Pathology 9 &10: Peripheral Venous and Lymphatic disease Flashcards
Vericose Veins
Abnormally dilated, tortuous veins
Causes of Vericose Veins (2)
Prolonged, increased intraluminal pressure
Loss of vessel wall support (vein walls are much more weak than arterial walls)
Which veins typically become vericose ?
Superficial veins
Upper and lower leg
Is there a familial link to Varicose veins ?
You know it ! Due to defective development of vein walls
The most troublesome complications from varicose veins are persistent edema in the extremity and Ischemic skin changes. What are some of theses skin changes ?
Stasis dermatitis
Cutaneous ulceration
These contribute to poor wound healing , infections –> osteomyelitis if infection gets into bone, This can lead to amputation of the limb
What causes esophageal varices to form ?
Portal Vein HTN ( Cirrhotic liver)
With cirrhosis and subsequent portal vein hypternsion, where else might you see varices ?
Rectum (hemorrhoids) Periumbilical veins (caput medusae)
What is the most dangerous association with esophageal varicoses ?
RUPTURE –> massive upper GI bleeds
What non-cirrhotic conditions can lead to hemorrhoids ?
Prolonged pelvic vascular congestion
Pregnancy
Chronic constipation
Increased intra-abdominal pressure
Thrombophelbitis/Phlebothrombitis
Venous thrombosis and inflammation
What are some state that can potentiate Venous Thrombosis ?
cardiac failure, neoplasia, pregnancy, obesity, post-op state, prolonged bed rest/immobilization
Where do most cases of thrombophlebitis occur in the body ?
Deep leg veins
Other sites:
Periprostatic venous plexus (males)
Pelvic venous plexus (females)
Large veins in the skull and the dural sinuses (infection or inflammation)
Portal vein thrombosis
Peritoneal infections, appendicitis, salpingitis
and pelvic abscesses
Explain how carcinoma can lead to thrombophlebitis
Elaboration of pro-coagulant factors by the tumor cells
Migratory thrombophlebitis (thrombophlebitis migrans) exhibits will exhibit “trousseaus sign” or clotting in many areas of the body w/o order. What is this indicative of
Cancer (adenocarcinoma)
What is the most serious complication of DVT (phlebothrombosis of the deep leg veins) ?
Pulmonary embolism
What is often the first sign of Thrombophlebitis ?
PE ! Sadly, many don’t realize they have a DVT until it is too late.
Lymphangitis (define)
Acute inflammation in lymphatic channels
Dilated and filled with inflammatory infiltrate
Composed of neutrophils and histiocytes
Extends through the vessel wall
Perilymphatic tissues
Cellulitis or focal abscesses (severe cases
Most common agent of lymphangitis ?
Group A β-hemolytic streptococci
Clinical features of lymphangitis
Painful subcutaneous red streaks
Extend along the course of lymphatics
Painful enlargement of regional lymph nodes
If lymph nodes fail to block bacteria
Drainage into venous circulation
Bacteremia or septicemia
Primary Lymphedema
Isolated congenital defect (simple congenital lymphedema)
Often due to agenesis or hypoplasia or lymph tissue
Famililal Milroy disease
Heredofamilial congenital lymphedema
Lymphatic agenesis or hypoplasia
Secondary Lymphedema
Occlusion of lymphatic vessels
Abnormal accumulation of interstitial fluid
Caused by:
Malignancy (efacement of lymphnode and
destruction of lymph tissue)
Post surgical Post irradiation fibrosis Post inflamm scarring Obstruction due to filiarisis
Persistent edema seen in lymphedema leads to subcutaneous interstitial fibrous tissue and induration. Induration is often referred to by its strange appearance, what is it ?
Peau d’orange (orange peel)
In lymphedema, lymphatics may rupture leading to milky collections of lymph in potential spaces. What is a major lymph structure that could rupture leading to this ?
Thoracic duct
Places where lymph will accumulate by name:
Chylous ascites (peritoneum)
Chylothorax (pleural cavity)
Chylopericardium (pericardium
A common cause of lymph rupture is ….
malignant invasion.
What kind of tumors are primarily found in the aorta,plum art and vena cava ?
Connective Tissue Sarcoma’s
Extremely rare
Endothelium derived neoplasms include
Hemangioma, Lymphangioma and Angiosarcoma
Tumors arising from cells that support vessels include
Glomus tumor and hemangiopericytoma
Hemangioma
Benign and extremely common tumors compose of increased # of normal or abnormal vessels filled w/ blood.
