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VEINS AND LYMPHATICS
Dr Zainab
VENOUS DISEASES
• Varicose veins and
phlebothrombosis/thrombophlebitis account
for at least 90% of cases of clinically relevant
venous disease.
Varicose Veins
• Varicose veins are abnormally dilated
tortuous veins produced by chronically
increased intraluminal pressures and
weakened vessel wall support.
HEMORRHOIDS
• • Hemorrhoids are varicose dilations of the
venous plexus at the anorectal junction that
result from prolonged pelvic vascular
congestion associated with pregnancy or
straining to defecaTE
• THEY are a source of bleeding and are prone
to thrombosis and painful ulceration.
Thrombophlebitis and
Phlebothrombosis
• Thrombosis of deep leg veins accounts for
more than 90% of cases of thrombophlebitis
and phlebothrombosis.
Etiology
• Virchow’s triad:
–Venous stasis
–Damage of endothelium
–Hypercoagulability
Risk Factors for Virchow’s Triad
• Venous stasis —incompetent valves, inactivity,
obesity, heart failure, orthopedic and pelvic
surgeries
• Endothelial damage—trauma from various
causes, external pressure
• Hypercoagulability— blood disorders, sepsis,
pregnancy, hormones, smoking
Assessment of DVT
• 50% are asymptomatic unless the clot is
in the ileofemoral vein.
• Symptomatic patients and those with
ileofemoral clot have edema ,redness,
pain, warmth, decreased movement,
+Homan’s sign (20% reliable).
• Dx Tests: Duplex scanning, venogram, D-
dimer blood test
DVT
Other sites of venous thrombi
• Periprostatic venous plexus in males
• Pelvic venous plexus in females
• Large veins in the skull and the dural sinuses
• Peritoneal infections, including peritonitis,
appendicitis, salpingitis, and pelvic abscesses
• Conditions associated with hypercoagulability
(e.G., Polycythemia vera)
DVT RISK FACTORS
• PROLONGED IMMOBILIZATION RESULTING IN
VENOUS STASIS .
• THE POSTOPERATIVE STATE
• CONGESTIVE HEART FAILURE
• PREGNANCY
• ORAL CONTRACEPTIVE USE
• MALIGNANCY, OBESITY, MALE SEX, AND AGE
OVER 50 YEARS.
• INHERITED DEFECTS IN COAGULATION FACTORS
DVT
“Trousseau syndrome.”
• Episodes of vessel inflammation due to blood
clot (thrombophlebitis) which are recurrent or
appearing in different locations over time
(thrombophlebitis migrans or migratory
thrombophlebitis).
Homan sign
• In some cases OF DVT , pain is elicited by
pressure over affected veins,squeezing the calf
muscles, or forced dorsiflexion of the foot,
THIS IS CALLED HOMAN SIGN
COMPLICATION OF DVT
• PULMONARY EMBOLISM
Lymphangitis and Lymphedema
Lymphangitis
• Inflammation or an infection of the lymphatic
channels that occurs as a result of infection at
a site distal to the channel.
• Most common cause of lymphangitis in
humans is streptococcus pyogenes (group a
strep)
Lymphangitis
• Clinically, the inflamed lymphatics appear as red,
painful
• Subcutaneous streaks, usually associated with
tender enlargement of draining lymph nodes
(acute lymphadenitis).
• If bacteria are not contained within the lymph
nodes, they can pass into the venous circulation
and cause bacteremia orsepsis.
Lymphedema
… Also known as lymphoedema and lymphatic
edema, is a condition of localized fluid retention
and tissue swelling caused by a compromised
lymphatic system.
CAUSES OF LYMPHEDEMA
• Tumors involving either the lymphatic
channels or the regional lymph nodes
• Surgical procedures that sever lymphatic
connections (e.g., axillary lymph nodes in radical
mastectomy)
• Postradiation fibrosis
• Filariasis
• Postinflammatory thrombosis and scarring
Tumors of blood vessels
Benign Tumors and Tumor-Like
Conditions
Vascular Ectasias
• Ectasia is a generic term for any local dilation
of a structure while
• telangiectasia is permanent dilation of
preexisting small vessels (capillaries, venules,
and arterioles, usually in the skin or mucous
membranes) that forms a discrete red lesion.
