B/2. Renal tumors Flashcards

1
Q

BENIGN Renal tumors

A
  • Angiomyolipoma
  • Oncocytoma
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2
Q

Angiomyolipoma def

A

benign renal tumors that arise from perivascular epithelioid cells
and consist of:
* blood vessels,
* smooth muscle, and
* mature fat cells

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3
Q

most common benign renal tumor

A

angiomyolipoma

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4
Q

Epidemiology Angiomyolipoma

A

most common benign renal tumor,
F > M(4:1)

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5
Q

angiomyolipoma etiology

A

: sporadic

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6
Q

Angiomyolipoma symptoms

A

mostly asymptomatic;
large tumors can present with
* hematuria,
* retroperitoneal hemorrhage,
* impaired renal function

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7
Q

Angiomyolipoma Pathology, hows growth rate?

A

slow-growing;
epithelioid angiomyolipoma have a greater number of epithelioid
cells, acidophilic and granular cytoplasm, less fat than classic type

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8
Q

Angiomyolipoma diagnostics

A
  • US
  • CT
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9
Q

Angiomyolipoma treatment

A

Treatment: surgical resection if >4 cm

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10
Q

Oncocytoma def

A

benign epithelial tumor arising in the collecting duct from the intercalated tubular cells

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11
Q

Oncocytoma symptoms

A
  • painless hematuria,
  • abdominal mass,
  • flank pain
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12
Q

Oncocytoma gross pathology

A

smooth, clearly defined brown tumor
with central radial scar;
large acidophilic cells,
excessive amount of mitochondria resulting in granular cytoplasm
without perinuclear clearing (unlike chromophobe RCC)

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13
Q

Oncocytoma diagnostics

A
  • US
  • CT
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14
Q

Oncocytoma treatment

A

often resected in order to exclude RCC

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15
Q

Oncocytoma prognosis

A

may transform into malignant oncocytic RCC

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15
Q

Oncocytoma prognosis

A

may transform into malignant oncocytic RCC

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16
Q

painless hematuria can be symptom of ?

A

Oncocytoma

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17
Q

MALIGNANT renal tumor

A

RENAL CELL CARCINOMA

18
Q

RENAL CELL CARCINOMA epidemiology
gender
age
%

A
  • Incidence increases continuously during the last years
  • RCC accounts for >90% of malignant kidney neoplasms
  • More common in males
  • 50-70 years but can present at any age
19
Q

what tumor accounts for >90% of malignant kidney neoplasms

A

RCC

20
Q

RENAL CELL CARCINOMA more common in which sex

A

males (2:1)

21
Q

peak age incidence of RCC

A

ages 50-70
but can present at any age

22
Q

RENAL CELL CARCINOMA risk factors

A
  • Smoking
  • Renal cystic diseases
  • end-stage kidney disease
  • Von-Hippel-Lindau syndrome (AD mutation, tumor suppressor gene, located on chromosome 3);
    bilateral RCC, hemangioblastoma, angiomatosis, pheochromocytoma
23
Q

Von-Hippel-Lindau syndrome

A
  • (AD mutation, tumor suppressor gene, located on chromosome 3);
    bilateral RCC, hemangioblastoma, angiomatosis, pheochromocytoma

Von HIPPEL-Lindau syndrome:
Hemangioblastoma, Increased risk of renal cell carcinoma, Pheochromocytoma, Pancreatic lesions (cysts, cystadenomas, and neuroendocrine tumors), Eye Lesions (retinal angiomas or hemangioblastomas)

24
Q

VHL normally inhibits

A

VHL normally inhibits HIF-1 alpha (Hypoxia Inducible Factor alpha ) > mutation results in increased activity of VEGF and PDGF pathways

25
Q

RENAL CELL CARCINOMA clinical findings

A
  • Up to 70% of cases are asymptomatic
  • Classic triad: hematuria, abdominal mass, flank pain
  • Constitutional: fever, weight loss, anemia
  • Paraneoplastic syndromes:
    *PTHrP > hypercalcemia
    *EPO > erythrocytosis
    *ACTH> cushing syndrome
    *Renin > hypertension
26
Q

How is RCC usually discovered?