Rare turn malignant
Where do you find most hemangiomas
head and neck (superficial)
Where can you find internal hemangiomas
The liver (1/3 of all)
Capillary hemangioma
Most common
Composed of a proliferation of benign capillaries
Found in:
Skin and subcutaneous tissues
Mucous membranes of the oral cavities and lips
Liver, spleen, and kidneys
Physical description of capillary hemangioma
Bright red to blue
Millimeters to several centimeters in diameter
Flat or elevated
Histologic description of Capillary Hemangioma
Unencapsulated aggregates of closely packed, thin-walled capillaries
Blood-filled and lined by flattened endothelium
Scant connective tissue stroma separates vessels
Lumens may be partially or completely thrombosed
Describe “strawberry type” capillary hemangioma
AKA Juvenile hemangioma of the skin of newborns
Extremely common
May be multiple
Grows rapidly in the first few months
Fades at 1 to 3 years of age
Completely regresses by age 7 (75% to 90% of
cases)
Carvenous Hemangioma
Large(compared to the capillary hemangioma), dilated vascular channels
Less well circumscribed
Involve deep structures
Brain hemangiomas--most problematic
Pressure symptoms or ruptureCavernous Hemangiomas are a component of what disease
Von Hippel Lindau disease (Cerebellum,brain stem and retina)
Physical description of Cavernous Hemangioma
Red-blue, soft, spongy masses
1 to 2 cm in diameter
Sharply defined
Composed of large, cavernous blood-filled vascular spaces
Separated by a modest connective tissue
stroma
In Cavernous hemangioma, intravascular thrombosis is associated with …
dystrophic calcification
Pyogenic Granuloma
Essentially it is a capillary hemangioma which has ulcerated (acute inflammation )
Rapidly growing pedunculated red nodule
Skin, gingival or oral mucosa
Bleeds easily; often ulcerated
Due to proliferating capillaries –> extensive edema
Shows acute and chronic inflammatory infiltrate
Lymphangiomas
Benign proliferations of lymph tissue
Simple lymphangioma
Small lymphatic channels in the head, ncke and axial sub cue tissues.
Elevated or pedunculated (1/2/ cm in diameter)
No erythrocytes
Cavernous lymphangioma
Neck or axila of children
Large (15 cm in diameter) –> gross deformity of the neck
Massively dialated, seperated by intervening connective tissue stroma
Cavernous lymphangioma is associated with what genetic disease ?
Turner syndrome
GLomus tumor
Benign, exquisitely painful tumors
Arises from modified smooth muscle cells of the glomus body (Specialized arteriovenous structure involved in thermoregulation).
Located anywher eon the skin but often found under the nails on digits
Physical description of GLomus tumor
Round, slightly elevated, red-blue
Firm nodules (<1 cm in diameter)
Aggregates, nests, and masses of specialized glomus cells
Branching vascular channels
Vascualar ectasias
Group of abnormally prominent capillaries, venules and arterioles
Creates a focal red lesion usually in skin or
mucous membranes
Nevus flammeus
“Birthmark”
Most common form of ectasia
Head or neck; light pink to deep purple
Vascular dilation of blood vessels in dermis
Port Wine Stain
Special form of nevus flammeus; grows with child
Thickens skin surface
Distributed over Trigeminal Nerve
What disease is Port Wine Stain often associated with ?
Sturge-Weber syndrome
encephalotrigeminal angiomatosis
Rare congenital disorder
Associations
Venous angiomatous masses in cortical
leptomeninges
Ipsilateral facial port wine nevi
Mental retardation, seizures, hemiplegia
Spider Telangiectasia
Non neoplastic lesion that blanches when pressure is applied to it
Focal network of subcutaneous small arteries or arterioles
Arranged in a radial fashion around a central core
Who mostly gets Spider Telangiectasia ?
Pregnant women or patients with cirrhosis
Increased estrogen levels ! Estrogen not broken
down in liver due to cirrhosis
Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Disease)
Dont confuse with Sturge Weber (associated with port wine stain)
Autosomal dominant disorder
Multiple aneurysmal telangiectasias
Skin and oral mucous membranes
Respiratory, GI, urinary tracts
Present from birth
Lesions rupture (occasional)
Serious epistaxis, GI bleed, or hematuria
Bacillary Angiomatis
Vascular proliferation due to opportunistic infections in immunocompromised patients
Involve skin bone brain others.
Due to gran negative bacilli in the Bartonella family.
