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Pathology > Childhood diseases III > Flashcards

Childhood diseases III Flashcards

1
Q

What are the four major types of fibrous tumors in infancy? Which one is malignant?

A
  1. Infantile myofibromatosis
  2. Aggressive infantile fibromatosis
  3. Infantile digital fibroma
  4. Congenital infantile fibrosarcoma = malignant
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2
Q

Can behavior of a tumor be predicted on histology alone?

A

no

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3
Q

What is the most common fibrous tumor in infants?

A

Infantile myofibromatosis

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4
Q

What are the histologic characteristics of of infantile myofibromatosis? What marker do they have with immunochemistry?

A

Non-pleomorphic, spindle shaped

Muscle specific actin

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5
Q

What distinguishes a benign infantile fibromatosis from an aggressive one?

A

RBC infiltration

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6
Q

What are the histologic characteristics of a malignant fibrosarcoma?

A

High grade atypia and high mitotic index

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7
Q

What is the incidence of teratomas

A

1/20,000-40,000

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8
Q

What is the source of a teratoma?

A

Single, polypotent cell

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9
Q

What is the most common SOILD tumor (malignant or not) of childhood?

A

Teratoma

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10
Q

Most teratoma are where? In which gender?

A

Sacrococcygeal in girls

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11
Q

What percent of teratomas are associated with congenital malformations?

A

10%

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12
Q

What is the presentation of myofibromatosis?

A

Benign fibrous tumors in which the cells express muscle specific actin

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13
Q

What percent of teratomas are malignant

A

12%

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14
Q

What are the histologic characteristics of teratomas? (3)

A

Epithelial cells, (endoderm) fibroblasts, and cartilage (mesoderm) all in a teratoma

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15
Q

Teratoma are from how many germ layers? (see slide)

A

2 or 3

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16
Q

What is the prognosis for teratomas?

A

Malignancy in 12%

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17
Q

What are the origins of most childhood malignancies? (3)

A

Hematopoietic
Nervous
Renal/adrenal

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18
Q

Malignancy occur in what percent of teratoma with immature tissue microscopically?

A

10-12%

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19
Q

Why do childhood cancers have a better prognosis than adult cancers?

A

Chance of spontaneous regression

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20
Q

What are the complications of teratomas?

A

Depends on location

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21
Q

What is the most common fibrous tumor in infants?

A

Infantile myofibromatosis

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22
Q

Childhood malignancies have a relationship with what?

A

Developmental abberations

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23
Q

What is increased in the blood/urine of infants with neuroblastomas?

A

Increase catecholamines (VMA and HVA)

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24
Q

Neuroblastomas are from what cells?

A

Primitive sympathetic cells/adrenal medulla

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25
Q

What are the three histological characteristics of neuroblastomas?

A
  1. Small blue round cells w/ little cytoplasm
  2. Rosette structure
  3. Dense neurosecretory granules (under EM)
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26
Q

What percent of all childhood CAs are neuroblastomas?

A

10%

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27
Q

How can you see the dense neurosecretory granules in neuroblastoma? What are contained within these?

A

Electronic microscope

Catecholamines

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28
Q

What is Wilms tumor?

A

most common primary malignant tumor of the kidney in children

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29
Q

What is the most common fibrous tumor in infants?

A

Infantile myofibromatosis

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30
Q

What are the histologic characteristics of of infantile myofibromatosis? What marker do they have with immunochemistry?

A

Non-pleomorphic, spindle shaped

Muscle specific actin

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31
Q

What is increased in the blood/urine of infants with neuroblastomas?

A

Increase catecholamines

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32
Q

What distinguishes a benign infantile fibromatosis from an aggressive one?

A

RBC infiltration

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33
Q

What are the histological characteristics of a Wilms tumor?

A

Tightly packed blue cells with the blastemal component and interspersed primitive tubules

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34
Q

What happens to the adrenal glands in neuroblastomas?

A

Hyperplasia

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35
Q

What are the three histological characteristics of neuroblastomas?

A
  1. Small blue round cells
  2. Rosette structure
  3. Dense neurosecretory granules
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36
Q

What are the histologic characteristics of a fibrosarcoma?

A

High grade atypia and high mitotic index

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37
Q

What is the rosette structure?

A

Round structures histologically

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38
Q

What is the prognosis for Wilms tumor? When is it really bad?

A

Very good with nephrectomy and chemo

bad if metastases or diffuse anaplasia

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39
Q

How can you seen the dense neurosecretory granules in neuroblastoma? What are contained within these?

A

Electronic microscope

Catecholamines

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40
Q

What is Wilms tumor associated with?

A

Mutations and congenital malformations

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41
Q

What do you have to differentiate between if you see an infant with CA causing a distended abdomen? How?

