Kidney Congenital Abnormalities, Cystic Lesions, Kidney Tumors Flashcards Preview

MHD > Kidney Congenital Abnormalities, Cystic Lesions, Kidney Tumors > Flashcards

Flashcards in Kidney Congenital Abnormalities, Cystic Lesions, Kidney Tumors Deck (41):
1

List the 3 main stages of kidney development in utero

Pronephros
Mesonephros
Metanephros

2

When does the pronephros appear? Where does it appear?

Pronephros forms around the 4th week

Moves from cervical region to caudal

3

What structures join to form the ureteric bud?

Mesonephric duct and the cloaca

4

What % of people are born with potentially significant malformations of the kidneys?

10%

5

What is a horseshoe kidney?

Fused kidneys with a common pole under the inferior mesenteric artery

Pt has one fused kidney instead of two

6

What is renal dysplasia?

Abnormal development of the kidney/nephrons

NOT related to neoplasia

7

What is the primary pathological issue in Cystic Renal Dysplasia?

Abnormality in metanephric differentiation

Presence of immature elements like mesenchyme and cartilage

8

What is the common gross appearance of Cystic Renal Dysplasia?

"Bunch of grapes"
See cysts and cartilage present outside of the kidney

9

What is the inheritance of Childhood Autosomal Recessive Polycystic Kidney Disease (ARPKD)?

Autosomal recessive, so very rare

10

What is the pathology associated with ARPKD?

Enlarged kidneys, but they retain a normal shape
Saccular dilations of the renal tubules

11

What are the primary symptoms of Adult Polycystic Kidney Disease (APKD)?

May be asymptomatic
Pain
Colic
Mass
Hemorrhage

12

What is the inheritance pattern of APKD?

Autosomal dominant

13

Describe the pathology of APKD

Huge kidney size (normal shape)
Many cysts present in the kidney and liver
May also see intracranial berry aneurysms

14

APKD
Treatment

Kidney transplant
Perhaps liver transplant too depending on function

15

APKD
Prognosis

Renal failure in middle age/later life depending on the mutation present

16

Acquired Cystic Disease
Where are the cysts seen?

Both the renal cortex and medulla

17

Acquired Cystic Disease
What should you do follow-up tests for?

Detection of cancer

18

Simple Cysts
Symptoms

Often asymptomatic

19

Simple Cysts
Why would one need to be removed?

Removed for fear of renal cancer

20

What is the general rule of thumb with all kidney tumors? (They are all presumed to be...)

All kidney tumors are presumed to be malignant until proven otherwise

21

List the two benign kidney tumors

Oncocytoma
Angiomyolipoma

22

Oncocytoma
What is it? What does it look like radiologically?

Benign tumor of the kidney, but looks like renal cell carcinoma by imaging

23

Oncocytoma
How are they usually treated?

They are benign, but they are commonly removed for fear of malignancy

24

Angiomyolipoma
What is this tumor made of? How can it be diagnosed?

Vessels, smooth muscle, and fat

May be diagnosed radiologically due to high fat density

25

Angiomyolipoma
What complication are you worried about?

Worried about potential for rupture from large vessels present with abnormal walls

You would remove the angiomyolipoma to lower this risk

26

Renal Cell Carcinoma
Triad of symptoms

Costovertebral pain
Mass
Hematuria

(Could also present with fever, malaise, weakness, weight loss, polycythemia, hypercalcemia, HTN)

27

Where is Renal Cell Carcinoma most likely to metastasize?

Lungs and Bones

28

Clear Cell RCC
Where is the mutation?

Mutation on chromosome 3 leading to inactivation of the VHL gene (a tumor suppressor)

29

Clear Cell RCC
What does the mutation lead to an accumulation of?

Accumulation of transcription factor HIF-1a and an overexpression of its target genes

Facilitates cellular adaptation to tissue hypoxia

30

Where does Clear Cell RCC commonly originate from?

Proximal tubular epithelium

31

Describe the gross appearance of Clear Cell RCC

"Hypernephroma" due to similarity in appearance to adrenal gland

32

Clear Cell RCC
Prognosis

Stage dependent

33

Clear Cell RCC
Treatment

Surgical removal

RCC is very chemoresistant, so surgery is usually the best option

34

VHL Syndrome
Pathogenesis

Multiple bilateral cysts and tumors with high vascularity and clear cells

35

Papillary RCC
Describe the pathogenesis. How is it acquired?

Hereditary
See multiple bilateral tumors with papillary histology

36

Papillary RCC
Prognosis

Better prognosis than Clear Cell RCC

37

What is the most common pediatric renal cancer?

Wilm's Tumor

38

What is the mutation leading to Wilm's tumor?

Loss of function mutations in WT1 and WT2

39

Wilm's tumor is a triphaic tumor. What are the three pathological characteristics?

Malignant blastema (small undifferentiated blue cells)
Tubules
Stroma

40

What are treatment options for Wilm's Tumor? How should you approach this treatment?

They are very soft tumors and it is crucial to correctly stage the tumor.

If there is extrarenal extension of the tumor, the child will need chemotherapy following removal of the tumor. If the tumor was limited to the kidneys, they will not need chemo.

41

Wilm's Tumor
Prognosis

Excellent - 90% survival at 5 years

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