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49 Cards in this Set

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What sonographic features are important when evaluating a complicated cyst?
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
What is the threshold in terms of Hounsfield units for a RCC?
15 HU (enhancement)
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?
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).
When do you want to biopsy a renal mass?
When you suspect an infected cyst, renal abscess, and/or to differentiate metastatic disease or lymphoma from a primary RCC.
Describe the Bosniak classification system.
What are the HU of a simple cyst?
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.
How often is a radiographically and clinically suggestive RCC found to be benign after surgical extirpation?
20% of small, solid, enhancing masses are found to be benign.
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?
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
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?
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
Can oncocytoma be differentiated from RCC with radiographic studies? How should renal oncocytomas be treated?
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.
What is an AML? What are its characteristics radiographically? What sydrome is it associated with?
--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
Describe tuberous sclerosis. Does everyone with TS have AML? How are AMLs in TS patients different? What is the difference in growth rate?
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)
What is Wunderlich’s syndrome?
What increases the risk of hemorrhage from AML?
Who should be treated if they have an AML?
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.
Immunoreactivity for what monoclonal antibody is characteristic of AML, and how is it used?
Positive reaction to HMB-45 is characteristic for AML and can be used to differentiate AML from sarcoma
What is a cystic nephroma?
What bimodal ages get it and what are sx?
How are they managed?
--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.
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?
Juxtaglomerular tumor (reninoma) are often small (< 3cm), solitary, and hypovascular. They secrete rennin and present with hypertension, hypokalemia, polydipsia, polyuria, myalgia, and headaches
What is the difference in terms of surgical management of RCC in young adults and children?
A lymphadenectomy should be performed for improved survival in this population
What is von-Hippel Lindau disease?
What are the major manifestations of VHL?
What pre-op considerations?
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)
Where is the VHL tumor suppressor gene located?
How does a VHL mutation lead to RCC?
What are targets of medical therapy?
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)
What is Birt-Hogg-Dube syndrome?
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..
How does Tarceva (erlotinab) work
It targets EGFR (epidermal growth factor receptor)
Name the histologic types of RCC and their respective percentage breakdowns. Which histologic type of RCC portends the worst prognosis, conventional, chromophilic, or chromophobic?
Conventional (70-80%), chromophilic aka papillary (10-15%), chromophobic (3-5%), collecting duct (1%), unclassified (1%). Conventional has the worst prognosis
What cytogenetic abnormalities are associated with papillary (chromophilic) RCC? Is it usually multicentric or solitary?
--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
Who are more predisposed to papillary RCC?

What is the single most distinctive and defining pathologic feature of chromophobic RCC?
Those with ESRD (Dialysis) or acquired renal cystic disease.

PHOBE: Microvesicles are most defining feature.
What population is renal medullary carcinoma prevalent in?
African Americans with sickle cell trait, often diagnosed in the third decade of life
What is the classic (or “too late”) triad for the presentation of RCC? How do at least half of RCCs present?
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.
How often are paraneoplastic syndromes found in patients with RCC
20% of patients with RCC have paraneoplastic syndromes with the production of parathyroid like hormone peptides leading to hypercalcemia being the most common
How do you treat hypercalcemia associated with RCC
Hydration and lasix, and if severe bisphosphonates and calcitonin
What is Stauffer’s syndrome? What is found on liver biopsy with Stauffer’s syndrome?
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
How often should patients with autosomal dominant polycystic kidney disease (ADPCKD) be imaged to screen for RCC and why
Never. There is no increased risk for RCC in ADPCKD, and imaging is difficult related to the altered intrarenal architecture
What patients should be targeted for screening for RCC? When should patients with ESRD begin screening for RCC
Patients with ESRD, acquired renal cystic disease, TS, and familial RCC. The third year on dialysis
How should you image a patient if you suspect an IVC thrombus
MRI, although multiplanar CT may be equivalent. Venacavography is reserved for patients with equivocal MRI findings or with MRI contraindications
What is the single most important prognostic factor for RCC
Pathologic stage
Define a radical nephrectomy
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
What is the recommended post-operative surveillance after nephrectomy for localized RCC?
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)
What is hyperfiltration renal injury? How is it treated
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
When can observation be proposed as a treatment option?
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
How often does IVC involvement with RCC occur? What is the cure rate?
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
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?
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
What’s the incidence of local recurrence after radical nephrectomy, and how often is this recurrence isolated?
2-4% of cases. Only about 40% of local recurrences are isolated, therefore a thorough metastatic workup is necessary.
What is the role for radiation with RCC?
Palliation of symptomatic osseous metastatic lesions
How does Sorafenib (Sutent) work?

bevacizumab?

What are two cytokines that are used as therapy for metastatic RCC, and what are their response rates?
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
What percentage of renal tumors are sarcomas?
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.
Is renal lymphoma more common with non-Hodgkin’s or Hodgkin’s lymphoma?
Non-Hodgkin’s lymphoma
What are the most common malignant neoplasms in the kidney?
Mets by a wide margin, with 12% of patient dying of cancer with renal metastases
T1a
T1b
T2a
T2b
T1a: <4cm
T1b: 4-7cm
T2a: 7-10cm
T2b: >10cm
all limited to the kidney
T3a
T3b
T3c
T4
T3a: renal vein (or branch) or perinephric fat
T3b: cava below diaphragm
T3c: cava above diaphragm
T4: beyond Gerota's (inc adrenal gland invasion)
Stage I - IV and associated survival
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
Fuhrman Grading
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
Hereditary Leiomyomatosis RCC
Cutaneous Leiomyomas
Uterine FIBROIDS (LMs)
RCC (aggressive type 2 papillary)
Autosomal Dominant with 100% penetrance