This document discusses the classification and characteristics of vascular tumors and malformations. It begins with an overview of modern classification systems developed in 1982 by the ISSVA, which use a binary system of vascular tumors (like hemangiomas) and malformations (like venous malformations). The document then goes into detail about different types of hemangiomas, vascular malformations, their presentation, diagnosis and treatment options.

2.  Introduction
 Classification
 Hemangioma vascular tumors
 Vascular malformations
12/20/2013 2

3.  Vascular nevi were labeled as angiomas and
were grouped together without clear
distinction between the different types
 Modern classification developed in 1982,
which got imprimatur of ISSVA afterwards
12/20/2013 3

4.  Binary classification
› Hemangiomas
› Vascular malformations
12/20/2013 4

5.  Hemangiomas
Common tumor of infancy that exhibits rapid post-
natal growth and slow regression during childhood
 Vascular malformations
Congenitally formed abnormal vascular channels
comprising quiescent endothelial cells and
characterized by their inability to regress
12/20/2013 5

6.  ISSVA classification
(International Society for the Study of
Vascular Anomalies)
Vascular Tumors
Vascular Malformations
12/20/2013 6

7.  ISSVA classification
12/20/2013 7
Tumours Malformations
Hemangioma Slow flow
Hemangioendothelioma • Capillary malformation
Angiosarcoma • Lymphatic malformation
Miscellaneous • Venous malformation
Fast flow
• Arteriovenous malformation
• Combined

9.  Consist of mature, differentiated endothelial
cells that behave aberrantly but without
dysplasia
 Characterized by rapid growth followed with
slow involution
 Are of different kinds
12/20/2013 9

10.  Appears in neonatalhood, commonly within
first 2 wks of life
 Types involving viscera and deeper
subcutaneous structures may not manifest
until 2-3 mo of life
 30-40% are nascent at birth
12/20/2013 10

11.  Epidemiologically…
› Occur in a 3-5:1 female: male ratio
› Present in 12% of Caucasian infants, with a
lower incidence in other races
› Up to 30% incidence in premature infants
› 20% are multi-focal
› 60% of these lesions are situated in the head
and neck area
12/20/2013 11

12.  Etiology
Hemangioma genesis begins from somatic
mutation of a single endothelial cell
These progenitor cells, whether are embolized
placental cells or immunophenotypic alteration of
primitive cells, leads to clonal expansion.
Expression of GLUT-1
12/20/2013 12

13.  Three phases:
1. Proliferating phase
2. Involuting phase
3. Involuted phase
12/20/2013 13

14.  Proliferating phase
› Rapid growth in first 6-8 mo
 Rapid division of pericytes and endothelial cells
› As the tumour permeates superficial dermis the
skin becomes raised, bosselated and a vivid
crimson colour.
› If the tumour proliferates in the lower dermis and
subcutis the overlying skin may be only slightly
raised and of a bluish hue.
12/20/2013 14

15.  Involuting phase
› Peak is before first year, later growth is
proportionate to the child
› Traces disappear by 5-7 years
12/20/2013 15

16.  Involuted phase
› Regression completed at age 5 year in 50% and
at age 7 year in 70%
› Normal skin restored in 50%
› Remnant marks include:
 Telangiectasia, yellowish hypoelastic patch,
fibrofatty residuum, laxity, scarring…
12/20/2013 16

17.  Skeletal alteration
› Rarely caused by hemangioma
› Cartilage/bone overgrowth on the face
12/20/2013 17

18.  Present fully grown at birth, no rapid
neonatal proliferation
 Similar to infantile type except negativity for
GLUT-1 staining
 Two types
1. Rapidly involuting (RICH)
2. Non-involuting (NICH)
12/20/2013 18

19.  Present at birth and regress completely
within 2 years
 There may be sufficient shunting to cause
high output congestive heart failure
12/20/2013 19

20.  Present at birth and demonstrate
proportional growth without regression
12/20/2013 20

21. 12/20/2013 21

22.  If multiple hemangioma ( >5 lesions) child is
at risk of harboring visceral ones
 Intra-hepatic hemangioma
› Triad of CHF, hepatomegaly, anemia
 Hemangiomatosis
› Involvement of 3 or more organ systems
12/20/2013 22

23.  Hepatic hemangioma occurs in 13% of
cutaneous lesions
12/20/2013 23

24.  Associated anomalies
› PHACES
(Posterior fossa, facial Hemangioma, Cardiac
defect, Eye anomaly, Sternal defect)
› Lumbosacral hemangioma
 Occult spinal dysraphism
› Perineum and lower limb involvement
 Urogenital and anorectal anomalies
12/20/2013 24

