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Fitz 9th > 118 - Vasular Tumors > Flashcards

118 - Vasular Tumors Flashcards

1
Q

Vascular anomalies are broadly divided into

A

Vascular malformations

Vascular tumors

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2
Q

Errors of vascular morphogenesis

A

Vascular malformation

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3
Q

Vascular tumors are subdivided into

A

Benign
Locally aggressive or borderline
Malignant

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4
Q

Benign Vascular Tumors

A 
Infantile hemangioma
Congenital hemangioma
Tufted hemangioma
Spindle-cell hemangioma
Epithelioid hemangioma
Pyogenic granuloma
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5
Q

Locally Aggressive Vascular Tumors

A 
Kaposiform hemangioendothelioma
Retiform hemangioendothelioma
PILA, Dabska tumor
Composite hemangioendothelioma
Kaposi sarcoma
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6
Q

Malignant Vascular Tumors

A

Angiosarcoma

Epithelioid hemangioendothelioma

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7
Q

Simple Vascular Malformations

A 
Capillary malformation
Lymphatic malformation
Venous malformation
Arteriovenous malformation
Arteriovenous fistula
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8
Q

Combined Vascular Malformations

A

CVM, CLM
LVM, CLVM
CAVM
CLAVM

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9
Q

Most common tumor of infancy

A

Infantile hemangioma

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10
Q

Infantile hemangiomas are more common in (males/females)

A

Females

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11
Q

Appears to be the most significant risk factor for IH development

A

Low birth weight (especially those weighing less than 2500g)

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12
Q

Prenatal risk factors for IH development

A

Advanced maternal age (older than 30 years of age)
Preeclampsia
Placenta previa
Other placental anomalies

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13
Q

80% of IHs have completed all growth by _____ of age

A

5 months

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14
Q

The late proliferative stage of ongoing slower growth that occurs after peak rapid growth typically ends by _____ of age

A

9 months

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15
Q

Deep IHs are more likely to have a (shorter/longer) proliferative phase

A

Longer

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16
Q

Evidence of involution of IH, often referred to as _____, involves change to a dull red, then gray or milky-white color, followed by flattening and softening

A

Graying

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17
Q

Graying in IH is usually apparent by _____ of age

A

1 year

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18
Q

More than 90% of IHs have completed involution by ______ of age

A

3.5 to 4 years

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19
Q

(Superficial/Deep) IHs are more likely to develop residual skin changes following involution

A

Superficial

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20
Q

Cutaneous IH classification based on tumor depth

A

Superficial
Deep
Combined/mixed

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21
Q

Cutaneous IH classification based on distribution

A

Localized
Segmental
Indeterminate
Multifocal

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22
Q

Most common of the morphologic subtypes of IH

A

Superficial

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23
Q

Facial segmental IH is associated with risk of

A

PHACE syndrome

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24
Q

PHACE/S syndrome

A 
Posterior fossa brain malformations
Hemangiomas of the face
Arterial anomalies
Cardiac anomalies
Eye abnormalities
Sternal defects
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25
Q

Segmental IHs on the lower body confer risk of

A

Myelopathy and genitourinary anomalies in LUMBAR syndrome

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26
Q

LUMBAR syndrome

A 
Lower body hemangioma and other cutaneous defects
Urogenital anomalies
Ulceration
Myelopathy 
Bony deformities
Anorectal malformations
Arterial anomalies
Renal anomalies
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27
Q

Multifocal IHs present with ______ or more IHs

A

6

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28
Q

Involvement of the following areas should alert clinicians to possible increased risk of complications

A 
Central face (especially the nose and perioral skin)
Periocular area
Neck
Mandibular region
Perineum
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29
Q

Complications of IH

A 
Ulceration 
Severe bleeding
Scarring 
Pain
Infection
Airway obstruction
Congestive heart failure
Visual compromise
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30
Q

Facial segmental IH patterns have been labeled as 4 segments

A

Frontotemporal (S1)
Maxillary (S2)
Mandibular (S3)
Frontonasal (S4)

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31
Q

Segments most highly associated with PHACE

A

Frontotemporal (S1)

Mandibular (S3)

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32
Q

Most common extracutaneous features of PHACE

A

Congenital vascular anomalies

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33
Q

PELVIS syndrome

A 
Perineal hemangioma
External genitalia malformations
Lipomyelomeningocele
Vesicorenal abnormalities
Imperforate anus
Skin tag
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34
Q

SACRAL syndrome

A 
Spinal dysraphism
Anogenital anomalies
Cutaneous anomalies
Renal and urologic anomalies
Angioma of lumbosacral localization
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35
Q

Most commonly associated extracutaneous abnormality in segmental IHs on the lower body involving the perineum or lumbosacral area

