12.8 Renal Neoplasia Flashcards

1
Q

paraneoplastic syndrome

  • what is it
  • examples (4)
A

tumors that excrete hormones

  1. EPO
  2. Renin
  3. PTHrP
  4. ACTH
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1
Q

large, unilateral flank mass, hematuria, HTN

-think what

A

Wilm’s tumor

-HTN due to renin secretion

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2
Q

renal cell carcinoma:

-classic symptoms

A

Classic triad:

  1. hematuria
  2. flank pain
  3. palpable mass

however, only 10% does triad of symptoms actually happen

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3
Q

Wilms tumor

-classic symptoms

A
  1. large, unilateral palpable mass
  2. hematuria
  3. HTN (from renin secretion)
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4
Q

hematuria, palpable mass, flank pain

-think what

A

think RCC

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4
Q

Denys-Drash syndrome

what is it (3 things)

how related to kidney

assoc with what pathogenesis

A
  1. Wilms tumor,
  2. progressive glomerular disease
  3. male pseudohermaphroditism

assoc with WT1 mutations

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5
Q

RCC: what you see on:

gross exam

histology

A
  1. yellow mass
  2. clear cytoplasm (‘clear cell type’) is common
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6
Q

renal tumor in a child, think what?

A

Wilm’s tumor

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7
Q

Beckwith-Wiedemann syndrome

symptoms (4)

A
  1. Wilms tumor
  2. neonatal hypoglycemia
  3. muscular hemihypertrophy
  4. organomegaly (including tongue)

WT2 mutations

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8
Q

renal cell carcinoma

-in addition to classic symptoms, what additional ones to know? (4)

A

classic triad: palpable mass, flank pain, hematuria

  1. fever
  2. weight loss
  3. paraneoplastic syndrome! (eg EPO, renin, PTHrP, ACTH)
  4. left sided varicocele (remember anatomy)
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8
Q

RCC:

how do hereditary and sporadic types present differently?

A
  1. age: sporadic is adult males >60, hereditary is younger adults
  2. sporadic: usu unilateral, hereditary: often bilateral
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9
Q

WAGR syndrome

  • what is it
  • how related to kidney
  • associated with what pathogenesis
A
  • Wilms tumor
  • Aniridia–no iris
  • Genital abnormalities
  • Retardation (mental and motor)
  • assoc with deletion of WT1 tumor suppressor gene
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10
Q

why would varicocele present with RCC?

which side?

A

left side b/c spermatic vein drains into L renal vein

(RCC loves to invade renal vein)

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12
Q

Wilm’s tumor

-classic population

A

-most common malignant renal tumor in children

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14
Q

angiomyolipoma

  • what is it
  • what is the important association?
A
  • all in the name: hamartoma of blood vessels, muscles, and adipose tissue
  • assoc with tuberous sclerosis
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15
Q

RCC:

describe pathogenesis

A
  • loss of VHL (3p) tumor suppressor gene, leads to:
    1. increased IGF-1 (growth factor)
    2. increased HIF (hypoxia induceable factor) transcription factor (increases VEGF and PDGF)
17
Q

RCC:

describe types

A
  1. sporadic
    - classically in adult males >60, smoking is major risk factor, classically single tumor in upper pole of kidney
  2. hereditary
    - younger adults, often bilateral
    - Von Hippel Lindau disease is associated with VHL gene inactivation, increasing risk for RCC
18
Q

Wilm’s tumor

-what syndromes may you see it with? (3)

A
  1. WAGR syndrome
  2. Denys-Drash syndrome
  3. Beckwith-Wiedemann syndrome
19
Q

Wilm’s tumor

-made of what, mech

A

-made of blastema (immature kidney mesenchyme), primitive glomeruli and tubules, and stromal cells

20
Q

Von Hippel Lindau disease

  • how assoc with kidney?
  • what else increased risk?
A
  • increased risk of hereditary RCC
  • b/c VHL disease is inactivation of VHL gene.
  • also increased risk for hemangioblastoma of cerebellum