagnostic study of choice for renal cancer?

Answer 15

CT urogram

Question 16

what test is the best way to evaluate extent of renal vein / IVC involvement?

Answer 16

MRI

Question 17

what are the indications for renal biopsy?

Answer 17

• prior to surveillance / ablation
• indeterminate lesion in high risk patient
• rule out metastasis
• primary tumor dx in metastatic disease

Question 18

what are the sites of metastasis for renal cancer?

Answer 18

• retroperitoneal lymph nodes
• lung
• liver
• bone
• brain

Question 19

what is the name of the grading system for clear cell RCC? what are the criteria?

Answer 19

• fuhrman system

- increasing nuclear size / irregularity / prominence

Question 20

which is more important for prognosis - grade or stage?

Answer 20

stage

Question 21

what are the treatment options for RCC?

Answer 21

• active surveillance
• radical nephrectomy
• partial nephrectomy
• thermal / cryo/ radio frequency ablation

Question 22

what is hyperfiltration injury? what can it lead to? what is the first sign

Answer 22

• glomerular hyperfiltration occurs in remaining tissue following removal of functional renal tissue
• can lead to focal segmental GN (first sign is proteinuria)

Question 23

what is the only therapy shown to produce a complete and durable remission of RCC? what are the downsides?

Answer 23

• IL-2 and IFN

- poor response rate, highly toxic

Question 24

what is the most common finding in tumors of the renal pelvis and ureter?

Answer 24

filling defect in collecting system with contrast

Question 25

diagnosis for renal pelvis and ureter tumors is made using which tests?

Answer 25

• urine cytology
• cystoscopy
• ureteroscopy

Question 26

what is the gold standard treatment for tumors of the renal pelvis and ureter?

Answer 26

radical nephroureterectomy (kidney, ureter, bladder cuff)