25.  Radiologic characteristics
› At proliferating phase remarkable shunting
pattern
› Gold standard is MRI, with contrast
12/20/2013 25

26.  Options are:
 Observation
 Local treatment for ulceration & bleeding
 Pharmacologic
 Surgical
12/20/2013 26

27.  Ulceration & bleeding
› In 5% cases, usually at lip and anogenital area
› Topical antibiotics
› Pressure for bleeding
› Debridement for superficial eschar
12/20/2013 27

28.  Corticosteroid
 For well localized (< 2.5 cmΦ), intra lesional
injection
Triamnicolone 3-5 mg/Kg per procedure, 3-5
injections over 6-8 wk interval
 For life threatening ones, systemic
Predinsolone 2-3 mg/Kg/day for 4-6wks…
 Parenteral for threatened upper airway and
visual field
12/20/2013 28

29.  Corticosteroid…
› Response rate 85%; Regression, stabilization of
growth, softening…
› Complication
 Cushingoid facies
 Diminished length and weight gain
 Myopathy, premature telarche, cardiomyopathy,
hirsuitism
12/20/2013 29

30.  Interferonα -2a
› Is second line treatment
› Indications
 Failure of corticosteroid treatment
 Complication and contraindication of corticosteroid
 Refusal to take parentral medication
› Dose
 2-3mU/m2 SC daily for 6-10 months
› Response rate >80%
12/20/2013 30

31.  Interferonα -2a…
› Complications
 Fever for 1-2 wks
 Reversible toxicities
Elevated transaminases, neutropenia, anemia
 Spastic diplegia
12/20/2013 31

32.  Chemotherapy
› Vincristine- another 2nd line
› Indications
 Kaposiform hemangioendothelioma with
thrombocytopenia
 Corticosteroid related problems
› Administered via central IV line
› Above 80% response
› Side effects:
 Peripheral neuropathy, constipation, hair loss,
sepsis
12/20/2013 32

33.  Laser therapy
› Can penetrated upto dermis level only, not
helpful for bulky lesions
› Indication
 For telangiectasia at involution phase
› Complication
 Ulceration, hypopigmentation, 2nd degree skin loss,
scarring
12/20/2013 33

34.  Surgical therapy
› Circular excision with purse-string closure
› Transverse excision- for eye lid, lip, neck
› Indications are depending on the phase of
Infantile hemangioma
› Always indicated for NICH
12/20/2013 34

35.  Indications for Surgical therapy…
 At proliferating phase (infancy)
› Obstruction- eyelid, subglottis
› Deformation- ambylopia, prominent ear
› Severe bleeding and ulceration irresponsive
otherwise
› Predictable scar or hair loss
› Liver hemangioma resulting in cardiac failure
12/20/2013 35

36.  Indications for Surgical therapy…
 At Involuting phase (early childhood)
› Hemangioma at nasal tip
› Protuberant involuting labial hemangioma
› For scar management
› Inevitable resection for residuum, expanded
skin..
› Staged resection & reconstruction
12/20/2013 36

37.  Indications for Surgical therapy…
 At Involuted phase (late childhood)
› Damaged skin
› Fibrofatty residuum
› Distortion of anatomic structures
› Staged reconstruction
12/20/2013 37

38.  Rare vasoproliferative tumors that present at
or shortly after birth.
 KH has both vascular and lymphatic
components, consisting of irregular
infiltrating nodules of compressed vessels
12/20/2013 38

39.  Expansion into regional nodes and soft
tissues is common in KH
 Associated with secondary destructive
osseous changes
 Needs aggressive local surgical excision in
absence of distant metastasis
12/20/2013 39

40.  Location
› Typically trunk, shoulder, thigh, retroperitoneum
› On skin adjacent petechiae & ecchymosis
 Associated with invasive vascular tumours
› KH
› Tufted angioma (TA)
12/20/2013 40

41.  Complicates with platelet trapping syndrome
› Thrombocytopenia, usually <10,000/mm3
› Risk of internal bleeding
 Management
› Admittance to a hospital, steroid infusions
› Antiplatelet/antifibrinolytic medications with or without
embolization of the lesion.
› Pharmacotherapy
› Surgical resection when possible.
12/20/2013 41

43.  Congenital morphogenic anomalies of
various vessels that can present at any age
 Most are sporadic, some have autosomal
inheritance pattern
 Types
› Slow flow
› Fast flow
12/20/2013 43