A

Neuroblastoma vs Wilms tumor

Biopsy with NSE = neuroblastoma.
Small round nuclei = neuroblastoma

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42
Q

What is it important to check the contralateral kidney with Wilms tumor?

A

Don’t’ excise it if their other kidney does not work

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43
Q

What are the gross characteristics of a Wilms tumor?

A

tan to gray color, with a well circumscribed margin

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44
Q

What are the histological characteristics of a Wilms tumor?

A

Tightly packed blue cells with the blastemal component and interspersed primitive tubules

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45
Q

How do you differentiate between Rosette structures with Wilm tumor?

A

No small round cells in Wilm tumor

46
Q

Most teratoma are where? In which gender?

A

Sacrococcygeal in girls

47
Q

What is the most common cause of a **solid tumor ** (benign or malignant) in infants? Most common Malignancy?

A

Tumor = Sacrococcygeal teratoma

Malignancy = neuroblastoma

48
Q

What are the most common sites of rhabdomyosarcomas?

A

Head and neck

GU tract

49
Q

What is Sarcoma botryoides?

A

A subtype of embryonal rhabdomyosarcoma that develops in walls of hollow, mucosal lined structures such as the nasopharynx. This has the best prognosis of all rhabdomyosarcomas.

50
Q

Malignancy occur in what percent of teratoma with immature tissue microscopically?

A

10-12%

51
Q

Childhood malignancies have a chance of doing what?

A

Spontaneous Regression

52
Q

What are the complications of teratomas?

A

Depends on location

53
Q

Childhood malignancy is the (BLANK) leading cause of death in age 5-14?

A

second

54
Q

Childhood malignancies have a relationship with what?

A

Developmental abberations

55
Q

What is increased in the blood/urine of infants with neuroblastomas?

A

Increase catecholamines (VMA and HVA)

56
Q

What is the tissue marker for neuroblastoma in the blood?

A

Neuron specific enolase (NSE)

57
Q

Neuroblastomas are from what cells?

A

Primitive sympathetic cells/adrenal medulla

58
Q

What are the three histological characteristics of neuroblastomas?

A
  1. Small blue round cells w/ little cytoplasm
  2. Rosette structure
  3. Dense neurosecretory granules (under EM)
59
Q

How can you see the dense neurosecretory granules in neuroblastoma? What are contained within these?

A

Electronic microscope

Catecholamines

60
Q

What is Wilms tumor?

A

most common primary malignant tumor of the kidney in children

61
Q

How do neuroblastomas present? (4)

A

Abdominal mass
Weight loss
Proptosis
Periorbital ecchymosis

62
Q 
IN an infant:
Abdominal mass
Diarrhea
Weight loss
Proptosis
Periorbital ecchymosis = ?
A

Neuroblastoma

63
Q

What is increased in the blood/urine of infants with neuroblastomas?

A

Increase catecholamines

64
Q

What is the tissue marker for neuroblastoma?

A

Neuron specific enolase

65
Q

Blueberry muffin baby = ?

A

Neuroblastoma

66
Q

What are the histological characteristics of a Wilms tumor?

A

Tightly packed blue cells with the blastemal component and interspersed primitive tubules

67
Q

What happens to the adrenal glands in neuroblastomas?

A

Hyperplasia

68
Q

What are the three histological characteristics of neuroblastomas?

A
  1. Small blue round cells
  2. Rosette structure
  3. Dense neurosecretory granules
69
Q

What is WAGR syndrome? What causes it?

A

Aniridia
MR

Germline deletion 11p13 in WT1

70
Q
  1. Small blue round cells
  2. Rosette structure
  3. Dense neurosecretory granules

= ?

A

Neuroblastoma

71
Q

What is the rosette structure?

A

Round structures histologically

72
Q

What is the prognosis for Wilms tumor? When is it really bad?

A

Very good with nephrectomy and chemo

bad if metastases or diffuse anaplasia

73
Q

How can you seen the dense neurosecretory granules in neuroblastoma? What are contained within these?

A

Electronic microscope

Catecholamines

74
Q

What is Wilms tumor associated with?

A

Mutations and congenital malformations

75
Q

What are the three subtypes of rhabdomyosarcomas? Which has the worst prognosis? Best?

A

Embryonal rhabdomyosarcomas (60% of cases) = best

Alveolar rhabdomyosarcomas

Pleomorphic rhabdomyosarcomas = worst

76
Q

What is the gene that is mutated in Wilms tumor?

A

WT1

77
Q

What is the clinical presentation of Wilms tumor?

A

Abdominal mass
Hematuria
HTN

78
Q

What are the two congential malformations associated with Wilms syndrome?

A
  1. WAGR syndrome

2. Denys-drash syndrome

79
Q

What is Denys-drash syndrome? What genetic mutation causes it?

