CARDIOVASCULAR SYSTEM TUMORS

Tumors of Blood Vessels

BENIGN TUMORS & Tumor-Like Conditions

of VESSELS Tumors• Hemangiomas

• Hemangiomas (Capillary & Cavernous, and others)• Epithelioid hemangiomas • Papillary endothelial hyperplasia

• Glomangiomas (glomus tumors) • Telangiectasias• Lymphangioma

Tumor-like coditions: Pyogenic granuloma

Hemangiomas • Mostly hamartomas. • The endothelial cells are of course clonal and show

some enhanced responses to some growth factors.• Capillary hemangiomas (little vessels) and

cavernous hemangiomas (big vessels) are common on the skin.

• Stork bites (leylek izi) backs of the neck and/or forehead of a baby) usually involute (i.e., thrombose and organize) after a few years, while cherry angiomas of the skin start popping up after age 20 or so.

• Port-wine stain (one form of "nevus flammeus"), fashionable in the Gorbachev era, can be treated by laser.

• Sturge-Weber syndrome: a hemangioma in the meninges, generally with an overlying port-wine stain in the check the eyelids.

Glomangiomas (glomus tumors)Painful tumors of the smooth muscle of the

human glomus organs, little thermoregulatory left-overs from mammalian evolution and found in

• the fingertips,• toes, • coccyx.

Telangiectasias"Dilatations of the ends of little vessels"

• Osler-Weber-Rendu telangiectasias result from any of several autosomal dominant genes. • Patients have little vascular malformations

connecting little arteries and little veins along their whole GI tract (often easiest to see on the lips), and often lots of other places.

• These are prone to bleed.

• The familiar "liver spider" is a centrally dilated artery supplying several little arterioles which blanch when the "spider's body" is pressed.

Lymphangiomas

• Hamartoma• The best-known is "cystic hygroma" of

the necks of babies.

Tumorlike conditions• Pyogenic granuloma:

• oral and skin growths; • granuloma gravidarum: if pops up the

gums of pregnant women grossly, it looks like a rotten cherry and bleeds very easily

• Microscopically: granulation tissue.

MALIGNANT TUMORS of VESSELS

• Hemangioendothelioma • Angiosarcoma • Hemangiopericytoma• Kaposi's sarcoma • Lymphangiosarcoma

Hemangioendothelioma• Low-grade malignancy of the endothelium.

Angiosarcoma• Best-known is epidemic hepatic angiosarcoma,

caused by exposure to vinyl chloride or "Thorotrast" contrast medium.

• At other sites, they often follow therapeutic radiation.

• Epithelioid angiosarcomas (epithelioid hemangioendotheliomas) are common, and look like carcinomas.

Hemangiopericytoma• Low-grade malignancy of the

pericytes. • The tumor cells interlace with

vessels, beautifully demonstrated in reticulin-stained preparations.

Kaposi's sarcoma

• The cause is herpesvirus 8 (HHV-8). • Classic Kaposi's sarcoma mostly involved the

legs of older men, and seldom caused major problems.

• Epidemic non-AIDS related Kaposi's is a disease mostly affecting young men in central Africa. It's more aggressive than classic Kaposi's, but not so much as in AIDS-related Kaposi's.

• Renal transplant patients are prone to yet another Kaposi's variant.

Lymphangiosarcoma

• Cancers of the lymphatics, • Generally arising in lymphedema:

• after mastectomy (Stewart-Treves syndrome),

• after exposure to radiation.

HEART TUMORS

• Metastatic NeoplasmsMetastatic Neoplasms• The most common tumor of the heart is a metastatic

tumor; tumor metastases to the heart occur in about 5% of patients dying of cancer.

• In descending order these tumors are carcinoma of the lung, lymphoma, breast cancer, leukemia, melanoma, carcinomas of the liver, and colon.

• Primary NeoplasmsPrimary Neoplasms • Primary cardiac tumors are uncommon; in addition,

most primary cardiac tumors are also (thankfully) benign.

• In descending order of frequency (adults) the primary cardiac tumors are: myxomas, fibromas, lipomas, papillary fibroelastomas, rhabdomyomas, and angiosarcomas (this last one is malignant).

• Benign neoplasms occur 3 times more often than malignant tumors. • Of these sarcomas, angiosarcomas are the most

common histologic type and occur more frequently in males. These tumors seed blood directly, thus metastases are common and widespread.