These lesions can be congenital or acquired
and are not true neoplasms.
TYPES
• Nevus flammeus (a “birthmark”), the most
common form of vascular ectasia, is a light
pink to deep purple flat lesion on the head or
neck composed of dilated vessels.
• Most regress spontaneously.
port wine stain
• Congenital disorder and special form of nevus
flammeus.
• They grow during childhood, thicken the skin
surface, and do not fade with time.
• Such lesions occurring in the distribution of
the trigeminal nerve are associated with the
Sturge- Weber syndrome (also called
encephalotrigeminal angiomatosis).
Spider telangiectasias
• Non-neoplastic vascular lesions AND manifest
as pulsatile arrays of dilated subcutaneous
arteries or arterioles (the “legs” of the spider)
about a central core (the spider’s “body”) that
blanch with pressure
• Commonly occur on the face, neck, or upper
chest and most frequently are associated with
hyperestrogenic states (e.G., In pregnant
women or patients with cirrhosis).
Hereditary hemorrhagic
telangiectasia (HHT),
• Also known as osler–weber–rendu disease
and osler–weber–rendu syndrome
• Rare autosomal dominant genetic disorder
that leads to abnormal blood vessel formation
in the skin, mucous membranes, and often in
organs such as the lungs, liver, and brain.
Contd
• Caused by mutations in genes that encode
components of the tgf-β signaling pathway in ecs.
• Congenital
• Spontaneously rupture, causing serious
epistaxis,gastrointestinal bleeding, or hematuria.
Hemangiomas
Benign (non-cancerous) tumor in infants. This
abnormal cluster of small blood vessels appears
on or under the skin, typically within one to
three weeks after birth.
• Malignant transformation is rare.
Capillary hemangiomas
• Most common type
• Occur in the skin, subcutaneous tissues, and
mucous
• Membranes of the oral cavities and lips, as well
as in the
• Liver, spleen, and kidney
• Histologically,they are composed of thin-walled
capillaries with scant stroma
• • juvenile hemangiomas
Juvenile hemangiomas
• • (Also-called “strawberry hemangiomas”)
• Of the newborn skin are extremely common (1 in
• 200 births) and can be multiple
• These grow rapidly for a few months but then
fade by 1 to 3 years of age, with complete
regression by 7 years of age in the vast majority
Of cases.
Pyogenic granulomas
• Are capillary hemangiomas
• Rapidly growing
• Red pedunculated lesions on the skin,
gingival, or oral mucosa.
Contd
• Microscopically they resemble exuberant granulation
tissue
• bleed easily and often ulcerate
• Roughly one fourth of the lesions develop after
trauma, reaching a size of 1 to 2 cm within a few weekS
• TREATMENT : Curettage and cautery usually are
curative
• Pregnancy tumor (granuloma gravidarum) is a pyogenic
granuloma that occurs infrequently in the gingiva of
pregnant women. These lesions may spontaneously
regress (especially after pregnancy) or undergo fibrosis,
but occasionally require surgical excision.
Cavernous hemangiomas
• COMPOSED OF LARGE, DILATED VASCULAR CHANNELS
• THEY ARE MORE INFILTRATIVE, FREQUENTLY INVOLVE
DEEP STRUCTURES, AND DO NOT SPONTANEOUSLY
REGRESS. THESE LESIONS ARE LOCALLY DESTRUCTIVE
• INTRAVASCULAR THROMBOSIS AND DYSTROPHIC
CALCIFICATION ARE COMMON.
• THEY ARE VULNERABLE TO TRAUMATIC ULCERATION
AND BLEEDING.
MORPHOLOGY
• MICROSCOPIC EXAMINATION:
• MASS IS SHARPLY DEFINED BUT
UNENCAPSULATED AND IS COMPOSED OF
LARGE BLOOD-FILLED VASCULAR SPACES
SEPARATED BY CONNECTIVE TISSUE STROMA
Lymphangioma
BENIGN LYMPHATIC COUNTERPART OF
HEMANGIOMAS.
