Vascular tumors & a brief discussion on varicose veins Ali Al Khader, M.D. Faculty of Medicine Al-Balqa’ Applied University Email: ali.alkhader@bau.edu.jo

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

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) 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

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

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) Visit http://doctorv.ca/cosmetic-services/telangiectasia-broken-facial-blood-vessels/spider-angioma-spider-hemangioma-detailed-page/ for references

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

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

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

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

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

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

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 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)

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