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Flashcards in Childhood diseases III Deck (112):
1

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

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

2

Can behavior of a tumor be predicted on histology alone?

no

3

What is the most common **fibrous** tumor in infants?

Infantile myofibromatosis

4

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

Non-pleomorphic, spindle shaped

Muscle specific actin

5

What distinguishes a benign infantile fibromatosis from an aggressive one?

RBC infiltration

6

What are the histologic characteristics of a malignant fibrosarcoma?

High grade atypia and high mitotic index

7

What is the incidence of teratomas

1/20,000-40,000

8

What is the source of a teratoma?

Single, polypotent cell

9

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

Teratoma

10

Most teratoma are where? In which gender?

Sacrococcygeal in girls

11

What percent of teratomas are associated with congenital malformations?

10%

12

What is the presentation of myofibromatosis?

Benign fibrous tumors in which the cells express muscle specific actin

13

What percent of teratomas are malignant

12%

14

What are the histologic characteristics of teratomas? (3)

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

15

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

2 or 3

16

What is the prognosis for teratomas?

Malignancy in 12%

17

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

Hematopoietic
Nervous
Renal/adrenal

18

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

10-12%

19

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

Chance of spontaneous regression

20

What are the complications of teratomas?

Depends on location

21

What is the most common **fibrous** tumor in infants?

Infantile myofibromatosis

22

Childhood malignancies have a relationship with what?

Developmental abberations

23

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

Increase catecholamines (VMA and HVA)

24

Neuroblastomas are from what cells?

Primitive sympathetic cells/adrenal medulla

25

What are the three histological characteristics of neuroblastomas?

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

26

What percent of all childhood CAs are neuroblastomas?

10%

27

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

Electronic microscope

Catecholamines

28

What is Wilms tumor?

most common primary malignant **tumor of the kidney** in children

29

What is the most common fibrous tumor in infants?

Infantile myofibromatosis

30

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

Non-pleomorphic, spindle shaped

Muscle specific actin

31

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

Increase catecholamines

32

What distinguishes a benign infantile fibromatosis from an aggressive one?

RBC infiltration

33

What are the histological characteristics of a Wilms tumor?

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

34

What happens to the adrenal glands in neuroblastomas?

Hyperplasia

35

What are the three histological characteristics of neuroblastomas?

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

36

What are the histologic characteristics of a fibrosarcoma?

High grade atypia and high mitotic index

37

What is the rosette structure?

Round structures histologically

38

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

Very good with nephrectomy and chemo

bad if metastases or diffuse anaplasia

39

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

Electronic microscope

Catecholamines

40

What is Wilms tumor associated with?

Mutations and congenital malformations

41

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

Neuroblastoma vs Wilms tumor

Biopsy with NSE = neuroblastoma.
Small round nuclei = neuroblastoma

42

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

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

43

What are the gross characteristics of a Wilms tumor?

tan to gray color, with a well circumscribed margin

44

What are the histological characteristics of a Wilms tumor?

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

45

How do you differentiate between Rosette structures with Wilm tumor?

No small round cells in Wilm tumor

46

Most teratoma are where? In which gender?

Sacrococcygeal in girls

47

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

Tumor = Sacrococcygeal teratoma

Malignancy = neuroblastoma

48

What are the most common sites of rhabdomyosarcomas?

Head and neck
GU tract

49

What is Sarcoma botryoides?

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

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

10-12%

51

Childhood malignancies have a chance of doing what?

Spontaneous Regression

52

What are the complications of teratomas?

Depends on location

53

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

second

54

Childhood malignancies have a relationship with what?

Developmental abberations

55

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

Increase catecholamines (VMA and HVA)

56

What is the tissue marker for neuroblastoma in the blood?

Neuron specific enolase (NSE)

57

Neuroblastomas are from what cells?

Primitive sympathetic cells/adrenal medulla

58

What are the three histological characteristics of neuroblastomas?

