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Nephrology > Renal Tumors > Flashcards

Renal Tumors Flashcards

1
Q

What are two pediatric renal neoplasms?

A

Nephroblastoma (Wilms tumor)

Mesoblastic nephroma

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2
Q

What are two Benign Adult renal neoplasms?

A

Papillary adenoma (?)
Oncocytoma
Angiomyolipoma

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3
Q

What are two Malignant Adult renal neoplasms?

A

Renal Cell Carcinomas

Urothelial Carcinomas

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4
Q

What is the most common pediatric renal neoplasm? What is the peak age of occurence

A

Wilms tumor
1-4 years old
4th leading cause of cancer in children

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5
Q

What is Wilms Tumor (Nephroblastoma) comprised of?

A

Embryonal nephrogenic elements:

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6
Q

What is the Hallmark appearance of Wilms Tumor?

A

Triphasic neoplasm: present in varying proportions
1.Blastema: cells with little cytoplasma, grow in nests or cords(trabeculae)

  1. Epithelium: Primitive tubules and glomeruli
  2. Stroma: spindle cells [Undifferentiated, Skeletal muscle (most common), Cartilage]
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7
Q

What is the rule of “5s”

A
  1. Wilms tumor accounts for 5% of ped cancers
  2. 5% Bilateral
  3. 5% Multicentric (if in one kidney)
  4. 5% Anaplastic = WORSE PROGNOSIS
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8
Q

What is the most common area of metastasis for Wilms tumor?

A

Lungs

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9
Q

What are 3 syndromes associated with Wilms tumor?

A
  1. Beckwith-Wiedeman syndrome
  2. WAGR syndrome
  3. Denys-Drash syndrome
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10
Q
  1. What are 2 Symptoms of Beckwith-Wiedeman syndrome?
  2. What is the gene mutation?
  3. What percentage develop Wilms Tumor?
A
  1. Organomegaly, Hemihypertrophy
  2. WT2 gene mutation
  3. 5% develop Wilms tumor
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11
Q
  1. What are 4 Symptoms of WAGR syndrome?
  2. What is the gene mutation?
  3. What percentage develop Wilms Tumor?
A
  1. Wilms-Aniridia-Genital anomaly-Retardation
  2. WT1
  3. 33% develop Wilms tumor
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12
Q
  1. What are 3 Symptoms of Denys-Drash syndrome?
  2. What is the gene mutation?
  3. What percentage develop Wilms Tumor?
A
  1. Glomerulonephritis, male pseudohermaphroditism, Wilms
  2. WT1
  3. 90% develop Wilms tumor
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13
Q

What is the typical clinical presentation of Wilms tumor?

A
  1. Abdominal mass/pain - parents notice when changing diaper
  2. Intestinal obstruction
  3. Hematuria
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14
Q

What is the treatment for Wilms Tumor? What 3 features cause a bad prognosis

A

Nephrectomy + Chemotherapy = 80-90% cure rate

bad prognosis = ANAPLASIA, age >2, large size

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15
Q

What is the pathogenesis of Wilms tumor?

A

Mutation in tumor suppressor genes on CHROMOSOME 11 (11p13-11p15.5)

  • -WT1 gene: growth promoting proteins (IGF-2)
  • -WT2 gene: interacts with growth promoting fx
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16
Q

What is the most common renal tumor of INFANCY? (0-1yrs old)

A

Congenital Mesoblastic Nephroma

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17
Q

What is a Congenital Mesoblastic Nephroma composed of?

A

SPINDLED CELLS (immature mesodermal tissue): smooth muscle, cartilage

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18
Q

What is the presentation of Congenital Mesoblastic Nephroma? what is the chance of metastasis?

A

Palpable mass often found by prenatal imaging

metastaes are rare: lungs, brain, rarely bone

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19
Q

What is the treatment for Congenital Mesoblastic Nephroma?

A

Nephrectomy with Wide margins (remove perinephric fat)

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20
Q

Why is Papillary adenoma controversial in its categorization as a benign Adult renal neoplasm?

A

current view is to regard ALL adenomas as potentially malignant (regardless of size)

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21
Q

What is the most common renal epithelial neoplasm in Adults?

A

Renal Papillary Adenoma

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22
Q

What is the size categorization for Renal Papillary Adenoma?

A 
<0.5cm = benign
<3.0cm = probably wont metastasize
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23
Q

How can chronic dialysis affect neoplasticity

A

chronic dialysis -> develop Cystic Renal Disease -> 33% develop papillary adenoma -> if multiple adenomas = Adenomatosis

24
Q

How are Renal Papillary Adenomas usually discovered

A

incidentally (because they are asymptomatic)

25
Q

What are morphologic features of Renal Papillary Adenomas

A
  1. Well circumscribed
  2. nonencapsulated
  3. subcapsular (under renal capsule)
26
Q

What is the histopathology of Renal Papillary Adenoma

A
  1. Well-circumscribed
  2. Bland-looking cells
  3. Eosinophilic, not clear cytoplasm
  4. fibrovascular cored lined by bland cells
27
Q

What is an Adult epithelial neoplasm >3.0 cm called?

A

Papillary renal cell carcinoma

28
Q

What are 4 clinical characteristics of Renal Oncocytoma?

A
  • Central Scar within lesion
  • M:F = 2:1; median age: 60-65
  • 5% of resected renal neoplasms
  • Rare metastases (benign)
29
Q

What is the appearance of Renal Oncocytoma?

