Vasoformative disorders are distinct type of lesions that covers benign, lesions of intermediate malignancy and malignant disorders along with syndromes. very useful for oral path, oral medicine and as well as general pathology and residents.

1. Vasoformative tumors

2. Contents
• Introduction
• Classification
• Benign tumors
• Malignant tumors
• Vascular Malformations
• Summary and conclusion
• References

3. • DIVIDED INTO TWO MAIN GROUPS: TUMORS AND
MALFORMATIONS.
• LARGE AND COMPLEX GROUP OF LESIONS.
• THESE CLINICAL ENTITIES ARE EXTREMELY DIFFERENT.
• BUT OFTEN CONFUSED, AS EVIDENCED BY THE HISTORY AND
MEDICAL LITERATURE.

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• The term hemangioma refers to the common tumor of
infancy that has a proliferative endothelium.
• Exhibits rapid postnatal growth and slow regression during
childhood; this tumor never appears in an adolescent or
adult.
• Whereas vascular malformations are comprised of
abnormally formed channels.
• That are lined by stable endothelium, present at birth,
never regress and often expand.

5. HISTORY
• The word “birthmark” was one of the first ways used to
described vascular anomalies,.
• Based on the folk belief that a mother’s emotions can leave
an imprint on her unborn fetus.
• In the late 1860s, Dugas and Fisher disproved this belief and
concluded that birthmarks were malformations resulting from
flawed embryologic development.
• Around the mid- 19th century, Virchow proposed a
histopathologic classification based on the size and
appearance of the vessels.

6. • Then in 1982, thanks to technologic advances Mulliken and
Glowacki classified them based on histology, biology, and
clinical presentation.
• They divided vascular anomalies into two major categories:
tumors and malformations.
• In 1996, the International Society for the Study of Vascular
Anomalies (ISSVA) adopted and modified Mulliken and
Glowacki’s original classification.
• This classification system is now widely accepted to properly
diagnose and manage vascular anomalies

7. CLASSIFICATIONS

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10. Heamangiomas
• All vascular tumors (except congenital hemangiomas) are
not clinically present at birth.
• Have a period of rapid growth, and spontaneously involute
(except noninvoluting congenital hemangiomas [nich]).
• A malformation is characterized as an error in
development of vascular embryologic tissue.
• Vascular malformations can be capillary, venous, lymphatic,
and/or arterial in nature.

11. • The term hemangioma has traditionally been used to describe
a variety of developmental vascular anomalies
• In recent years, great progress has been made in the
classification and understanding of these vascular lesions.
• Currently, hemangiomas are considered to be benign tumors
of infancy.
• That are characterized by a rapid growth phase with
endothelial cell proliferation, followed by gradual involution.
Seminars in Plastic Surgery Vol. 28 No. 2/2014

13. PATHOGENESIS

14. Clinical presentation
• Most common tumor of infancy (10%)
• Slight female predominance
• 60% arise in head and neck – cosmetic concerns followed by the
trunk (25%) and the extremities (15%).
• Which are grouped into Infantile Hemangiomas (IHs) and
Congenital Hemangiomas (CHs)
• Eighty percent of hemangiomas occur as single lesions, but 20% of
affected patient s will have multiple tumors.

15. Infant with two red, nodular masses
on the tragus, posterior scalp and neck strawberry hemangioma
Neville 2nd edition

16. Juvenile hemangioma
• The juvenile hemangioma is a form of capillary hemangioma
which occurs during infancy at a rate of about 1 in every 200
live births.
• About one-fifth of the cases are multiple.
• During the early stage it may resemble a common birthmark
in that it is a flat, red lesion that intensifies in color when the
infant strains or cries.
• With time it acquires an elevated, protruding appearance that
distinguishes it from birthmarks and has earned it the fanciful
designation of strawberry nevus .

17. • DEEPLY situated lesions impart little color to the
overlying skin.
• Consequently may be misdiagnosed preoperatively.
• These tumors may be located on any body surface but
are most common in the region of the head and neck.
• Particularly the parotid, where they seemingly follow the
distribution of cutaneous nerves and arteries

18. Clinical appearance of juvenile hemangioma

19. Juvenile (cellular) hemangioma. low-power
photomicrograph showing a cellular mass of
vascular endothelial cells arranged
in lobular aggregates.
High-power view showing a highly cellular
endothelial proliferation forming
occasional indistinct vascular lumina.

