Renal Tumors

Front 1

What sonographic features are important when evaluating a complicated cyst?

Back 1

The thickness and contour of the cyst wall,
number and thickness of any septa,
presence of any calcifications,
density of the renal cyst fluid,
presence of solid components

Front 2

What is the threshold in terms of Hounsfield units for a RCC?

Back 2

15 HU (enhancement)

Front 3

What is a renal pseudotumor and what are the CT characteristics? What radiologic test can be used to differentiate between benign a pseudotumor or a malignancy?

Back 3

A renal pseudotumor is caused by a hypertrophied Column of Bertin, renal dysmorphism, or an unusually shaped kidney. On CT you see an enhancing mass that is isodense with the rest of the parenchyma. A DMSA scan can be used to differentiate between a benign pseudotumor or a malignancy (increased density if the mass is a pseudotumor, decreased density if it’s a cyst or solid tumor).

Front 4

When do you want to biopsy a renal mass?

Back 4

When you suspect an infected cyst, renal abscess, and/or to differentiate metastatic disease or lymphoma from a primary RCC.

Front 5

Describe the Bosniak classification system.
What are the HU of a simple cyst?

Back 5

Simple cyst HU 0-20...if >20 then get u/s to confirm simple fluid then = hyperdense cyst.

I: Uncomplicated, simple cyst. HU 0-20.

II: few septae, minimal calcium in the wall or septum, infected cysts and hyperdense cysts. <3cm. Chance of malignancy is 0-5%. not followed.

IIF: 4-5 septae present, thick or nodular Ca in the septum walls. >3cm. Follow with repeat imaging at 6 months, then spread out if stable.

III: can’t be confidently distinguished from malignant neoplasms. irregular margin, thickened septa, and thick irregular calcifications. 50% are malignant. remove!

IV: Complex renal cysts with large cystic components, irregular margins, SOLID enhancing portions providing diagnosis of malignancy. 75-90% are malignant. Should be surgically removed.

Front 6

How often is a radiographically and clinically suggestive RCC found to be benign after surgical extirpation?

Back 6

20% of small, solid, enhancing masses are found to be benign.

Front 7

What is a metanephric adenoma? What chromosomal abnormality can be associated with metanephric adenomas, and what other subtype of RCC is associated with the same abnormality?

Back 7

pathologic diagnosis. Highly basophilic epithelial cells are seen on histology. rarely if ever metastatic, but because they can’t be reliably diagnosed clinically or radiographically they are surgically removed because of the concern for malignancy.

Polysomy of chromosomes 7 and 17 = papillary RCC

Front 8

What is a oncocytoma?
From which renal cell type does the oncocytoma arise? What subtype of RCC has a histologic similarity to oncocytoma and why?

Back 8

benign renal mass that represents 3-7% of all solid renal masses which are light brown or tan, well circumscribed, homogenous in appearance, and may contain a central "stellate" scar.
-- It arises from the distal tubule.
--Chromophobic RCC also arises from the distal tubule and is histologically similar

Front 9

Can oncocytoma be differentiated from RCC with radiographic studies? How should renal oncocytomas be treated?

Back 9

No. Given the uncertainty of the pre-operative diagnosis and the fact that RCC may be present in oncocytoma masses, these lesions should be surgically removed or ablated.

Front 10

What is an AML? What are its characteristics radiographically? What sydrome is it associated with?

Back 10

--Benign neoplasm consisting of adipose tissue, smooth muscle, and thick walled vessels.
--areas of negative attenuation (below -20) indicative of fat - that excludes RCC. On MRI, the criteria is enhancement on T1.
--Tuberous Sclerosis

Front 11

Describe tuberous sclerosis. Does everyone with TS have AML? How are AMLs in TS patients different? What is the difference in growth rate?