A

Myelopathy, presenting as tethered cord or lipomyelo(meningo)cele

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36
Q

Infants with periocular hemangiomas are at risk for

A

Anisometropia
Amblyopia
Permanent visual loss

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37
Q

Most common ocular complication of periocular hemangiomas

A

Amblyopia

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38
Q

Most favorable prognostic sign to herald normal vision following involution is

A

Absence of asymmetrical refractive error

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39
Q

Beard area hemangiomas carry a 60% risk of causing

A

Symptomatic airway disease

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40
Q

Recommended for infants presenting with 5 or more cutaneous IHs

A

Screening hepatic and abdominal ultrasound

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41
Q

Most common extracutaneous site for IH

A

Liver

42
Q

Focal hepatic hemagiomas are not true IHs, but are analogous to

A

RICH occurring in the liver

43
Q

Diffuse infantile hepatic hemangiomas can cause

A

Abdominal compartment syndrome

Severe hypothyroidism

44
Q

Most common complication of IH

A

Ulceration

45
Q

Sensitive marker of impending ulceration in IH

A

Early gray-white discoloration in patients younger than 3 months

46
Q

Development of new blood vessels from existing blood vessels

A

Angiogenesis

47
Q

De novo development of blood vessels

A

Vasculogenesis

48
Q

Studies show IH to be if fetal, rather than maternal origin, and demonstrate immature mesenchymal features with similarities to the _____, an early embryologic vascular structure

A

Cardinal vein

49
Q

Most reliable histologic marker of IH

Red blood cell glucose transporter protein

A

GLUT1

50
Q

GLUT1 expression is absent in the normal cutaneous vasculature but is found in

A

Placental blood vessels as well as in other so-called barrier tissues, such as the blood-brain barrier

51
Q

Postulated to trigger neovasculogenesis in infants

A

Hypoxia

52
Q

Hepatic hemangioma demonstrates high levels of _____ activity, which accelerates the degradation of thyroid hormone

A

Type III iodothyronine deiodinase

53
Q

The most important factor to affect prognosis in complicated IH is

A

Timing of specialist referral for management

54
Q

Only medication approved by the US FDA for the treatment of complicated IH

A

Propranolol

55
Q

In _____, it is recommended to use the lowest effective dose, consider a slower dose titration, and to give the medication in 3 divided doses

A

PHACE

56
Q

Common side effects of propranolol

A

Sleep disturbance
Acrocyanosis
Asymptomatic transient decrease in blood pressure or heart rate

57
Q

Rarely observed potential important adverse effects of propranolol

A 
Hypoglycemia
Symptomatic hypotension or bradycardia
Wheezing
Bronchospasm
Diarrhea
58
Q

In determining a patient’s candidacy for propranolol, family history must be obtained to ensure absence of

A

Congenital heart disease
Arrhythmia
Maternal connective tissue disease

59
Q

Dosing of propranolol for IH

A

Start at 1 mg/kg/day in 2 divided doses

Increase by 0.5 mg/kg/day increments every 3 to 7 days to a target dose between 2 and 3 mg/kgday

60
Q

Main benefit of atenolol (beta1-selective antagonist)

A

Lack of bronchial reactivity

61
Q

Advantage of nadolol (nonselective beta-antagonist)

A

Inability to cross the blood-brain barrier

62
Q

Nonselective beta blocker available in an ophthalmic preparation approved for the treatment of pediatric glaucoma
Eight to 10 times more potent than propanolol

A

Topical timolol

63
Q

Corticosteroid are most effective during the _____ phase of IH

A

Proliferative

64
Q

Dosing of prednisone or prednisolone for IH

A

2 to 3 mg/kg/day typically for 4-8 weeks followed by a tapering of varying length

65
Q

Short-term complications of systemic corticosteroids

A 
Cushingoid facies
Personality changes (irritability/fussiness)
Gastric irritation
Fungal infection
Diminished gain of height and weight
66
Q

Other complications of systemic corticosteroids

A

Hypertension
Steroid-induced myopathy
Immunosuppression
Transient adrenal insufficiency

67
Q

Children taking more than _____ mg/kg/day of prednisone for longer than _____ days are considered to have a deficit in cell-mediated immunity

A

2

14

68
Q

Reported adverse events of intralesional corticosteriods for periocular hemangiomas

A

Retinal artery embolization

Blindness

69
Q

PDL is primarily limited by its minimal depth of penetration (less than _____)

A

2 mm

70
Q

Even earlier excision may be indicated in cases where clinical characteristics, such as _____ dictate that scarring will inevitably occur

A

Pedunculated IH
Severe, recalcitrant ulceration
Extremely thick dermal involvement

71
Q

_____ years serve as a na appropriate threshold for decisions regarding surgery

A

Four

72
Q

Raised, violaceous tumor with large, radiating veins or with overlying telangiectasia and a halo of pallor
Involute spontaneously by or before 14 months of age