20. Capillary (lobular) hemangioma
• Capillary hemangiomas usually appear during the first few
years of life and are located in the skin or subcutaneous
tissue.
• Rare cases are familial; linkage analysis has localized the
mutation to chromosome 5 although the candidate gene has
not been identified.
• Typically elevated and red to purple in color, they are
composed of a proliferation of capillary-sized vessels lined by
flattened endothelium,

21. Histopathology
• Congenital hemangiomas have been classified
microscopically as capillary hemangiomas or cavernous
hemangiomas.
• Depending on whether the microscopic size of the
capillaries is small or large, respectively.
• The vascular spaces are lined by endothelium without
muscular support.
• Clinically, no significant difference is noted between
capillary and cavernous hemangiomas (Regezi 3rd edition)

22. Capillary hemangioma consisting of small vessels lined by flattened mature endothelium
Soft tissue tumors

23. Capillary heamangioma with large blood vesels

24. The natural history of Hemangioma is characterized by three
phases:
• Proliferating Phase
• Involuting Phase
• Involuted Phase

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Proliferating Phase
• Hemangioma grows rapidly during the first 6
to 8 months of infancy, the skin becomes
raised, bosselated, and a vivid crimson color.
• Palpation at this stage reveals a tense and
noncompressible mass.

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Involuting Phase
• Hemangioma reaches its peak before the first year;
and for a time thereafter, growth is proportionate to
that of the child.
• The first signs of the involuting phase appear as the
crimson color fades to a dull purplish color, the skin
gradually pales, and the tumor feels less tense.
• The involuting phase continues until the child is 5 to
10 years of age.

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Involuted Phase
• Regression is complete in 50% of children by age 5 years and
in 70% by age 7 years, with continued improvement until age
10 to 12 years.
• The hemangioma may persist in the form of residual tumor,
loose skin, telangiectasias, or scarring.

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Hemangiomas can be superficial, deep, or
visceral in location
• Superficial lesions: often exhibit the classic crimson color of
the so-called strawberry hemangioma.
• Deep lesions: Are those within the deep dermis or
subcutaneous tissues.
• Often present as pale blue or purple masses that may be
confused with venous malformations.
• Visceral lesions: Are not apparent on physical examination,
and in 50% of cases, there are no accompanying cutaneous
hemangiomas.
• In this setting, their presence may only be suggested by
physiologic findings such as hepatomegaly, congestive heart
failure, or stridor.

30. CONGENITAL HEMANGIOMAS
• Although congenital hemangiomas (chs) and ihs grossly
appear very similar, they are histopathologically,
pathogenetically, and immunophenotypically distinct.
• Congenital hemangiomas in general have no gender
preference.
• Are fully developed at birth, and test negative for the
immunohistochemical marker glut 1.
• Congenital hemangiomas exhibit proportional growth, are
usually solitary, and more commonly present on the
extremities.

31. • Congenital hemangiomas undergo the proliferative phase in
utero, which makes it possible to be diagnosed with prenatal
imaging.
• They are separated into two main types: rapidly involuting
congenital hemangiomas (RICHs) and non involuting
congenital hemangiomas (NICHs)

32. Rapidly Involuting Congenital
Hemangioma
• Rapidly involuting congenital hemangiomas undergo rapid
proliferation in utero and are grossly visible at birth.
• They usually start involuting weeks after birth and fully
involute around 2 years of age.
• They present as a raised, violaceous tumor with ectatic veins
surrounded by a pale rim of vasoconstriction.
• Histologically, variable-sized lobular capillaries with prominent
endothelial cells are seen.

33. • Involution usually starts just after birth centrally and
progresses toward the periphery, leaving behind a region of
thin atrophied skin with little subcutaneous fat.
• Lesions can be excised if they do not completely involute

34. Noninvoluting Congenital Hemangioma
• Noninvoluting congenital hemangiomas always appear fully
formed at birth as a solitary tumor and are more common in
males).
• They demonstrate proportional growth with the child and will
never involute.
• Typical lesions are round, average from 5 to 10 cm in
diameter, with coarse overlying telangiectasias.
• Most are warm to palpation with a component of fast arterial
flow that can demonstrated by Doppler US.

35. Histopathology of a proliferating infantile hemangioma with plump endothelial cells in the dermis
Medscape. com

36. Endothelium becomes flattened and
resembles that seen in adult forms American journal of pathology

37. • Interlobular areas contain dilated dysplastic veins.
• Surgical excision is treatment of choice since they are solitary
and do not involute

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39. Complications
• Complications occur in about 20% of hemangiomas.
• The most common problem is ulceration which may occur
with or without secondary infection.
• Although hemorrhage may be noted.
• Significant blood loss does not usually occur.
• Hemangiomas that occur in crucial areas can be associated
with significant morbidity.

40. • Periocular tumors often result in amblyopia (dimness of
vision) strabismus or astigmatism.
• Patients with multiple cutaneous hemangiomas or large facial
hemangiomas are at increased risk tor concomitant visceral
hemangiomas.
• Tumors in the neck and laryngeal region can lead to airway
obstruction.
• Congestive Heart Failure : usually occurs in one of two
settings: diffuse neonatal hemangiomatosis or large visceral
hemangiomas

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DIAGNOSTIC IMAGING
Hemangiomas are readily distinguished from other tumors by:
– Ultrasonography (US)
– Computerized tomography (CT)
– Magnetic resonance imaging (MRI)
– Arteriography

42. Vascular malformations
• Vascular malformations are present at birth and unlike
hemangiomas, do not go through a “rapid proliferative
phase” and they do not “involute”.
• They grow commensurately with the patient.
• Vascular malformations may be capillary, venous, arterial, or
combinations of these.
• Approximately 31% of these malformations are found in the
head and neck region

43. • Vascular malformations are congenital morphogenic
anomalies.
• Various vessels that can present at any age.
• They are subdivided into 2 categories:
• (1) slow- or low-flow and (2) fast- or high-flow malformations.
• Low-flow malformations contain combinations of capillary,
venous, and lymphatic components

44. Clinical presentation
• In many cases, the diagnosis of a vascular malformation can
be made from the patient’s history.
• Although present at birth, these lesions are often not
identified immediately.
• But only later on when the lesion enlarges enough to be
clinically identifiable.