Physical description of a Bacillary Angiomatosis
Skin lesions
Red papules and nodules
Rounded subcutaneous masses
Capillary proliferation with epithelioid endothelial cells
Nuclear atypia and mitoses
Lesions contain neutrophils, nuclear dust, and bacteria
Diagnosis of Bacillar Angiomatis
PCR !
Treatment of Bacillary Angiomatis
Macrolide antibiotics
Kaposi Sarcoma (Forms )
Intermediate Grade Neoplasm
Common in AIDS patients
Chronic KS
AKA classic or European KS
Lymphadenopathic KS
AKA African or endemic KS
Transplant-associated KS
AIDS-associated (epidemic) KS
Chronic Kaposi Sarcoma
Can see with underlying malignancy; not HIV
Multiple red to purple skin plaques or nodules
Distal lower extremities
Increase in size and number; spread proximally
Locally persistent; asymptomatic
Remain localized to skin and subcutaneous tissue
Who will you most likely see Chronic Kaposi in ?
Older men of Eastern European (Ashkenazi Jews) or Mediterranean descent
Lymphadenopathic KS
Same geographic distribution as Burkitt lymphoma
Prevalent among South African Bantu children
Not associated with HIV
Skin lesions are sparse
Lymphadenopathy
Extremely aggressive
Transplant-associated KS
Solid-organ transplantation
Long-term immunosuppression
Aggressive (even fatal)
Nodal, mucosal, and visceral involvement
Cutaneous lesions may be absent
occasionally these regress, may lower immunosupressives
AIDS-associated (epidemic) KS
Originally found in 1/3rd of AIDS patients Male homosexuals Regimens of antiretroviral therapy Incidence is less than 1% Lymph nodes or viscera Disseminates widely early in disease
What cuases Kaposi sarcoma ?
Human herpesvirus-8 (HHV-8)
Sexually transmitted or via saliva
Kaposi: Patch Stage
Red to purple macules
Confined to the distal lower extremities
Dilated irregular endothelial cell-lined vascular spaces
Interspersed lymphocytes, plasma cells, and macrophages
Kaposi: Plaque Stage
Over time, spread proximally
Larger, violaceous (more purple), raised
Dermal accumulations of dilated, jagged vascular channels
Lined and surrounded by plump spindle cells
Extravasated red cells, hemosiderin-laden macrophages, and other mononuclear inflammatory cells
Kaposi: Nodule Stage
Distinctly neoplastic Sheets of plump, proliferating spindle cells Dermis or subcutaneous tissues Small vessels and slit-like spaces Contain red cells More marked hemorrhage, hemosiderin pigment, and mononuclear inflammation Mitotic figures are common Heralds nodal and visceral involvement African and AIDS-associated variants
Hemangioendothelioma
Intermediate Grade Neoplasm wide spectrum
Epithelioid hemangioendothelioma
Vascular tumor of adults
Around medium-sized and large veins
Plump and cuboidal tumor cells
Inconspicuous well-defined vascular channels
Angiosarcomas
Group of malignant endothelial neoplasms
Anaplastic –> Revert back to stem cell like structure.
More often affects older adults
Skin soft tissue breast an liver
What are angiosarcomas associated with (chemicals) ?
Associated with carcinogen exposures
Arsenic (arsenical pesticides) Thorotrast Radioactive contrast agent formerly used for radiologic imaging Polyvinyl chloride (PVC)
Describe angiosarcoma and association with lymphedema
Several years after radical mastectomy
Lymph node resection no lymph drainage. Will
be in ipsilateral upper extremity.
Ipsilateral upper extremity
Tumor arises from lymphatic vessels (lymphangiosarcoma)
Physical description of Angiosarcoma
Small, sharply demarcated, asymptomatic, red nodules
Large, fleshy masses of red-tan to gray-white tissue
Margins blend with surrounding structures
Central areas of necrosis and hemorrhage
Histologic representation of angiosarcoma
Plump, anaplastic endothelial cells producing vascular channels
Wildly undifferentiated tumors
Solid spindle cell appearance and without
definite blood vessels
CD31 or von Willebrand factor
Locally Invasive, metastasize readily
Aggressive tumors 5 year survival rates approaching 30%
Hemangiopericytoma
Rare neoplasm
Derived from pericytes-myofibroblast-like cells
Arranged around capillaries and venues
Slowly enlarging, painless masses
Where are hemangiopericytomas most common ?
Most common
Lower extremities (thigh)
Retroperitoneum
Histologic description of Hemangiopericytoma
Numerous branching capillary channels
Gaping sinusoidal spaces enclosed within nests of spindle-shaped to round cells
Recur after excision
About half will metastasize
Hematogenously to lungs, bone, or liver