A
  1. Well circumscribed
  2. tan/yellow/MAHOGANY BROWN
  3. typically solitary (6 cm)
  4. Central stellate scar
30
Q

What is the Ddx of renal onchocytoma?

A

chromophobe RCC

31
Q

What do Renal Oncocytoma cells look like?

A

abundant granular eosinophilic cytoplasm

nuclei: smal, round, regular

32
Q

what is an Angiomylipoma (where does it originate)

A

Mesenchymal tumor (1% renal neoplasms)

33
Q

What 4 hereditary disorders are Angiomylipomas associated with?

A
  1. Tuberous sclerosis
  2. von Recklinghausen disease (NF1)
  3. von-Hippel Lindau syndrome
  4. ADPK (polycystic - adults)
34
Q

Although Angiomyolipomas are benign, what 2 ways can they cause malignancy?

A
  1. Massive hemorrhage

2. significant loss of fxnal renal parenchyma if tumor is large enough (Renal failure)

35
Q

What is the light microscopy and histogenesis of Angiomylipoma?

A

Histogenesis: Perivascular epithelioid cell
LM: mixture of mature ADIPOSE, spindled cells with SMOOTH MUSCLE features, and thick-walled BLOOD VESSELS

36
Q

What does multifocality suggest in Angiomyolipoma?

A

usually solitary
multifocality suggest tuberous sclerosis
(autosomal dominant neurocutaneous disorder)
–involves veins and regional lymph nodes

37
Q

What is the most common malignant renal neoplasm in adults? what are the demographics?

A

RCC (Clear cell) -85%

2/3 are MEN, >50 yo, 1% bilateral

38
Q

Where are urothelial carcinomas found? (TCC)

A

malignant renal epithelium
involves collecting system:
(Calyces, Pelvis, Ureter, Urinary Bladder)

39
Q

Where do RCC’s arise from?

A

Renal tubular/collecting duct epithelium

40
Q

What are the risk factors for RCC?

A 
Tobacco,
Tuberous sclerosis
VON HIPPEL LINDAU DIEASE
renal transplant
dialysis-acquired cystic renal disease
HTN
treated Neuroblastoma
41
Q

What are the clinical symptoms of RCC? (classic triad)

A
  1. Flank pain
  2. Palpable Mass
  3. Hematuria
42
Q

What are the most important prognosis factors?

A

Tumor stage

Fuhrman Nuclear Grade

43
Q

What are the metastatic characteristics of RCC

A

25% metastases
spread to: lung, bone, lymph nodes, adrenals, liver, brain
overall 5 year survival: 70% (even with extension of tumor into renal vein, IVC, RA

44
Q

What is the treatment for RCC?

A

Surgical Resection or Nephrectomy, Cryotherapy

(Chemo is minimally effective)
if Advanced (beyond Gertoas fascia): immunotherapy, cytokine therapy, VEGF inhibs, IL-2 therapy
45
Q

What does Trisomy 7 and MET mutations cause?

A

papillary neoplasms

46
Q

What do von Hippel Lindau (VHL)gene, Chromosome 3 mutations cause?

A 
Clear Cells RCC 
VHL gene (3p25)
47
Q

How is the Von Hippel Lindau involved with RCC?

A

VHL = part of ubiquitin ligase complex = acts as 1) TUMOR SUPPRESSOR
2) VEGF (helps metastasis)

48
Q

What is prognosis of CCRCC, pt presentation?

A

50% of pts die

Tumor cells release EPO = pts present with elevated hematocrit

49
Q

What are characteristics of Papillary RCC?

A
  1. 10%-15% of RCC (second most common)
  2. C-MET gene mutation
  3. associated with ESRD (DIALYSIS KIDNEY)
  4. > 3cm
  5. better prognosis than CCRCC
50
Q

What is the histological difference between Papillary adenoma and Papillary RCC?

A

Adenoma: fibrovascular cores with plane looking cells

RCC: fibrovascular cores containing FOAM CELLS

51
Q

What is the DDX for chromophobe RCC? and what are some characteristics?

A

DDX: CCRCC, oncocytoma

  1. 5% of RCC
  2. good prognosis
  3. Tx: neprectomy
52
Q

What is the microscopic appearance of Chromophobe RCC?

A
  1. well defined cell membrance, faintly granular cytoplasm
  2. PERINUCLEAR CLEAR HALO
  3. Raisinoid (irregular contours)
  4. pleomorphism and maybe binucleate
53
Q

What are characteristics of Collecting Duct Carcinoma?

A

Rare: <0.1% of RCC
WORST PROGNOSIS
presentation: PAINLESS GROSS HEMATURIA
50% have coexisting bladder urothelial carcinoma

54
Q

What are characteristics of Renal Medullary Carcinoma?

A
  1. Very rare, aggressive
  2. AFRICAN AMERICANS or MEDITERRANEAN
  3. SICKLE CELL!!
55
Q

Where does Renal Urothelial Carcinomas come from?

A

Neoplasms of TCC (Calyces, Pelvis)

multifocal (Ureter, Urinary bladder)

56
Q

What is the appearance of Renal Urothelial Carcinomas? What is the aggressive appearance?

A

Exophytic, Papillary growth pattern

–Flat = more aggressive

Nephrology (13 decks)

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