44.  Radiologic diagnosis
› US and color doppler studies
 To differentiate slow and fast flow anomalies
› MRI with contrast
 Informative portrayal of abnormal channels, flow
characteristics, and extent of involvement in tissue
planes
 Phleboliths are pathognomonic for VM
› Contrast enhanced CT
 For intra-osseuos anomalies or secondary bone
involvements
12/20/2013 44

45.  Radiologic diagnosis…
 Angiography
 It is used in conjunction with superselective
embolization as the primary treatment for AVM
 Done in conjunction with sclerotherapy for slow
flow malformations
12/20/2013 45

46.  CM is a macular, red, vascular stain that
presents at birth and persists throughout life
 Comprises regular, ectatic, thin-walled
capillary-to-venular sized channels located in
the papillary and upper reticular dermis.
 Some are red flags signaling underlying
abnormalities
12/20/2013 46

47.  Midline cephalic CM may indicate an
underlying occipital encephalocele, and a
dorsal CM can signal underlying cervical or
lumbosacral spinal dysraphism
 Sturge-Weber Syndrome
 Sometimes causes soft tissue and skeltal
hypertrophy
12/20/2013 47

48.  Treatment
› Flashlamp pulsed-dye laser, results in significant
lightening in 70-80%
 Better if initiated in infancy and childhood
› Surgical
 For excision of nodules
 For correction of vertical maxillary excess or mandibular
prognathism
12/20/2013 48

49.  Facial CM in association with ipsilateral leptomeningeal
and ocular vascular anomalies
 ‘Port-wine staining’ of V1 distribution
12/20/2013 49

50.  Leptomemingeal vascular malformations cause seizure,
hemiplegia, delay of cognitive & motor skills
 In the eye: glaucoma, retinal detachment & blindness
12/20/2013 50

51.  This distinctive condition presents as a
congenital, reticulated, serpiginous,
cutaneous vascular network
12/20/2013 51

52.  It is a benign condition and most resolve within first year
of life
 Coetaneous atrophy, vascular staining and venous
ectasia may persist
12/20/2013 52

53.  SPIDER TELANGIECTASIA
› In children on the dorsum of the hands,
forearms, and face
› In adults, they appear, in decreasing order, on
the face, neck, thorax, and arms
› Treatment
 Laser therapy
12/20/2013 53

54.  GENERALISED ESSENTIAL
TELANGIECTASIA
› Acquired lesion occurs almost exclusively in
adult women
› Flashlamp pulsed-dye laser therapy
12/20/2013 54

55.  Osler-Weber-Rendu syndrome
› Group of autosomal disorders
› Characterized by multi-system vascular
anomalies and recurrent hemorrhage
12/20/2013 55

56.  Discrete, spiderlike, bright red maculopapules, typically
with a diameter of 1-4 mm
 Location
› Face, tongue, lips, nasal and oral mucous membranes,
conjunctiva, palmar aspect of the fingers, and nail beds
 Bleeding
› Present with epistaxis
› hematemesis, hematuria, melena, into CNS
12/20/2013 56

57.  Presentation
› Evident at birth or detected before 2 years
› Also can be detected with prenatal US
 Types
› Microcystic……………………………………Lymp
hangioma
› Macrocystic………………………………..Cystic
Hygroma
› Combined
 Almost never regresses by its own
12/20/2013 57

58.  Anomalous channels, vesicles, or pouches
filled with lymphatic fluid
12/20/2013 58

59.  Cystic Hygroma
12/20/2013 59

60.  lymphangioma circumscriptum
› Limited to dermis level
12/20/2013 60

61.  Facial involvement…
12/20/2013 61

62.  Cervicofacial involvement…
› May cause airway obstruction
12/20/2013 62

63.  In cervicoaxillary…
› Recurrent pleural & pericardial effusion
 In extremity…
› Lymphedema
 In viscera..
› Hypoalbuminemia from protein loosing
enteropathy
12/20/2013 63

64.  Can Complicate with
› Intralesional bleeding
› Cellulitis
 In case of systemic infections LM flare up
12/20/2013 64

65.  Treatment
› Conservative
› Sclerotherapy
 For large cysts aspiration of fluid & instillation of
sclerosing agents
 Absolute ethanol
 Doxycyline
 Sodium tetradecyl sulfate
› Surgical resection- is the mainstay
 For cutaneous LM, resection to level of deep fascia
and closure with SSG
12/20/2013 65