A

Rapidly involuting congenital hemangioma

73
Q

Somatic activating missense mutations in the genes _____ are reported to cause RICH and NICH

A

GNAQ

GNA11

74
Q

Also called angioblastoma of Nakagawa

A

Tufted angioma

75
Q

Demonstrate vascular tufts of tightly packed capillaries, randomly dispersed throughout the dermis in a typical “cannonball distribution” with crescenteric spaces surrounding the vascular tufts, and lymphatic-like spaces within the tumor stroma

A

Tufted angioma

76
Q

CD31+

D2-40, LYVE1, PROX1 are partially positive

A

Tufted angioma

77
Q

Kaposiform hemangioendothelioma does not occur in the

A

Liver

78
Q

Presence of platelet trapping within a vascular tumor resulting in a profoundly severe thrombocytopenia (typically less than 30000/microliter) ad associated with microangiopathic hemolytic anemia and consumption of clotting factors resulting in low fibrinogen, elevated D-dimers, and varying degrees of decreased coagulation factors

A

Kasabach-Merritt phenomenon

79
Q

Kasabach-Merritt Phenomenon is a complication almost exclusively of

A

Tufted angioma

Kaposiform hemangioendothelioma

80
Q

Involvement of KHE across more than _____ anatomic region increases the risk of developing KMP

A

1

81
Q

KHE presenting at a later age, with superficial tumors or tumors involving only bone, appears to have a (lower/higher) risk of KMP

A

Lower

82
Q

Considered first-line treatment for KHE and are often used in combination with vincristine

A

Systemic corticosteroids

83
Q

Rare benign vascular tumor most often seen with Maffucci syndrome

A

Spindle-cell hemangioma

84
Q

Genetic mutation associated with Maffucci syndrome

A

IDH1

IDH2

85
Q

Nodular, dense, spindle cell proliferation associated with dilated dysplastic veins

A

Spindle-cell hemangioma

86
Q

Solitary, red, rapidly growing papule or nodule, often with a subtle collarette of scale
Typical locations include the cheek and forehead

A

Pyogenic granuloma

87
Q

Band-Aid disease - often develop an eroded surface, with subsequent bleeding which can be profuse

A

Pyogenic granuloma

88
Q

Multiple cutaneous vascular papules and plaques that are usually congenital with development of new lesions over time

A

Multifocal lymphagioendotheliomatosis with thrombocytopenia

89
Q

Most infants affected with _____ have intermittent thrombocytopenia and lesions in the GI tract, leading to GI bleeding

A

Multifocal lymphagioendotheliomatosis with thrombocytopenia

90
Q

Thin-walled vessels, some hobnailed endothelial cells, and intraluminal papillary projections

A

Multifocal lymphagioendotheliomatosis with thrombocytopenia (similar to Dabska tumor)

91
Q

Also known as Dabska tumor

A

Papillary intralymphatic angioendothelioma

92
Q

Dermal nodule or a diffuse violaceous swelling on the head, neck, or extremities that is seen almost exclusively in the pediatric age group

A

Papillary intralymphatic angioendothelioma/Dabska tumor

93
Q

Enlarged vessels with thick walls surrounded with lymphoid aggregates and smaller lymphatic vessules

A

Papillary intralymphatic angioendothelioma/Dabska tumor

94
Q

The presence of _____ is the sine qua non of papillary intralymphatic angioendothelioma/Dabska tumor

A

“Hobnail” endothelial cells inside tumoral vessels

95
Q

Occurs primarily in adults and has a significant overlap with PILA/Dabska tumor

A

Retiform hemangioendothelioma

96
Q

Retiform pattern of long, arborizing blood vessels lined by monomorphic hobnail endothelial cells
Prominent lymphocytic infiltrate with focal presence of hyaline collagenous core comprising papillae

A

Retiform hemangioendothelioma

97
Q

Combination of benign and malignant vascular components

Identification of at least 2 hemangioendothelioma variants is necessary to render this diagnosis

A

Composite hemangioendothelioma

98
Q

Violaceous papule, often surrounded by a pale rim and peripheral ecchymotic halo, which fades with time

A

Targetoid hemosiderotic hemangioma

99
Q

Dilated intraluminal papillary projections dissecting into collagen bundles in the subcutis
Extravasated erythrocytes and hemosiderin are present

A

Targetoid hemosiderotic hemangioma

100
Q

Also called sudoriparous angioma

A

Congenital eccrine angiomatous hamartoma

101
Q

Ill-defined plaques with increased lanugo hair and sweating at the site of the lesion

A

Congenital eccrine angiomatous hamartoma

102
Q

Closely packed eccrine sweat glands associated with dilated capillaries, a few dysplastic venous channels, and a dense collagenous matrix

A

Congenital eccrine angiomatous hamartoma

Fitz 9th (108 decks)

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