• Signs and symptoms of these tumors at presentation are generalized, nonspecific, and mimic several other systemic diseases.

• Right-sided tumors • congestive heart failure (CHF), • edema, • jugular venous distention, • Ascites,• pericardial effusions. • Vena cava syndrome, pulmonary embolism, and restrictive

cardiomyopathy are some of the complications.

• Left-sided tumors • embolism, • ischemic attacks, • cerebrovascular and peripheral-vascular accidents. • Based on their size and position, they may induce

arrhythmias and interfere with ventricular compliance.

Benign tumors

• Rhabdomyoma• Hamartoma

• most frequently found tumors in children.

• They are associated with tuberous sclerosis in about 50-80% of patients.

• These tumors are frequently multiple, involving ventricular free and septal walls, and have a yellowish-gray color.

• They vary from small to extremely large.

• Fibromas

(Papillary fibroelastoma)• Hamartoma• Usually single and large, • most commonly in the left

ventricular free wall,• 40% are diagnosed in infants

younger than 1 year. • These tend to be firm

nonencapsulated tumors derived from fibroblasts.

• It looks like a sea anemone on the pulmonic valve.

• can cause embolic stoke.

• Teratomas • Single, encapsulated, grayish-tan tumors • most often in the pericardium.

• Angiomas • A vascular tumor• can occur in any part of the heart (with a

preference for right-sided chambers)• hemangioma or lymphangioma.• Hemangiomas are red, hemorrhagic, sessile, or

polypoid subendocardial nodules that vary from small to large and occasionally have been associated with hemorrhagic cardiac tamponade.

• These vascular vessels communicate between themselves within the myocardium.

• They can infiltrate the intraventricular septum near the conduction system where they may cause heart block.

• Myxoma • A myxoma is a benign tumor, usually irregular in

shape and jellylike in consistency.

• Half of all primary heart tumors are myxomas.

• A lesion of endothelial origin that arises as a ball from the atrial septum and fills the left atrium.

• Grossly, it's a typically benign, soft tumor.

• The tumor may damage the mitral valve.

• Myxomas can plug the mitral valve (sudden death), or cause emboli.

• They often are found attached to the atrial septum and mitral valve apparatus in the left atrium (>85%).

• Histologic Findings: • In general, myxomas are globular, hard,

and mottled lesions with hemorrhage. • Histologically, they are composed of

stellate or globular myxoma cells, endothelial cells, macrophages, mature or immature smooth muscle cells, and a variety of intermediate forms embedded in an abundant acid mucopolysaccharide ground substance.

• Atrial myxomas• In adults, myxomas are the most common

primary tumor of the heart. • They arise in any of the 4 chambers or on the

heart valves; however, about 90% are located in the atria. Myxomas in the atria have a left-to-right ratio of approximately 4:1.

• Myxomas are mostly single and rarely multiple in several chambers.

• Tumors can be 1-10 cm or larger in diameter.

• The tumors can be sessile or pedunculated.

• Complications: • Stroke can be a complication.• Recurrent myxomas are common in patients

with Carney complex.• The most common postoperative complication is

atrial dysrhythmia.• Atrial myxomas may create ball-valve

obstructions that cause unexpected syncopal attacks, cardiac insufficiency, and sudden death in apparently healthy young children and adults.

• Cerebellar ischemic stroke can result from emboli from an atrial myxoma.

• Embolization to the brain, kidneys, and lungs can also occur.• May even occur in utero.

Carney complex • A familial multiple neoplasia and

lentiginosis syndrome:• (1) primary pigmented nodular adrenocortical

disease (PPNAD), a pituitary-independent, primary adrenal form of hypercortisolism;

• (2) lentigines, ephelides, and blue nevi of the skin and mucosae;

• (3) a variety of nonendocrine and endocrine tumors. • myxomas (skin, heart, breast, and other sites);

psammomatous melanotic schwannoma; growth hormone–producing pituitary adenoma; testicular Sertoli-cell tumor.

• Echocardiograms revealed dense echoes arising from the space between the mitral leaflets; these findings were consistent with the histologic features of a myxoma.

• Myxomas recur in approximately 12-22% of familial cases and in about 1-2% of sporadic cases.

Malignant tumors• Cardiac angiosarcoma• Rhabdomyosarcoma• Fibrosarcoma

Complications: • arrhythmia, • congestive heart failure,• thromboembolism, • decrease in ventricular function, • Metastasis.