Malignant Renal tumors

Question 1

What is the most accurate imaging study for characterizing a renal mass?

a. Intravenous pyelography
b. Ultrasonography
c. Computed tomography (CT) with and without contrast enhancement
d. CT-positron emission tomography (PET) scan
e. Renal arteriography

Answer 1

c. Computed tomography (CT) with and without contrast enhancement. A dedicated triple-phase renal CT scan remains the single most important radiographic image to delineate the nature of a renal mass. In general, any renal mass that enhances with administration of intravenous contrast material on CT scanning should be considered a RCC until proved otherwise.

Question 2

2. A hyperdense renal cyst may also be termed a:
   a. probable malignancy.
   b. Bosniak II cyst.
   c. Bosniak III cyst.
   d. Bosniak IV cyst.
   e. probable angiomyolipoma.

Answer 2

b. Bosniak II cyst. Category II lesions are minimally complicated cysts that are benign but have some radiologic findings that cause concern. Classic hyperdense renal cysts are small (<3 cm), round, sharply marginated, and do not enhance after administration of contrast material. Hyperdense cysts that are 3 cm or larger are classified as Bosniak IIF lesions.

Question 3

The most generally accepted indication for core needle biopsy of a renal mass is a suspected clinical diagnosis of:

a. renal cell carcinoma (RCC).
b. renal oncocytoma.
c. renal cyst.
d. renal metastasis.
e. renal angiomyolipoma.

Answer 3

d. Renal metastasis. The traditionally accepted indications for biopsy of a renal mass are when a renal abscess or infected cyst is suspected, or when differentiating RCC from metastatic malignancy or renal lymphoma. Core needle biopsy is now performed with increased frequency for the evaluation of renal masses in other circumstances, particularly for patients in whom a wide variety of treatment options are under consideration.

Question 4

According to the American Urological Association (AUA) guidelines (Fig. 97.1), recommended postoperative radiographic surveillance of the chest after radical nephrectomy for T1N0M0 RCC is:

a. no imaging studies.
b. chest radiograph at 1 year.
c. chest radiograph annually for 3 years.
d. chest CT at 1 year and then chest radiograph annually for 2 years.
e. chest radiograph annually for 5 years.

Answer 4

Chest radiograph annually for 3 years. Surveillance for recurrent malignancy after radical nephrectomy for RCC can be tailored according to the initial pathologic tumor stage. This patient is low risk (pT1N0M0), and the AUA guidelines recommend an annual chest radiograph for 3 years and only as clinically indicated beyond that time period.

Question 5

The European Organization for Research and Treatment of Cancer 30904 study randomly assigned patients to radical versus partial nephrectomy. Which of the following was an inclusion criterion?

a. Clinical T1a tumor (<4.0 cm)
b. Tumor size <5.0 cm
c. Estimated glomerular filtration rate (GFR) >60 mL/min/1.73 m2
d. No hypertension
e. Age <70 years

Answer 5

b. Tumor size <5.0 cm. A solitary tumor and a normal contralateral kidney were also required, but criteria for the latter were not well defined, at least based on current perspectives about functional assessment that depend on eGFR rather than serum creatinine level.

Question 6

The European Organization for Research and Treatment of Cancer 30904 study randomly assigned patients to radical versus partial nephrectomy. Which of the following was an inclusion criterion?

a. Clinical T1a tumor (<4.0 cm)
b. Tumor size <5.0 cm
c. Estimated glomerular filtration rate (GFR) >60 mL/min/1.73 m2
d. No hypertension
e. Age <70 years

Answer 6

5. Surveillance for recurrent malignancy after nephron-sparing surgery for RCC can be tailored according to the initial pathologic tumor stage. This patient is intermediate to high risk, and the AUA Guidelines recommend a baseline abdominal scan (CT or MRI) within 3 to 6 months following surgery with continued imaging (ultrasonography, CT, or MRI) every 6 months for at least 3 years and annually thereafter to year 5. Imaging beyond 5 years may be performed at the discretion of the clinician.

Question 7

According to the AUA guidelines, following partial nephrectomy for pathologic stage T3aN0M0 RCC, it is recommended to perform surveillance abdominal CT scanning with what frequency?

a. Never
b. Every 6 months for at least 3 years and then annually to year 5
c. Every year to year 5
d. Every 2 years
e. Every year for 2 years and then at year 5

Answer 7

b. Every 6 months for at least 3 years and then annually to year 5. Surveillance for recurrent malignancy after nephron-sparing surgery for RCC can be tailored according to the initial pathologic tumor stage. This patient is intermediate to high risk, and the AUA Guidelines recommend a baseline abdominal scan (CT or MRI) within 3 to 6 months following surgery with continued imaging (ultrasonography, CT, or MRI) every 6 months for at least 3 years and annually thereafter to year 5. Imaging beyond 5 years may be performed at the discretion of the clinician.