• SIMPLE (CAPILLARY) LYMPHANGIOMAS ARE
SLIGHTLY ELEVATED OR SOMETIMES PEDUNCULATED
LESIONS UP TO 1 TO 2 CM IN DIAMETER THAT OCCUR
PREDOMINANTLY IN THE HEAD, NECK, AND AXILLARY
SUBCUTANEOUS TISSUES.
• HISTOLOGICALLY LYMPHANGIOMAS ARE COMPOSED
OF NETWORKS OF ENDOTHELIUM-LINED SPACES THAT
ARE DISTINGUISHED FROM CAPILLARY CHANNELS
ONLY BY THE ABSENCE OF BLOOD CELLS.
Cavernous lymphangiomas
(cystic hygromas)
• Found in the neck or axilla of children, and
more rarely in the retroperitoneum. Cavernous
lymphangiomas can be large (up to 15 cm),
filling the axilla or producing gross deformities
of the neck
contd
• These lesions are composed of massively
dilated lymphatic spaces lined by ecs and
separated by intervening connective tissue
stroma containing lymphoid aggregates. The
tumor margins are indistinct and
unencapsulated, making definitive resection
difficult.Cavernous lymphangiomas of the
neck are common in turner syndrome.
Glomus Tumors (Glomangiomas)
• Benign, exquisitely painful tumors arising from
specialized smcs of glomus bodies,
arteriovenous structures involved in
thermoregulation.
• Most commonly are found in the distal
portion of the digits, especially under the
fingernails.
• Excision is curative.
Intermediate-Grade (Borderline)
Tumors
• Kaposi sarcoma
• Hemangioendotheliomas
KAPOSI SARCOMA
• KAPOSI SARCOMA (KS) IS A VASCULAR
NEOPLASM CAUSED BY KAPOSI SARCOMA
HERPESVIRUS (KSHV, ALSO KNOWN AS
HUMAN HERPESVIRUS-8, OR HHV-8).
• MOST COMMON IN PATIENTS WITH AIDS AND
ITS PRESENCE IS USED AS A CRITERION FOR
THE DIAGNOSIS.
TYPES
• CLASSIC KS
• ENDEMIC AFRICAN KS
• TRANSPLANTATION-ASSOCIATED KS
…AIDS-ASSOCIATED (EPIDEMIC) KS
• Classic KS
• A DISORDER OF OLDER MEN
• CAN BE ASSOCIATED WITH MALIGNANCY OR ALTERED
IMMUNITY BUT IS NOT ASSOCIATED WITH HIV
INFECTION.
• CLASSIC KS MANIFESTS AS MULTIPLE RED-PURPLE SKIN
PLAQUES OR NODULES, USUALLY ON THE DISTAL
LOWER EXTREMITIES
• PROGRESSIVELY INCREASE IN SIZE AND NUMBER AND
SPREAD PROXIMALLY.
• THE TUMORS ARE ASYMPTOMATIC AND REMAIN
LOCALIZED TO THE SKIN AND SUBCUTANEOUS TISSUE.
Endemic African KS
• TYPICALLY OCCURS IN YOUNGER (UNDER 40 YEARS OF
AGE) HIV-SERONEGATIVE INDIVIDUALS
• INVOLVES LYMPH NODES MUCH MORE FREQUENTLY
THAN IN THE CLASSIC VARIANT
• A PARTICULARLY SEVERE FORM, WITH PROMINENT
LYMPH NODE AND VISCERAL INVOLVEMENT, OCCURS
IN PREPUBERTAL CHILDREN
• PROGNOSIS IS POOR, WITH AN ALMOST 100%
MORTALITY WITHIN 3 YEARS.
• Transplantation-associated KS
• occurs in solid organ transplant
• recipients in the setting of T-cell immunosuppression.
• In these patients, the risk for KS is increased 100-fold
• It pursues an aggressive course and often involves
lymph nodes, mucosa, and viscera
• Cutaneous lesions may be absent
• Lesions often regress with attenuation of
immunosuppression, but at the risk for organ rejection.
AIDS-associated (epidemic) KS
• IT IS THE MOST COMMON HIV-RELATED
MALIGNANCY.
