Campbell Malignant Renal Tumors 2021

Question 1

Strongest predictors of malignancy in a renal mass (2)

Answer 1

Male sex

Increasing tumor size

Question 2

___ imaging provides the most accurate characterization of renal masses while assessing for locally advanced features and intra-abdominal metastases and readily excludes angiomyolipoma by identifying intralesional fat.

Answer 2

Multiphasic, cross-sectional imaging

CT and magnetic resonance imaging (MRI) are the most widely used and studied

Question 3

CT: Enhancement ___ HU is indicative of RCC, but does NOT preclude ___.

MRI: Enhancement ___ with IV gadolinium contrast is suggestive of RCC, helpful for masses ___ cm.

Answer 3

Greater than 15 to 20 Hounsfield units (HU)
Does not preclude benign histology

MRI: > 20% enhancement
masses < 2 cm

Question 4

Solid massess with areas of ___ indicative of fat are diagnostic for ___.

Answer 4

< -20 HU

AML

Question 5

Ultrasound criteria for simple cysts

Answer 5

Smooth cyst wall
Round or oval shape
No internal echoes
Through-transmission with strong acoustic shadowing posteriorly

Question 6

Imaging option for:

Complex cyst or solid mass, with significant contrast allergy

Answer 6

MRI with or without gadolinium

Question 7

Imaging option for:

Complex cyst or solid mass, no significant contrast allergy, eGFR > 45 or 30-45

Answer 7

Dedicated renal CT with and without contrast or MRI with and without gadolinium

(if eGFR 30-45, add periprocedural hydration)

Question 8

Imaging option for:

Complex cyst or solid mass, no significant contrast allergy, eGFR < 30

Answer 8

Noncontrast CT or MRI without gadolinium

Ultrasound or CEUS

Question 9

Uncommon but potentially serious complication with MRI + gadolinium

Answer 9

Nephrogenic systemic fibrosis (NSF)

most common:

• PXs on dialysis, with CKD stage 4 and 5
• Significant acute kidney injury
• Patients receiving multiple doses of gadolinium-based contrast agents

Question 10

Gadolinium should only be used with eGFR ___

Answer 10

> 30 mL/min

Question 11

Bosniak I

Answer 11

Water density
Homogeneous, hairline thin wall No septa
No calcification
No enhancement

0% risk of malignancy

Surveillance not necessary

Question 12

Bosniak II

Answer 12

Few hairline septa in which “perceived” enhancement may be present Fine calcification or short segment of slightly thickened calcification in
wall or septa
No unequivocal enhancement
Hyperdense lesion: ≤3 cm, well marginated, with no unequivocal enhancement

Minimal risk of malignancy

Surveillance not necessary

Question 13

Bosniak IIF

Answer 13

Multiple hairline thin septa
Minimal smooth wall thickening
“Perceived” enhancement of wall or septae may be present Calcification may be thick or nodular but must be without enhancement Generally well marginated
No unequivocal enhancement

3%–5% risk of malignancy
IF with: Hyperdense lesion: >3 cm or totally intrarenal with no enhancement
5%-10% risk of malignancy

Periodic surveillance

Question 14

Bosniak III

Answer 14

“Indeterminate,” thickened irregular or smooth walls or septa in which measurable enhancement is present

50% risk of malignancy
Surgical excision

Question 15

Bosniak IV

Answer 15

Clearly malignant lesions that can have all the criteria of category III but also contain enhancing soft-tissue components

75-90% risk of malignancy
Surgical excision

Question 16

Clear cell and papillary variants arise from the ____.

Chromophobe and collecting duct carcinoma arise from the ____.

Answer 16

Clear cell and papillary: PCT

Chromophobe and collecting duct carcinoma: DCT

Question 17

Most generally accepted environmental risk factor for RCC

Answer 17

Tobacco exposure

** All forms of tobacco use have been implicated, and risk increases with cumulative dose or pack-years

Question 18

RCC risk factors

Answer 18

Tobacco exposure
Obesity
Hypertension

Question 19

Familial form of clear cell RCC: ____. Its major manifestations include: ___

Answer 19

von Hippel-Lindau (VHL) disease

RCC (50% of patients with VHL)
Pheochromocytoma
Retinal angiomas
Hemangioblastomas of the brainstem, cerebellum, or spinal cord

** Highly vascular tumors can lead to substantial morbidity

Question 20

VHL protein and RCC

Answer 20

VHL keeps HIF levels low through ubiquitin-mediated degradation
Inactivation of VHL gene –> accumulation of HIFs (esp. HIF-2alpha–> Increased VEGF expression –> pronounced neovascularity in clear cell RCC

Question 21

Second most common histologic subtype of RCC

Answer 21

Papillary RCC

Question 22

Prompt surgical management is recommended for patients with ___, due to aggressive tumor behavior vs. other familial RCC syndromes.

