118 - Vasular Tumors Flashcards
(102 cards)
1
Q
Vascular anomalies are broadly divided into
A
Vascular malformations
Vascular tumors
2
Q
Errors of vascular morphogenesis
A
Vascular malformation
3
Q
Vascular tumors are subdivided into
A
Benign
Locally aggressive or borderline
Malignant
4
Q
Benign Vascular Tumors
A
Infantile hemangioma Congenital hemangioma Tufted hemangioma Spindle-cell hemangioma Epithelioid hemangioma Pyogenic granuloma
5
Q
Locally Aggressive Vascular Tumors
A
Kaposiform hemangioendothelioma Retiform hemangioendothelioma PILA, Dabska tumor Composite hemangioendothelioma Kaposi sarcoma
6
Q
Malignant Vascular Tumors
A
Angiosarcoma
Epithelioid hemangioendothelioma
7
Q
Simple Vascular Malformations
A
Capillary malformation Lymphatic malformation Venous malformation Arteriovenous malformation Arteriovenous fistula
8
Q
Combined Vascular Malformations
A
CVM, CLM
LVM, CLVM
CAVM
CLAVM
9
Q
Most common tumor of infancy
A
Infantile hemangioma
10
Q
Infantile hemangiomas are more common in (males/females)
A
Females
11
Q
Appears to be the most significant risk factor for IH development
A
Low birth weight (especially those weighing less than 2500g)
12
Q
Prenatal risk factors for IH development
A
Advanced maternal age (older than 30 years of age)
Preeclampsia
Placenta previa
Other placental anomalies
13
Q
80% of IHs have completed all growth by _____ of age
A
5 months
14
Q
The late proliferative stage of ongoing slower growth that occurs after peak rapid growth typically ends by _____ of age
A
9 months
15
Q
Deep IHs are more likely to have a (shorter/longer) proliferative phase
A
Longer
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
17
Q
Graying in IH is usually apparent by _____ of age
A
1 year
18
Q
More than 90% of IHs have completed involution by ______ of age
A
3.5 to 4 years
19
Q
(Superficial/Deep) IHs are more likely to develop residual skin changes following involution
A
Superficial
20
Q
Cutaneous IH classification based on tumor depth
A
Superficial
Deep
Combined/mixed
21
Q
Cutaneous IH classification based on distribution
A
Localized
Segmental
Indeterminate
Multifocal
22
Q
Most common of the morphologic subtypes of IH
A
Superficial
23
Q
Facial segmental IH is associated with risk of
A
PHACE syndrome
24
Q
PHACE/S syndrome
A
Posterior fossa brain malformations Hemangiomas of the face Arterial anomalies Cardiac anomalies Eye abnormalities Sternal defects
25
Segmental IHs on the lower body confer risk of
Myelopathy and genitourinary anomalies in LUMBAR syndrome
26
LUMBAR syndrome
```
Lower body hemangioma and other cutaneous defects
Urogenital anomalies
Ulceration
Myelopathy
Bony deformities
Anorectal malformations
Arterial anomalies
Renal anomalies
```
27
Multifocal IHs present with ______ or more IHs
6
28
Involvement of the following areas should alert clinicians to possible increased risk of complications
```
Central face (especially the nose and perioral skin)
Periocular area
Neck
Mandibular region
Perineum
```
29
Complications of IH
```
Ulceration
Severe bleeding
Scarring
Pain
Infection
Airway obstruction
Congestive heart failure
Visual compromise
```
30
Facial segmental IH patterns have been labeled as 4 segments
Frontotemporal (S1)
Maxillary (S2)
Mandibular (S3)
Frontonasal (S4)
31
Segments most highly associated with PHACE
Frontotemporal (S1)
| Mandibular (S3)
32
Most common extracutaneous features of PHACE
Congenital vascular anomalies
33
PELVIS syndrome
```
Perineal hemangioma
External genitalia malformations
Lipomyelomeningocele
Vesicorenal abnormalities
Imperforate anus
Skin tag
```
34
SACRAL syndrome
```
Spinal dysraphism
Anogenital anomalies
Cutaneous anomalies
Renal and urologic anomalies
Angioma of lumbosacral localization
```
35
Most commonly associated extracutaneous abnormality in segmental IHs on the lower body involving the perineum or lumbosacral area
Myelopathy, presenting as tethered cord or lipomyelo(meningo)cele
36
