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04. RCC (937) > Campbell Malignant Renal Tumors 2021 > Flashcards

Campbell Malignant Renal Tumors 2021 Flashcards

1
Q

Strongest predictors of malignancy in a renal mass (2)

A

Male sex

Increasing tumor size

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2
Q

___ 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.

A

Multiphasic, cross-sectional imaging

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

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3
Q

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.

A

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

MRI: > 20% enhancement
masses < 2 cm

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4
Q

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

A

< -20 HU

AML

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5
Q

Ultrasound criteria for simple cysts

A

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

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6
Q

Imaging option for:

Complex cyst or solid mass, with significant contrast allergy

A

MRI with or without gadolinium

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7
Q

Imaging option for:

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

A

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

(if eGFR 30-45, add periprocedural hydration)

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8
Q

Imaging option for:

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

A

Noncontrast CT or MRI without gadolinium

Ultrasound or CEUS

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9
Q

Uncommon but potentially serious complication with MRI + gadolinium

A

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
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10
Q

Gadolinium should only be used with eGFR ___

A

> 30 mL/min

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11
Q

Bosniak I

A

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

0% risk of malignancy

Surveillance not necessary

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12
Q

Bosniak II

A

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

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13
Q

Bosniak IIF

A

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

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14
Q

Bosniak III

A

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

50% risk of malignancy
Surgical excision

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15
Q

Bosniak IV

A

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

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16
Q

Clear cell and papillary variants arise from the ____.

Chromophobe and collecting duct carcinoma arise from the ____.

A

Clear cell and papillary: PCT

Chromophobe and collecting duct carcinoma: DCT

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17
Q

Most generally accepted environmental risk factor for RCC

A

Tobacco exposure

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

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18
Q

RCC risk factors

A

Tobacco exposure
Obesity
Hypertension

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19
Q

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

A

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

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20
Q

VHL protein and RCC

A

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

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21
Q

Second most common histologic subtype of RCC

A

Papillary RCC

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22
Q

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

A

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
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23
Q

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

A

Succinate Dehydrogenase Renal Cell Carcinoma (SDHRCC)

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24
Q

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

A

Birt-Hogg-Dube syndrome

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

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25
Q

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

A

Cowden Syndrome

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26
Q 
AML
Cortical tubers
Subependymal nodules
Subependymal giant cell astrocytomas (SEGA)
Pulmonary lymphangiofibromas
Multifocal, bilateral, large
Typically clear cell histology
A

Tuberous sclerosis complex (TSC)

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27
Q

Contributes to RCC chemorefractory nature:

A

Energy-dependent efflux pumps for hydrophobic cytotoxic compounds

** BUT: cisplatin is not extruded by these proteins, so there must be other chemotherapy resistance mechanisms

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28
Q

mTOR inhibitors

A

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
29
Q

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

A

10-20%

Papillary RCC and familial RCC

30
Q

All RCCs are ___ derived from ___ cells.

A

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

31
Q 
Originates from proximal tubule
Aggressive
Hypervascular tumor
Typically well- circumscribed, lobulated, golden yellow tumor 
VHL-associated
Chromosome 3p deletions
70-80% of RCC
A

Clear cell RCC

32
Q 
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
A

Papillary RCC Type 1

33
Q 
Proximal tubule
Hypovascular
Fleshy tumor with fibrous pseudocapsule
Eosinophilic cells, high grade nuceli
HPRC, Fumarate hydratase gene mutation in HLRCC
5-10% of RCC
A

Papillary RCC Type 2

34
Q 
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
A

Chromophobe RCC

35
Q 
ESRD and VHL
Good prognosis
Low-grade clear epithelial cells in linear papillae 
VHL disease
~5% of RCC
A

Clear cell papillary RCC

36
Q 
Collecting duct
Poor prognosis
Complex highly infiltrative cords within inflamed stroma
Firm, centrally located tumor
Multiple chromosomal losses
RARE <1%
A

CA of the collecting ducts of Bellini

37
Q 
Collecting duct
Dismal prognosis
Poorly differentiated cells, lacelike appearance
Extensive hemorrhage and necrosis
Sickle-cell trait association
A

Renal medullary carcinoma

38
Q 
Metastasizes early
Extremely poor prognosis
Hypovascular
HLRCC
FH mutation
A

HLRCC associated RCC

39
Q

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

A

Succinate-dehydrogenase deficient renal carcinoma

40
Q

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

A

MiT family translocation RCC

41
Q

T1a

A

Organ-confined ≤4.0 cm

42
Q

T1b

A

Organ-confined >4.0 cm to 7.0 cm

43
Q

T2a

A

Organ-confined >7.0 to 10.0 cm

44
Q

T2b

A

Organ-confined >10.0 cm

45
Q

T3a

A

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

46
Q

T3b

A

Extension into IVC below diaphragm

47
Q

T3c

A

Extension into IVC above diaphragm or invasion of IVC wall

48
Q

T4

A 
Direct adrenal involvement
Locally advanced (invasion beyond Gerota fascia)
49
Q

N1

A

Regional LN involvement

50
Q

M1

A

Systemic metastasis

51
Q

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

A

MRI

52
Q

Metastatic workup should include:

A 
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)
53
Q

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

A

Sarcomatoid and rhabdoid differentiation

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

54
Q

Classic triad (a.k.a. “too late triad”)

A

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.”

55
Q

MOST COMMON paraneoplastic syndrome in RCC

A

Elevated ESR

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

56
Q

Management of paraneoplastic hypercalcemia

A

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

57
Q

Hypertension in RCC mechanism

A

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

58
Q

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

A

Surgical excision

Hypercalcemia: treated medically

59
Q

The SINGLE MOST IMPORTANT prognostic factor for RCC

A

Pathologic stage

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

60
Q

Factors favoring active surveillance in RCC

A

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

61
Q

RCC surgical principles:

LND for ___.

Adrenalectomy for ___.

Minimally invasive
considered when ___.

A

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.

62
Q

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

A
  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.
63
Q

Thermal ablative therapies include: ___ and ___

For management of stage ___ renal masses smaller than ___ cm.

A 
Cryosurgery
Radiofrequency ablation (RFA)

cT1a
< 3.0 cm

64
Q

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.

A

−19.4°C

65
Q

Stage I IVC thrombus

A

I: adjacent to the ostium of the renal vein

66
Q

Stage II IVC thombus

A

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

67
Q

Stage III IVC thrombus

A

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

68
Q

Stage IV IVC thrombus

A

IV: extending above the diaphragm

04. RCC (937) (10 decks)

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