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MOD > Malignant Tumors of Infancy and Childhood (10) > Flashcards

Malignant Tumors of Infancy and Childhood (10) Flashcards

1
Q

2 common malignant neoplasms of childhood?

A

Neuroblastoma

Wilms Tumor

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2
Q

Where are Neuroblastomas found?

A

Sympathetic ganglia and Adrenal Medulla

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3
Q

Most common extracranial solid tumor of childhood?

A

Neuroblastomas

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4
Q

What are Neuroblastomas made of?

A

Primordial NCCs

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5
Q

Most Neuroblastomas are sporadic or familal?

A

Sporadic

- 1-2% are familial

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6
Q

Familal Neuroblastomas usually present how?

A

Involving BOTH adrenals or at MULTIPLE autonomic sites

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7
Q

Familial Neuroblastomas have a germline mutation in?

A

ALK gene (anaplastic Lymphoma Kinase)

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8
Q

ALK gene

A

Anaplastic Lymphoma Kinase gene

– Familial Neuroblastomas

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9
Q

With Neuroblastomas, an age younger than ____ has a more favorable outcome

A

18 months

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10
Q

Where are a majority of Neuroblastomas found?

A

Adrenal medulla

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11
Q

How do Neuroblastomas usually look?

A

In situ lesions = small

Soft, gray, tan, tissue

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12
Q

If a Neuroblastoma is an in situ lesion (small), when they regress what will be left behind?

A

Calcification of tissue

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13
Q

An Adrenal Medulla or sympathetic ganglia neoplasm?

A

Neuroblastoma

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14
Q

What are Homer-Wright Pseudorosettes and what are they seen with?

A

Tumor cells aligned in a circle with Neuropil in the middle

- Seen with neuroblastomas

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15
Q

What is neuropil?

A

Faint eosinophilic material seen in Neuroblastomas

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16
Q

What are Homer-Wright Pseudorosettes and what are they seen with?

A

Tumor cells aligned in a circle with Neuropil in the middle

- Seen with Neuroblastomas

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17
Q

If there is a presence of _____, neuroblastomas have a better outcome

A

Schwannian stroma

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18
Q

Symptoms of a Neuroblastoma if the child is less than 2 years old?

A

Abdominal mass
Fever
Weight loss maybe

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19
Q

Symptoms of a Neuroblastoma if the child is over 2 years old?

A

Bone pain
Respiratory issues
== METASTASIZED

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20
Q

Where are 2 common places for a Neuroblastoma to metastasize?

A
  1. Periorbital region - proptosis

2. Cutaneous - Blueberry muffin baby

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21
Q

Proptosis and Blueberry muffin baby signify?

A

Neuroblastoma that has metastasized

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22
Q

Regardless of location, what do Neuroblastomas produce?

A

Catecholamines!!

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23
Q

Elevated catecholamines can diagnose?

A

Neuroblastomas

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24
Q

With Neuroblastomas, there will be an increased in what excreted in the urine?

A

VMA

HVA

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25
Q

Neuroblastomas - early or late stage diagnoses are more common?

A

Late

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26
Q

Regardless of tumor stage, an age less than ____ has better outcomes for neuroblastomas

A

18 months

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27
Q

Amplification of ____ is associated with BAD outcomes for Neuroblastomas

A

MYC

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28
Q

On what chromosome is MYC?

29
Q

Increased MYC suggests?

A

Worse outcome for Neuroblastomas

30
Q

If a child is younger than 2 with a neuroblastoma, what can be used to estimate the outcome?

31
Q

What ploidy is associated with better outcomes of a child with Neuroblastoma that is less than 2 years old?

A

Hyperdiploid

32
Q

What can detect MYC amplification?

33
Q

Nephroblastoma

A

Wilms Tumor

34
Q

Between what ages are Wilms tumors the most common?

A

2-5 years old

35
Q

Most common RENAL tumor of childhood?

A

Wilms tumor

36
Q

Bilateral Wilms tumors are either?

A
  1. Synchronous - growing at same time

2. Metachronous - one after the other

37
Q

How is the age of onset of bilateral wilms tumors different than unilateral?

