VASCULAR LESIONS OF ORAL

CAVITY

M.N. Anusha
III Year PG
 CLASSIFICATION

REACTIVE INFECTIVE
• Pyogenic • Bacillary
granuloma angiomatosis

BENIGN BORDERLINE MALIGNANT

• Hemangioma • Hemangioendothelioma • Angiosarcoma
• Angioma • Hemangiopericytoma • Kaposi Sarcoma
• Angioendothelioma
 PYOGENIC GRANULOMA

It is a distinctive clinical entity originating as a Pyogenic

organisms
response of the tissues to a nonspecific infection. It is a (botryomyco
tic infection,
tumor like growth that is considered as an exaggerated, staphylococc
conditioned response to minor trauma. i or
streptococci)

ETIOLOGY

Pregnancy
Trauma -
tumour -
pathway for
increasing
the
levels of
invasion of
progestero
nonspecific
ne and
types of
estrogen -
microorgani
angiogenesi
sms.
s
Pyogenic granuloma | MyPathologyReport.ca
Lomeli Martinez SM, Carrillo Contreras NG, Gómez Sandoval JR, Zepeda Nuño JS, Gomez Mireles JC, Varela
Hernández JJ, Mercado-González AE, Bayardo González RA, Gutiérrez-Maldonado AF. Oral Pyogenic
Granuloma: A Narrative Review. Int J Mol Sci. 2023 Nov 28;24(23):16885. doi: 10.3390/ijms242316885.
PMID: 38069207; PMCID: PMC10706684.
 CLINICAL FEATURES
• Site: Gingiva (facial, lingual, IDP), lips, tongue and buccal mucosa.

• The lesion is usually an elevated, pedunculated or sessile vascular

mass with a smooth, lobulated, or even a warty surface, which
commonly is ulcerated and shows a tendency for hemorrhage either
spontaneously or upon slight trauma.

• It is deep red or red purple, depending upon its vascularity, painless,

and rather soft in consistency.

• Sometimes there is exudation of purulent material.

HISTOPATHOLOGY
 BACILLARY ANGIOMATOSIS

• Bacillary angiomatosis is a reactive vascular proliferation caused by

infection with the bacteria Bartonella henselae and Bartonella quintana.

• It occurs almost exclusively in immunocompromised patients, typically men

with human immunodeficiency virus (HIV) infection.

• Presents as multiple, elevated, pink skin or mucosal lesions.

• This process may also involve visceral organs, such as the liver.
 HISTOPATHOLOGY Epithelioid
endothelial cells
in association
• Bacillary angiomatosis usually shows a with marked
vaguely lobular growth pattern, with acute
capillary-sized vessels lined by epithelioid inflammation.
endothelial cells with clear cytoplasm.

• Numerous stromal neutrophils are seen, and

amorphous, eosinophilic aggregates that
contain fibrin and bacilli.
Showing
epithelioid
• The bacilli may be identified with a
endothelia
Warthin–Starry stain.
l cells,
neutrophil
s, and
eosinophili
c debris.
 HEMANGIOMA
 Rapid
endothelial cell
proliferation in
early infancy,
followed by
• Hemangiomas are lesions that are not present at involution over
birth. time.

• They manifest within the first month of life, exhibit  Another

a rapid proliferative phase, and slowly involute to hypothesis
nonexistent. postulates that
placental cells,
such as the
• Hemangiomas of the oral cavity are not common
trophoblast,
pathologic entities, but the head and neck are may be the cell
common sites. of origin for
hemangiomas
 PATHOPHYSIOLOGY

The more common

Undifferentia Retiform Final
capillary
ted capillary developmen developmen
network tal stage tal stage hemangioma
stage • Begins at • Begins at two represents an
• Primitive about 48 days months of arrest in the
mesenchyme of embryonic development
development development of
is nourished by
an interlacing • Replacement the mesenchyme
system of • Separate of the primordia in the
blood spaces venous and immature
arterial stems plexiform undifferentiated
• Non appear on network by the capillary network
distinguishable either side of mature
arterial and the capillary vascular stage. As
venous network channels differentiation
channels progresses,
MULLIKEN AND GLOWACKI CLASSIFICATION
WATSON AND MCCARTHY CLASSIFICATION

Angioblastic/
Capillary Cavernous Racemose
hemangioma hemangioma Hypertrophic hemangioma
hemangioma

Diffuse Nevus Hereditary

Metastasizing vinosus/
Systemic hemorrhagic
hemangioma Port wine
hemangioma stain telangiectasia
 CLINICAL FEATURES
• Appears as a flat or raised lesion of the mucosa,
usually deep red or bluish red and seldom well INVESTIGATIONS
circumscribed.
• Angiography
• They are readily compressible and fill slowly when • Ultrasonography
released. Common sites : Lips, tongue, buccal mucosa • Contrast-enhanced MRI
and palate. • Radiographically, intraosseous hemangioma
shows honeycombed pattern that is well
• The tumor often is traumatized and undergoes demarcated from normal bone.
ulceration and secondary infection. • CT scans often show an expansile process with
a high-density amorphous mass
• Certain tiny vascular formations of lip vessels called
‘microcherry’, ‘glomerulus’ and ‘venous lake’ have
been described.
 Angiography showing the main
vessels supplying the
intraosseous hemangioma.

