Literature revision of vascular tumors

1. VASCULAR TUMORS
Literature Revision

2. DR. LEONARDO BALLESTAS MALDONADO
GENERAL SURGERY RESIDENT
UNIVERSIDAD DEL VALLE
DR. RENE TIMARAN
VASCULAR SURGERY TEACHER
HOSPITAL SAN VICENTE DE PAUL
UNIVERSIDAD DE ANTIOQUIA

3. Clinic case
• Female
• 2 weeks after birth
• Premature antecedent
• GLUT-1 positive
DIAGNOSIS

4. “Not every skin lesion looking like a
strawberry is a hemangioma; not all
hemangiomas look like strawberries”
J.B. Mulliken, MD

5. Evidence
• A search of the evidence in the database:
Medline, Embase, Cohcrane, Tripdatabase,
Scielo
• Key words: Hemangioma, vascular anomalies,
kaposiform hemangioendothelioma, infantile
hemangioma, pyogenic granuloma, congenital
hemangioma

7. Introduction
• Vascular anomalies are the most common skin and soft
tissue lesions observed in infants and children
• Older nomenclature continues to cause confusion,
misunderstood diagnoses, and potencial mismagement
• In 1982, Milliken and Glowacki proposed a
classification system for vascular anomalies based on
their clinical behavior and endothelial cell
characteristic
Kilcline C, Frieden IJ. Infantile hemangiomas: how common are they? A systematic review of the medical literature. Pediatr
Dermatol 2008;25(2):168–73.

8. ISSVA Classification
Society for the Study of Vascular Anomalies
Vascular tumors Vascular malformation Vascullar anomalliies
Hemangioma Vascular malformation

9. Vascular anomalies
Vascular Tumors Vascular malformaciones
Infantile
hemangioma
Congenital
hemangioma
NICH RICH
• Capillary malformation
• Venous malformation
• Lymphatic malformation
Slow flow Fast flow
• Arterial malformation
• Arteriovenous fistula
• Arteriovenous malformation
Combined types

10. Differentiating Features
TUMOR
• True tumors, with
proliferation of the vascular
endothelium
• >3:1 female : male
• Small or absent at birth
• Rapid growth during infancy
• Self-limited
• Diagnosis: Clinical +
appearence
MALFORMATION
• No tumor, comprised of
dysplastic vessels
• 1:1 female : male
• Present at birth
• Growth proportional to
child
• Never disappear
• Diagnosis: MRI, Doppler
ultrasonography,
angiography

11. Vascular tumors
• Infantile Hemangioma
• Congenital Hemangioma
• Kaposiform Hemangioendothelioma
• Pyogenic Granuloma

12. Infantile Hemangioma (IH)
• Bening endothelial cell tumor
• Most common tumor of infancy/childhood
• Incidence of 4.5% overall
• It is more frequent in caucasian, premature infants and
females (3:1 to 5:1)
• Perinatal characteristics associated with a higher risk of IH
include preeclampsia, multiple gestation, and low birth
weight
Hemangioma Investigator Group, Haggstrom AN, Drolet BA, Baselga E, et al. Prospective study of infan- tile hemangiomas:
demographic, prenatal, and peri- natal characteristics. J Pediatr 2007;150(3):291–4.

13. Pathogenesis
• Despite the frequency of this tumor,
understanding of the pathogenetic mechanisms
• Generally believed to be a complex interaction of
both genetic and environmental factors
• Histologically, have markedly increased cellularity
with clusters of plump cells that are positive for
markers of immature endothelial cells
Drolet BA, Swanson EA, Frieden IJ, et al. Infantile hemangiomas: an emerging health issue linked to an increased rate of low birth
weight infants. J Pediatr 2008;153(5):712 No-715.

14. Pathogenesis
• Endothelial-like cells of the hemangioma
expressed GLUT-1, the erythrocyte-type
glucose transporter protein that has been
shown to be upregulated in zones of hypoxia
• GLUT-1 seems to be an exclusive marker for IH
and is an invaluable tool used to distinguish
hemangiomas from other vascular lesions.
NorthPE,WanerM,MizerackiA,etal.GLUT1:anewly discovered immunohistochemical marker for juvenile hemangiomas. Hum Pathol
2000;31(1):11–22

15. Diagnosis
• History and physical
examination
• Radiographic imaging
• Histopathology – GLUT-1

16. History
• The diagnosis is typically made clinically based on
its appearance and characteristic behavior
• Between 30% and 50% of lesions are visible at
birth as a small, pale spot, telangiectatic stain or
ecchymotic area
• Appears weeks/months afther birth (the median
age appearance is 2 weeks)

17. Localitation
• Most hemangiomas are
single (80%)
Head an neck 60%
Trunk 25%
Extremities 15%

18. Natural growth infantile hemangioma
Age
(Years)
1 2 3 4 5 6 7 8
Growth
■ Proliferating
■ Involuting
■ Involuted

19. Proliferation stage

20. Involution stage

21. Residual changes

22. classified by their depth of soft tissue
involvement

23. Classification based on morphology
• Localized or
segmental or
indeterminate
• Segmental
hemangiomas are at
higher risk of
complications and
associated anomalies

