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 % 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.