Ref - Elif B, Derya Y, Gulperi K, Sevgi B. Intraosseous hemangioma in the mandible: a

case report. J Clin Exp Dent. 2017;9:e153–6. doi: 10.4317/jced.52864
 HISTOLOGIC FEATURES

• The usual hemangioma is composed of many small capillaries lined by a

single layer of endothelial cells supported by a connective tissue stroma of
varying density.

• Some cases show rather remarkable endothelial cell proliferation.

• The cavernous form of hemangioma consists of large dilated blood sinuses

with thin walls, each showing an endothelial lining.

• The sinusoidal spaces usually are filled with blood, although an admixture
with occasional lymphatic vessels occurs in some instances.
 INTRAMUSCULAR HEMANGIOMA

• The intramuscular hemangioma is one special form of hemangioma

which is quite rare in the head and neck region.

• It arises within normal skeletal muscle, comprises less than 1% of all

hemangiomas and is important chiefly because of the problem in
differential diagnosis and of treatment of the lesion.

• Intramuscular hemangiomas represent a challenge on diagnosis

because they exhibit few signs on clinical examination.

• Often times, the extent of the lesion is not clinically apparent on

examination, and imaging studies frequently define more extensive
lesions than suspected.
 ASSOCIATED SYNDROMES
 Rendu-Osler-Weber syndrome (autosomal dominant inheritance, multiple
telangiectasias, occasional GI tract involvement, occasional CNS involvement).

 Sturge-Weber-Dimitri syndrome (noninherited and nonfamilial, port-wine

stain, leptomeningeal angiomas).

 Kasabach-Merritt syndrome (thrombocytopenic purpura associated with

hemangioma, consumptive coagulopathy, micro angiopathic hemolysis,
intralesional fibrinolysis).

 Maffucci syndrome (hemangiomas of the mucous membranes,

dyschondroplasia).

 Von Hippel-Lindau syndrome (genetic transmission variable, hemangiomas of

the cerebellum or the retina, cysts of the viscera).

 Klippel-Trenaunay-Weber syndrome (port-wine stain, angiomatosis of the

extremities).
 HEMANGIOENDOTHELIOMA
• Hemangioendothelioma is a varied group of proliferative and
neoplastic vascular lesions, which have a biological behavior that
falls somewhere between the benign hemangioma and malignant
angiosarcoma.

• Chromosomal translocation involving chromosomes 1 and 3 [t(1;3)

(p36.3;q25)] - Epithelioid hemangioendothelioma.
 CLINICAL FEATURES

• Age : The tumor is may be found at any age or The malignant hemangioendothelioma is
even at birth. Commonly seen during the similar to the hemangioma in appearance and
second and third decades of life. is usually manifested clinically as a flat or
slightly raised lesion of varying size, dark
• Gender : Female predominance – 2:1 red or bluish red, sometimes ulcerated and
showing a tendency to bleed after even slight
• Site : Skin and subcutaneous tissues. Oral - lips, trauma.
palate, gingiva, tongue and centrally within the
maxilla and mandible. Bone may be involved by the tumor,
producing a destructive process.
• Pain and localized swelling may be present.
 HISTOPATHOLOGICAL FEATURES

• The lesion is poorly circumscribed, usually exhibiting biphasic proliferation

of venous or capillary vessels.

• Presence of dilated and congested veins with inactive endothelial cell nuclei
and with occasional thrombi or phleboliths.

• These are intermixed with solid sheets of epithelioid (epithelioid

hemangioendothelioma) or spindle shaped (spindle cell
hemangioendothelioma) mesenchymal cells with minimal dysplasia, few
mitotic figures, and minimal differentiation toward a vascular lumen or
channel.
 EPITHELOID HEMANGIOENDOTHELIOMA

The epithelioid cells have abundant eosinophilic cytoplasm, may contain vacuoles
(primitive lumina), and may be admixed with smooth muscle bundles.

Special stain -
Ulex europaeus
MARKERS -
CD31, CD34

Januzis, G., Sakalys, D., Krukis, M.M. et al. Maxillary epithelioid hemangioendothelioma: an especially rare
malignant tumor mimicking periodontal disease. BMC Oral Health 20, 309 (2020).
 SPINDLE CELL
HEMANGIOENDOTHELIOMA

• The lesional cells of the spindle cell

hemangioendothelioma are rather bland, bipolar
mesenchymal fibroblast like cells which may contain
vacuoles, presumed to be abortive or primitive
vascular lumina.