Question 8

Following partial nephrectomy of a solitary kidney, what is the most effective method of screening for hyperfiltration nephropathy?

a. Urinary dipstick test for protein
b. Albumin-to-creatinine ratio
c. Iothalamate glomerular filtration rate (GFR) measurement
d. Serum creatinine measurement
e. Renal biopsy

Answer 8

b. Albumin-to-creatinine ratio. Patients who undergo nephronsparing surgery for RCC may be left with a relatively small amount of renal tissue. These patients are at risk for long-term renal functional impairment from hyperfiltration renal inMury. Because proteinuria is the initial manifestation of the phenomenon, an albumin-to-creatinine ratio should be obtained yearly in patients with a solitary remnant kidney to screen for hyperfiltration nephropathy. Traditionally a 24-hour urine protein was obtained on an annual basis, but this is more burdensome, and for screening purposes the albumin-to-creatinine ratio is more suitable. If positive, more rigorous testing, such as the 24-hour urine protein, can be considered to more accurately quantify the degree of proteinuria.

Question 9

The most accurate and practical assessment of renal function for routine use after nephrectomy is:

a. serum creatinine measurement.
b. urinary dipstick test for protein.
c. 24-hours urinary protein measurement.
d. iothalamate GFR measurement.
e. serum creatinine–based estimation of GFR and analysis for proteinuria.

Answer 9

8. e. Serum creatinine–based estimation of GFR and analysis for proteinuria. Identification and classification of CKD is best performed by assessing cause, estimated GFR (eGFR), and extent of albuminuria. At present, there are several formulas in clinical use, including the MDRD and CKD-EPI formulas, each of which is an improvement over using serum creatinine alone for identification of patients with or at risk for CKD. Serum levels of creatinine are dependent on gender, muscle mass, and other factors and can therefore lead to an underestimation of kidney disease in certain populations, such as thin, elderly women. Urinary creatinine measurement is impractical and provides only marginally more valuable information than serum creatinine. Urinary protein measurement can identify patients with early signs of kidney disease (proteinuria), and there are multiple methods to assess this, including 24-hour urinary protein and spot urine studies (albuminto-creatinine ratio, protein-to-creatinine ratio); none has been shown to be better than GFR estimation as a screening test. Direct measurement of GFR using iothalamate (or other agents) is costly and not routinely available; it is therefore impractical in most settings.

Question 10

What is an important prerequisite for successful cryoablation of a renal tumor?

a. Slow freezing
b. Rapid thawing
c. A single prolonged freeze-thaw cycle
d. A double freeze-thaw cycle
e. Freezing of tumor to a temperature of −10°C

Answer 10

D. A double freeze-thaw cycle. Renal cryosurgery is an ablative nephron-sparing treatment option for RCC that can be performed percutaneously under radiographic guidance or laparoscopically under direct vision and ultrasound guidance. The aim of cryosurgery is to ablate the same predetermined volume of tissue that would have been removed had a conventional surgical excision been performed. Established critical prerequisites for successful cryosurgery include rapid freezing, gradual thawing, and a repetition of the freeze-thaw cycle.

Question 11

Which two imaging modalities are the preferred and most accurate for demonstrating the presence and extent of an inferior vena caval tumor thrombus?

a. Abdominal ultrasonography and CT
b. MRI and renal artery angiography
c. CT and MRI
d. MRI and contrast venacavography
e. Contrast venacavography and transesophageal ultrasonography

Answer 11

c. CT and MRI. Both CT and MRI are noninvasive and accurate modalities for demonstrating both the presence and the distal extent of vena caval involvement. Although MRI has been recommended as the test of choice at most centers, many studies have demonstrated that a properly performed multiplanar CT also provides sufficient information for surgical planning, and it has become the preferred diagnostic study at many centers.

Question 12

In patients undergoing complete surgical excision of RCC, the lowest 5-year survival rate is associated with which factor?

a. Perinephric fat involvement
b. Clear cell histology
c. Subdiaphragmatic inferior vena caval involvement
d. Intra-atrial tumor thrombus
e. Lymph node involvement

Answer 12

E. Lymph node involvement. In most studies, the presence of lymph node or distant metastases has carried a dismal prognosis that is much more pronounced than the other distractors.