Answer 22

HLRCC

• • develop cutaneous and uterine leiomyomas and type 2 papillary RCC
• • often solitary and unilateral, and they are more likely to be aggressive than other forms of familial RCC

Question 23

Shares features with HLRCC, early onset (< 40 years old), bilateral, multifocal, often with pheochromocytomas and head and neck paragangliomas

Answer 23

Succinate Dehydrogenase Renal Cell Carcinoma (SDHRCC)

Question 24

Cutaneous fibrofolliculomas
Lung cysts
Spontaenous pneumothoraces
Distal nephron-derived renal tumors

Answer 24

Birt-Hogg-Dube syndrome

** include: chromophobe RCC, oncocytomas, and hybrid oncocytic tumors that exhibit features of both of these entities

Question 25

PTEN tumor suppressor gene 34% lifetime risk of RCC 10% risk of epithelial thyroid carcinomas Usually papillary histology

Answer 25

Cowden Syndrome

Question 26

``` AML Cortical tubers Subependymal nodules Subependymal giant cell astrocytomas (SEGA) Pulmonary lymphangiofibromas Multifocal, bilateral, large Typically clear cell histology ```

Answer 26

Tuberous sclerosis complex (TSC)

Question 27

Contributes to RCC chemorefractory nature:

Answer 27

Energy-dependent efflux pumps for hydrophobic cytotoxic compounds ** BUT: cisplatin is not extruded by these proteins, so there must be other chemotherapy resistance mechanisms

Question 28

mTOR inhibitors

Answer 28

Temsirolimus Everolimus * * Efficacious in patients where TKIs have failed * * mTOR pathway leads to increased expression of HIFs and other growth-promoting and potentially tumorigenic sequelae

Question 29

Multicentricity is found in ___ of cases, is more common in association with ___.

Answer 29

10-20% | Papillary RCC and familial RCC

Question 30

All RCCs are ___ derived from ___ cells.

Answer 30

All RCCs are, by definition, ADENOCARCINOMAS, derived from RENAL TUBULAR EPITHELIAL cells.

Question 31

``` Originates from proximal tubule Aggressive Hypervascular tumor Typically well- circumscribed, lobulated, golden yellow tumor VHL-associated Chromosome 3p deletions 70-80% of RCC ```

Answer 31

Clear cell RCC

Question 32

``` Proximal tubule Hypovascular Fleshy tumor with fibrous pseudocapsule Basophilic cells, low-grade nuclei HPRC, altered MET proto-oncogene status in 81% 5-10% of RCC ```

Answer 32

Papillary RCC Type 1

Question 33

``` Proximal tubule Hypovascular Fleshy tumor with fibrous pseudocapsule Eosinophilic cells, high grade nuceli HPRC, Fumarate hydratase gene mutation in HLRCC 5-10% of RCC ```

Answer 33

Papillary RCC Type 2

Question 34

``` Intercalated cells of collecting duct “Plant cells” with pale cytoplasm, perinuclear clearing or “halo,” nuclear “raisins,” and prominent cell borders BHD syndrome TP53 and PTEN mutations Folliculin gene mutation ```

Answer 34

Chromophobe RCC

Question 35

``` ESRD and VHL Good prognosis Low-grade clear epithelial cells in linear papillae VHL disease ~5% of RCC ```

Answer 35

Clear cell papillary RCC

Question 36

``` Collecting duct Poor prognosis Complex highly infiltrative cords within inflamed stroma Firm, centrally located tumor Multiple chromosomal losses RARE <1% ```

Answer 36

CA of the collecting ducts of Bellini

Question 37

``` Collecting duct Dismal prognosis Poorly differentiated cells, lacelike appearance Extensive hemorrhage and necrosis Sickle-cell trait association ```

Answer 37

Renal medullary carcinoma

Question 38

``` Metastasizes early Extremely poor prognosis Hypovascular HLRCC FH mutation ```

Answer 38

HLRCC associated RCC

Question 39

Young adults, SDH mutation HIF-VEGF pathway Vacuolated eosinophilic or clear cells Solitary lesion

Answer 39

Succinate-dehydrogenase deficient renal carcinoma

Question 40

Children and young adults: 40% of pediatric RCC Clear cells with papillary architecture; variable histo Well-circumscribed, tan-yellow

Answer 40

MiT family translocation RCC

Question 41

T1a

Answer 41

Organ-confined ≤4.0 cm

Question 42

T1b

Answer 42

Organ-confined >4.0 cm to 7.0 cm

Question 43

T2a

Answer 43

Organ-confined >7.0 to 10.0 cm

Question 44

T2b

Answer 44

Organ-confined >10.0 cm

Question 45

T3a

Answer 45

Invasion of pelvicalyceal system Invasion of perinephric or renal sinus fat Extension into renal vein or branches

Question 46

T3b

Answer 46

Extension into IVC below diaphragm

Question 47

T3c

Answer 47

Extension into IVC above diaphragm or invasion of IVC wall

Question 48

T4

Answer 48