• AIDS-ASSOCIATED KS OFTEN INVOLVES LYMPH
NODES AND DISSEMINATES WIDELY TO
VISCERA EARLY IN ITS COURSE. MOST
PATIENTS EVENTUALLY DIE OF OPPORTUNISTIC
INFECTIONS RATHER THAN FROM KS.
Pathogenesis
• Virtually all KS lesions are infected by KSHV (HHV-
8)
• Like Epstein-Barr virus, KSHV is a γ-herpesvirus
• It is transmitted both through sexual contact and
potentially via oral secretions and cutaneous
exposures
• KSHV and altered T-cell immunity probably are
required for KS development
• In older adults, diminished T-cell immunity may
be related to aging.
MORPHOLOGY
• In classic kaposi sarcoma
• Cutaneous lesions progress through three stages:
patch, plaque, and nodule.
• Patches are pink, red, or purple macules, typically
confined to the distal lower extremities
• Microscopic examination
dilated, irregular, and angulated blood vessels lined
by ecs and an interspersed infiltrate of chronic
inflammatory cells, sometimes containing hemosiderin
• These leions can be difficult to distinguish from
granulation tissue.
contd
• With time, lesions spread proximally and
become larger, violaceous,raised plaques
composed of dilated, jagged dermal vascular
channels lined and surrounded by plump
spindle cells. Other prominent features
include extravasated red cells, hemosiderin-
laden macrophages, and other mononuclear
cells.
CONTD
• Eventually nodular, more overtly neoplastic, lesions
appear.
• These are composed of plump, proliferating spindle
cells
• Mostly located in the dermis or subcutaneous tissues
• Often with interspersed slitlike spaces
• The spindle cells express both EC and SMC markers.
Hemorrhage and hemosiderin deposition are more
pronounced, and mitotic figures are common.
• Nodular stage often is accompanied by nodal and
visceral involvement, particularly in the african and
aids-associated variants.
Clinical Features
• Most primary KSHV infections are
asymptomatic.
• Classic KS is initially largely restricted to the
surface of the body, and surgical resection
usually is adequate for an excellent prognosis.
Malignant Tumors
• Angiosarcomas

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Veins and lymphatics

  • 2. VENOUS DISEASES • Varicose veins and phlebothrombosis/thrombophlebitis account for at least 90% of cases of clinically relevant venous disease.
  • 3. Varicose Veins • Varicose veins are abnormally dilated tortuous veins produced by chronically increased intraluminal pressures and weakened vessel wall support.
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  • 26. HEMORRHOIDS • • Hemorrhoids are varicose dilations of the venous plexus at the anorectal junction that result from prolonged pelvic vascular congestion associated with pregnancy or straining to defecaTE • THEY are a source of bleeding and are prone to thrombosis and painful ulceration.
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  • 32. Thrombophlebitis and Phlebothrombosis • Thrombosis of deep leg veins accounts for more than 90% of cases of thrombophlebitis and phlebothrombosis.
  • 33. Etiology • Virchow’s triad: –Venous stasis –Damage of endothelium –Hypercoagulability
  • 34. Risk Factors for Virchow’s Triad • Venous stasis —incompetent valves, inactivity, obesity, heart failure, orthopedic and pelvic surgeries • Endothelial damage—trauma from various causes, external pressure • Hypercoagulability— blood disorders, sepsis, pregnancy, hormones, smoking
  • 35. Assessment of DVT • 50% are asymptomatic unless the clot is in the ileofemoral vein. • Symptomatic patients and those with ileofemoral clot have edema ,redness, pain, warmth, decreased movement, +Homan’s sign (20% reliable). • Dx Tests: Duplex scanning, venogram, D- dimer blood test
  • 36. DVT
  • 37. Other sites of venous thrombi • Periprostatic venous plexus in males • Pelvic venous plexus in females • Large veins in the skull and the dural sinuses • Peritoneal infections, including peritonitis, appendicitis, salpingitis, and pelvic abscesses • Conditions associated with hypercoagulability (e.G., Polycythemia vera)
  • 38. DVT RISK FACTORS • PROLONGED IMMOBILIZATION RESULTING IN VENOUS STASIS . • THE POSTOPERATIVE STATE • CONGESTIVE HEART FAILURE • PREGNANCY • ORAL CONTRACEPTIVE USE • MALIGNANCY, OBESITY, MALE SEX, AND AGE OVER 50 YEARS. • INHERITED DEFECTS IN COAGULATION FACTORS
  • 39. DVT
  • 40. “Trousseau syndrome.” • Episodes of vessel inflammation due to blood clot (thrombophlebitis) which are recurrent or appearing in different locations over time (thrombophlebitis migrans or migratory thrombophlebitis).
