Diseases of the Blood vessels - 2

Dr. Geener .K .John

Recommended reading –
Pages 378- 396 from
Robbins – Basic Pathology 10th edition
SLO# 1 : Discuss the morphological types of aneurysms with their
causes and complications.
SLO# 2 : Discuss the pathogenesis and morphology of Syphilitic
aortitis.
SLO# 3 : Describe changes in Aortic dissection.
SLO# 4 : Discuss the clinical presentation, microscopic changes and
complications of vasculitis.
SLO# 5 : Classify the vascular tumors.
SLO# 6 : Discuss the gross and microscopic features of capillary and
cavernous hemangioma.
SLO# 7 : Discuss the aetiology gross and microscopic features of
angiosarcoma.
 Aneurysm
Localised abnormal dilatation of a blood vessel
or the wall of heart – due to weakening of
the wall
hematoma

True & false aneurysm

A pseudoaneurysm happens as a result
of injury to a blood vessel. The artery
leaks blood, which then pools near the
damaged spot. It's different from a true
aneurysm, which happens when the
wall of a blood vessel stretches and
forms a bulge. Most pseudoaneurysms
are complications from medical
procedures.
 Morphological classification
The bulge in this type of
an aneurysm is formed
Saccular only on one side of the
vessel

Fusiform The bulge in this type of an

aneurysm is formed on all sides
of a blood vessel
Cylindrical

Berry most common

The bulge in this type of an aneurysm looks like a berry on a narrow stem. It is
the most common type of brain aneurysm.
Causes
Atherosclerosis
Hypertension
Cystic medial degeneration
Syphilis
Inadequate connective tissue synthesis
Marfan syndrome
Ehlers-Danlos syndrome
Congenital defects – berry aneurysm
Infections – mycotic aneurysm
Atherosclerotic aneurysm
Most common sites – abdominal aorta (AAA),
followed by descending thoracic aorta & arch
Pathogenesis –
Media undergoes degeneration &
necrosis thinning & weakening
Gross – site is below the origin of
renal artery & above the bifurcation
of aorta. Saccular or fusiform in shape
Frequently contains a mural thrombus
Complications
Obstruction of a branch vessel
resulting in ischemia
Embolism from atheromatous
material or from thrombus
Compression on adjacent structures
Ureter, Vertebral bodies
Abdominal mass
Rupture – risk is related to size
Syphilitic aortitis
A feature of tertiary syphilis
Aneurysm is a major complication of aortitis
Pathogenesis – inflammation (obliterative
endarteritis) around vasa vasorum of thoracic
aorta  ischemic medial injury
Morphology –
Most common sites – ascending aorta & arch
Contraction of fibrous scar  wrinkling called
. There is patchy wrinkling and thickening due to
“tree bark appearance” irregular scarring and fibrosis.

Involvement of aortic root aortic incompetence

& LVH, known as ‘cor bovinum”
Aortic dissection
Blood enter through a tear in intima
and spreads along the planes of
the media forming a blood filled
channel in the aortic wall
HT is the most imp. risk factor
Another distal tear produces
“double barreled aorta”
 Vasculitis
Inflammation of vessel wall
Infectious or non infectious (more imp)
Pathogenesis of non infectious – immunological
1- Immune complex deposition

Serum sickness, SLE

2- Anti neutrophil cytoplasmic Abs. (ANCA) mediated

PR3- ANCA in Granulomatosis with polyangiitis

MPO- ANCA in Microscopic polyangiitis
3- Direct injury by anti endothelial cell Abs.

