Board Review Renal Tumors

Front 1

what's the most common benign solid renal tumor?

Back 1

renal medullary fibroma; females, most less than 1cm, 50% bilateral

Front 2

when should you bx a renal mass?

Back 2

when clinical or radiologic evidence suggests diagnosis other than RCCa or when a definite dx is necessary

Front 3

what is the incidence of RCC?

Back 3

3%; 30000 new each year; 12000 deaths; 90% of kidney tumors

Front 4

synchronous mets occur in what % of RCC pts?

Back 4

1/3; metachronous mets 30-50% at 5 yr follow-up; prognosis worse with synchronous

Front 5

how often does RCC invade adjacent organs?

Back 5

10%; very poor prognosis: 5yr survival less than 5%

Front 6

what are the recurrence rates of partial nephx?

Back 6

ipsilateral kidney 5-10%; 3-5% in contralateral kidney

Front 7

what % of RCC occur bilaterally?

Back 7

1-2%; metachronous in 3-5%

Front 8

what is then incidence of the different histologic types of RCC?

Back 8

clear cell (70%), papillary (15%), chromophobe (5%), sarcomatoid (2%), collecting duct (1%), and renal medullary carcinoma (less than 1%)

Front 9

what cystic renal dz are assoc with an inc incidence of RCC?

Back 9

acquired renal cystic dz (4-6fold); VHL (76% have cysts, 35-38%); TS (2%)

Front 10

what types of RCC are more aggressive?

Back 10

sarcomatoid, renal medullary carcinoma, collecting duct carcinoma

Front 11

which type of RCC is assoc with psammoma bodies?

Back 11

papillary type; 15% incidence of multifocality

Front 12

What is the predominant grp of pts in whom renal medullary type RCC appears?

Back 12

almost exclusively with sickle cell trait or hemoglobin SC disease; aggressive; young pts

Front 13

what is cell type of origin of most RCC?

Back 13

proximal convoluted tubule; Bellini tumors is collecting duct; renal medullary carcinoma from calyceal epithelium

Front 14

what are risk factors for RCC?

Back 14

smoking, men, urban dwellers; 50-70 yo, VHL, horseshoe kidney, acquired renal cystic dz, obesity

Front 15

What is VHL?

Back 15

rare, AD, multiorgan syndrome assoc with 35% incidence of RCC, 75% with renal, epididymal, and pancreatic cysts, cerebellar hemangioblastomas, retinal angiomas, and pheo; RCC tends to be multiple and bilateral (80%); 1/3 of pts die from RCC

Front 16

what is the fcn of the VHL gene?

Back 16

tumor suppressor gene

Front 17

where are the most common chromosomal changes observed in RCC?

Back 17

3p: familial, sporadic, VHL; hereditary papillary RCC t(X;1), trisomy 17 and trisomy or tetrasomy 7

Front 18

T/F: RCC have a true histologic capsule.

Back 18

False

Front 19

T/F: nuclear grade is a predictor of survival that is independent of pathologic stage.

Back 19

True. exception is spindle cells: indicate poor prognosis independent of nuclear grade

Front 20

what is the bosniak classification?

Back 20

I: simple cyst; II: septated, thin peripheral calc (15% malignant); III: complex cyst, hyperdense, thick calc, thick septa (50%); IV: enhancing thick wall; irr borders, thick calc (95%)

Front 21

how common are paraneoplastic syndromes assoc with RCC?

Back 21

30%

Front 22

what is Stauffer syndrome?

Back 22

reversible hepatic dysfcn in the absence of mets; frequently seen with clear cell RCC; abnl LFT's, fever, hepatic necrosis, typically resolves with nephx; persistence or recurrence is poor prognostic sign

Front 23

what other dz is assoc with Stauffer syndrome?

Back 23

XGP

Front 24

what is incidence of venous involvement in RCC?

Back 24

25% renal vein; 5-10% IVC; 1% atrial

Front 25

where are the most common sites of mets from RCC?