  • 41. Homan sign • In some cases OF DVT , pain is elicited by pressure over affected veins,squeezing the calf muscles, or forced dorsiflexion of the foot, THIS IS CALLED HOMAN SIGN
  • 42. COMPLICATION OF DVT • PULMONARY EMBOLISM
  • 44. Lymphangitis • Inflammation or an infection of the lymphatic channels that occurs as a result of infection at a site distal to the channel. • Most common cause of lymphangitis in humans is streptococcus pyogenes (group a strep)
  • 45. Lymphangitis • Clinically, the inflamed lymphatics appear as red, painful • Subcutaneous streaks, usually associated with tender enlargement of draining lymph nodes (acute lymphadenitis). • If bacteria are not contained within the lymph nodes, they can pass into the venous circulation and cause bacteremia orsepsis.
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  • 48. Lymphedema … Also known as lymphoedema and lymphatic edema, is a condition of localized fluid retention and tissue swelling caused by a compromised lymphatic system.
  • 49.
  • 50. CAUSES OF LYMPHEDEMA • Tumors involving either the lymphatic channels or the regional lymph nodes • Surgical procedures that sever lymphatic connections (e.g., axillary lymph nodes in radical mastectomy) • Postradiation fibrosis • Filariasis • Postinflammatory thrombosis and scarring
  • 51. Tumors of blood vessels
  • 52.
  • 53. Benign Tumors and Tumor-Like Conditions
  • 54. Vascular Ectasias • Ectasia is a generic term for any local dilation of a structure while • telangiectasia is permanent dilation of preexisting small vessels (capillaries, venules, and arterioles, usually in the skin or mucous membranes) that forms a discrete red lesion. These lesions can be congenital or acquired and are not true neoplasms.
  • 55. TYPES • Nevus flammeus (a “birthmark”), the most common form of vascular ectasia, is a light pink to deep purple flat lesion on the head or neck composed of dilated vessels. • Most regress spontaneously.
  • 56. port wine stain • Congenital disorder and special form of nevus flammeus. • They grow during childhood, thicken the skin surface, and do not fade with time. • Such lesions occurring in the distribution of the trigeminal nerve are associated with the Sturge- Weber syndrome (also called encephalotrigeminal angiomatosis).
  • 57. Spider telangiectasias • Non-neoplastic vascular lesions AND manifest as pulsatile arrays of dilated subcutaneous arteries or arterioles (the “legs” of the spider) about a central core (the spider’s “body”) that blanch with pressure • Commonly occur on the face, neck, or upper chest and most frequently are associated with hyperestrogenic states (e.G., In pregnant women or patients with cirrhosis).
  • 58. Hereditary hemorrhagic telangiectasia (HHT), • Also known as osler–weber–rendu disease and osler–weber–rendu syndrome • Rare autosomal dominant genetic disorder that leads to abnormal blood vessel formation in the skin, mucous membranes, and often in organs such as the lungs, liver, and brain.
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  • 61. Contd • Caused by mutations in genes that encode components of the tgf-β signaling pathway in ecs. • Congenital • Spontaneously rupture, causing serious epistaxis,gastrointestinal bleeding, or hematuria.
  • 62. Hemangiomas Benign (non-cancerous) tumor in infants. This abnormal cluster of small blood vessels appears on or under the skin, typically within one to three weeks after birth. • Malignant transformation is rare.
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  • 70. Capillary hemangiomas • Most common type • Occur in the skin, subcutaneous tissues, and mucous • Membranes of the oral cavities and lips, as well as in the • Liver, spleen, and kidney • Histologically,they are composed of thin-walled capillaries with scant stroma • • juvenile hemangiomas
  • 71.
