Benign stuff Flashcards

1
Q

What can be used to ablate a benign, symptomatic renal mass with over 90% success rate?

A

95% EtOH

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2
Q

Peripelvic or renal sinus cysts originate from lymphatics or parenchyma?

A

Lymphatics

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3
Q

A hyperdense benign renal cyst defers how on CT non-contrast vs. CT with contrast

A

they look the same; similar HUs on both (i.e. non-enhancing with contrast)

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4
Q

Two mutations for ADPKD?

A

Chromosome 16p (PKD1; polycystin-1)

Chromosome 4 (PKD2; polycystin-2)

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5
Q

Why may urinalysis be negative even though cysts are infected in PKD?

A

the cortical renal cysts are not contiguous with the collecting tubules

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6
Q

Lipophilic antibiotics penetrate infected cysts better than hydrophilic antibiotics; therefore, which abx are recommended?

A

Ciprofloxacin
Bactrim (trimethoprim/sulfmethoxazole)
Clindamycin
Chloramphenicol

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7
Q

If infection of cysts cannot be cleared with antibiotics, then what do you do?

A

Percutaneous drainage

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8
Q

ADPKD are also associated with abnormalities in what other two organs?

A

Hepatic cysts in the liver

Berry aneurysms in the brain
- screen with MRA after age 30

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9
Q

Is ADPKD associated with increased RCC?

A

NO

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10
Q

ARPKD mutation?

A

Chromosome 6 (PKHD1, fibrocystin)

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11
Q

Is ARPKD associated with cysts in other organs?

A

No

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12
Q

What happens in the liver in ARPKD?

A

Congenital hepatic fibrosis, biliary atresia

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13
Q

Rate of renal failure in ADPKD? ARPKD?

A

ADPKD = ~50% by adulthood (30 years)

ARPKD = 100%, and at young age

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14
Q

What is nephronophthisis?

A

MEDULLARY cystic disease formed at the corticomedullary junction
- due to AR mutation in ciliary proteins

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15
Q

Do ppl with nephronophthisis develop ESRD?

A

yes, inevitable

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16
Q

The three forms of nephronophthisis are infantile, juvenile, and adolescent. Which is most common, and what is it associated with?

A

Juvenile is MC

Associated with retinitis pigmentosa.

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17
Q

Treatment of nephronophthisis?

A

Supportive care. Transplant when ESRD

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18
Q

What is the benign disorder with cysts that develop in the collecting ducts?

A

Medullary sponge kidney

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19
Q

What mutation is associated in medullary sponge kidney?

What syndrome has high prevalence of medullary sponge kidney?

A

RET oncogene, although the majority are sporadic

Beckwith-Wiedemann Syndrome/hemihypertrophy

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20
Q

Imaging buzzword associated with medullary sponge kidney?

A

“paint brush-like” appearance of calyces on KUB

21
Q

Nephrocalcinosis has many etiologies, including medullary sponge kidney. What are the other etiologies?

A
  1. Hyper PTH
  2. Distal RTA (type 1)
  3. Renal TB
  4. Papillary necrosis
  5. Hyperoxaluria
  6. Medullary sponge kidney
22
Q

Multicystic dysplastic kidney is a functional/non-functional kidney?

A

non-functional; renal maldevelopment causing many cortical cysts and absent calyceal system

23
Q

MCKD is associated with increased what in the contralateral, healthy kidney?

A
  1. Vesicoureteral reflux, **therefore recommend screening with VCUG
  2. UPJO
24
Q

MCKD is associated with normal/abnormal renal function in an infant?

A

Normal. The contralateral kidney hypertrophys

25
Q

What is the treatment for MCKD?

A

None usually; the kidney will likely involute.

*Nephrectomy if uncontrolled HTN, pain, or SOB due to diaphragm irritation

26
Q

Oncocytomas are derived from which part of the nephron?

Which subtype of RCC is also derived from the same part, and therefore has similar histologic findings?

A

Distal tubules

*Chromophobe RCC also originates from distal tubule

27
Q

Can you radiographically distinguish oncocytoma from RCC?

A

No. Therefore, treat it as a RCC

28
Q

What syndrome is associated with increased development of oncocytomas and chromophobe RCCs?

Hint: also see cutaneous fibrofolliculomas and pulmonary cysts

A

Birt-Hogg-Dube syndrome

29
Q

Definitive treatment of an oncocytoma?

A

Since you don’t know it’s oncocytoma until its taken out….

Partial/radical nephrectomy or thermal ablation

30
Q

What is the benign tumor derived from perivascular epitheliod cells and has adipose, smooth muscle, and blood vessels?

A

Angiomyolipoma

31
Q

AMLs have strong male/female predilection?

A

Female (80%)

32
Q

What is the variant of AML that has a paucity of fat and about 1/3 malignant potential?

A

Epitheliod

33
Q

What is the imaging finding for AML that is pathognomonic?

A

Negative HU’s on CT

34
Q

AML has a histologic stain of _____, which melanoma is also positive for.

A

HMB-45 positive

35
Q

What is Wunderlich syndrome?

A

massive retroperitoneal hemorrhage of an AML

- occurs in 10% of AMLs and more likely in pregnancy

36
Q

Lenk’s Triad of Wunderlich syndrome

A

acute flank pain
palpable tender abdominal mass
symptoms of internal bleeding (tachycardia, hypoT, shock)

37
Q

The eponyms for flank ecchymosis and umbilical ecchymosis?

A

flank ecchymosis = Grey Turner sign

umbilical ecchymosis = Cullen’s sign

38
Q

What are the 3 MC etiologies of retroperitoneal hemorrhage?

A
  1. AAA
  2. RCC
  3. AML
39
Q

Treatment options for AML?

A
  1. None if small, asymptomatic
    * *2. If > 4cm, then partial/radical nephrectomy or embolization (due to risk of rupture)
  2. If epitheliod variant, treat as RCC (surveillance vs. remove)
40
Q

What syndrome has a strong association of AML’s?

Hint: also see sebaceous adenoma, seizures, mental retardation?

A

Tuberous sclerosis

“zits, fits, deficits”, and AMLs

41
Q

Tuberous sclerosis is Auto dominant or Auto recessive?

A

AD

42
Q

Mutations for TS?

A

9q34 (TSC1)

16p14 (TSC2)

43
Q

AML’s in a pt with TS behave differently how?

A

They grow faster

- 5% annual normally; in TS, 20% annual growth rate

44
Q

What chemotherapy drug is indicated in patients with TS and have AMLs?

A

Everolimus (mTOR inhibitor)

- also treats the subependymal giant cell astrocytoma (SEGA) seen in TS and also help with the increased risk of RCC

45
Q

This benign tumor is a Bosniak 3-4 cystic renal mass and is seen in perimenopausal women as it is related to estrogen therapy.

What does it stain positive for?

Treatment

A

Mixed Epithelial Stromal Tumor of the Kidney (MESTK)

Stains positive for ESTrogen/progesterone receptors

Treatment is partial/radical nephrectomy (Bosniak 3-4)

46
Q

What differentiates between a benign papillary adenoma and a papillary RCC?

A

Size. Adenoma MUST be < 5mm; if > 5mm then its papillary RCC

47
Q

Treatment of papillary adenoma?

A

Remove or ablate

48
Q

Mutation seen in papillary adenoma

A

same as in papillary RCC, trisomy of chromo 7 and 17