Question 13

A 45-year-old man has a 5-cm, exophytic RCC in the upper pole of a solitary left kidney and a single 2-cm left lower lung metastasis. What is the best treatment?

a. Initial targeted therapy, then partial nephrectomy
b. Partial nephrectomy, then targeted therapy
c. Staged partial nephrectomy and pulmonary lobectomy
d. Simultaneous partial nephrectomy and pulmonary lobectomy
e. Simultaneous radical nephrectomy and pulmonary lobectomy

Answer 13

d. Simultaneous partial nephrectomy and pulmonary lobectomy. The subset of patients with metastatic RCC and a solitary metastasis, estimated at between 1.6% and 3.2% of patients, may benefit from nephrectomy with resection of the metastatic lesion. This patient also needs partial nephrectomy to preclude the need for dialysis. Of note, both procedures could be performed with a minimally invasive approach under the same anesthesia

Question 14

13. A healthy 75-year-old man is referred after renal biopsy of a 3.0-cm centrally located renal mass. The biopsy is definitive for renal oncocytoma. The other kidney is normal, the serum creatinine level is 1.0 mg/dL, and there is no evidence of metastatic disease. What is the best next step?
    a. Open radical nephrectomy
    b. Laparoscopic nephroureterectomy
    c. Percutaneous thermal ablation (TA)
    d. Partial nephrectomy
    e. Observation with follow-up renal imaging

Answer 14

e. Observation with follow-up renal imaging. Renal mass biopsy is now performed with increased frequency and should be considered in an elderly patient such as this. For those in whom nonextirpative options are being considered, biopsy can provide important information, such as a definitive nonmalignant diagnosis (as in this example). Given the benign nature of renal oncocytomas, the best answer is observation with follow-up imaging at an interval between 6 and 12 months, if clinically warranted based on the patient’s overall health and life expectancy.

Question 15

14. Which of the following agents demonstrated an oncologic benefit in the postoperative adjuvant setting for patients at high risk of recurrence following nephrectomy?
    a. High-dose interleukin-2 (IL-2)
    b. Sunitinib
    c. Autologous tumor vaccine
    d. Pazopanib
    e. Interferon-α (IFN-α)

Answer 15

b. Sunitinib. Sunitinib demonstrated a significantly longer disease-free survival compared to placebo (6.8 vs. 5.6 years) in the phase 3 S-TRAC trial that randomized 615 men with pT2 (highgrade), pT3-4, or N1 clear cell RCC to sunitinib or placebo. Importantly, patients receiving therapy experienced significantly higher rates of toxic events and there was no difference in overall survival. All other therapies listed failed to demonstrate an oncologic benefit in the adjuvant setting.

Question 16

Based on the 2017 AUA Guidelines, which of the following patients would not need to be considered for genetic counseling?

a. A 45-year-old woman with a pT1a clear cell RCC.
b. A 68-year-old man with a pT1a RCC with histology suggestive of succinate dehydrogenase (SDH) deficiency
c. A 69-year-old man with 2 ipsilateral pT1a tumors, a 3.5-cm clear cell, and a 1.1-cm type 1 papillary
d. A 62-year-old woman with a pT1b chromophobe RCC and moderate-sized lung cysts
e. The 7-year-old son of a patient with von Hippel-Lindau (VHL) disease

Answer 16

c. A 69-year-old man with 2 ipsilateral pT1a tumors, a 3.5-cm clear cell, and a 1.1-cm type 1 papillary. A 69-year-old man with 2 ipsilateral pT1a tumors, a 3.5-cm clear cell and a 1.1-cm type 1 papillary probably does not need to consider genetic counseling, unless the family or personal history is otherwise suggestive of a familial syndrome. Genetic counseling is recommended for all patients with kidney cancer who are 46 years of age or younger. When histology suggests possible SDH deficiency, further testing should be pursued, and a history of chromophobe RCC and lung cysts or a prior spontaneous pneumothorax is suggestive for BHD syndrome. Relatives of patients with known familial RCC should be strongly considered for genetic counseling, and surveillance ideally will be initiated at an early age. An elderly patient with multifocal RCC may not need to be considered for genetic counseling, particularly if there is discordance in the histologies.

Question 17

Which two agents have the most similar mechanisms of action?

a. Sunitinib and temsirolimus
b. Pazopanib and axitinib
c. Pembrolizumab and ipilimumab
d. Nivolumab and cabozantinib
e. Atezolizumab and ipilimumab

Answer 17

B. Pazopanib and axitinib. Pazopanib and axitinib both target the VEGF pathway, and specifically the VEGF receptor, and are TKI. Sunitinib and cabozantinib are also TKIs, and temsirolimus is an mTOR inhibitor. Pembrolizumab, atezolizumab, ipilimumab, and nivolumab are all checkpoint inhibitors (Fig.