Infants with periocular hemangiomas are at risk for
Anisometropia
Amblyopia
Permanent visual loss
37
Most common ocular complication of periocular hemangiomas
Amblyopia
38
Most favorable prognostic sign to herald normal vision following involution is
Absence of asymmetrical refractive error
39
Beard area hemangiomas carry a 60% risk of causing
Symptomatic airway disease
40
Recommended for infants presenting with 5 or more cutaneous IHs
Screening hepatic and abdominal ultrasound
41
Most common extracutaneous site for IH
Liver
42
Focal hepatic hemagiomas are not true IHs, but are analogous to
RICH occurring in the liver
43
Diffuse infantile hepatic hemangiomas can cause
Abdominal compartment syndrome
| Severe hypothyroidism
44
Most common complication of IH
Ulceration
45
Sensitive marker of impending ulceration in IH
Early gray-white discoloration in patients younger than 3 months
46
Development of new blood vessels from existing blood vessels
Angiogenesis
47
De novo development of blood vessels
Vasculogenesis
48
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
Cardinal vein
49
Most reliable histologic marker of IH
| Red blood cell glucose transporter protein
GLUT1
50
GLUT1 expression is absent in the normal cutaneous vasculature but is found in
Placental blood vessels as well as in other so-called barrier tissues, such as the blood-brain barrier
51
Postulated to trigger neovasculogenesis in infants
Hypoxia
52
Hepatic hemangioma demonstrates high levels of _____ activity, which accelerates the degradation of thyroid hormone
Type III iodothyronine deiodinase
53
The most important factor to affect prognosis in complicated IH is
Timing of specialist referral for management
54
Only medication approved by the US FDA for the treatment of complicated IH
Propranolol
55
In _____, it is recommended to use the lowest effective dose, consider a slower dose titration, and to give the medication in 3 divided doses
PHACE
56
Common side effects of propranolol
Sleep disturbance
Acrocyanosis
Asymptomatic transient decrease in blood pressure or heart rate
57
Rarely observed potential important adverse effects of propranolol
```
Hypoglycemia
Symptomatic hypotension or bradycardia
Wheezing
Bronchospasm
Diarrhea
```
58
In determining a patient's candidacy for propranolol, family history must be obtained to ensure absence of
Congenital heart disease
Arrhythmia
Maternal connective tissue disease
59
Dosing of propranolol for IH
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
Main benefit of atenolol (beta1-selective antagonist)
Lack of bronchial reactivity
61
Advantage of nadolol (nonselective beta-antagonist)
Inability to cross the blood-brain barrier
62
Nonselective beta blocker available in an ophthalmic preparation approved for the treatment of pediatric glaucoma
Eight to 10 times more potent than propanolol
Topical timolol
63
Corticosteroid are most effective during the _____ phase of IH
Proliferative
64
Dosing of prednisone or prednisolone for IH
2 to 3 mg/kg/day typically for 4-8 weeks followed by a tapering of varying length
65
Short-term complications of systemic corticosteroids
```
Cushingoid facies
Personality changes (irritability/fussiness)
Gastric irritation
Fungal infection
Diminished gain of height and weight
```
66
Other complications of systemic corticosteroids
Hypertension
Steroid-induced myopathy
Immunosuppression
Transient adrenal insufficiency
67
Children taking more than _____ mg/kg/day of prednisone for longer than _____ days are considered to have a deficit in cell-mediated immunity
2
| 14
68
Reported adverse events of intralesional corticosteriods for periocular hemangiomas
Retinal artery embolization
| Blindness
69
PDL is primarily limited by its minimal depth of penetration (less than _____)
2 mm
70
Even earlier excision may be indicated in cases where clinical characteristics, such as _____ dictate that scarring will inevitably occur
Pedunculated IH
Severe, recalcitrant ulceration
