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Benign stuff Flashcards

(48 cards)

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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?

Study These Flashcards
A

“paint brush-like” appearance of calyces on KUB

21
Q

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

Study These Flashcards
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?

Study These Flashcards
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?

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

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

Study These Flashcards
A

Normal. The contralateral kidney hypertrophys

25
What is the treatment for MCKD?
None usually; the kidney will likely involute. *Nephrectomy if uncontrolled HTN, pain, or SOB due to diaphragm irritation
26
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?
Distal tubules *Chromophobe RCC also originates from distal tubule
27
Can you radiographically distinguish oncocytoma from RCC?
No. Therefore, treat it as a RCC
28
What syndrome is associated with increased development of oncocytomas and chromophobe RCCs? Hint: also see cutaneous fibrofolliculomas and pulmonary cysts
Birt-Hogg-Dube syndrome
29
Definitive treatment of an oncocytoma?
Since you don't know it's oncocytoma until its taken out.... Partial/radical nephrectomy or thermal ablation
30
What is the benign tumor derived from perivascular epitheliod cells and has adipose, smooth muscle, and blood vessels?
Angiomyolipoma
31
AMLs have strong male/female predilection?
Female (80%)
32
What is the variant of AML that has a paucity of fat and about 1/3 malignant potential?
Epitheliod
33
What is the imaging finding for AML that is pathognomonic?
Negative HU's on CT
34
AML has a histologic stain of _____, which melanoma is also positive for.
HMB-45 positive
35
What is Wunderlich syndrome?
massive retroperitoneal hemorrhage of an AML | - occurs in 10% of AMLs and more likely in pregnancy
36
Lenk's Triad of Wunderlich syndrome
acute flank pain palpable tender abdominal mass symptoms of internal bleeding (tachycardia, hypoT, shock)
37
The eponyms for flank ecchymosis and umbilical ecchymosis?
flank ecchymosis = Grey Turner sign umbilical ecchymosis = Cullen's sign
38
What are the 3 MC etiologies of retroperitoneal hemorrhage?
1. AAA 2. RCC 3. AML
39
Treatment options for AML?
1. None if small, asymptomatic * *2. If > 4cm, then partial/radical nephrectomy or embolization (due to risk of rupture) 3. If epitheliod variant, treat as RCC (surveillance vs. remove)
40
What syndrome has a strong association of AML's? Hint: also see sebaceous adenoma, seizures, mental retardation?
Tuberous sclerosis "zits, fits, deficits", and AMLs
41
Tuberous sclerosis is Auto dominant or Auto recessive?
AD
42
Mutations for TS?
9q34 (TSC1) 16p14 (TSC2)
43
AML's in a pt with TS behave differently how?
They grow faster | - 5% annual normally; in TS, 20% annual growth rate
44
What chemotherapy drug is indicated in patients with TS and have AMLs?
Everolimus (mTOR inhibitor) | - also treats the subependymal giant cell astrocytoma (SEGA) seen in TS and also help with the increased risk of RCC
45
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
Mixed Epithelial Stromal Tumor of the Kidney (MESTK) Stains positive for ESTrogen/progesterone receptors Treatment is partial/radical nephrectomy (Bosniak 3-4)
46
What differentiates between a benign papillary adenoma and a papillary RCC?
Size. Adenoma MUST be < 5mm; if > 5mm then its papillary RCC
47
Treatment of papillary adenoma?
Remove or ablate
48
Mutation seen in papillary adenoma
same as in papillary RCC, trisomy of chromo 7 and 17

Renal tumors (6 decks)

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