Vascular tumors & a brief discussion on varicose veins

Elsevier. Kumar et al. Robbins basic pathology 9th

Locally aggressive but metastasize infrequently

Classification

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

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

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)

Facial port wine nevi Ipsilateral venous angiomas in the cortical leptomeninges Mental retardation Seizures Hemiplegia Radiopacities of the skull

Telangiectasia: = permanent dilation of preexisting small vessels (capillaries, venules, and arterioles) …usually in the skin or mucous membranes …red lesion

Port wine stain

Visit https://www.dermquest.com/image-library/image/5044bfd1c97267166cd67289 for references

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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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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.

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

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

Elsevier. Kumar et al. Robbins basic pathology 9th …Courtesy of Christopher D.M. Fletcher, MD, Brigham and Women’s Hospital, Boston, Massachusetts.

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

Visit https://basicmedicalkey.com/infantile-juvenile-hemangioma/ for references

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Hemangiomas, cont’d

- 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

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 disease, in which vascular lesions

are commonly found in the cerebellum, brain stem, retina, pancreas,

and liver

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

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

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

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

• 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…and it is 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

Intermediate-Grade (Borderline) Tumors

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

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)

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

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 plexus …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)