Back 25

lung, bone, LN, liver, adrenals (1-10%), brain, heart, spleen, skin

Front 26

describe general 5 yr survival for RCC.

Back 26

organ confined T1-2 (85-90%); T3a (70-75%), T3b-c (40-60%), N+ (10-20%), mets (0-5%)

Front 27

what is 5 yr survival of nephx and resection of solitary lung met?

Back 27

30-35%

Front 28

what is the typical characteristics of RCC in kids?

Back 28

only 3-5%; around 9yo with abdominal mass; survival worse than Wilms'

Front 29

where can you see renal hemangiomas?

Back 29

klippel-trenaunay and sturge-weber

Front 30

what % of leukemia cases will have renal involvement?

Back 30

up to 50%; NHL more common than Hodgkin's; multiple, bilateral; Tx is systemic; most common secondary tumors of the kidney

Front 31

besides lymphoid, what other primary tumors can met to the kidney?

Back 31

lung (squamous), breast, uterine, melanoma

Front 32

What is tuberous sclerosis?

Back 32

AD (9q), multiple and bilateral AML; mental retardation, adenoma sebaceum; late childhood;

Front 33

T/F: most pts with AML have tuberous sclerosis?

Back 33

False. although AML occur in 80% of pts with TS, less than 40% of pts with AML have TS

Front 34

what % of renal cancers result in spontaneous perinephric hemorrhage?

Back 34

60%

Front 35

what % of AML pats develop acute hemorrhage?

Back 35

10%; lesions larger than 4cm at higher risk

Front 36

what chromosomal abnl assoc with AML?

Back 36

LOH 16p12 in 50% of AML assoc with TS and 10% sporadic

Front 37

what is the characteristic organelle seen in oncocytoma on electron microscopy?

Back 37

mitochondria; gives eosinophilic cytoplasm in polygonal, uniform cells of oncocytoma. mitotic figures are rare

Front 38

what is incidence of multifocal or bilateral oncocytoma?

Back 38

multifocal 3-5%; bilateral 3-5%

Front 39

how often does RCC occur with oncocytoma?

Back 39

10%

Front 40

how common are renal oncocytomas?

Back 40

10%; peak incidence 60-80

Front 41

what cell type of origin for oncocytoma?

Back 41

proximal convoluted tubule

Front 42

what's the most common renal tumor in horseshoe kidneys?

Back 42

RCC, then Wilms'

Front 43

what are classic features of cystic nephroma?

Back 43

uncommon multicystic tumor; solitary, unilateral, multilobular; middle-aged women in upper pole

Front 44

how often are mets found at time of presentation of RCC?

Back 44

1/3; 30-50% will develop mets over 5 yrs; incidence of adrenal mets at nephx is just under 4%

Front 45

what is the most common type of primary retroperitoneal cancer?

Back 45

liposarcoma

Front 46

what is Robertson's tumor?

Back 46

renin-secreting juxtaglomerular apparatus tumor; rare, young women may occur during pregnancy; rhomboid renin protogranules on electron microscopy

Front 47

describe presentation of gas embolism in laparoscopic nephx.

Back 47

rapid and sudden dec in end-tidal CO2 (seconds) is earliest sign; severe hypotension, typical [mill-wheel] cardiac murmur and desat happen later and assoc with poor prognosis

Front 48

how do you treat gas embolus?

Back 48

desufflation, steep L lateral decub/T-burg to minimize any R ventricular outlet obs; central venous cath aspiration

Front 49

what is tension pneumoperitoneum?

Back 49

high intraperitoneal pressures over time lead to vena caval compression which dec venous return leading to hypotension; direct pressure on diaphragm makes ventilation difficult; CO2 is absorbed causing acidosis, hypoxia, and arrhythmias; eventual cardiovascular collapse; Tx immediate desufflation, correcting T-burg, IV fluids, and hyperventilation with 100% O2