97.6). Pembrolizumab and nivolumab block PD-1, while

atezolizumab blocks PD-L1 and ipilimumab blocks CTLA-4.

Question 18

17. A 48-year-old woman with a history of seizure disorder presents with recurrent gross hematuria and left flank pain. Abdominal CT shows a large left perinephric hematoma associated with a 3.0-cm left renal angiomyolipoma. There are also multiple right renal angiomyolipomas ranging in size from 1.5 to 6.5 cm. What is the best management of the left renal lesion?
    a. Selective embolization
    b. Radical nephrectomy
    c. Observation
    d. Partial nephrectomy
    e. Laparoscopic exposure and renal cryoablative therapy

Answer 18

17. a. Selective embolization. Most patients with acute or potentially life-threatening hemorrhage will require total nephrectomy if exploration is performed, and if the patient has TSC, bilateral disease, preexisting renal insufficiency, or other medical or urologic disease that could affect renal function in the future, selective embolization should be considered. In such circumstances, selective embolization can temporize by controlling hemorrhage and in many cases will prove to be definitive treatment. This patient almost certainly has TSC given bilateral AMLs and history of seizures.

Question 19

18. Which of the following statements is TRUE regarding cystic nephromas occurring in adults?
    a. They are complex cystic lesions that are typically classified as Bosniak II to III.
    b. They are malignant 2% to 5% of the time.
    c. They are more common in men than in women.
    d. When suspected, they should be treated by radical nephrectomy.
    e. They are readily differentiated from cystic RCC on the basis of appropriate imaging studies.

Answer 19

A. They are complex cystic lesions that are typically classified as Bosniak II to III. Cystic nephromas are benign renal neoplasms that occur most commonly in middle-aged women. They appear to be genetically related to MEST but generally have a somewhat different radiographic appearance. Unlike MEST, which contain a solid stromal component and often appear as solid or Bosniak IV lesions on cross-sectional imaging, cystic nephromas are typically characterized as complex cystic lesions without a solid component

Question 20

19. Which environmental factor is most commonly accepted as a risk factor for RCC?
    a. Radiation therapy
    b. Antihypertensive medications
    c. Tobacco use
    d. Diuretics
    e. High-fat diet

Answer 20

19 c. Tobacco use. The most generally accepted environmental risk factor for RCC is tobacco use, although the relative associated risks have been modest, ranging from 1.4 to 2.3 when compared with controls. All forms of tobacco use have been implicated, with risk increasing with cumulative dose or pack-years. Other wellestablished risk factors include obesity and hypertension.

Question 21

20. Which of the following manifestations is common only in certain VHL families; specifically, which is common in those with type 2 and only rarely seen in type 1 VHL?
    a. RCC
    b. Pancreatic cysts or tumors
    c. Epididymal tumors
    d. Pheochromocytoma
    e. Inner ear tumors

Answer 21

d. Pheochromocytoma. The familial form of clear cell RCC is the VHL syndrome. MaJor manifestations include the development of RCC, pheochromocytoma, retinal angiomas, and hemangioblastomas of the brainstem, cerebellum, or spinal cord. Penetrance for all of these traits is far from complete, and some, such as pheochromocytomas, tend to be clustered in certain families but not in others.

Question 22

Common manifestations of VHL disease include:

a. RCC, pheochromocytoma, fibrofolliculoma
b. angiomyolipoma, pheochromocytoma, fibrofolliculoma
c. RCC, pheochromocytoma, hemangioblastoma
d. angiomyolipoma, pheochromocytoma, hemangioblastoma
e. RCC, thyroid carcinoma, leiomyoma

Answer 22

c. RCC, pheochromocytoma, hemangioblastoma. These are the three most common tumor types occurring in VHL disease. Facial fibrofolliculomas occur in BHD syndrome, and uterine leiomyomas are commonly seen in hereditary leiomyomatosis and RCC syndrome

Question 23

What is the most common cause of death in patients with the VHL syndrome?

a. Renal failure
b. Cerebellar hemangioblastoma
c. Unrelated medical disease
d. Pheochromocytoma
e. RCC

Answer 23

e. RCC. With improved management of the central nervous system manifestations of the disease, RCC is the most common cause of mortality in patients with VHL syndrome.

Question 24

The VHL syndrome tumor suppressor protein regulates the expression of which of the following mediators of biologic aggressiveness for RCC?

a. Basic fibroblast growth factor
b. Vascular endothelial cell growth factor
c. Epidermal growth factor receptor (EGFR)
d. Hepatocyte growth factor (scatter factor)
e. P-glycoprotein (multiple drug resistance efflux protein)

Answer 24

b. Vascular endothelial cell growth factor. Inactivation or mutation of the VHL gene leads to dysregulated expression of hypoxia inducible factor-1, an intracellular protein that plays an important role in regulating cellular responses to hypoxia, starvation, and other stresses. This in turn leads to a several-fold upregulation of the expression of VEGF, the primary proangiogenic growth factor in RCC, contributing to the pronounced neovascularity associated with this carcinoma.