  • 72. Juvenile hemangiomas • • (Also-called “strawberry hemangiomas”) • Of the newborn skin are extremely common (1 in • 200 births) and can be multiple • These grow rapidly for a few months but then fade by 1 to 3 years of age, with complete regression by 7 years of age in the vast majority Of cases.
  • 73.
  • 74. Pyogenic granulomas • Are capillary hemangiomas • Rapidly growing • Red pedunculated lesions on the skin, gingival, or oral mucosa.
  • 75. Contd • Microscopically they resemble exuberant granulation tissue • bleed easily and often ulcerate • Roughly one fourth of the lesions develop after trauma, reaching a size of 1 to 2 cm within a few weekS • TREATMENT : Curettage and cautery usually are curative • Pregnancy tumor (granuloma gravidarum) is a pyogenic granuloma that occurs infrequently in the gingiva of pregnant women. These lesions may spontaneously regress (especially after pregnancy) or undergo fibrosis, but occasionally require surgical excision.
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  • 80. Cavernous hemangiomas • COMPOSED OF LARGE, DILATED VASCULAR CHANNELS • THEY ARE MORE INFILTRATIVE, FREQUENTLY INVOLVE DEEP STRUCTURES, AND DO NOT SPONTANEOUSLY REGRESS. THESE LESIONS ARE LOCALLY DESTRUCTIVE • INTRAVASCULAR THROMBOSIS AND DYSTROPHIC CALCIFICATION ARE COMMON. • THEY ARE VULNERABLE TO TRAUMATIC ULCERATION AND BLEEDING.
  • 81. MORPHOLOGY • MICROSCOPIC EXAMINATION: • MASS IS SHARPLY DEFINED BUT UNENCAPSULATED AND IS COMPOSED OF LARGE BLOOD-FILLED VASCULAR SPACES SEPARATED BY CONNECTIVE TISSUE STROMA
  • 82. Lymphangioma BENIGN LYMPHATIC COUNTERPART OF HEMANGIOMAS. • SIMPLE (CAPILLARY) LYMPHANGIOMAS ARE SLIGHTLY ELEVATED OR SOMETIMES PEDUNCULATED LESIONS UP TO 1 TO 2 CM IN DIAMETER THAT OCCUR PREDOMINANTLY IN THE HEAD, NECK, AND AXILLARY SUBCUTANEOUS TISSUES. • HISTOLOGICALLY LYMPHANGIOMAS ARE COMPOSED OF NETWORKS OF ENDOTHELIUM-LINED SPACES THAT ARE DISTINGUISHED FROM CAPILLARY CHANNELS ONLY BY THE ABSENCE OF BLOOD CELLS.
  • 83. Cavernous lymphangiomas (cystic hygromas) • Found in the neck or axilla of children, and more rarely in the retroperitoneum. Cavernous lymphangiomas can be large (up to 15 cm), filling the axilla or producing gross deformities of the neck
  • 84. contd • These lesions are composed of massively dilated lymphatic spaces lined by ecs and separated by intervening connective tissue stroma containing lymphoid aggregates. The tumor margins are indistinct and unencapsulated, making definitive resection difficult.Cavernous lymphangiomas of the neck are common in turner syndrome.
  • 85.
  • 86. Glomus Tumors (Glomangiomas) • Benign, exquisitely painful tumors arising from specialized smcs of glomus bodies, arteriovenous structures involved in thermoregulation. • Most commonly are found in the distal portion of the digits, especially under the fingernails. • Excision is curative.
  • 87. Intermediate-Grade (Borderline) Tumors • Kaposi sarcoma • Hemangioendotheliomas
  • 88. KAPOSI SARCOMA • KAPOSI SARCOMA (KS) IS A VASCULAR NEOPLASM CAUSED BY KAPOSI SARCOMA HERPESVIRUS (KSHV, ALSO KNOWN AS HUMAN HERPESVIRUS-8, OR HHV-8). • MOST COMMON IN PATIENTS WITH AIDS AND ITS PRESENCE IS USED AS A CRITERION FOR THE DIAGNOSIS.
