1. Vascular tumors & a brief discussion on varicose veins
Ali Al Khader, M.D.
Faculty of Medicine
Al-Balqa’ Applied University

2. Classification Locally aggressive but metastasize infrequently
Robbins basic pathology 9th edition

3. Overview Can arise from:
endothelium (e.g., hemangioma, lymphangioma, angiosarcoma) or
cells that support or surround blood vessels (e.g., glomus tumor)
Those of large vessels are extremely rare and mostly sarcomas
Benign lesions can be differentiated easily from anaplastic malignant ones
…but occasionally the distinction between benign and malignant can be
difficult

4. Overview, cont’d Benign tumors:
-obvious vascular channels filled with blood cells or lymph…lined by a
monolayer of normal-appearing endothelial cells
Malignant tumors are
-More cellular
-Cytologic atypia
-usually do not form well-organized vessels
-confirmation of the endothelial derivation may require
immunohistochemical detection of endothelial cell–specific markers,
such as CD31 or von Willebrand factor

5. Benign Tumors and Tumor-Like Conditions
Vascular Ectasias
…congenital or acquired…not true neoplasms
-Nevus flammeus (a “birthmark”)
…light pink to deep purple flat lesion
…on the head or neck
…regress spontaneously
-Port wine stain
…a special form of nevus flammeus
…grow during childhood
…do not fade with time
…can be associated with Sturge-Weber syndrome (also called encephalotrigeminal
angiomatosis)
Telangiectasia:
= permanent dilation of preexisting small vessels (capillaries, venules, and arterioles)
…usually in the skin or mucous membranes
…red lesion
Facial port wine nevi
Ipsilateral venous angiomas in the cortical leptomeninges
Mental retardation
Seizures
Hemiplegia
Radiopacities of the skull

6. Port wine stain
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7. Vascular Ectasias, cont’d
-Spider telangiectasias …radial, often pulsatile arrays of dilated subcutaneous arteries or arterioles (the “legs” of the spider) about a central core (the spider’s “body”) …blanch with pressure …face, neck, or upper chest …hyperestrogenic states (e.g., in pregnant women or patients with cirrhosis)
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8. Vascular Ectasias, cont’d
Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)
…autosomal dominant
…problems in TGF-β signaling pathway in endothelial cells
…present at birth
…widely distributed over the skin and oral mucous membranes
…as well as in the respiratory, gastrointestinal, and urinary tracts
…can spontaneously rupture…epistaxis, GI bleeding, hematuria…etc.

9. Hemangiomas
Very common…7% of all benign tumors of infancy and childhood
Usually: head & neck
…may be widely distributed… = angiomatosis
1/3 of internal hemangiomas are in the liver
Malignant transformation is very rare

10. Hemangiomas, cont’d Histologic and clinical variants
Capillary hemangiomas …the most common
...skin, subcutaneous tissues, and mucous
membranes of the oral cavities and lips, as
well as in the liver, spleen, and kidneys
…histologically: thin-walled capillaries with
scant stroma

11. Hemangiomas, cont’d
- Juvenile hemangiomas (so-called strawberry hemangiomas) …of the newborn skin …extremely common (1 in 200 births) and can be multiple …grow rapidly for a few months but then fade by the age of 1 to 3 years …complete regression by age 7 in the vast majority
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12. Hemangiomas, cont’d Pregnancy tumor (granuloma gravidarum)
Pyogenic granulomas
…a subtype of capillary
hemangioma
…rapidly growing
…red pedunculated lesions on the
skin, gingival, or oral mucosa
…bleed easily and are often
ulcerated
…25%: caused by trauma
…curettage and cautery usually are
curative
Pregnancy tumor (granuloma gravidarum)
…a pyogenic granuloma
…that occurs infrequently in the
gingiva of pregnant women
…These lesions may spontaneously
regress (especially after pregnancy)
or undergo fibrosis
…Occasionally require surgical
excision