Question 25

24. What do the hereditary papillary RCC syndrome and VHL syndrome have in common?
    a. Mode of genetic transmission
    b. Chromosome 3 abnormalities
    c. Propensity toward tumor formation in multiple organ systems
    d. Inactivation of a tumor suppressor gene
    e. Nearly complete penetrance

Answer 25

a. Mode of genetic transmission. Studies of families with hereditary papillary renal carcinoma (HPRC) have demonstrated an autosomal dominant mode of transmission, similar to VHL disease. VHL is caused by inactivation or mutation of a tumor suppressor gene, whereas HPRC is caused by activation of an oncogene.

Question 26

25. Mutation of the met proto-oncogene in hereditary papillary RCC leads to:
    a. increased expression of hepatocyte growth factor.
    b. increased sensitivity to vascular endothelial growth factor (VEGF).
    c. inactivation of a tumor suppressor gene that regulates cellular proliferation.
    d. constitutive activation of the receptor for hepatocyte growth factor.
    e. increased expression of VEGF

Answer 26

25. d. Constitutive activation of the receptor for hepatocyte growth factor. Missense mutations of the met proto-oncogene at 7q31 were found to segregate with the disease, implicating it as the relevant genetic locus. The protein product of this gene is the receptor tyrosine kinase for the hepatocyte growth factor, which plays an important role in regulating the proliferation and differentiation of epithelial and endothelial cells in a wide variety of organs, including the kidney. Most of the mutations in hereditary papillary RCC have been found in the tyrosine kinase domain of met and lead to constitutive activation.

Question 27

26. P-glycoprotein is a transmembrane protein that is involved in:
    a. immunotolerance.
    b. resistance to high-dose IL-2 therapy.
    c. resistance to cisplatin therapy.
    d. resistance to radiation therapy.
    e. efflux of large hydrophobic compounds, including many cytotoxic drugs

Answer 27

26. e. Efflux of large hydrophobic compounds, including many cytotoxic drugs. P-glycoprotein is a 170-kDa transmembrane protein expressed by 80% to 90% of RCCs that acts as an energydependent efflux pump for a wide variety of large hydrophobic compounds, including several cytotoxic drugs.

Question 28

Pathology demonstrates venous involvement limited to the main renal vein along with contralateral adrenal involvement with RCC. There is also a 6-cm bulky retroperitoneal lymph node replaced with cancer. What is the stage? a. pT3aN1M0

b. pT3aN2M0
c. pT3aN1M1
d. pT3bN1M1
e. pT4N2M0

Answer 28

27. c. pT3aN1M1. Isolated renal vein involvement is now classified as T3a, and nodal classification has been simplified such that all nodal involvement is now classified as N1. Contiguous invasion of the ipsilateral adrenal is now classified as T4, but metastatic involvement of the contralateral (or ipsilateral) adrenal gland is classified as M1, reflecting a likely hematogenous pattern of dissemination.

Question 29

Which of the following is most likely to demonstrate an infiltrative growth pattern?

a. Clear cell RCC
b. Sarcomatoid variants of RCC
c. Papillary RCC
d. Chromophobe RCC
e. Oncocytoma

Answer 29

b. Sarcomatoid variants of RCC. Most RCCs are round to ovoid and circumscribed by a pseudocapsule of compressed parenchyma and fibrous tissue rather than a true histologic capsule. Unlike upper tract transitional cell carcinomas (TCCs), most RCCs are not grossly infiltrative, with the notable exception of some sarcomatoid variants.

Question 30

29. What is the most common mutation identified in sporadic clear cell RCC?
    a. Activation of the met proto-oncogene
    b. Activation of the VHL gene
    c. Inactivation of the VHL gene
    d. Inactivation of p53
    e. Inactivation of genes on chromosome 9

Answer 30

29. c. Inactivation of the VHL gene. Chromosome 3 alterations and VHL mutations are common in conventional RCC, and mutation or inactivation of this gene has been found in over 75% of sporadic cases.

Question 31

30. Which of the following cytogenetic abnormalities is commonly associated with type 1 papillary RCC?
    a. Trisomy of chromosome 7
    b. Trisomy of the Y chromosome
    c. Loss of chromosome 17
    d. Loss of all or parts of chromosome 3
    e. Loss of chromosome 7

Answer 31

0. a. Trisomy of chromosome 7. The cytogenetic abnormalities associated with type 1 papillary RCC are characteristic and include trisomy of chromosomes 7 and 17.