A
  • Up to 70% of cases are asymptomatic; most commonly detected as an incidental finding on radiograph or other abdominal imaging
27
Q

List Paraneoplastic syndromes in RCC

A
  • PTHrP > hypercalcemia
  • EPO > erythrocytosis
  • ACTH> cushing syndrome
  • Renin > hypertension
28
Q

classic triad in RCC

A
  • hematuria,
  • abdominal mass,
  • flank pain
29
Q

histopathological classification of RCC

A
  • Clear cell RCC 60% Arises from PCT cells
  • Papillary (chromophilic) RCC 5-15% Arises from PCT cells
  • Chromophobe RCC 5-10% Indolent clinical course
  • Oncocytic RCC 5-10% Considered benign neoplasm
  • Collecting duct (Bellini) RCC < 1% Arises from medullary collecting ducts; aggressive
30
Q

RCC Diagnostics

A
  • The standard evaluation of patients with suspected renal cell tumor includes:
    1. Renal US
    2. CT scan of the abdomen and pelvis
    3. CXR
    4. Urinalysis
    5. Urine cytology
  • If metastatic disease is suspected from the chest radiograph, a CT of the chest is warranted
  • MRI is useful in evaluating the IVC in cases of suspected
    tumor involvement or invasion by thrombus.
31
Q

when is MRI useful in RCC

A

MRI is useful in evaluating the IVC in cases of suspected
tumor involvement or invasion by thrombus.

32
Q

In clinical practice, any solid renal mass should be considered

A

malignant until proven otherwise

33
Q

staging of RCC
T1a, T1b

A

T1a Tumor < 4 cm in largest diameter, confined to the kidney
T1b Tumor 4-7 cm in largest diameter, confined to the kidney

34
Q

staging of RCC
T2

A

T2 Tumor > 7 cm in largest, confined to the kidney

35
Q

staging of RCC
T3a, T3b, T3c

A
  • T3a Tumor invades adrenal gland or perinephric tissue, but not beyond Gerota’s fascia
  • T3b Tumor invades renal vein or IVC below diaphragm
  • T3c Tumor invades IVC above diaphragm
36
Q

staging of RCC
T4

A

T4 Tumor invades beyond Gerota’s fascia

37
Q

metastasis of RCC

A

lymphatics,
lung,
bone,
brain,
liver

38
Q

which LN get involved in RCC

A

renal hilar
lymph nodes

39
Q

Treatment of RCC
T1, T2

A
  • Radical nephrectomy en-bloc removal:
    *of Gerota’s fascia including kidney,
    *adrenal gland, and
    *hilar lymph nodes
  • Nephron-sparing approach/Partial nephrectomy (laparoscopic or open surgery):
    *appropriate choice if T1a
    *or for patients who have only one functional kidney (depending on the size and location of the lesion)
40
Q

when is nephron sparing approach/partial nephrectomy used in RCC

A

(laparoscopic or open surgery)
1. appropriate choice if T1a
or
2. for patients who have only one functional kidney (depending on the size and location of the lesion)

41
Q

Advanced/
metastatic RCC
treatment

A
  • Consider surgery to alleviate hematuria/pain from primary tumor, or cytoreductive nephrectomy before systemic treatment is initiated
  • Targeted therapy
    -Sunitinib, sorafenib, pazopanib, axitinib (oral TKI targetting VEGF and PDGF)
    -Bevacizumab Anti-VEGF monoclonal Ab
    -Everolimus, temserolimus (mTOR inhibitors, used if patient fails to respond
    to TKI therapy
  • Immunotherapy
    -Pembrolizumab, nivolumab (anti-PD1 monoclonal Ab)
    -Ipilimumab (anti-CTLA4 monoclonal Ab)
    -Cytokines : IL-2 (Aldesleukin), IFN-alpha; not commonly used nowadays
42
Q

when to consider surgery in advanced metastatic RCC

A

Consider surgery to alleviate hematuria/pain from primary tumor,
or
cytoreductive nephrectomy before systemic treatment is initiated