• Epithelioid cells are usually seen in small numbers in

scattered areas and the associated dilated venous
channels are more prone to contain thrombi and
phleboliths than are those of the epithelioid
hemangioendothelioma.

Morais TML, Sánchez-Romero C, Ribeiro L, Faé DS, Verner FS, de Almeida OP, de Aquino SN.
Kaposiform Hemangioendothelioma of the Oral Cavity: A Rare Tumor with an Unusual Location. Head
Neck Pathol. 2021 Dec;15(4):1421-1425. doi: 10.1007/s12105-021-01316-6. Epub 2021 Mar 13. PMID:
33715098; PMCID: PMC8633177.
 OTHER TYPES

Kaposiform
• Admixture of cells. Similar to capillary hemangioma.
hemangioendothelioma
Polymorphous • Consists of mixture of solid and primitive vascular and angiomatous
hemangioendothelioma endothelial areas.

Nodule of kaposiform
hemangioendothelioma, showing
Kaposi sarcoma– like spindled cells
and better formed small capillaries.

Stromal fibrosis, crescentic vascular spaces, and

spindled endothelial proliferation in kaposiform
hemangioendothelioma.
Textbook of bone and soft tissue pathology – Andrew
L.Folpe, Carrie Y. Inwards
 HEMANGIOPERICYTOMA

• Hemangiopericytoma is a tumor thought to be derived from

pericytes.

• Distinctly different vascular neoplasm which is usually benign

but has a definite malignant counterpart.

• Head and neck lesions represent 16–25% of all reported

hemangiopericytomas, and the tumor represents 2–3% of all
soft tissue sarcomas in humans.
 CLINICAL FEATURES
• Age : Prior to the second decade or after the seventh decade.

• Gender : There is no gender predilection.

• Rapidly enlarging red or bluish mass, sessile or somewhat pedunculated, soft or

rubbery, is usually painless, may demonstrate surface lobularity or telangiectasia and is
relatively well demarcated from the surrounding mucosa.

• Intraosseous examples have been reported.

• The oral/pharyngeal mucosa is one of the most common locations for infantile
hemangiopericytoma.

• This lesion is usually multiple and congenital, and often demonstrates an alarmingly
rapid rate of enlargement after birth.
 HISTOPATHOLOGIC FEATURES
• Numerous vascular channels with plump endothelial nuclei and a
surrounding, tightly packed proliferation of oval and spindled cells,
hyperchromatic nuclei and a moderate amount of cytoplasm.

• The cells have indistinct cytoplasmic borders.

• The tumor cells are more spindled and arranged in either short
fascicles or in a disorganized fashion (patternless pattern)

• The branching vascular channels of varying sizes is often described

as a ‘staghorn’ or ‘antler-like’ pattern. Reticulin staining demonstrates
pericytes.
• Older, less aggressive lesions tend to have less cellularity and may
have a largely mucoid interstitial appearance, which can be mistaken Lesional cells are immunoreactive
for myxoid lipoma or myxoid liposarcoma. for vimentin, factor XIIIa antigen,
HLA-DR antigen and QBEND/10
• Focal cartilage production may rarely be seen and such lesions must (CD34).
be differentiated from mesenchymal chondrosarcoma.
HSP Atlas - Histopathology Atlas (umk.pl)
 KAPOSI SARCOMA

• Kaposi’s sarcoma (Multiple Idiopathic Hemorrhagic Sarcoma) is a multicentric

proliferation of vascular and spindle cell components

• Kaposi’s sarcoma has four major clinical presentations.

• Oral involvement in this form of the disease is quite unusual but when it occurs it does
so as soft, bluish nodules of the palatal mucosa or gingiva.

• Cutaneous multifocal blue-red nodules develop on the lower extremities and slowly
increase in size and numbers, with some lesions regressing while new ones are forming
on adjacent or distant skin.
 • The classic variant • Presents as a localized
is often associated or generalized
with altered enlargement of lymph
immune states as node chains, including
well as the cervical nodes.
lymphoreticular • Visceral involvement
and other and minimal skin or
malignancies. mucous membrane
involvement.
Endemic
• In the head and neck
Classic (Chronic) (Lymphadenopathic region, salivary glands
, African) may be affected.

Immunosuppressio
nassociated AIDS-related
(Transplant)

• Seen in 1–4% of renal • Seen in approximately

transplant patients 40% of homosexual
AIDS patients
• Manifested one or two
• Young adults or early
years after
transplantation. middle-aged males

• Sarcomatous • Cutaneous lesions,

involvement occurs on oral lesions can also
 CLINICAL FEATURES

• Site : Gingiva, tongue, uvula, tonsils, pharynx, and trachea.

