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Nelson - Cancer and Benign Tumors > Ch. 502 - Retinoblastoma & Ch. 505.1 - Hemangiomas > Flashcards

Ch. 502 - Retinoblastoma & Ch. 505.1 - Hemangiomas Flashcards

1
Q

The most common benign tumors of infancy

A

Hemangiomas

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2
Q

Hmeangiomas occur in approx ___% of term infants

A

5-10

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3
Q

Risk of hemangioma is 3-5x higher in boys vs girls

A

Girls

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4
Q

The risk for hemangiomas is doubled in term vs preterm

A

Preterm

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5
Q

Risk for hemangioma is higher in offspring of women who had

A

Chorionic villus sampling

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6
Q

Hemangiomas grow rapidly within ___ year/s

A

1

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7
Q

Slowing of growth of hemangioma is noted within

A

Next 5 years after year 1

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8
Q

Involution of hemangioma takes place at ___ y/o

A

10-15

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9
Q

More than 50% of all hemangiomas are located in the

A

Head and neck

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10
Q

T/F Most hemangiomas are solitary

A

T

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11
Q

Presence of more than 1 hemangioma increases the likelihood of

A

Visceral hemangiomas

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12
Q

Primary site of visceral involvement of hemangiomas

A

Liver

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13
Q

Other sites of visceral hemangiomas besides the liver

A

Brain, intestine, lung

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14
Q

Infantile hemangiomas can be diff. from other lesions by the expression of

A

GLUT1

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15
Q

T/F Most hemangiomas require no therapy

A

T

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16
Q

Common complication of hemangioma that leads to secondary infection

A

Ulceration

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17
Q

Large hepatic hemangiomas or hemangioendotheliomas may result in

A

Hepatomegaly, anemia, thrombocytopenia, and high-output heart failure

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18
Q

Characterized by rapidly enlarging lesion, thrombocytopenia, microangiopathic haemolytic anemia, and coagulopathy as a result of platelet andRBC trapping and activation of the clotting system within the vasculature of the hemangioma

A

Kasabach-Merritt Syndrome

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19
Q

T/F Kasabach-Merritt syndrome is associated with infantile hemangiomas but not with kaposiform hemangioendotheliomas

A

F, other way around

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20
Q

T/F Midline hemangioma in the lumbosacral area indicates the need for an MRI to search for underlying asymptomatic neurologic abnormalities

A

T

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21
Q

MC intraocular tumor in children

A

Retinoblastoma (Rb)

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22
Q

Rb progresses to metastatic disease and death in ___% of children worldwide

A

> 50

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23
Q

Rb accounts for ___% of all pediatric malignancies

A

4

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24
Q

Median age of diagnosis of Rb

A

2 y/o

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25
Q

More than 90% of Rb cases are diagnosed in children by what age

A

Younger than 5 y/o

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26
Q

T/F Majority of Rb are unilateral

A

T, 66-75%

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27
Q

T/F Bilateral Rb is more common in older children

A

F, younger particularly less than 1 y/o

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28
Q

T/F Bilateral Rb is always heritable

A

T

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29
Q

T/F There is a possible risk of Rb in children conceived by in vitro fertilization

A

T

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30
Q

Hereditary vs Sporadic: Diagnosed at a younger age

A

Hereditary

31
Q

Hereditary vs Sporadic: Multifocal

A

Hereditary

32
Q

Hereditary vs Sporadic: Unilateral

A

Sporadic

33
Q

Hereditary vs Sporadic: Unifocal

A

Sporadic

34
Q

Hereditary vs Sporadic: Loss of function of RB1 gene

A

Both

35
Q

Hereditary vs Sporadic: 2 mutational events

A

Both

36
Q

Hereditary vs Sporadic: First mutation through germinal cells then second mutation subsequently in somatic retinal cells