13. Hemangiomas, cont’d Cavernous hemangiomas
…composed of large, dilated vascular channels
…more infiltrative, frequently involve deep structures, and do not
spontaneously regress
…usually clinically not problematic but may be infiltrative and require
surgery or may ulcerate and bleed due to trauma
…one component of von Hippel-Lindau diseas, in which vascular lesions
are commonly found in the cerebellum, brain stem, retina, pancreas,
and liver

14. Lymphangiomas Benign lymphatic counterpart of hemangiomas
1- Simple (capillary) lymphangiomas
…most commonly head, neck, and axillary subcutaneous tissues
…endothelium-lined spaces that can be distinguished from capillary
channels only by the absence of blood cells

15. Lymphangiomas, cont’d
2- Cavernous lymphangiomas (cystic hygromas) …neck or axilla of children, and more rarely in the retroperitoneum …can be large (up to 15 cm), filling the axilla or producing gross deformities of the neck …cavernous lymphangiomas of the neck are common in Turner syndrome …massively dilated lymphatic spaces lined by endothelial cells and separated by intervening connective tissue stroma containing lymphoid aggregates …infiltrative and complete resection is difficult

16. Glomus Tumors (Glomangiomas)
Benign
Exquisitely painful tumors
Arise from specialized smooth muscle cells 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

17. Bacillary Angiomatosis
In the immunocompromised
Can involve the skin, bone, brain, and other organs
Proliferation of capillary-sized vessels with inflammation
The endothelial cells show atypia
Bartonella spp.
HIF-1 alpha & VEGF by host cells…induced by bacteria
Cured by antibiotics

18. Intermediate-Grade (Borderline) Tumors
Kaposi sarcoma
…viral cause (G protein (induces VEGF), viral homologue of cyclin D &
inhibition of p53)
…most commonly found in AIDS patients…a criterion for diagnosing
AIDS…the most common malignancy in AIDS patients
…multiple red-purple lesions
…the most common tumor in central Africa (endemic type + AIDS-related type)
…4 types of KS based on demographics
…3 stages of cutaneous lesions: patch-plaque-nodule

19. Kaposi sarcoma Classic
…older men of Mediterranean, Middle Eastern, or Eastern European descent (especially Ashkenazic
Jews)
…usually on the distal lower extremities and spreads proximally
Endemic African
…under age 40
…a severe form of it occurs in prepubertal children (almost 100% mortality in 3 years)…nodal and
visceral
Transplantation-associated
…nodal, visceral and mucosal more than cutaneous
…may regress with removal of immunosuppression
AIDS-related
…spread in the body
…AIDS patients mostly die due to opportunistic infection more than KS

20. Malignant tumors …Angiosarcoma, a brief discussion
Older adults
Most commonly: skin, soft tissue, breast and liver
In chronic lymphedema (like in breast
cancer due to surgery or radiation)
…lymphangiosarcoma
Radiation and foreign bodies are also
risk factors
-arsenical pesticides
-Thorotrast (a radioactive contrast
agent formerly used for radiologic
imaging)
-polyvinyl chloride (a widely used plastic, and one of the best known examples of human chemical carcinogenesis)

21. Varicose veins
Chronically increased intraluminal pressures and weakened vessel wall support…the venous valves become incompetent due to dilation
superficial veins
20% of men and a third of women
Pregnancy (compression of IVC) is a risk factor
Obesity is a risk factor
Familial tendency…premature varicosities
lower extremity stasis, congestion, edema, pain, and thrombosis
…secondary ischemic skin changes, including stasis dermatitis and ulcerations
…may become chronic with superimposed infections
Embolization is very rare

22. Varicosities in other 2 sites
Esophageal varices
may occur in addition to hemorrhoids and caput medusa…in portal HTN
(due to cirrhosis (mostly) or portal vein or hepatic vein obstruction)
Hemorrhoids…dilation of anorectal junction venous plexuse
…from prolonged pelvic vascular congestion associated with
pregnancy or straining to defecate
…will cause bleeding (mainly in internal hemorrhoids) or pain (mainly in
thrombosed external hemorrhoids)

23. Thank You