Back 11

20-30% of AMLs are in patients with TS
--TS is an AD disorder characterized by mental retardation, epilepsy, and adenoma sebaceum. Hamartomas affect retina, heart, lung, brain.
--Only 60% of TS patients get AMLs. Only 2% get RCC.
--AMLs in TS patients are more likely to be bilateral and multicentric, more likely symptomatic,
--tendency toward accelerated growth of approximately 20% per year in contrast with a 5% for a solitary AML.
--pts can also have hypopigmented spots, periungual fibromas (nail beds)

Front 12

What is Wunderlich’s syndrome?
What increases the risk of hemorrhage from AML?
Who should be treated if they have an AML?

Back 12

Massive retroperitoneal hemorrhage caused by AML which is found in up to 10% of patients with AML.
--Pregnancy increases the risk of hemorrhage from AML.
--4 CM IS MAGIC #. Treat those >4 cm with symptoms, women of child bearing age, those who don’t have access to good emergency care. Can observe >4 is pt/MD agree.

Front 13

Immunoreactivity for what monoclonal antibody is characteristic of AML, and how is it used?

Back 13

Positive reaction to HMB-45 is characteristic for AML and can be used to differentiate AML from sarcoma

Front 14

What is a cystic nephroma?
What bimodal ages get it and what are sx?
How are they managed?

Back 14

--a benign multilocular renal mass with characteristic hobnailed pattern microscopically. It typically is classified as a Bosniack III or IV renal cyst.
--male children <2, and females >40. Kids have asx abdominal mass. Adults p/w symptomatic abdominal pain, hematuria, UTI, or hypertension.
--Tx = rad nx..b/c of concern for cystic RCC in adults and Wilms in kids.
---Pre-op radiographic studies can’t reliably r/o malignancy.

Front 15

What diagnosis must be considered in any patient with hyperreninemia and hypertension in whom renal artery stenosis has been excluded? What other symptoms can be present?

Back 15

Juxtaglomerular tumor (reninoma) are often small (< 3cm), solitary, and hypovascular. They secrete rennin and present with hypertension, hypokalemia, polydipsia, polyuria, myalgia, and headaches

Front 16

What is the difference in terms of surgical management of RCC in young adults and children?

Back 16

A lymphadenectomy should be performed for improved survival in this population

Front 17

What is von-Hippel Lindau disease?
What are the major manifestations of VHL?
What pre-op considerations?

Back 17

AD familial ccRCC.
--50% with VHL get RCC, often early age of onset, bilateral and multifocal involvement.
--Other syndrome features are:
pheochromocytomas,
retinal angiomas,
hemangioblastomas of the brain stem, cerebellum, cord,
renal and pancreatic cysts, (75%)
inner ear tumors,
papillary cystadenomas of the epididymis!!!!!!

don't operate until tumors at least 3cm...get MR brain and spine on everyone, get pre-op catecholamines in case they have a pheo (can be extra-adrenal)

Front 18

Where is the VHL tumor suppressor gene located?
How does a VHL mutation lead to RCC?
What are targets of medical therapy?

Back 18

Chromosome 3p25.

The VHL protein complex targets hypoxia-inducible factor 1 (HIF-1) for degradation, keeping HIF-1 levels low under normal conditions. HIF-1 plays an important role in regulating cellular responses to hypoxia, starvation, and other stresses. The VHL mutation leads to increased intracellular HIF-1, which leads to upregulation of vascular endothelial growth factor (VEGF), the proangiogenic growth factor in RCC.

Targets are: VEGF, PDGF, EGFR ...
mTOR inhibitors also target HIF and are used in metastatic setting (Temsirolimus, Everolimus)

Front 19

What is Birt-Hogg-Dube syndrome?

Back 19

This is an AD syndrome in which patients develop:
cutaneous fibrofolliculomas (look like goose bumps)
lung cysts,
spontaneous pneumothoraces,
renal tumors derived from the distal tubule = chromophobe RCC & oncocytoma. some can get ccRCC too...9%
--Defect on Ch 17..