A

Hereditary

37
Q

Hereditary vs Sporadic: 2 mutations occur in somatic retinal cells

A

Sporadic

38
Q

RB1 gene is located on what chromosome

A

13q14

39
Q

RB1 gene encodes for

A

Retinoblastoma protein, a tumor suppressor protein

40
Q

Model of oncogenesis in which 2 mutational events are required for tumor development (as in Rb)

A

Knudson’s “two-hit” model of oncogenesis

41
Q

T/F All first-degree relatives of children with known or suspected hereditary Rb should have retinal exam

A

T

42
Q

Cell formation found histologically in Rb tumors

A

Flexner-Wintersteiner rosettes

43
Q

Why Rb tumors have necrotic and calcified areas

A

Tend to outgrow their blood supply

44
Q

Endophytic vs exophytic Rb: Arise from the inner surface of the retina

A

Endophytic

45
Q

Endophytic vs exophytic Rb: Grow from the outer retinal layer

A

Exophytic

46
Q

Endophytic vs exophytic Rb: Can cause retinal detachment

A

Exophytic

47
Q

Endophytic vs exophytic Rb: Vitreous seeding

A

Endophytic

48
Q

Type of Rb tumor that grows intraretinally and remain flat

A

Diffuse infiltrating type

49
Q

Type of Rb tumor that can cause iris neovascularization

A

Diffuse infiltrating type

50
Q

T/F Rb tumors can be both endophytic and exophytic

A

T

51
Q

Means by which Rb tumors can spread

A

Direct extension, hematogenous, lymphatic

52
Q

Route by which Rb can spread to the CNS

A

Choroid > optic nerve > lamia cribrosa > CNS

53
Q

Rb classically presents with ___

A

Leukocoria

54
Q

Often the initial presenting complaint in patients with Rb

A

Strabismus

55
Q

T/F Majority of Rb can be detected by routine ophtha screening in the context of a positive family history

A

F, only approx 10%

56
Q

Characteristic ophtha finding in Rb

A

Chalky, white-gray retinal mass with a soft friable consistency

57
Q

T/F Imaging studies are diagnostic for Rb

A

F

58
Q

T/F Biopsy is done to confirm Rb

A

F, contraindicated!

59
Q

Rb disease in which both eyes and the pineal region is involved

A

Trilateral Rb

60
Q

Trilateral Rb is seen in ~___% of patients with hereditary Rb

A

60

61
Q

T/F Metastatic disease is often present at diagnosis

A

F, rarely present

62
Q

Treatment of Rb is determined by

A

Size and location of tumors

63
Q

Primary goal of Rb treatment

A

CURE

64
Q

Secondary goal of Rb treatment

A

Preserving vision and the eye itself; decreasing risk of late side effects (mainly secondary malignancies)

65
Q

Indication for enucleation in Rb

A

No potential for salvage of useful vision (unresponsive and/or recurrent)

66
Q

Treatment for bilateral Rb

A

Chemoreduction + focal therapy (laser photocoagulation or cryotherapy

67
Q

Treatment for large Rbs

A

Multiagent therapy, VEC (vinc, etop, carboplatin)

68
Q

Treatment for large Rb if multi agent chemo fails

A

External-beam irradiation

69
Q

T/F In general, Rb has poor prognosis

A

F, poor only if Rb has spread outside the eye

70
Q

Routine ophtha exam in children with Rb should continue until

A

Older than 7 y/o

71
Q

T/F Trilateral Rb is universally fatal

A

T

72
Q

Germline RB1 mutations are at significant risk for development of second malignancies, especially

A

1) Osteosarcoma 2) Soft tissue sarcomas 3) Malignant melaoma

73
Q

Factors that increase risk of second malignancy in Rb

A

1) Germline RB1 mutation 2) Radiation therapy

74
Q

Adverse of effects of radiation therapy in Rb

A

1) Second malignancy 2) Cataracts 3) Orbital growth deformities 4) Lacrimal dysfunction 5) Late retinal vascular injury

Nelson - Cancer and Benign Tumors (1 decks)

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