Question 32

31. What percentage of RCCs are chromophobe cell carcinomas?
    a. 0% to 2%
    b. 3% to 5%
    c. 8% to 10%
    d. 12% to 15%
    e. 18% to 25%

Answer 32

31. b. 3% to 5%. Chromophobe cell carcinoma is a distinctive histologic subtype of RCC that appears to be derived from the cortical portion of the collecting duct. It represents 3% to 5% of all RCCs.

Question 33

32. Most renal medullary carcinomas are:
    a. found in patients with sickle cell disease.
    b. diagnosed in the fifth decade of life.
    c. responsive to high-dose chemotherapy.
    d. genetically and histologically similar to papillary RCC.
    e. metastatic at the time of diagnosis.

Answer 33

32. e. Metastatic at the time of diagnosis. Renal medullary carcinoma is a rare histologic subtype of RCC that occurs almost exclusively in association with sickle cell trait. It is typically diagnosed in young African Americans, often in the third decade of life. Many cases are both locally advanced and metastatic at the time of diagnosis. Most patients have not responded to therapy and have succumbed to their disease in a few to several months.

Question 34

3. Which paraneoplastic syndrome associated with RCC can often be managed or palliated medically?
   a. Polycythemia
   b. Stauffer syndrome
   c. Neuropathy
   d. Hypercalcemia
   e. Cachexia

Answer 34

d. Hypercalcemia. Hypercalcemia has been reported in up to 13% of patients with RCC and can be due to either paraneoplastic phenomena or osteolytic metastatic involvement of the bone. The production of parathyroid hormonelike peptides is the most common paraneoplastic etiology, although tumor-derived 1,25-dihydroxyvitamin D 3 and prostaglandins
may contribute in a minority of cases. Medical management includes vigorous hydration followed by diuresis with furosemide and the selective use of bisphosphonates, corticosteroids, and/or calcitonin.

Question 35

34. A healthy 64-year-old man is found to have a 6.0-cm solid, heterogeneous mass in the hilum of the right kidney. CT of the abdomen and pelvis shows interaortocaval lymph nodes enlarged to 2.5 cm. A chest radiograph and a bone scan are negative, and the contralateral kidney is normal. The serum creatinine level is 1.0 mg/dL. What is the best next step?
    a. Right radical nephrectomy and regional or extended lymph node dissection
    b. Abdominal exploration, sampling of the enlarged lymph nodes, and possible radical nephrectomy pending frozen section analysis
    c. CT-guided percutaneous biopsy of the lymph nodes
    d. CT-guided percutaneous biopsy of the tumor mass
    e. Systemic therapy followed by radical nephrectomy

Answer 35

4. a. Right radical nephrectomy and regional or extended lymph node dissection. An aggressive surgical approach is still preferred because it will likely prolong survival and represents the only realistic chance for a cure. Lymph nodes in this size range are most likely malignant, and this patient will likely need to consider adjuvant clinical trials. An extended lymph node dissection includes the interaortocaval nodes and nodes alongside and behind the ipsilateral great vessel from the crus of the diaphragm to the ipsilateral common iliac artery.

Question 36

35. Which of the following patients would be the best candidate for percutaneous biopsy or fine-needle aspiration of a renal mass?
    a. A 42-year-old man with a 2.5-cm Bosniak III complex renal cyst
    b. An 88-year-old man with a 1.7-cm solid, enhancing renal mass
    c. A 32-year-old woman with bilateral solid, enhancing renal masses ranging in size from 1.5 to 4.0 cm
    d. A 48-year-old woman with a 3.5-cm solid, enhancing renal mass with fat density present
    e. A 38-year-old woman with a fever, a urinary tract infection, and a 3.5-cm solid/cystic, enhancing renal mass

Answer 36

35. e. A 38-year-old woman with a fever, urinary tract infection, and a 3.5-cm solid/cystic, enhancing renal mass. Patients with flank pain, a febrile urinary tract infection, and a renal mass should be offered percutaneous biopsy and/or aspiration to establish a diagnosis of renal abscess rather than malignancy, and to provide drainage if abscess is confirmed.

Question 37

6. A 67-year-old man undergoes radical nephrectomy and inferior vena caval thrombectomy (level 2 tumor thrombus). The primary tumor is otherwise confined to the kidney, and the lymph nodes are not involved. What is the approximate 5-year cancer-free survival rate?
   a. 0% to 15%
   b. 16% to 30%
   c. 31% to 50%
   d. 51% to 70%
   e. 71% to 90%

Answer 37

36. c. 31% to 50%. Venous involvement with tumor thrombi was once thought to be a dismal sign for patients with RCC, but more recent studies suggest that many patients with tumor thrombi can be salvaged with an aggressive surgical approach. These studies document 31% to 50% 5-year survival rates for patients with IVC thrombus thrombi, as long as the tumor is otherwise confined to the kidney.