Extremely thick dermal involvement
71
_____ years serve as a na appropriate threshold for decisions regarding surgery
Four
72
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
Rapidly involuting congenital hemangioma
73
Somatic activating missense mutations in the genes _____ are reported to cause RICH and NICH
GNAQ
| GNA11
74
Also called angioblastoma of Nakagawa
Tufted angioma
75
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
Tufted angioma
76
CD31+
| D2-40, LYVE1, PROX1 are partially positive
Tufted angioma
77
Kaposiform hemangioendothelioma does not occur in the
Liver
78
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
Kasabach-Merritt phenomenon
79
Kasabach-Merritt Phenomenon is a complication almost exclusively of
Tufted angioma
| Kaposiform hemangioendothelioma
80
Involvement of KHE across more than _____ anatomic region increases the risk of developing KMP
1
81
KHE presenting at a later age, with superficial tumors or tumors involving only bone, appears to have a (lower/higher) risk of KMP
Lower
82
Considered first-line treatment for KHE and are often used in combination with vincristine
Systemic corticosteroids
83
Rare benign vascular tumor most often seen with Maffucci syndrome
Spindle-cell hemangioma
84
Genetic mutation associated with Maffucci syndrome
IDH1
| IDH2
85
Nodular, dense, spindle cell proliferation associated with dilated dysplastic veins
Spindle-cell hemangioma
86
Solitary, red, rapidly growing papule or nodule, often with a subtle collarette of scale
Typical locations include the cheek and forehead
Pyogenic granuloma
87
Band-Aid disease - often develop an eroded surface, with subsequent bleeding which can be profuse
Pyogenic granuloma
88
Multiple cutaneous vascular papules and plaques that are usually congenital with development of new lesions over time
Multifocal lymphagioendotheliomatosis with thrombocytopenia
89
Most infants affected with _____ have intermittent thrombocytopenia and lesions in the GI tract, leading to GI bleeding
Multifocal lymphagioendotheliomatosis with thrombocytopenia
90
Thin-walled vessels, some hobnailed endothelial cells, and intraluminal papillary projections
Multifocal lymphagioendotheliomatosis with thrombocytopenia (similar to Dabska tumor)
91
Also known as Dabska tumor
Papillary intralymphatic angioendothelioma
92
Dermal nodule or a diffuse violaceous swelling on the head, neck, or extremities that is seen almost exclusively in the pediatric age group
Papillary intralymphatic angioendothelioma/Dabska tumor
93
Enlarged vessels with thick walls surrounded with lymphoid aggregates and smaller lymphatic vessules
Papillary intralymphatic angioendothelioma/Dabska tumor
94
The presence of _____ is the sine qua non of papillary intralymphatic angioendothelioma/Dabska tumor
"Hobnail" endothelial cells inside tumoral vessels
95
Occurs primarily in adults and has a significant overlap with PILA/Dabska tumor
Retiform hemangioendothelioma
96
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
Retiform hemangioendothelioma
97
Combination of benign and malignant vascular components
| Identification of at least 2 hemangioendothelioma variants is necessary to render this diagnosis
Composite hemangioendothelioma
98
Violaceous papule, often surrounded by a pale rim and peripheral ecchymotic halo, which fades with time
Targetoid hemosiderotic hemangioma
99
Dilated intraluminal papillary projections dissecting into collagen bundles in the subcutis
Extravasated erythrocytes and hemosiderin are present
Targetoid hemosiderotic hemangioma
100
Also called sudoriparous angioma
Congenital eccrine angiomatous hamartoma
101
Ill-defined plaques with increased lanugo hair and sweating at the site of the lesion
Congenital eccrine angiomatous hamartoma
102
Closely packed eccrine sweat glands associated with dilated capillaries, a few dysplastic venous channels, and a dense collagenous matrix
Congenital eccrine angiomatous hamartoma