24. Ultrasound
• Differentiate deep hemangiomas to venous
malformations
• To evaluate the response to drug treatment
• Findings
 Dense parenchyma rapid flow
 Minor arterial resistance
 Increased venous velocity
 Effect of soft tissue mass
•Connors III JP, Mulliken JB. Vascular Tumors and Malformations in Childhood. Vascular Surgery 6th ed 2005

25. Magnetic resonance
• Test "reference”
• Findings
 Intermediate intensity parenchymal tissue (T1), and
moderate hyperintensity (T2)
 Empty Flow (arteriovenous shunts)
 Glasses high flow or low flow
 Reducing the size and number of vessels irrigating /
draining
 lobularity
 Body fat avascular
•Connors III JP, Mulliken JB. Vascular Tumors and Malformations in Childhood. Vascular Surgery 6th ed 2005

26. Complications
• Although most IHs are uncomplicated and do
not require treatment
• 24% of those referred to tertiary institutions
had complications
• Size, location, and subtype (localized vs
segmental) are major factors to consider in
evaluating an infant’s risk
Haggstrom AN, Drolet BA, Baselga E, et al. Prospective study of infantile hemangiomas: clinical characteristics predicting
complications and treat- ment. Pediatrics 2006;118(3):882–7.

28. Ulceration
• Is the most common complication and can result
in pain, infection, bleeding, and permanent
scarring
• Larger in size or of the segmental subtype are
more likely to develop ulceration
• The cause of ulceration is not well understood,
but maceration and friction are likely contributing
factors given the higher frequency in locations
prone to this
Chamlin SL, Haggstrom AN, Drolet BA, et al. Multi- center prospective study of ulcerated hemangi- omas. J Pediatr
2007;151(6):684–9, 689-e1.

29. Ulceration

30. Visual compromise
• Threat to vision is a common reason for
treatment in the IH population
• Infants are at particular risk because stimulus
deprivation for as little as 1 week can interrupt
visual development and result in permanent
visual impairment
• Periocular IH may cause ptosis, strabismus, and
anisometropia, each of which may result in
astigmatism, amblyopia, or blindness
Al Dhaybi R, Superstein R, Milet A, et al. Treatment of periocular infantile hemangiomas with propranolol: case series of 18
children. Ophthalmology 2011; 118(6):1184–8.

31. Visceral involvement and
complications
• Hemangiomatosis – 30%
• The lesions are usually less
than 5 mm in diameter and
domelike
• Are recognized to have a
higher risk of visceral
hemangiomas, with liver and
gastrointestinal involvement
being most common
• Should be screened with
ultrasound to rule out lesions
in internal organs

32. Anomalies associated with anatomic
localization of HI
• The presence of IH in
particular locations can
be a marker for
underlying or
associated anomalies
• The “beard”
distribution has been
associated with the
presence of airway
hemangiomas
Orlow SJ, Isakoff MS, Blei F. Increased risk of symp- tomatic hemangiomas of the airway in association with cutaneous
hemangiomas in a “beard” distribution. J Pediatr 1997;131(4):643–6.

33. Anomalies associated with anatomic
localization of HI
In the lumbosacral area
have also been reported in
association with occult
spinal dysraphism
Girard C, Bigorre M, Guillot B, et al. PELVIS syndrome. Arch Dermatol 2006;142(7):884–8.

34. PHACE syndrome
The neurocutaneous
disorder characterized by
posterior fossa
abnormalities [P], large
facial hemangiomas [H],
arterial anomalies [A],
cardiac defects [C], and
eye anomalies [E]
Frieden IJ, Reese V, Cohen D. PHACE syndrome. The association of posterior fossa brain malforma- tions, hemangiomas, arterial
anomalies, coarctation of the aorta and cardiac defects, and eye abnormal- ities. Arch Dermatol 1996;132(3):307–11.

35. Treatment Objetives
• Prevent or reverse the life-threatening
complications or function
• Prevent or minimize disfigurement
• Minimize psychosocial stress for the patient and
family
• Properly treat ulceration to minimize scarring,
bleeding, infection and pain

36. Conservative treatment
• Observation is the mainstay of management
because 90% of IHs are small and localized
and do not involve aesthetically or
functionally important areas
• Up to 38% of hemangiomas referred to
tertiary care specialists require systemic
treatment because of complications

37. B-Blockers
• The use of propranolol for IH quickly gained favor,
because it has been perceived to have a lower
side-effect profile than other systemic therapies
• Its mechanism of action in the treatment of IH is
unknown
• Possible mechanisms of action purported include
vasocon- striction, inhibition of angiogenesis, and
induction of apoptosis
Leaute-Labreze C, Dumas de la Roque E, Hubiche T, et al. Propranolol for severe hemangiomas of infancy. N Engl J Med
2008;358(24):2649–51

38. Systemic Corticosteroids
• Response to treatment is variable, regression in one
third of cases, stabilization of growth in another third
of cases, and minimal to no response in the final third
of cases
• Adverse effects are common and include irritability,
gastrointestinal upset, sleep disturbance, cushingoid
facies, adrenal suppression, immunosuppression,
hypertension, bone demineralization, cardiomyopathy,
and growth retardation
• The duration of treatment is variable
Barrio VR, Drolet BA. Treatment of hemangiomas of infancy. Dermatol Ther 2005;18(2):151–9.

