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Renal II Exam 1 > Puthoff Lecture 3 > Flashcards

Puthoff Lecture 3 Flashcards

1
Q

Inheritance of adult polycystic kidney disease?

A

Autosomal dominant

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2
Q

What does mahogany-brown and well-circumscribed with central stellate scar and large morphology describe?

A

Renal oncocytoma

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3
Q

Which form of ARPKD (children) is being described?

20% CDs cystic, hepatic fibrosis, portal HTN, die in early childhood?

A

Infantile

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4
Q

What subclass of RCC has papillary growth pattern, trisomy of 7 (loss of MET), 16, 17, loss of Y?

Prognosis?

A

Papillary carcinoma

Better

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5
Q

Inheritance pattern for Nephronopthisis Familial form?

A

Autosomal recessive

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6
Q

What are the extra-renal manifestations associated with ADPKD?

A

40% hepatic cysts
4-10% DIE of subarachnoid hemorrhage
25% mitral valve prolapse
82% diverticular disease

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7
Q

What tumor occurs in mostly men, around 60-80 y/o and has cigarette smoking, HTN, obesity, and TS ask RFs?

A

RCC

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8
Q

Which form of ARPKD (children) is being described?

Less than 10% CDs, cystic, most do not survive past adolescence

A

Juvenile

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9
Q

What genetics are associated with renal papillary adenoma?

A

Trisomy 7 and 17

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10
Q

Wilms tumor tri phasic pattern composed of what?

A

Stromal
Epithelial
Blastemal

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11
Q

What is the classic triad of Sx for RCC?

A

Hematuria
CVA pain
Palpable flank mass

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12
Q

Renal oncocytomas arise from what?

Present when?

A

Type A intercalated cells

Adulthood

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13
Q

Composition of most common type of kidney stone?

A

Calcium oxalate and phosphate

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14
Q

Xp11 translocation carcinoma has what gene translocation?

A

TFE3

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15
Q

What cancers metastasize TO the kidney?

A

Lung, melanoma, breast, GI, pancreas

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16
Q

What are the most common subtypes of autosomal recessive polycystic kidney disease?

A

Perinatal

Neonatal

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17
Q

What group of diseases is the most common genetic cause of ESRD in children and young adults?

A

Nephronopthisis

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18
Q

Order of most common childhood malignant tumors?

A
  1. Acute leukemia
  2. Neuroblastoma
  3. Retinoblastoma
  4. WILMS TUMOR
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19
Q

What RCC subtypes have a “better” prognosis?

A

Papillary

Chromophobe

20
Q

What RCC subtypes have a “average” prognosis?

A

Clear cell

21
Q

Where does collecting [Bellini] Duct carcinoma arise from?

Prognosis?

A

Medullary location

Poor

22
Q

What is the most critical prognostic element in Wilms tumor?

A

Presence or absence of diffuse anaplasia

23
Q

What stages are unfavorable in neuroblastomas?

What ages?

Absence or presence of scwhannian stroma is unfavorable?

A

3, 4

> 18 months

Absence

24
Q

What form of autosomal recessive PKD has the worst prognosis?

A

Perinatal, only live a few hours

25
Q

What subclass of RCC makes up 70-80% of RCC and is non-papillary, mostly sporadic, and has deletion of chromosome 3?

What gene is lost here?

A

Clear cell carcinoma

VHL

26
Q

Urothelial carcinoma of the kidney arises from where?

What clinical manifestation?

Prognosis?

A

Urothelium of renal pelvis

Hematuria

Poor

27
Q

What disease shows morphology of cysts localized to the corticomedullary junction and medulla?

A

Medullary cystic disease

28
Q

What is the only chromosome expression or loss that confers a favorable prognosis for neuroblastomas?

A

TRKA

29
Q

Dialysis acquired cystic disease predisposes to development of what?

A

RCC

30
Q

Multiple lesions seen with papillary and clear cell types of carcinomas indicate what?

A

Hereditary form

31
Q

PKD1 gene located on what chromosome?

PKD2?

A

16

4

32
Q

What gene is mutated in the juvenile Nephronopthisis forms?

A

NPHP1 to NPHP11

33
Q

What genes are mutated in medullary cystic disease?

Inheritance pattern of adult-onset form?

A

MCKD1 and 2

Autosomal dominant

34
Q

What is the most common benign kidney neoplasm?

What defines it?

Morphology of it?

A

Renal papillary adenoma

Less than 1cm

Cortical, discrete, yellow-gray, small

35
Q

What is described by bilateral very large kidneys, hematuria, flank pain, and chronic renal failure age 40-60?

A

ADPKD (adult)

36
Q

What histomorphology of WILMS Tumor is favorable?

Unfavorable?

A

Tri phasic

Anaplastic

37
Q

What disease morphology is described by well-circumscribed margins, tan-to-gray color, large, expansile tumor in lower pole in kidney?

A

Wilms tumor

38
Q

Which disorders are considered ciliopathies?

A

PKD
Medullary cystic disease
Nephronophthisis

39
Q

What subtype of RCC arises from type B intercalated cells and has pale eosinophilic cytoplasm with nuclear halos?

Prognosis?

A

Chromophobe

Better

40
Q

Nephrogenic rests are precursor lesions for what?

A

Wilms Tumor

41
Q

What tumor has a strong association with tuberous sclerosis and presents in middle-aged women?

Loss of what gene?

A

Angiomyolipoma

TSC1 or TSC2

42
Q

90% of WILMS tumor arise with no congenital abnormalities, but 10% have associations with what?

A

WT1, Beta-catenin

43
Q

Inheritance pattern of hereditary RCC?

A

Autosomal dominant

44
Q

Peak age of WILMS tumor?

A

2-5

45
Q

What RCC subtypes have a “poor” prognosis?

A

CD
Sarcomatoid
Medullary

Renal II Exam 1 (6 decks)

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