Question 38

37. Which of the following is NOT a predictor of cancer-specific survival after nephrectomy for RCC?
    a. Pathologic stage
    b. Tumor size
    c. Nuclear grade
    d. Patient age
    e. Histologic necrosis

Answer 38

37. d. Patient age. Although patient age and comorbidity are important predictors of overall survival in patients with RCC and strongly affect the choice of treatment in these patients, they have no effect on the likelihood of dying of cancer-specific causes. Each of the other factors is a predictor of cancer-specific mortality from RCC and has been incorporated into one or more RCC prognostic algorithms for cancer-specific outcomes.

Question 39

38. Which of the following statements about renal lymphoma is TRUE?
    a. 5% to 10% of all lymphomas involving the kidney are primary tumors.
    b. The radiographic patterns manifested by renal lymphoma are diverse and can be difficult to differentiate from RCC.
    c. Percutaneous biopsy is rarely indicated if renal lymphoma is suspected.
    d. Renal failure associated with renal lymphoma is most often due to extensive parenchymal replacement by the malignancy.
    e. The most common pattern of renal involvement is by direct extension from adMacent retroperitoneal lymph nodes.

Answer 39

38. b. The radiographic patterns manifested by renal lymphoma are diverse and can be difficult to differentiate from RCC. Five different radiographic patterns have been described for lymphoma involving the kidney, including a solitary mass, occasionally making it difficult to differentiate from RCC.

Question 40

38. Which of the following statements about renal lymphoma is TRUE?
    a. 5% to 10% of all lymphomas involving the kidney are primary tumors.
    b. The radiographic patterns manifested by renal lymphoma are diverse and can be difficult to differentiate from RCC.
    c. Percutaneous biopsy is rarely indicated if renal lymphoma is suspected.
    d. Renal failure associated with renal lymphoma is most often due to extensive parenchymal replacement by the malignancy.
    e. The most common pattern of renal involvement is by direct extension from adMacent retroperitoneal lymph nodes.

Answer 40

b. The radiographic patterns manifested by renal lymphoma are diverse and can be difficult to differentiate from RCC. Five different radiographic patterns have been described for lymphoma involving the kidney, including a solitary mass, occasionally making it difficult to differentiate from RCC.

Question 41

39. Which of the following would be considered diagnostic for renal angiomyolipoma (AML)?
    a. Hyperechoic pattern on ultrasonography
    b. Enhancement of >30 Hounsfield units on CT scan
    c. Small area measuring less than −20 Hounsfield units on nonenhanced CT
    d. Aneurysmal changes on renal arteriogram e. Positive signal on T2 images of MRI

Answer 41

c. Small area measuring less than −20 Hounsfield units on nonenhanced CT. The presence of even a small focus of fat, as evidenced by a density less than − 20 HU on a non-enhanced CT scan, is diagnostic for AML. The findings described in a, b, and d are all suggestive of, but not diagnostic for, renal AML.

Question 42

The main limitation of renal mass biopsy is:

a. risk of needle tract seeding.
b. difficulty differentiating the eosinophilic variants of RCC from renal oncocytoma.
c. risk of pneumothorax.
d. risk of hemorrhage.
e. inability to determine subtype of RCC.

Answer 42

40. b. Difficulty differentiating the eosinophilic variants of RCC from renal oncocytoma. The main limitation of renal mass biopsy is difficulty differentiating renal oncocytoma, the most common benign renal mass, from eosinophilic variants of conventional, papillary, and chromophobe RCC on biopsy material. While the non-diagnostic rate is between 10% and 15%, the risk of complications is low in the modern era with the use of smallergauge needles, and needle tract seeding with RCC appears to be a rare event

Question 43

41. Which of the following tumors is most likely to be a malignant RCC?
    a. 2.5-cm hyperechoic complex cyst, with no enhancement with IV contrast
    b. 6.0-cm complex cyst with four thin septae
    c. 5.0-cm cyst with thin, curvilinear calcification
    d. 11-cm cyst with water density and homogeneous nature
    e. 3.0-cm solid lesion with fat associated with calcification

Answer 43

41. e. 3.0-cm solid lesion with fat associated with calcification.

Tumors with calcification associated with fat are uncommon but are almost always malignant RCC. In this setting the fat is thought to be a reactive process related to tumor necrosis. Calcification is virtually never seen in association with AML. The lesions described in a–c are Bosniak II renal cysts, with risk of malignancy of <10%. The lesion described in d is a simple cyst and highly likely to be benign despite its large size.