Kawasaki disease
 Giant cell arteritis
Most common vasculitis. Usually > 70 years
Usually affects temporal artery - thickened &
cord like
Microscopy -Granulomatous
reaction around fragmented
internal elastic lamina
Takayasu arteritis
Typically involve aortic arch
& it’s major branches
“Pulseless disease”
Histology same as giant cell
arteritis but ♀ < 50
Kawasaki disease
Mucocutaneous lymph node syndrome
Erosions of oral mucosa, skin & conjunctiva
An acute febrile illness of infancy and
childhood - associated with an arteritis
affecting large, medium-sized vessels.
Clinical significance – involvement of
coronary arteries  acquired heart disease
in children.
a condition that mainly affects children under the age of 5
Granulomatosis with polyangiitis
(Wegener granulomatosis)
Characterized by the triad of
(1) Granulomas of the upper respiratory tract (ear,
nose, sinuses, throat) or the lower respiratory tract
(lung) or both

(2) Vasculitis affecting small to medium-sized vessels,

most prominent in the lungs and upper airways
an uncommon disorder that causes inflammation of the blood
vessels in your nose, sinuses, throat, lungs and kidneys.
(3) Glomerulonephritis
PR3- ANCA positive in most cases
Thromboangiitis obliterans
Buerger disease
Presents with intermittent
claudication
Almost exclusively in men under 35 years who
are heavy smokers of cigarettes
Characterized by segmental, thrombosing,
acute and chronic inflammation of medium-
sized and small arteries, principally the tibial
and radial arteries
Vascular tumors - Classification
Benign Neoplasms
Haemangioma
Capillary hemangioma
Juvenile hemangioma
Pyogenic granuloma
Cavernous hemangioma
Lymphangioma
Capillary lymphangioma
Cavernous lymphangioma
Glomus tumour
Classification Contd.
Intermediate-Grade Neoplasms
Kaposi sarcoma
Hemangioendothelioma
Hemangiopericytoma

Malignant Neoplasms
Angiosarcoma
Capillary hemangioma
Most common vascular tumour
Commonly seen in the skin, subcutaneous tissues and
mucous membranes of the oral cavities and lips
Gross – Size vary from a few millimeters up to several
centimeters in diameter, they are bright red to blue
and level with
the surface of the skin or
slightly elevated, with intact
covering epithelium
Microscopy
Usually lobulated but unencapsulated,
aggregates of closely packed, thin-walled
capillaries, usually blood filled and lined by a
flattened endothelium
 A cavernous hemangioma
happens when capillaries – small
blood vessels that connect arteries
Cavernous hemangioma and veins – swell and form a
noncancerous mass called an
angioma.
Gross - The usual cavernous hemangioma is a
red-blue, soft, spongy mass 1 to 2 cm in
diameter
Microscopy - The mass is sharply defined, but
not encapsulated, and made up of large,
cavernous vascular spaces, partly or
completely filled with
blood, separated by a scant
connective tissue stroma
 noncancerous fluid-filled cysts that

Lymphangioma form in children, often on the head

and neck

Simple (Capillary) lymphangioma - Composed

of small lymphatic channels, tend to occur
subcutaneously in the head and neck region
and in the axilla.
Cavernous lymphangioma (Cystic Hygroma)
Occur in children in the neck or axilla and only
rarely retroperitoneally.
Intermediate grade tumors
Kaposi sarcoma
Caused by Kaposi sarcoma herpes virus(KSHV)
Important because of its frequent occurrence in
patients with AIDS.
Hemangioendothelioma
Clinically & histologically intermediate between the
benign, well-differentiated hemangiomas and the
frankly anaplastic angiosarcomas
Hemangiopericytoma
These tumors are derived from pericytes, which are
normally arranged along capillaries and venules.
Angiosarcoma
Malignant endothelial neoplasms
Hepatic angiosarcomas are associated with
distinct carcinogens
Arsenic
Thorotrast
Polyvinyl chloride (PVC)
Can follow lymphedema after mastectomy
Morphology
Gross – can be found anywhere in the body
but most often skin, soft tissue, breast and
Angiosarcoma is a rare cancer that develops
liver in the inner lining of blood vessels and lymph
vessels
Begin as small nodules,
become large fleshy mass
with central necrosis &
hemorrhage
Microscopy
Degree of differentiation can vary
Undifferentiated tumors show spindle cells
arranged as sheets
Endothelial origin demonstrated using
endothelial cell markers CD 31 & vWF

CD 31+