45. Photograph shows skin involvement with red
discoloration and enlargement of underlying
soft tissues

46. • Venous malformations are
bluish, soft and easily
compressible, and
auscultation reveals no
bruits.
• These malformations can
vary from superficial,
localized.

47. Oral Manifestations
• In the oral cavity, these can present at any site.
• Most commonly occur on anterior two-thirds of the tongue,
palate, and gingival and buccal mucosa.
• Vascular malformations may be associated with underlying
disease or systemic anomalies.

48. Associated Syndromes
• Bonnet-Dechaume-Blanc syndrome or Wyburn-Mason
syndrome.
• Parkes-Weber syndrome.
• Capillary malformation-AVM syndrome.
• Cobb syndrome.

49. Arterio-venous malformations
• High-flow lesions
 Result from persistent direct arterial and venous
communication.
 Although they are present from birth, may not become
noticeable until later in childhood or adulthood.
 A palpable thrill or bruit often is noticeable because of the
fast vascular flow through these lesions.

50. High-flow vascular malformation of the lower lip

51. Intra-bony vascular malformations
• “lntrabony“ hemangiomas”
• May occur and probably represent either venousor arterio-
venous malformations.
• In the jaws, such lesions are detected most often in patients
between 10 and 20 years of age.
• They are considerably more common in females than males
and occur twice as often in the mandible as the maxilla.

52. • The radiographic appearance of intrabony vascular
malformations is variable.
• Most commonly, the lesion shows a multilocular radiolucent
defect.
• The individual loculations may be small (honeycomb
appearance) or large (soap bubble appearance).
• In other cases, the lesion may present as an ill-defined
radiolucent area or a well defined cyst like radiolucency

53. Intrabony venous malformation . Well
circumscribed radiolucency that contains
fine trabeculations.

54. Lymphangioma
• Lymphatic malformations have the propensity to enlarge
more than any other vascular malformation.
• They occur most commonly in the neck and axilla (55% to
95%).

55. Lymphangioma. Vesicle- like appearance of a tumor of the right lateral tongue.

56. •
Lesion of the tongue showing dilated
lymphatic vessels beneath the epithelium
and in the deeper connective tissues.
High-power photomicrograph showing
dilated, lymph-filled vessels immediately
below the atrophic surface epithelium.

57. Malignant tumors
• Angiosarcoma
• Extremely rare (50% in head
and neck)
• Prognosis on tumor size,
grade.
• Radiation minimally
effective.
• Poor survival
The images show abundant small blood vessels lined
by large atypical endothelial cells

58. Kaposi’s Sarcoma
• Viral-induced
• Four entities:
• Classic
• Endemic
• Immunosuppressed
• AIDS-related
• Surgery or combination of
• Radiation therapy and
• Chemotherapy
Plaques on ala and tip of nose

59. An HIV-positive patient presented with an Intraoral Kaposi’s sarcoma
lesion with an overlying candidiasis infection.

60. Early lesion of Kaposi sarcoma illustrating irregular proliferation of
miniature vessels .

61. Hemangiopericytoma
Pericytes of Zimmerman
• 25% in head and neck.
• Surgical treatment.
• Grade important on
prognosis.
• Radiation or chemotherapy
for selected cases
Heamangiopericytoma on buccal vestibule

62. Staghorn blood vessels with surrounding pericytes

63. Hemangioendothelioma
• Group of vascular
neoplasms that may be
considered benign as well
as malignant.
Budding, hobnail-like endothelial cells visible.

64. Refrences
• Kaul A, Gupta A, Singh S, Gupta G. Oral hemangioma simulating gingivitis. Univ Res J Dent
2014;4:175-8.
• Dasgupta, R, and Fishman S. "ISSVA classification." In Seminars in pediatric surgery, 2014;
23(4),158-61. WB Saunders. DOI: 10.1053/j.sempedsurg.2014.06.016
• Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and Maxillofacial Pathology3th ed. India:
Elsevier; 2009.
• Rajendran R, Shivpathasundaram S. Shefars text book of oral pathology. Fifth ed: Elsevier
publications.
• Enzinger and Weiss's. Soft tissue tumors. 5th edition. Elsvier: 2008. p 825-903.
• Krishnan M, Kumar K S, Sowmiya T. Hemangiopericytoma - The need for a protocol-based
treatment plan. Indian J Dent Res 2011;22:497.