66.  Most common vascular anomalies, 1-4% of
population
 Comprises thin-walled vascular spaces
surrounded by abnormally formed layers of
smooth muscle
 The dysplastic venous networks drain to
adjacent veins, many of which are varicose and
lack valves.
12/20/2013 66

67.  Presentation
› Bluish, soft, compressible lesions usually evident
at birth; Usualy solitary
› They swell when dependent
› Grow proportionately with child
› Also occur in the oronasopharynx, genitalia,
bladder, brain, spinal cord, liver, spleen, lungs,
skeletal muscles, and bones
12/20/2013 67

68.  Presentation…
› Pain and stiffness in the area, especially at
mornings
› Episodic thrombosis
› Phleboliths on imaging studies, as early as 2
years
› When mulltiple cutaneous and visceral
involvement, suggest familial inheritance
12/20/2013 68

69.  FAMILIAL GLOMANGIOMAS
› multiple, often tender, blue nodular or plaque like
venous anomalies occurring anywhere on the skin
› Autosomal dominant inheritance
12/20/2013 69

70.  BLUE RUBBER BLEB NEVUS
› Sporadic disorder
› Cutaneous lesions associated with sessile or
polypoid GIT lesions
› May cause recurrent GI bleeding
12/20/2013 70

71.  Craniofacial VM
› Facial asymmetry
› Orbital involvement
 Enophthalmos and exophthalmos depending on
position
› Intra-oral…
 Speech impairment and mal-occlusion
› Pharyngeal & laryngeal
 Obstructive sleep apnea
 Extremity
 Undergrowth because of disuse
 Hemarthrosis
12/20/2013 71

72.  Bleeding profile
› Localized intra-vascular coagulopathy (LIC)
› Risk of DIC following trauma
But platelets are minimally diminished (100-
150,000/ mm3)
Hypofibrogenemia, increased D-dimer or fibrin-
split products
12/20/2013 72

73.  Treatment
› Sclerotherapy
 For small nodules I/lesional injections
 For large & I/muscular ones, under GA and
fluoroscopic control
 Complications
 Blistering
 Full-thickness necrosis
 Neural deficit
 Systemic- renal toxicity and cardiac arrest
 Multiple sessions should be given
12/20/2013 73

74.  Treatment…
› Surgical resection
 After sclerotherapy for functional or cosmetic
impairment
 Pre-op anti-coagulation consideration
 Contour resection for hand, foot
 Endoscopic banding or resection for GI lesions
› Other
 Elastic stockings
 Low dose aspirin for painful phlebothrombosis
12/20/2013 74

75.  The epicenter of an AVM is called the nidus
and consists of arterial feeders, and ectatic
veins
 Comprises thickened fibromuscular walls,
and fibrotic stroma.
12/20/2013 75

76.  Presentation
› present at birth, most are seen during infancy
› Intracranial AVM is more common than extracranial
AVM, followed, infrequency of location by limbs,
trunk, and viscera
› Blush mistaken as hemangioma
› Puberty & trauma trigger expansion
› Mass develops under vascular stain, with localized
warmth, bruit, thrill
12/20/2013 76

77.  Consequences
› Ischemia
› Ulceration
› Pain
› Intermittent bleeding
› Cardiac failure
12/20/2013 77

78.  Stage I (Quiescence):
› Pink-blush stain, warmth, and AV shunting by
continous Doppler or 20-MHz color Doppler
 Stage II (Expansion):
› Same as stage I, plus enlargement, pulsations, thrill,
bruit, and tottuous/tense veins
 Stage III (Destruction):
› Same as above, plus dystrophic changes, ulceration,
bleeding persistent pain, and expansion/destruction
 Stage IV (Decompensation):
› Same as stage II, plus cardiac failure
12/20/2013 78

79.  Treatment:
› Stage I & II treatments are debatable
› For endangering signs
› Angiography followed by super-selective
embolization*
› Then, resection in 24-72 hrs time
 Resection of nidus together with overlying skin
12/20/2013 79

80. 12/20/2013 80

81.  Treatment
› Shoe-lift for leg discrepancy >1.5 cm
› Percutaneous epiphysiodhesis for upper limb, >2
cm discrepancy
› Super-selective embolization for complications
from fast-flow lesions, after 3-4 years
› Elastic compressive stockings
› Staged contour resection & selective
amputations for foot hypertrophy
› Sclerotherapy
12/20/2013 81

82. 12/20/2013
82

83. 12/20/2013 83