A

Wilm’s tumor +
Nephropathy
Gonadal dysgenesis

WT1 mutation

80
Q

What percent of Wilms tumors have denys-drash syndrome? WAGR syndrome?

A

90%

33 %

81
Q

What is the marker for NTDs?

A

Alpha Fetoprotein (AFP)

82
Q

What is the prognosis for Wilms tumor? When is it really bad?

A

Very good with nephrectomy and chemo

bad if metastases

83
Q

How do you differentiate between Rosette structures with Wilm tumor?

A

No small round cells in Wilm tumor

84
Q

What is the most common SARCOMA of childhood?

A

rhabdomyosarcomas

85
Q

What are the two congential malformations associated with Wilms syndrome?

A
  1. WAGR syndrome

2. Denys-drash syndrome

86
Q

What are rhabdomyosarcomas?

A

a type of cancer, specifically a sarcoma (cancer of connective tissues), in which the cancer cells are thought to arise from skeletal muscle progenitors. It can also be found attached to muscle tissue, wrapped around intestines, or in any anatomic location. It mostly occurs in areas naturally lacking in skeletal muscle, such as the head, neck, and genitourinary tract.

87
Q

What is the most common sarcoma of childhood?

A

rhabdomyosarcomas

88
Q

What are the three types of rhabdomyosarcomas? Which has the worst prognosis? Best?

A

Embryonal rhabdomyosarcomas = best

Alveolar rhabdomyosarcomas

Pleomorphic rhabdomyosarcomas = worst

89
Q

What are the histological characteristics of an embryonal rhabdomyosarcomas?

A

Malignant cells ranging from primitive and round to spindled and eosinophilic

90
Q

What are the histological characteristics of alveolar rhabdomyosarcomas?

A

tumor is traverse by a network of fibrous septae

Cells in the center are discohesive, while those are the periphery adhere to the septae

Tumor cells are uniform round with little cytoplasm

91
Q

What are the histological characteristics of pleomorphic rhabdomyosarcomas?

A

Numerous large, sometimes multinucleated bizarre eosinophilic tumor cells.

92
Q

What is the marker for rhabdomyocytes?

A

Myogenin

93
Q

What is the treatment for rhabdomyosarcomas?

A

Surgery + chemo/radiation

94
Q

What percent of children diagnosed with rhabdomyosarcomas survive 5 years?

A

50%

95
Q

Fetal EtOH syndrome (FAS) = what 5 signs?

A
  1. MR
  2. Microcephaly
  3. Short palpebral fissures
  4. Maxillary hypoplasia
  5. ASDs
96
Q

Can FAS have epicanthal folds?

A

Yes, just like Down

97
Q

What are neural tube defects?

A

AN opening in the spinal cord or brain that occurs very early in human development

98
Q

What vit deficiency is associated with NTDs?

A

Folic acid

99
Q

What is the marker for NTDs?

A

AFP

100
Q

What is the indicator of fetal lung maturity?

A

Surfactant (lecithin-sphingomyelin ratio)

101
Q

What are the three types of teratomas?

A

(1) benign teratomas containing well-differentiated, adult tissue
(2) immature teratomas containing embryonic tissue that is not frankly malignant
(3) malignant teratomas.

102
Q

True or false: Malignant tumors in children tend to regress, differentiate, and respond better to treatment

A

True

103
Q

What are neuroblastomas?

A

A malignant tumor arising from primitive sympathetic cells

104
Q

What is the most important clinical marker for neuroblastoma?

A

N-myc amplification

105
Q

What are the two catecholamine derivatives, and one other cytokine that are elevated in Neuroblastoma?

A

homovanillic acid [HVA]
vanillylmandelic acid [VMA])
serum neuron-specific enolase

106
Q

What is stage 1, 2A, and 2B of neuroblastoma?

A

The tumor is localized, and contralateral lymph nodes are not invaded

107
Q

What is stage 4S of Neuroblastoma? Prognosis?

A

defined as a small primary tumor in the abdomen or thoracic cavity, with metastasis in the liver or bone marrow and skin (or both) (not in the cortical bone), in infants

108
Q

Wilms tumor is frequently associated with what?

A

beta-catenin mutations

109
Q

What are the clinical features of Wilm’s tumor? Prognosis?

A

Abdominal enlargement, mass, pain.

Prognosis depends on the histology of the tumor, but generally good

110
Q

What is the most common soft tissue sarcoma in children?

A

Rhabdomyosarcoma

111
Q

What is sarcoma botryoides? Prognosis?

A

A subtype of embryonal rhabdomyosarcoma–best prognosis of all the rhabdomyosarcomas

112
Q

What is the cell marker (stain) for rhabdomyosarcoma?

A

myogenin

Pathology (32 decks)

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