  • 89.
  • 90.
  • 91.
  • 92. TYPES • CLASSIC KS • ENDEMIC AFRICAN KS • TRANSPLANTATION-ASSOCIATED KS …AIDS-ASSOCIATED (EPIDEMIC) KS
  • 93. • Classic KS • A DISORDER OF OLDER MEN • CAN BE ASSOCIATED WITH MALIGNANCY OR ALTERED IMMUNITY BUT IS NOT ASSOCIATED WITH HIV INFECTION. • CLASSIC KS MANIFESTS AS MULTIPLE RED-PURPLE SKIN PLAQUES OR NODULES, USUALLY ON THE DISTAL LOWER EXTREMITIES • PROGRESSIVELY INCREASE IN SIZE AND NUMBER AND SPREAD PROXIMALLY. • THE TUMORS ARE ASYMPTOMATIC AND REMAIN LOCALIZED TO THE SKIN AND SUBCUTANEOUS TISSUE.
  • 94.
  • 95.
  • 96. Endemic African KS • TYPICALLY OCCURS IN YOUNGER (UNDER 40 YEARS OF AGE) HIV-SERONEGATIVE INDIVIDUALS • INVOLVES LYMPH NODES MUCH MORE FREQUENTLY THAN IN THE CLASSIC VARIANT • A PARTICULARLY SEVERE FORM, WITH PROMINENT LYMPH NODE AND VISCERAL INVOLVEMENT, OCCURS IN PREPUBERTAL CHILDREN • PROGNOSIS IS POOR, WITH AN ALMOST 100% MORTALITY WITHIN 3 YEARS.
  • 97. • Transplantation-associated KS • occurs in solid organ transplant • recipients in the setting of T-cell immunosuppression. • In these patients, the risk for KS is increased 100-fold • It pursues an aggressive course and often involves lymph nodes, mucosa, and viscera • Cutaneous lesions may be absent • Lesions often regress with attenuation of immunosuppression, but at the risk for organ rejection.
  • 98. AIDS-associated (epidemic) KS • IT IS THE MOST COMMON HIV-RELATED MALIGNANCY. • AIDS-ASSOCIATED KS OFTEN INVOLVES LYMPH NODES AND DISSEMINATES WIDELY TO VISCERA EARLY IN ITS COURSE. MOST PATIENTS EVENTUALLY DIE OF OPPORTUNISTIC INFECTIONS RATHER THAN FROM KS.
  • 99. Pathogenesis • Virtually all KS lesions are infected by KSHV (HHV- 8) • Like Epstein-Barr virus, KSHV is a γ-herpesvirus • It is transmitted both through sexual contact and potentially via oral secretions and cutaneous exposures • KSHV and altered T-cell immunity probably are required for KS development • In older adults, diminished T-cell immunity may be related to aging.
  • 100. MORPHOLOGY • In classic kaposi sarcoma • Cutaneous lesions progress through three stages: patch, plaque, and nodule. • Patches are pink, red, or purple macules, typically confined to the distal lower extremities • Microscopic examination dilated, irregular, and angulated blood vessels lined by ecs and an interspersed infiltrate of chronic inflammatory cells, sometimes containing hemosiderin • These leions can be difficult to distinguish from granulation tissue.
  • 101. contd • With time, lesions spread proximally and become larger, violaceous,raised plaques composed of dilated, jagged dermal vascular channels lined and surrounded by plump spindle cells. Other prominent features include extravasated red cells, hemosiderin- laden macrophages, and other mononuclear cells.
  • 102. CONTD • Eventually nodular, more overtly neoplastic, lesions appear. • These are composed of plump, proliferating spindle cells • Mostly located in the dermis or subcutaneous tissues • Often with interspersed slitlike spaces • The spindle cells express both EC and SMC markers. Hemorrhage and hemosiderin deposition are more pronounced, and mitotic figures are common. • Nodular stage often is accompanied by nodal and visceral involvement, particularly in the african and aids-associated variants.
  • 103. Clinical Features • Most primary KSHV infections are asymptomatic. • Classic KS is initially largely restricted to the surface of the body, and surgical resection usually is adequate for an excellent prognosis.