• Early oral mucosal sarcomas are flat and slightly blue, red or
purple plaques, either focal or diffuse, may be completely
asymptomatic.

• With time, lesions become more deeply discolored and

surface papules and soft nodules develop, or may become
exophytic and ulcerated, and may bleed, usually remaining
less than 2 cm in size.

• Cervical lymph nodes and salivary gland enlargement may

also be seen.

• Sometimes associated with overlying candidiasis.

 HISTOPATHOLOGY

PATCH STAGE

PLAQUE STAGE

NODULAR STAGE
Proliferation of small blood Increased number of
vessels and capillaries capillaries or dilated Histologic features similar
Plump, mildly atypical vascular channels to plaque stage but are
endothelial cells Proliferating sheets of more prominent
Mononuclear sarcomatous or atypical
inflammatory cell infiltrate spindle cells
Inconspicuous perivascular Lesional cells – enlarged,
proliferation of spindle hyperchromatic nuclei,
cells with minimal atypia mild to moderate
pleomorphism
Minimal mitotic activity

Immunoreactivity – CD31, CD34

Ulex europaeus
 Patch stage - Newly formed
vessels protruding into a
larger vascular space
characteristic of the
promontory sign
Plaque stage - Large numbers of intracellular
and extracellular eosinophilic hyaline
globules are visible in this field. The arrows
indicate so-called "autolumination", with
paranuclear vacuoles containing
erythrocytes

Nodular stage – Numerous blood filled spaces

in a background of malignant spindle cells
Ref - Grayson, Wayne & Pantanowitz, Liron. (2008). Histological variants of Kaposi
sarcoma. Diagnostic pathology. 3. 31. 10.1186/1746-1596-3-31.
 ANGIOSARCOMA

• Angiosarcomas are rare, aggressive malignant sarcomas arising from

vascular endothelial cells.

• They constitute 2% of all the soft tissue sarcomas.

• Tumors may arise at any anatomic location, but the skin of the head
and neck and breast are frequently involved Vimentin
Factor VIII-related
• Angiosarcoma of the oral cavity is extremely rare antigen
p53
• Angiosarcomas are rare in children and young adults.

• Usually presents as a non-healing wound - Proliferative lesion with a

bluish hue and bleeds on touch

Ref - Terada T. Angiosarcoma of the oral cavity. Head Neck Pathol. 2011
Mar;5(1):67-70. doi: 10.1007/s12105-010-0211-5. Epub 2010 Oct 6.
PMID: 20924728; PMCID: PMC3037457.
Solid tumor composed of atypical spindle and polygonal cells
with hyperchromatic nuclei and nucleoli (fig. A). Mitotic figure
were scattered. The tumor cells occasionally showed
intracytoplasmic vacuole. In some areas, vasoformative
channels containing red blood cells were recognized (fig. B).
Lymphoid tissues with follicles were also scattered within the
tumor. The tumor was invasive into the surrounding tissue
(fig. C) and lymphovascular permeation was recognized in
certain areas.
 REFERENCES
1. Shafers textbook of oral pathology – 9TH edition

2. Textbook of Oral & Maxillofacial pathology – Allen and Neville – 1 st south Asian edition

3. Bone and soft tissue pathology - Andrew L. Folpe, Carrie Y. Inwards

4. Terada T. Angiosarcoma of the oral cavity. Head Neck Pathol. 2011 Mar;5(1):67-70. doi:
10.1007/s12105-010-0211-5. Epub 2010 Oct 6. PMID: 20924728; PMCID: PMC3037457.

5. Grayson, Wayne & Pantanowitz, Liron. (2008). Histological variants of Kaposi sarcoma. Diagnostic
pathology. 3. 31. 10.1186/1746-1596-3-31

6. Januzis, G., Sakalys, D., Krukis, M.M. et al. Maxillary epithelioid hemangioendothelioma: an especially
rare malignant tumor mimicking periodontal disease. BMC Oral Health 20, 309 (2020).
https://doi.org/10.1186/s12903-020-01291-4

7. Lomeli Martinez SM, Carrillo Contreras NG, Gómez Sandoval JR, Zepeda Nuño JS, Gomez Mireles JC,
Varela Hernández JJ, Mercado-González AE, Bayardo González RA, Gutiérrez-Maldonado AF. Oral
Pyogenic Granuloma: A Narrative Review. Int J Mol Sci. 2023 Nov 28;24(23):16885. doi:
10.3390/ijms242316885. PMID: 38069207; PMCID: PMC10706684.

8. HSP – Histopathology Atlas