Vascular Tumors

Hemangiomas are composed of blood vessels. Are these benign or malignant?

Benign tumors of blood vessels

Kaposi's sarcoma can arise in AIDS patients, transplant patients, and older Eastern European males. How are these treated?

AIDS patients --Treat AIDS so the immune system will be able to fight Transplant patients --Reduce the immunosuppression so the immune system can fight Older Eastern European males --Surgical excision

There are three populations that are affected by Kaposi's sarcoma. What are they? -The typical one? -Another immunosuppressed one? -Old white guys?

AIDS patients Transplant patients Older Eastern European males

Angiosarcomas are malignant tumors of blood vessels. How dangerous are these and why?

Aggressive --Usually diagnosis is delayed

Angiosarcomas arise after radiation for breast cancer or Hodgkin's. Where do angiosarcomas arise?

Arise around where the radiation was --Head, neck, breast

A glomus tumor arises from the smooth muscle of a glomus body. Where do these arise?

Arise underneath the fingernails --Where the glomus bodies shunt normally work to shunt blood away from extremities

Bacillary angiomatosis is a benign capillary proliferation involving the skin and visceral organs. What population does this arise in?

Arises in AIDS patients

What is a glomus body?

Arteriovenous shunts in the dermis of the extremities

A cystic hygroma causes a buildup of lymph fluid. What syndrome is this associated with?

Associated with Turner's syndrome

A pyogenic granuloma is a vascular mass on the skin that can ulcerate and bleed easily. What are these masses associated with?

Associated with trauma and pregnancy

Bacillary angiomatosis is similar to Kaposi's sarcoma, but benign. Like Kaposi's sarcoma, this is due to an infection. What organism is responsible?

Bartonella henselae --Normally causes cat-scratch disease

What is bacillary angiomatosis? -Benign or malignant? -Type of cells involved? -What tissue is affected?

Benign capillary proliferation involving the skin and visceral organs

What type of cells compose an angiosarcoma?

Blood vessels

What types of cells compose a hemangioma?

Blood vessels

Angiosarcomas arise after radiation for a previous cancer. What types of cancers are typical? -Most common in women? -Common in adolescents?

Breast cancer Hodgkin's Lymphoma

Strawberry hemangiomas are a fairly common benign tumor of blood vessels in kids. How do they appear? -Color? -Do they blanch when pressed? -Borders?

Bright red compressible plaques with sharp borders

Sturge-Weber Disease: The capillaries in the pia mater can be messed up. How can this present?

Can develop AVM in the brain Can develop seizures --"Ipsilateral leptomeningeal angiomatosis"

Sturge-Weber Disease: This is a congenital vascular disorder that affects capillaries. What capillaries are specifically affected?

Capillaries in opthalmic nerve distribution (CN V1) Capillaries in pia mater Capillaries in the eye

Hemangiomas are benign tumors of blood vessels. What are the two different types? -Child type? -Adult type?

Child type = Strawberry hemangioma --Kids like strawberries more than cherries Adult type = Cherry hemangioma --Adults like cherries more as they grow up`

Lymphangiosarcoma is a malignant tumor of lymphatic vessels. What is the cause of this?

Chronic lymphedema --Lymph nodes/vessels are removed and lymph cannot drain

Strawberry hemangiomas grow rapidly but then regress on their own. By what age are they gone?

Completely regressed by 5-8 years old

Sturge-Weber Disease: What is this?

Congenital vascular disorder that affects capillaries

Cystic hygromas are associated with Turner's syndrome. What do these lead to?

Cystic hygroma in the neck leads to the 'webbed neck' seen in Turner's syndrome

What type of tumor is a cystic hygroma?

Cystic hygroma is a lymphangioma in the neck

Sturge-Weber Disease: The capillaries in the eye can be messed up. How can this present?

Early-onset glaucoma

Strawberry hemangiomas appear as bright red compressible plaques with sharp borders. How are they normally dealt with?

Grow rapidly, but then regress on their own

Kaposi's sarcoma is a malignant tumor that arises in AIDS patients. What is the responsible organism?

HHV-8

Angiosarcoma is a tumor of blood vessels. Is this benign or malignant?

Malignant tumor of blood vessels

What is a lymphangiosarcoma?

Malignant tumor of lymphatic vessels

Your patient had a radical mastectomy and now presents with a mass in her right arm near the axilla. What does she have?

Lymphangiosarcoma

A cystic hygroma is a lymphangioma of the neck. What does this mean?

Malformations of the lymph system, so lymph fluid builds up and can't be drained

HHV-8 leads to Kaposi's sarcoma in AIDS patients. What cell type is involved?

Malignant proliferation of endothelial cells

Kaposi's sarcoma appear as purple lesions (flat, to plaques, to nodules). Where do these lesions arise? -Most common? -Other sites?

Most commonly on the skin --Can also involved the visceral organs --Mouth, GI tract, respiratory tract

Angiosarcomas are malignant tumors of blood vessels. What causes these?

Most often due to radiation for previous cancers

Sturge-Weber Disease: The capillaries in the opthalmic nerve distribution are messed up. How does this present?

Nevus flammeus (port-wine birthmark) in the CN V1 distribution

Cherry hemangiomas appear as small, bright red cutaneous papules. Do these regress on their own like strawberry hemangiomas do?

No --Cherry hemangiomas do not regress

Angiosarcomas can arise in the liver, not related to previous radiation. What are the risk factors for liver angiosarcoma? -3 unique exposures

PVC Arsenic Thorotrast (thorium dioxide)

A glomus tumor arises underneath the fingernails. How do these present? -Pain? -Color? -Location?

Painful, red-blue tumor under the fingernails --Mass under fingernail = painful --Red-blue = these are normally AV shunts

Strawberry hemangiomas are usually present at birth or first few weeks of life. How common are these?

Pretty common --1 in 200 births

Kaposi's sarcoma is a malignant proliferation of endothelial cells. How does this present on the skin? -Color? -How do they appear as they progress?

Purple lesions --Begin as flat patches --Progress to raised plaques --Progress to nodules that ulcerate

Lymphangiosarcomas arise from chronic lymphedema. What can cause this?

Radical mastectomy --Remove the axillary lymph nodes too

Glomus bodies are AV shunts in the dermis of the extremities. What is their purpose?

Shunt blood away from the extremities when exposed to cold temperatures

Cherry hemangiomas are benign tumors of blood vessels in adults. How do these appear? -Size? -Color?

Small, bright red cutaneous papules --Look like little dots, much small than strawberry hemangioma

Glomus bodies are AV shunts that control temperature regulation. What is a glomus tumor?

Tumor that arises from the smooth muscle of a glomus body

Strawberry hemangiomas are benign tumors of blood vessels in children. When do these appear?

Usually present at birth or within first few weeks of life

What is a pyogenic granuloma? -What type of tissue is it? -Color? -How does it appear on the skin? -What complications happen easily?

Vascular, red pedunculated mass that ulcerates and bleeds easily --Looks like a bubble of blood on the skin

Bacillary angiomatosis is a benign capillary proliferation that arises in AIDS patients. What other tumor does this sound very similar to?

Very similar to Kaposi's sarcoma --BUT this is benign, not malignant