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

59

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

Electronic microscope

Catecholamines

60

What is Wilms tumor?

most common primary malignant **tumor of the kidney** in children

61

How do neuroblastomas present? (4)

**Abdominal mass**
**Weight loss**
Proptosis
Periorbital ecchymosis

62

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

Neuroblastoma

63

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

Increase catecholamines

64

What is the tissue marker for neuroblastoma?

Neuron specific enolase

65

Blueberry muffin baby = ?

Neuroblastoma

66

What are the histological characteristics of a Wilms tumor?

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

67

What happens to the adrenal glands in neuroblastomas?

Hyperplasia

68

What are the three histological characteristics of neuroblastomas?

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

69

What is WAGR syndrome? What causes it?

Aniridia
MR

**Germline deletion** 11p13 in WT1

70

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

= ?

Neuroblastoma

71

What is the rosette structure?

Round structures histologically

72

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

Very good with nephrectomy and chemo

bad if metastases or diffuse anaplasia

73

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

Electronic microscope

Catecholamines

74

What is Wilms tumor associated with?

Mutations and congenital malformations

75

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

Embryonal rhabdomyosarcomas (60% of cases) = best

Alveolar rhabdomyosarcomas

Pleomorphic rhabdomyosarcomas = worst

76

What is the gene that is mutated in Wilms tumor?

WT1

77

What is the clinical presentation of Wilms tumor?

Abdominal mass
Hematuria
HTN

78

What are the two congential malformations associated with Wilms syndrome?

1. WAGR syndrome
2. Denys-drash syndrome

79

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

Wilm's tumor +
Nephropathy
Gonadal dysgenesis

WT1 mutation

80

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

90%
33 %

81

What is the marker for NTDs?

Alpha Fetoprotein (AFP)

82

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

Very good with nephrectomy and chemo

bad if metastases

83

How do you differentiate between Rosette structures with Wilm tumor?

No small round cells in Wilm tumor

84

What is the most common SARCOMA of childhood?

rhabdomyosarcomas

85

What are the two congential malformations associated with Wilms syndrome?

1. WAGR syndrome
2. Denys-drash syndrome

86

What are rhabdomyosarcomas?

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

What is the most common sarcoma of childhood?

rhabdomyosarcomas

88

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

Embryonal rhabdomyosarcomas = best

Alveolar rhabdomyosarcomas

Pleomorphic rhabdomyosarcomas = worst

89

What are the histological characteristics of an embryonal rhabdomyosarcomas?

Malignant cells ranging from primitive and round to spindled and eosinophilic

90

What are the histological characteristics of alveolar rhabdomyosarcomas?

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

What are the histological characteristics of pleomorphic rhabdomyosarcomas?

Numerous large, sometimes multinucleated bizarre eosinophilic tumor cells.

92

What is the marker for rhabdomyocytes?

Myogenin

93

What is the treatment for rhabdomyosarcomas?

Surgery + chemo/radiation

94

What percent of children diagnosed with rhabdomyosarcomas survive 5 years?

50%

95

Fetal EtOH syndrome (FAS) = what 5 signs?

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

96

Can FAS have epicanthal folds?

Yes, just like Down

97

What are neural tube defects?

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

98

What vit deficiency is associated with NTDs?

Folic acid

99

What is the marker for NTDs?

AFP

100

What is the indicator of fetal lung maturity?

Surfactant (lecithin-sphingomyelin ratio)

101

What are the three types of teratomas?

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

102

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

True

103

What are neuroblastomas?

A malignant tumor arising from primitive sympathetic cells

104

What is the most important clinical marker for neuroblastoma?

N-myc amplification

105

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

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

106

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

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

107

What is stage 4S of Neuroblastoma? Prognosis?

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

Wilms tumor is frequently associated with what?

beta-catenin mutations

109

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

Abdominal enlargement, mass, pain.

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

110

What is the most common soft tissue sarcoma in children?

Rhabdomyosarcoma

111

What is sarcoma botryoides? Prognosis?

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

112

What is the cell marker (stain) for rhabdomyosarcoma?

myogenin