Front 20

How does Tarceva (erlotinab) work

Back 20

It targets EGFR (epidermal growth factor receptor)

Front 21

Name the histologic types of RCC and their respective percentage breakdowns. Which histologic type of RCC portends the worst prognosis, conventional, chromophilic, or chromophobic?

Back 21

Conventional (70-80%), chromophilic aka papillary (10-15%), chromophobic (3-5%), collecting duct (1%), unclassified (1%). Conventional has the worst prognosis

Front 22

What cytogenetic abnormalities are associated with papillary (chromophilic) RCC? Is it usually multicentric or solitary?

Back 22

--Trisomy of chromosomes 7 and 17 and loss of the Y chromosome.
--Activating mutations of the met proto-oncogene on chromosome 7q31 is another common, feature.
--Multicentric tumors (multiple tumors arising independently) occur in 40% of cases

Front 23

Who are more predisposed to papillary RCC?

What is the single most distinctive and defining pathologic feature of chromophobic RCC?

Back 23

Those with ESRD (Dialysis) or acquired renal cystic disease.

PHOBE: Microvesicles are most defining feature.

Front 24

What population is renal medullary carcinoma prevalent in?

Back 24

African Americans with sickle cell trait, often diagnosed in the third decade of life

Front 25

What is the classic (or “too late”) triad for the presentation of RCC? How do at least half of RCCs present?

Back 25

The classic triad is flank pain, gross hematuria, and a palpable abdominal mass. This is rarely found because of early detection with increased use of CT and other radiographic studies. 50% found incidentally.

Front 26

How often are paraneoplastic syndromes found in patients with RCC

Back 26

20% of patients with RCC have paraneoplastic syndromes with the production of parathyroid like hormone peptides leading to hypercalcemia being the most common

Front 27

How do you treat hypercalcemia associated with RCC

Back 27

Hydration and lasix, and if severe bisphosphonates and calcitonin

Front 28

What is Stauffer’s syndrome? What is found on liver biopsy with Stauffer’s syndrome?

Back 28

Nonmetastatic hepatic dysfunction leading to elevated LFTs present in 3-20% of cases of RCC. Hepatic function normalizes 60-70% of the time after radical nephrectomy. If you have a persistent elevation of LFTs, you are dealing with metastatic disease. On pathology: nonspecific hepatitis associated with a prominent lymphocytic infiltrate

Front 29

How often should patients with autosomal dominant polycystic kidney disease (ADPCKD) be imaged to screen for RCC and why

Back 29

Never. There is no increased risk for RCC in ADPCKD, and imaging is difficult related to the altered intrarenal architecture

Front 30

What patients should be targeted for screening for RCC? When should patients with ESRD begin screening for RCC

Back 30

Patients with ESRD, acquired renal cystic disease, TS, and familial RCC. The third year on dialysis

Front 31

How should you image a patient if you suspect an IVC thrombus

Back 31

MRI, although multiplanar CT may be equivalent. Venacavography is reserved for patients with equivocal MRI findings or with MRI contraindications

Front 32

What is the single most important prognostic factor for RCC

Back 32

Pathologic stage

Front 33

Define a radical nephrectomy

Back 33

A radical nephrectomy includes removing the kidney while it is in Gerota’s fascia, performing a regional lymphadenectomy, and removing the ipsilateral adrenal gland. We do not perform a regional lymphadenectomy and we only remove the ipsilateral adrenal gland if it is involved on pre-operative imaging

Front 34

What is the recommended post-operative surveillance after nephrectomy for localized RCC?

Back 34

Yearly H&P with blood tests for T1N0M0. Yearly H&P, blood test, cxray for T2N0M0 and every 2 years CT. For T3N0M0, H&P/blood tests and chest x-ray every 6 months for 3 years, then yearly. At 1 year should get abdominal CT, then every 2 years (radical). Every 6 months for 3 years, then ever 2 years (partial)

Front 35

What is hyperfiltration renal injury? How is it treated

Back 35

This may occur after partial nephrectomy because of the increased filtration of the remainder of the kidney. Manifested by proteinuria, FSGS, and renal failure. Treated with dietary restriction of protein and ACE inhibitors

Front 36

When can observation be proposed as a treatment option?