Question 44

42. A common and pathogenic cytogenetic finding in children with RCC is:
    a. VHL mutation.
    b. cMET oncogene mutation.
    c. p53 mutation.
    d. TFE3 gene fusions.
    e. PTEN mutations.

Answer 44

42. d. TFE3 gene fusions. Mutations or translocations resulting in TFE3 gene fusions are common in RCC occurring in the pediatric population. Although these cancers often present with advanced stage, the t(X;17) variant frequently follows an indolent course, while t(X;1) cancers can recur with late lymph node metastases.

Question 45

43. According to the 2017 AUA Guidelines, which of the following statements about TA is TRUE?
    a. TA should be prioritized in elderly patients with comorbidities.
    b. In general, a tumor size threshold of 2.5 cm should be applied for decision making about TA.
    c. Renal mass biopsy should be performed at the time of TA whenever it might change management.
    d. Local recurrence is more common after TA than partial nephrectomy, but when repeat ablation is taken into account, similar local control rates are observed.
    e. Repeat ablation is feasible in some patients, but surgical resection is often required after failed TA.

Answer 45

d. Local recurrence is more common after TA than partial nephrectomy, but when repeat ablation is taken into account, similar local control rates are observed. Renal mass biopsy should always be performed prior to or concomitant with TA, as it will help guide surveillance. Most local failures of TA can be salvaged with repeat TA, and only a small minority of such patients end up requiring salvage surgery. Success rates TA, for tumors <3.0 cm have been encouraging, and this threshold is advocated in the Guidelines as a general reference point. TA can be a good choice in some elderly patients with comorbidities, but active surveillance is also a reasonable option and in reality is often a better choice for many such patients.

Question 46

44. What is the mechanism of action for nivolumab?
    a. Activates CTLA-4
    b. Blocks CTLA-4
    c. Activates PD-1
    d. Blocks PD-1
    e. Blocks PD-L1

Answer 46

44. c. Activates PD-1. The mechanism of action of nivolumab is to block PD-1 and thus activate the immune system. Pembrolizumab also blocks PD-1, while atezolizumab blocks PDL1 and ipilimumab blocks CTLA-4.

Question 47

According to the 8th edition of the AJCC TNM classification (2016), which of the following tumors would NOT be considered stage pT3a?

a. 4.3 cm RCC with renal sinus fat invasion
b. 12 cm RCC with involvement of the renal vein
c. 5.6 cm RCC with invasion into the perinephric fat
d. 4.8 cm RCC with invasion into the adrenal gland

Answer 47

45. d. 4.8 cm RCC with invasion into the adrenal gland. Direct invasion of the adrenal gland is pT4 and metastatic involvement of the adrenal gland is pM1. All of the other characteristics are indicative of pT3a tumors, including collecting system involvement (a modification made in the 8th edition).

Question 48

46. Most tumors at various sites in the VHL syndrome share the following characteristic:
    a. Malignant behavior
    b. Hypervascularity
    c. Rapid growth rate
    d. High nuclear grade
    e. Symptomatic presentation

Answer 48

46. b. Hypervascularity. Tumors in the VHL syndrome include adrenal pheochromocytoma, retinal angiomas, cerebellar and brainstem hemangioblastoma, RCC, and others. Most are relatively slow growing and asymptomatic if patients are evaluated and screened in a proactive manner. The common feature is that almost all are hypervascular.

Question 49

47. One major difference between hereditary papillary RCC syndrome and VHL syndrome is:
    a. pattern of genetic inheritance.
    b. age of onset.
    c. gender distribution.
    d. incidence of metastasis.
    e. incidence of associated tumors in nonrenal organ systems.

Answer 49

47. e. Incidence of associated tumors in nonrenal organ systems.

The incidence of nonrenal tumors is low in the hereditary papillary RCC syndrome in contrast to VHL syndrome, in which patients commonly develop tumors in the eyes, spinal cord, cerebellum, adrenal glands, inner ear, epididymis, and pancreas.

Question 50

48. Which syndrome is most likely to exhibit aggressive behavior of RCC?
    a. VHL syndrome
    b. Hereditary papillary RCC syndrome
    c. Hereditary leiomyomatosis and RCC syndrome
    d. Birt-Hogg-Dubé (BHD) syndrome
    e. Familial oncocytosis

Answer 50

48. c. Hereditary leiomyomatosis and RCC syndrome. Malignant behavior is particularly common in the hereditary leiomyomatosis and RCC syndrome, and proactive and aggressive surgical management is recommended.