Renal and renal pelvic tumors

Question 0

Vhl associated with which type of RCC

Answer 0

Clear cell

Question 1

Location of VHL gene?

Answer 1

3p25

Question 2

What’s the cMet gene?

Answer 2

Oncogene associated with papillary RCC.

Question 3

Where is the cMet gene located?

Answer 3

7q31. Papillary type I. Mutated in HPRCC

Question 4

Fibrofolliculomas of head and neck, pneumothorax, pulmonary cysts, renal mass. What’s the disease

Answer 4

Birt Hogg Dube.

Tumor either chromophobe or oncocytoma.

Question 5

Location of BHD gene

Answer 5

17p11

Question 6

4 hereditary RCC syndromes

Answer 6

VHL
HPRC hereditary papillary RCC
BHD
HLRCC hereditary leiomyoma RCC 
All autosomal dominant

Question 7

Renal angiomas, endolymphatic yolk sac rumors, hemangioblastomas of cns, pancreatic tumors, clear cell RCC, pheo. Syndrome?

Answer 7

Vhl

Question 8

Fibroids, type 2 papillary RCC. Syndrome

Answer 8

Hlrcc

Question 9

What’s the difference between type I and type II papillary RCC?

Answer 9

Path: type I more basophilic and scant cytoplasm.
Clinical: type II more aggressive

Question 10

RCC associated with African American race

Answer 10

Medullary

Poor prognosis Mean survival 12-15 mo

Question 11

What’s the T stage for a renal mass with ivc thrombus above the diaphragm or with ivc wall invasion?

Answer 11

T3C (2010 ajjcc tnm v7)

Question 12

What’s stage III RCC?

Answer 12

T1-2N1M0 or

T3N0-1M0

Question 13

Sensitivity of CT scan for tumor thrombus

Answer 13

78-96%

Question 14

Initial metastatic work up for renal masses

Answer 14

Cxr
Review CT carefully
LFTs

If symptomatic: bone scan, brain imaging, chest ct

Question 15

Cancer risk of bosniak III cyst

Answer 15

50%

Question 16

Most common sites of recurrence of RCC

Answer 16

Lung>bone>liver>adrenal>cns

Most occur within 2 years

Question 17

True or false. Resection of clinically negative nodes in RCC increases PFS

Answer 17

False. EORTC 30881

Question 18

Risk of temporary or permanent dialysis in pt undergoing PNx in solitary kidney

Answer 18

5-8%. Important to mention for consent.

Question 19

RCC Size threshold for intervention in pts with VHL syndrome?

Answer 19

3 cm

Question 20

What is a level 2 tumor thrombus?

Answer 20

Extending into ivc to below hepatics

Question 21

Response rates to interferon alpha in metaStatic RCC?

Answer 21

12%.

1.8% complete response

Question 22

Mental retardation, adenoma sebaceum, epilepsy. What kind of renal massis associated?

Answer 22

AML

Pt has tuberous sclerosis

Question 23

Pt with renal tumor, htn, hypokalemia, polydipsia, polyuria, myalgias and headaches. What kind of tumor?

Answer 23

Reninoma

Question 24

What's stauffers syndrome?

Answer 24

Paraneoplastic ( non metastatic) liver dysfunction. May be due to elevated IL6

Question 25

Hereditary risks of UTUC?

Answer 25

Lynch syndrome II | Balkan nephropathy

Question 26

Locus of gene involved in HLRCC?

Answer 26

1q

Question 27

Locus of gene associated with tuberous sclerosis

Answer 27

9q

Question 28

What's the mechanism of action of cisplatin

Answer 28

Inhibition of DNA cross linking

Question 29

What is translocation carcinoma of the kidney

Answer 29

Affect young adults TFE3 and TFEB Genes involved Papillary or nested architecture w clear cells Presents high stage

Question 30

Risk of metastasis in small renal masses?

Answer 30

1-2% At a median follow up of 3 years

Question 31

Common primary sites that metastasize to kidney

Answer 31

Lung, breast, colon, melanoma

Question 32

What's WAGR

Answer 32

Wilms Aniridia Gu malformation Mental retardation

Question 33

Name the syndrome: | Pseudohermaphroditism, mesangial sclerosis, wilms tumor

Answer 33

Denys drash

Question 34

Name the syndrome: macroglossia, hemihypertrophy, omphalocele, visceromegaly, wilms tumor

Answer 34

Beckwith-wiedemann

Question 35

Name 3 syndromes associated with Wilms

Answer 35

WAGR Denys drash Beckwith wiedemann

Question 36

Poor prognostic indicator in wilms histology

Answer 36

Anaplasia

Question 37

Does Balkan nephropathy affect the rates of bladder cancer?

Answer 37

No

Question 38

Where does UTUC occur most often?

Answer 38

Lower ureter 70% Mid ureter 25% Upper ureter 5%

Question 39

ddx renal sinus mass?

Answer 39

``` RCC Urothelial ca Lymphoma RENAL ARTERY ANEURYSM!!! Use MRI or US to confirm ```

Question 40

Ureteral pseudodivericulosis is associated with?

Answer 40

Urothelial malignancy (usually bladder)