Renal and renal pelvic tumors Flashcards

0
Q

Vhl associated with which type of RCC

A

Clear cell

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1
Q

Location of VHL gene?

A

3p25

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2
Q

What’s the cMet gene?

A

Oncogene associated with papillary RCC.

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3
Q

Where is the cMet gene located?

A

7q31. Papillary type I. Mutated in HPRCC

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4
Q

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

A

Birt Hogg Dube.

Tumor either chromophobe or oncocytoma.

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5
Q

Location of BHD gene

A

17p11

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6
Q

4 hereditary RCC syndromes

A
VHL
HPRC hereditary papillary RCC
BHD
HLRCC hereditary leiomyoma RCC 
All autosomal dominant
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7
Q

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

A

Vhl

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8
Q

Fibroids, type 2 papillary RCC. Syndrome

A

Hlrcc

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9
Q

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

A

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

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10
Q

RCC associated with African American race

A

Medullary

Poor prognosis Mean survival 12-15 mo

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11
Q

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

A

T3C (2010 ajjcc tnm v7)

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12
Q

What’s stage III RCC?

A

T1-2N1M0 or

T3N0-1M0

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13
Q

Sensitivity of CT scan for tumor thrombus

A

78-96%

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14
Q

Initial metastatic work up for renal masses

A

Cxr
Review CT carefully
LFTs

If symptomatic: bone scan, brain imaging, chest ct

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15
Q

Cancer risk of bosniak III cyst

A

50%

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16
Q

Most common sites of recurrence of RCC

A

Lung>bone>liver>adrenal>cns

Most occur within 2 years

17
Q

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

A

False. EORTC 30881

18
Q

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

A

5-8%. Important to mention for consent.

19
Q

RCC Size threshold for intervention in pts with VHL syndrome?

A

3 cm

20
Q

What is a level 2 tumor thrombus?

A

Extending into ivc to below hepatics

21
Q

Response rates to interferon alpha in metaStatic RCC?

A

12%.

1.8% complete response

22
Q

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

A

AML

Pt has tuberous sclerosis

23
Q

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

A

Reninoma

24
Q

What’s stauffers syndrome?

A

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

25
Q

Hereditary risks of UTUC?

A

Lynch syndrome II

Balkan nephropathy

26
Q

Locus of gene involved in HLRCC?

A

1q

27
Q

Locus of gene associated with tuberous sclerosis

A

9q

28
Q

What’s the mechanism of action of cisplatin

A

Inhibition of DNA cross linking

29
Q

What is translocation carcinoma of the kidney

A

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

30
Q

Risk of metastasis in small renal masses?

A

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

31
Q

Common primary sites that metastasize to kidney

A

Lung, breast, colon, melanoma

32
Q

What’s WAGR

A

Wilms
Aniridia
Gu malformation
Mental retardation

33
Q

Name the syndrome:

Pseudohermaphroditism, mesangial sclerosis, wilms tumor

A

Denys drash

34
Q

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

A

Beckwith-wiedemann

35
Q

Name 3 syndromes associated with Wilms

A

WAGR
Denys drash
Beckwith wiedemann

36
Q

Poor prognostic indicator in wilms histology

A

Anaplasia

37
Q

Does Balkan nephropathy affect the rates of bladder cancer?

A

No

38
Q

Where does UTUC occur most often?

A

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

39
Q

ddx renal sinus mass?

A
RCC
Urothelial ca
Lymphoma
RENAL ARTERY ANEURYSM!!!
Use MRI or US to confirm
40
Q

Ureteral pseudodivericulosis is associated with?

A

Urothelial malignancy (usually bladder)