``` Direct adrenal involvement Locally advanced (invasion beyond Gerota fascia) ```

Question 49

N1

Answer 49

Regional LN involvement

Question 50

M1

Answer 50

Systemic metastasis

Question 51

___ is the premier study for invasion into adjavent structures and evaluation and staging of IVC thrombus

Answer 51

MRI

Question 52

Metastatic workup should include:

Answer 52

``` Chest radiograph Abdominal and pelvic CT or MRI Liver function tests Bone scintigraphy (for elevated alk phos, bone pain, or poor performance status) Chest CT (if abnormal CXR findings) ```

Question 53

No longer recognized as a distinct histologic subtype of RCC: ___ and ___

Answer 53

Sarcomatoid and rhabdoid differentiation ** Spindle cell histology, positive staining for vimentin, infiltrative growth pattern, aggressive local and metastatic behavior, and poor prognosis

Question 54

Classic triad (a.k.a. "too late triad")

Answer 54

Gross hematuria Palpable abdominal mass Flank pain ** The classic triad of flank pain, gross hematuria, and palpable abdominal mass is now rarely seen. This is fortunate because this constellation of findings almost always denotes advanced disease and some refer to it as the “too late triad.”

Question 55

MOST COMMON paraneoplastic syndrome in RCC

Answer 55

Elevated ESR ** The most common of these syndromes is elevated erythrocyte sedimentation rate, which accounts for more than 50% of identified paraneoplastic syndromes.

Question 56

Management of paraneoplastic hypercalcemia

Answer 56

Vigorous hydration Furosemide Bisphosphonates (zoledronic acid 4 mg IV q4weeks) Denosumab therapy if no response to zoledronic)

Question 57

Hypertension in RCC mechanism

Answer 57

Increased production of renin Encasement of the renal artery or branches -- renal artery stenosis Polycythemia from EPO production AV fistula within the tumor

Question 58

In general, treatment of paraneoplastic syndromes requires ___ except for ___.

Answer 58

Surgical excision | Hypercalcemia: treated medically

Question 59

The SINGLE MOST IMPORTANT prognostic factor for RCC

Answer 59

Pathologic stage ** Lymph node involvement is associated with 5- and 10-year survival rates of 5% to 30% and 0 to 5%, respectively

Question 60

Factors favoring active surveillance in RCC

Answer 60

Elderly Life expectancy <5 years High comorbidities Excessive perioperative risk Frailty (poor functional status) Patient preference for AS Marginal renal function Tumor size <3cm Tumor growth <5mm/year Non-infiltrative Low complexity Favorable histology

Question 61

RCC surgical principles: LND for ___. Adrenalectomy for ___. Minimally invasive considered when ___.

Answer 61

LND for clinically concerning regional lymphadenopathy. Adrenalectomy for intraop/imaging findings of invasion or metastasis of adrenal Min invasive when it would not compromise oncologic, functional and perioperative outcomes.

Question 62

Radical nephrectomy generally preferred only if ALL of these criteria are met:

Answer 62

1. High tumor complexity and PN would be challenging even in experienced hands 2. No preexisting CKD or proteinuria; and 3. Normal contralateral kidney and new baseline GFR will likely be >45 mL/ min/1.73 m2.

Question 63

Thermal ablative therapies include: ___ and ___ For management of stage ___ renal masses smaller than ___ cm.

Answer 63

``` Cryosurgery Radiofrequency ablation (RFA) ``` cT1a < 3.0 cm

Question 64

Complete and reliable tissue necrosis with cryoablation is con- sistently achieved only at temperatures of ___ or lower. Iceball must extend beyond visible margins of tumor.

Answer 64

−19.4°C

Question 65

Stage I IVC thrombus

Answer 65

I: adjacent to the ostium of the renal vein

Question 66

Stage II IVC thombus

Answer 66

II: extending up to the lower aspect of the liver and below the hepatic veins

Question 67

Stage III IVC thrombus

Answer 67

III: involving the intrahepatic portion of the IVC but below the diaphragm

Question 68

Stage IV IVC thrombus

Answer 68

IV: extending above the diaphragm