Chapter 20: Related Neoplasia Flashcards

1
Q

Which 3 ethnicities are most commonly affected by Wilms Tumor?

A

Asians > Whites > Blacks

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2
Q

How is Wilms tumor most often discovered?

A
  • As large abdominal mass by parent or doc
  • May exhibit pain, microscopic hematuria, and HTN
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3
Q

Why is the presence of nephrogenic rests in a child a significant finding?

A
  • Putative precursor lesions of Wilms Tumors
  • 25-40% of unilateral
  • 100% of bilateral arise from nephrogenic rests!
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4
Q

Between what ages is peak prevalence for Wilms Tumor?

A

2-5 yo

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5
Q

Which 3 groups of congenital malformation/familial syndromes have associated Wilms Tumor?

A
  1. WAGR –> Wilms tumor + Aniridia + Genital anomalies + Retardation
  2. Denys-Drash Syndrome (90% have Wilms)
  3. Beckwith-Wiedemann Syndrome
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6
Q

What is the most common way that Wilms Tumor is acquired?

A

90% are SPORADIC arising in previously healthy children w/ no congenital abnormalities

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7
Q

Which 2 congenital malformation syndromes associated with Wilms Tumors have a strong association with the WT1 gene?

A
  1. WAGR syndrome
  2. Denys-Drash syndrome
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8
Q

What is the classic histomorphology/pattern of Wilms Tumor?

A
  • Classic triphasic combination of:
    1) Blastemal cells
    2) Stromal cells
    3) Epithelial cells

*Mimics the germinal development of the normal kindey

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9
Q

Anaplasia in the histomorphology of Wilms Tumor tends to be associated with what mutations?

Resistance to?

A

p53 mutations w/ resistance to chemotherapy

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10
Q

What is the most common genitourinary malformation seen in affected males and females with WAGR syndrome?

A
  • Males = undescended testes
  • Females = streak gonads or uterine malformations
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11
Q

What is the characteristic glomerular lesion seen in patients with Denys-Drash syndrome?

A

Diffuse mesangial sclerosis

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12
Q

In addition to Wilms tumors, patients with Denys-Drash syndrome are at an increased risk for what other tumor?

A

Gonadoblastoma (germ cell tumor)

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13
Q

Beckwith-Wiedemann syndrome is associated with mutations of which chromosomal region and with what associated gene?

A

WT2 associated w/ IGF2

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14
Q

Beckwith-Wiedemann syndrome is characterized by what classic findings?

A
  • Enlargement of body organs (organomegaly)
  • Macroglossia
  • Hemihypertrophy
  • Omphalocele
  • Adrenal cytomegaly
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15
Q

What is the characteristic gross morphology of Wilms Tumors?

A
  • Well-circumscribed margins
  • Tan-to-gray color w/ foci of hemorrhage, cysts, and necrosis
  • Large, expansile tumor in lower pole of kidney
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16
Q

What is the most common primary renal tumor of childhood?

A

Wilms Tumor

17
Q

What is the most critical prognostic element (i.e., unfavorable vs. favorable histopathologic features) of Wilms Tumors?

A

Presence or absence of diffuse anaplasia

18
Q

Genetic mutations in which 2 genes may have a synergistic role in development of Wilms Tumors?

A

WT1 and β-catenin

19
Q

Why is the histological presence of schwannian stroma in a neuroblastoma important?

A

Associated w/ favorable prognosis

20
Q

Common sites for metastasis of Neuroblastoma?

A
  • Liver
  • Lungs
  • Bones and Bone Marrow
  • Periorbital region –> proptosis and ecchymosis
21
Q

In neonates, disseminated neuroblastomas may present with multiple cutaneous metastases causing a deep blue discoloration of the skin, known clinically as what?

A

“Blueberry muffin baby”

22
Q

In children, under 2 years of age, how do neuroblastomas typically present?

A
  • Large abdominal mass
  • Fever
  • Weight loss
23
Q

Which 4 stages are associated with favorable prognosis of Neuroblastomas?

A
  • Stage 1
  • Stage 2A
  • Stage 2B
  • Stage 4S
24
Q

How is the presence of TRKA expression vs. TRKB expression related to prognosis of Neuroblastomas?

A
  • Presence of TRKA expression = Favorable
  • Presence of TRKB expression = Unfavorable
25
Q

Which DNA ploidy of neuroblastomas is favorable and which is unfavorable for prognosis?

A
  • Hyperdiploid (whole chromosomal gains) = favorable
  • Near-diploid (segmental chromosomal losses) = unfavorable
26
Q

How is age of diagnosis related to prognosis in neuroblastoma?

A
  • <18 months old = favorable
  • >18 months old = unfavorable
27
Q

Amplification of which oncogene in neuroblastomas has possibly the most profound impact on prognosis?

A

MYCN

28
Q

Hemizygous loss of genetic material on which chromosome may be the most common deletion event in neuroblastomas?

A

Chromosome 11q