Benign vascular tumours

1. BENIGN TUMORS OF
CONNECTIVE TISSUE -
VASCULAR ORIGIN

2. Normal Vascular structure
 Vasculature is divided into arterial and
venous components joined by a network
of capillaries.

3. Introduction
VASCULOGENESIS
 De novo development of
blood vessels from
primitive endothelial
cells.
 Early embryogenesis.
ANGIOGENESIS
 Formation of new
microvessels from
differentiated
endothelium.
 During embryogenesis
and postnatal state.

4. Etiopathogenesis
Sequence of events
 The undifferentiated capillary network stage
 The retiform developmental stage
 Final developmental stage

6. Hemangioma Vascular
malformation
 Appears weeks after
birth
 Rapid growth
 Spontaneous involution
 Abnormality of
endothelial cell
proliferation
 Results from increased
number of capillaries
 Often circumscribed
 Usually present at birth
 Progressive
enlargement
 No involution
 Abnormality of vessel
morphogenesis
 Results from dilatation
of arterioles,
veins/capillaries
 Poorly circumscribed

7. Hemangioma
 Most common soft tissue tumor during infancy and
childhood.
 Most hemangiomas are superficial lesions that have a
predilection for the head and neck region, but they may
also occur internally, such as in the liver.
 Composed of capillary vessels arranged in lobules, which
are subserved by a feeder vessel.
 Most cases persist if untreated but have limited growth
potential but some vascular tumors (infantile
hemangioma) regress altogether.

9.  Infantile hemangioma

10.  Usually 80% of all hemangiomas are single lesions,
but 20% of affected infants develop multiple
tumors
 In oral cavity, it can involve lip(63%), buccal
mucosa(14%), lateral border of tongue(14%),
maxillary sinus, maxilla, mandible and parotid
glands.

11. Classification: Watson & McCarthy
 Capillary
 Cavernous
 Angioblastic/ Hypertrophic
 Racemose
 Diffuse systemic hemangioma
 Metastasizing hemangioma
 Nevus vinosus /Port wine
 Hereditary hemorrhagic telengiectasis.

12. Demographics
Site
 Oral hemangiomas represent 14% of all
human hemangiomas
 60% hemangiomas are located in head and
neck area
 25% occur on the trunk
 15% on the extremities

13. Capillary hemangioma
Clinical features-
Age- Infants
Sex- Female>Male
Site- Skin and soft tissues, especially in the head and neck
area.

14.  ORAL MANIFESTATIONS:
 Appear as a flat or raised lesion of the
mucosa, usually deep red or bluish red and
seldom well circumscribed.
 They are readily compressible and fills
slowly when released.
 Site:
 Lips, Tongue, Buccal mucosa, Palate
 The tumor presents at birth or shortly thereafter
as a red-purple macule that slowly becomes raised
and then tends to regress in over 70% of cases
after a period of months to years.

15. Histological appearances
 The overall low-power
architecture in all cases,
regardless of the organ
involved, is that of a
multilobulated tumor
 In early lesions, the lobules
are highly cellular and
composed of mitotically
active, plump endothelial
cells forming tiny, rounded,
often uncanalized vascular
spaces

17.  As lesions mature, the vessels become
canalized and more easily recognized, then
often showing congested lumina and flat
endothelial cells.
 Older lesions become progressively fibrotic
with almost complete regression or absence
of the vascular elements.

19. LOBULAR CAPILLARY HEMANGIOMA/PYOGENIC
GRANULOMA
 The classical appearance is that of a solitary, rapidly
growing, ulcerated, bleeding, polypoid blue-red
nodule which is usually less than 2cm in diameter
H/F-
 Ulcerated epithelium, prominent acute inflammatory
infiltrate
 Core of tumor shows lobules of small capillaries, with
or without discernible lumina, lined by prominent
endothelial cells
 Stroma is loose and edematous
 Old lesions can show marked fibrosis.

22. Cavernous hemangioma
 Clinical features are similar to capillary hemangioma,
however, these lesions tend to be larger, deeper and
less well circumscribed
Histologic appearances
 Consist of poorly circumscribed, irregularly dilated
blood vessels lined by flat endothelium and with walls
of varying thickness.
 Areas resembling capillary hemangioma often can be
found focally, especially in the superficial portion

23. Dilated blood vessels, sinusoidal spaces lined
by flat endothelium and with walls of varying
thickness.
Cavernous hemangioma

24. Syndromes associated
 Rendu-Osler-Weber syndrome
 Sturge-Weber-Dimitri syndrome
 Kasabach-Merritt syndrome
 Maffucci syndrome
 von Hippel-Lindau syndrome

25.  Osler-Weber-Rendu
Disease
 Sturge Weber
Syndrome- Port Wine
stain

26. Lymphangioma
 Lymphangiomas are uncommon, benign
malformations of the lymphatic system
 True neoplasm/hamartoma?
 Lymphangiomatosis- multiple lesions seen in infancy
and childhood. It is a potentially life-threatening
disease with visceral involvement
Classification-
 Simple
 Cavernous
 Cellular or hypertrophic
 Diffuse systemic
 Cystic hygroma

27. Clinical features-
Age- At birth
Sex- F=M
Site- Tongue, palate, BM, gingiva and lips
C/P- Superficial- papillary lesion
Deep- nodular mass with no change in surface texture
Macroglossia, macrocheilia
Lymphangioma of alveolar ridge in neonates-blue dome
shaped

28. H/F-
- Numerous lymphatic vessels in loose fibrovascular stroma
- Vessels just beneath the surface epithelium fill or replace the
CT papillae, producing the surface change
- Hemangiolymphangioma- channels filled with blood and
lymph
- Lymphangiomyoma- associated with smooth muscle
component

29. 29

31. Hemangiopericytoma
 Benign tumor of pericytes
 Distinct entity?
Clinical features-
Age- 2nd
-7th
decade
Sex- F=M
C/P- Rapidly enlarging red/bluish mass
- Soft and rubbery in consistency
- Infantile type- rapid rate of enlargement

32. H/F-
- Well circumscribed lesion
- Numerous vascular channels with plump endothelial
cells surrounded by tightly packed oval and spindle cells
with hyperchromatic nuclei and moderate amt of
cytoplasm
- Stag-horn pattern
- Reticulin staining required to differentiate it from
hemangioendothelioma

33. Stag horn pattern

34. Hemangioendothelioma
 Vascular lesions with a biologic behavior
intermediate between hemangioma &
angiosarcoma
Clinical features-
Age- Infants
Sex- Female>Male
Site- skin and soft tissues, especially in the head and
neck area.
Commonest location in the oral cavity- lip, palate,
gingiva, tongue
C/P- similar to hemangioma

35. H/P-
 Poorly circumscribed lesion characterized by the
proliferation of endothelial cells and multiple
vascular spaces
Variants-
 Epitheloid
 Kaposiform
 Polymorphous
 Spindle cell