Dobson Renal Path #8 Flashcards

1
Q

Non smoker, renal mass, workup for flank pain and hematuria. Nephrectomy shows mass with mahogany brown cut surface and central scar. Large benign cells with eosinophilic cytoplasm and scattered large nucleoli are found on histology. what is the diagnosis?

A

Oncocytomoa

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2
Q

The eosinophilic appearance of the cytoplasm in oncocytooma is due to the overabundance of what organelle?

A

Mitochondria

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3
Q

Most common malignancy of kidney

A

renal cell carcinoma

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4
Q

Epidemiology of renal cell carcionma?

A
  • 60s-70’s
  • 2:1 males
  • most are sporadic but some are AD familial forms
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5
Q

What is Von Hippel Lindau syndrome?

A
  • Rare disorder in which tumors and cysts develop all over the body
  • most develop renal cysts and multiple bilateral renal cell carcinomas
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6
Q

Who has double the incidence of renal cell carcionma?

A
  • Cigarette smokers
  • also increase risk for those with ESRD CKD cystic disease and tuberous sclerosis (not double)
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7
Q

What are additional risk factors for renal cell carcinoma besides smoking and diseases?

A
  • Obesity
  • htn
  • unopposed estrogen
  • asbestos
  • petroleum
  • heavy metal
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8
Q

Where do renal carcinmas occur in the kidney?

A
  • can arise in any portion but most common to the poles
  • As they enlarge they can bulge into calyces and pelvis and eventually through the walls of the collecting system into the ureter
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9
Q

Where do renal cell carcinomas like to invade?

A
  • Venous tumor invasion with possible extension into the IV
    • renal vein invasion classifies this as T3 stage
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10
Q

Besdies RCC what other tumor has a tendency to invade the IVC?

A
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11
Q

What are clear cell carcinomas?

A
  • Solid unilateral masses involving the upper or lower pole
  • Arise from proximal tubular epithelium
  • Bright yellow grey whie sphewrical mass that distort renal outline
  • rounded or polygonal shape and abundant clear or granular cytoplasm with lipids and glycogen
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12
Q

What is the most common type of renal cell carcionma?

A

clear cell carcinoma makes up 70-80% of RCC

most cases are sporadic but associated with VHL syndrome,

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13
Q

What is papillary carcinoma?

A
  • 10-15% of renal cancers
  • occurs sporadically or familial
  • not associated with 3p deletions unlike clear cell carcinoma
  • seen with trisomies 7 and 17 and loss of Y in pale patients in sporadic form
  • seen with trisomy 7 in familial form
  • Intersitial foam cells are common
  • Psammoma bodies are present
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14
Q

What is chromophobe carcinoma?

A
  • prominent cell membranes and pale eosinohilic cytoplasm wit hhalo around nucleus
  • good prognosis
  • grow from intercalated cells of collecting duct
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15
Q

What is Xp11 translocation carcinoma?(who gets it, chromosome, whats expressed and what does it look like)

A
  • renal cell carcinoma that occurs in young patients and has translocations of TFE3 gene at Xp11.2
  • Clear cytoplasm with papillary architecture
  • overexpression of TFE3 transcription factor
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16
Q

How do renal cell carcionma present?

A
  • Flank pain
  • Palpable mass
  • Hematuria
    • intermittent and microscopic

weight loss, fever, malaise, weakness, intermittent fever

17
Q

Prognosis of RCC?

A
  • 5 yr survival rate is 70% and up to 100% if no metastases
  • Renal vein invasion takes it to 60%
18
Q
A

Wilms tumor

19
Q

What is the most common pediactric primary renal tumor of childhood?

A
  • Wilms tumor (nephroblastoma), can involve both kidneys simultaneously or one after the other, occurs btw 2-5 yrs old
20
Q

What is WAGI/R syndrome characterized by?

A
  • Wilms tumor
  • Aniridia
  • Genitourinary anomalies
  • Intellectual disability
  • Deletions of 11p13 (WT1 and PAX6 genes)
21
Q

What is Denys-Drash Syndrome characterized by?

A
  • gonadal dysgenesis
  • early onset nephropathy leding to renal failure
  • glomerular lesion in patients is diffuse mesangial sclerosis
  • At risk for wilms tumor and also gonadoblastomas
22
Q

Beckwith-Wiedemann syndrome?

A
  • At risk for wilms tumor
  • organomegaly
  • macroglossia
  • hemihypertrophy
  • omphalocele
  • abnormally large cells in adrenal cortex
23
Q

What do wilms tumors look like microscopically?

A
  • small blue cell or blasteamal component
  • 5% have anaplasia which correlates with presence of TP53 mutation with resistance to chemo
24
Q

Are mets common to the kidney?

A

No, melanoma maybe some lymphomas with systemic spread, leukemias since they are circulating but it is not common