39. Intralesional and topical
corticosteroids
• Reported to decrease the size or slow growth
• Are most effective for small and localized
cutaneous hemangiomas
• The efficacy is limited by the depth of its
penetration compared with the depth of
hemangioma involvement
Barrio VR, Drolet BA. Treatment of hemangiomas of infancy. Dermatol Ther 2005;18(2):151–9.

40. Vincristine
• Effective in the treatment of IH and has historically
been reserved for those IHs resistant to corticosteroids
or in patients intolerant of corticosteroids
• Constipation is the most common side effect, but
neuromyopathy, most commonly presenting as foot
drop, is a potentially serious side effect
• Administration of vincristine requires placement of a
central line; therefore, risks associated with this must
be considered as well
Barrio VR, Drolet BA. Treatment of hemangiomas of infancy. Dermatol Ther 2005;18(2):151–9.

41. Interferon
• Recombinant interferon-a an inhibitor of
angiogenesis, administered as a subcutaneous
injection
• Side effects include flulike symptoms of fever,
irritability, and malaise
• Its use is not recommended in earlier age

42. Laser therapy
• The mechanism for this is not well understood
• Controversy exists surrounding its use for the treatment of
proliferating IH as adverse outcomes including ulceration
and scarring have been described
• Tuse of laser for intact IH is limited by the depth of the
laser’s penetration (1 mm)
• A few studies describing its benefit in the treatment of
ulcerated hemangiomas in terms of both increasing
reepithelialization and decreasing pain
Witman PM, Wagner AM, Scherer K, et al. Complica- tions following pulsed dye laser treatment of super- ficial hemangiomas.
Lasers SurgMed 2006;38(2): 116–23.

43. Surgery
• Surgical excision may be an option for
function- threatening or life-threatening
hemangiomas when medical therapy fails or is
not tolerated
• More commonly its role is for removal of
residual fibrofatty tissue or correction of
scarring after involution

45. Congenital Hemangioma (CH)
• Unlike IH, CH is fully
developed at birth and does
not undergo postnatal
growth
• Rare (compared to infantile)
• Blue/gray hue, pale halo
(skin)
• Is more common in the
extremities and has an
equal sex distribution
Vascullarr anomalliies
Hemangioma

46. • 2 types
Non Involuting Congenital Hemangioma
(NICH) – persistent
Rapidly Involuting Congenital Hemangioma
(RICH) – resolved by 1 – 2 years

47. Growth patterns of hemangiomas
GROWTH NICH
RICH
IH
BIRTH 1 YR 2 YRS
AGE
RICH:rapidly involuting congenital hemangioma; NICH:noninvoluting congenital hemangioma. IH: Infantile Hemangioma

48. Treatment
• Most CHs are managed by observation
• RICHs begin to involute shortly after birth, and
most have resolved by 12 months of age
• NICH is a stable lesion that does not involute
or respond to pharmac logic treatment. Thus,
NICH causing disfigurement or functional
problems is treated by excision

49. Kaposiform Hemangioendothelioma
• Rare vascular neoplasm that is
locally aggressive but does not
metastasize
• Fifty percent of lesions are
present at birth and are
diagnosed during infancy
(58%), early childhood (1 to 10
years; 32%), or late child hood
(10 to 20 years; 10%)
• Has an equal sex distribution,
is solitary, and affects the head
or neck (40%), trunk (30%), or
extremity (30%)

50. Kasabach-Merritt phenomenon
• "Giant Hemangioma”
• Thrombocytopenia
(typically <10,000 mm3)
• Petechiae
• bleeding

51. Treatment
• A localized tumor can be resected, depending on
its location
• Patients with KMP require systemic treatment to
prevent lifethreatening complications
• Responds best to vincristine (90%), followed by
IFN (50%) and corticosteroid (10%)

52. Pyogenic granuloma
• Is a solitary a small red papule that grows rapidly, forming a stalk
• The malefemale ratio is 2 : 1
• It is commonly complicated by bleeding (64.2%) and epidermal ulceration
(36.3%)
• The presentation is inversely correlated with age
• They are distributed on the head or neck (62%), trunk (19%), upper
extremity (13%), or lower extremity (5%)
• Twentyfive percent of patients have a history of trauma or an underlying
cutaneous condition (including capillary malformation, dermatologic
disorder, viral infection, or insect bite).

53. Treatment
• Numerous treatment
methods have been
described: curettage, shave
excision, laser therapy, and
excision
• These modalities have a
recurrence rate of 43.5%
• Definitive treatment
requires fullthickness skin
excision, which has an
approximately 100% cure
rate

54. Clinic case
• Female
• 2 weeks after birth
• Premature antecedent
INFANTILE HEMANGIOMA

55. THANKS