A

10 months earlier onset for bilateral tumors

38
Q

Bilateral Wilms tumors are assumed to have?

A

Germline mutation

39
Q

What percentage of Wilms tumors are sporadic?

40
Q

What 3 malformation and chromosome abnormalities are associated with Wilms tumors?

A
  1. WAGR syndrome
  2. Denys Drash Syndrome
  3. Beckwith Wiedmann Syndrome
41
Q

WAGR syndrome

A

Wilms tumors
Aniridia (no iris)
Genitourinary anomalies
Retardation

42
Q

WAGR syndrome has a germline mutation of?

A

Deletion of 11p13 chromsome

43
Q

What is the percentage risk of developing a wilms tumor with WAGR syndrome?

44
Q

What gene is knocked out with WAGR syndrome?

A

WT1 gene - 1st hit

45
Q

WAGR syndrome requires a second hit of the WT1 gene to develop a Wilms tumor. What type of mutation will do that?

A

Nonsense or Frameshift of the remaining WT1 allele

46
Q

Symptoms of WAGR syndrome?

A

Wilms tumor
Aniridia (no iris)
Genitourinary anomalies
Retardation

47
Q

What is the percentage risk of developing a wilms tumor with Denys Drash Syndrome?

48
Q

What are the symptoms with Denys Drash Sydrome?

A

Gonadal Dysgenesis (no male testicular development) and early renal failure

49
Q

What are the symptoms with Denys Drash Syndrome?

A

Gonadal Dysgenesis (no male testicular development) and early renal failure

50
Q

What mutation is seen with Denys Drash Syndrome that can lead to a wilms tumor?

A

Missense mutation in zinc finger region of WT1 that affects its DNA binding properties

51
Q

If only one mutation in the zinc finger region of WT1 is present with Denys Drash Syndrome, what will result?

A

Gonadal Dysgenesis

NEED 2 HITS on WT1

52
Q

Those with Denys Drash Syndrome have an increased risk of developing?

A

Gonadoblastoma

53
Q

What mutation is seen with Beckwith - Wiedmann Syndrome?

A

11p15.5 (WT2) - distal to WT1

54
Q

What are the symptoms with Beckwith - Wiedmann Syndrome?

A

Enlargement of organs, macroglossia (big tongue), hemihypertrophy (growth of one side of body), large cells in adrenal cortex

55
Q

What are the symptoms with Beckwith - Wiedmann Syndrome?

A

Enlargement of organs, Macroglossia (big tongue), hemihypertrophy (growth of one side of body), large cells in adrenal cortex

56
Q

What is overexpressed in Beckwith - Wiedmann Syndrome that may lead to development of a Wilms tumor?

57
Q

With sporadic cases of wilms tumors how do they present?

A

Abdominal mass, that may extend across midline and into pelvis, hematuria, pain, hypertension

58
Q

Abdominal mass, extends across midline, hematuria and hypertension in a 2-5 year old suggests?

A

Wilms tumor

59
Q

How do wilms tumors look?

A

Large, solitary, well circumscribed mass of tan and gray

60
Q

What 3 cell types are commonly found in wilms tumors?

A
  1. Blastemal
  2. Stromal
  3. Epithelial
61
Q

Blastemal cells in a wilms tumor?

A

Sheets of blue cells

62
Q

Epithelial cells in a wilms tumor?

63
Q

If a wilms tumor has anaplasia (large hyperchromatic cells), what does that suggest?

A

TP53 mutations

64
Q

If a wilms tumor has anaplasia, what does that mean they are likely unresponsive to?

A

CHEMO - TP53 mutation

65
Q

Nephrogenic rests

A

Precursor lesions of wilms tumors in renal parenchyma

66
Q

Precursor lesions of wilms tumors in renal parenchyma

A

Nephrogenic rests

67
Q

With bilateral wilms tumors, how often are nephrogenic rests present?

A

100% of the time

68
Q

Nephrogenic rests increase the risk for?

A

Wilms tumors

MOD (68 decks)

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