Back 36

Patients with small (<3cm), solid, enhancing, well-marginated, homogeneous renal lesions, who are elderly or poor surgical risks can be managed with serial renal imaging at 6-12 month intervals

Front 37

How often does IVC involvement with RCC occur? What is the cure rate?

Back 37

IVC involvement occurs in 4-10% of patients and 45-70% of patients with RCC and IVC thrombus can be cured with radical nephrectomy and IVC thrombectomy

Front 38

What clinical symptoms would make you worry about an IVC thrombus? Is it possible to tell if an IVC thrombus is invading the wall radiographically?

Back 38

RCC patients with lower extremity edema, an isolated right-sided or non-reducible varicocele, dilated superficial abdominal veins, proteinuria, or a PE. No, unfortunately this bad prognostic indicator is seen intraoperatively

Front 39

What’s the incidence of local recurrence after radical nephrectomy, and how often is this recurrence isolated?

Back 39

2-4% of cases. Only about 40% of local recurrences are isolated, therefore a thorough metastatic workup is necessary.

Front 40

What is the role for radiation with RCC?

Back 40

Palliation of symptomatic osseous metastatic lesions

Front 41

How does Sorafenib (Sutent) work?

bevacizumab?

What are two cytokines that are used as therapy for metastatic RCC, and what are their response rates?

Back 41

Sorafenib is an oral tyrosine kinase inhibitor with an excellent side-effect profile. It is the mainstay of medical therapy for RCC.

It is an IgG1 monoclonal antibody that binds all VEGF isoforms,

Interferon alpha and IL-2. Interferon alpha monotherapy provides a response of 12%, with an improvement in median survival of 3.8 months. IL-2 has an overall response rate of 15%. Together they have a 19% response rate, but no improvement in overall survival

Front 42

What percentage of renal tumors are sarcomas?

Back 42

1-2% of renal tumors are sarcomas, most commonly leiomyosarcomas, with a peak presentation in the fifth decade of life. If you have a patient with a rapidly expanding tumor that looks like it is arising from the capsule a sarcoma should be in your differential. The most common site of metastasis is the lung and patients usually die within months.

Front 43

Is renal lymphoma more common with non-Hodgkin’s or Hodgkin’s lymphoma?

Back 43

Non-Hodgkin’s lymphoma

Front 44

What are the most common malignant neoplasms in the kidney?

Back 44

Mets by a wide margin, with 12% of patient dying of cancer with renal metastases

Front 45

T1a
T1b
T2a
T2b

Back 45

T1a: <4cm
T1b: 4-7cm
T2a: 7-10cm
T2b: >10cm
all limited to the kidney

Front 46

T3a
T3b
T3c
T4

Back 46

T3a: renal vein (or branch) or perinephric fat
T3b: cava below diaphragm
T3c: cava above diaphragm
T4: beyond Gerota's (inc adrenal gland invasion)

Front 47

Stage I - IV and associated survival

Back 47

Stage I: T1-2; 96% 5-yr surv
Stage II: T3a; 65-70%
Stage III: T3, N1; 20-50%
Stage IV: T4; M1; 20% 2-yr

Front 48

Fuhrman Grading

Back 48

intended only for ccRCC (ok for pap too) :
--Grade 1: round, small nuclei, ~10um, nucleoli not visible
--Grade 2: medium, slightly irregular nuclei, ~15um, nucleoli visible only at high power
--Grade 3: Nuclei more irregular, ~20um, nucleoli visible at low power

--Grade 4: even more bizzare looking than grade 3

Front 49

Hereditary Leiomyomatosis RCC

Back 49

Cutaneous Leiomyomas
Uterine FIBROIDS (LMs)
RCC (aggressive type 2 papillary)
Autosomal Dominant with 100% penetrance