CARDIOVASCULAR SYSTEM TUMORS

1. CARDIOVASCULAR SYSTEM TUMORS

2. Tumors of Blood Vessels

3. 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

4. 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.

7. 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.

8. 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.

9. Lymphangiomas • Hamartoma • The best-known is "cystic hygroma" of the necks of babies.

10. 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.

12. MALIGNANT TUMORS of VESSELS • Hemangioendothelioma • Angiosarcoma • Hemangiopericytoma • Kaposi's sarcoma • Lymphangiosarcoma

13. 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.

15. Hemangiopericytoma • Low-grade malignancy of the pericytes. • The tumor cells interlace with vessels, beautifully demonstrated in reticulin-stained preparations.

16. 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.

19. Lymphangiosarcoma • Cancers of the lymphatics, • Generally arising in lymphedema: • after mastectomy (Stewart-Treves syndrome), • after exposure to radiation.

20. HEART TUMORS

21. Metastatic 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 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).

22. 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.

23. 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.

24. 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.

25. 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.

26. 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.

27. 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%).

28. 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.

31. 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.

32. 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.

33. 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.

34. 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.

35. Malignant tumors • Cardiac angiosarcoma • Rhabdomyosarcoma • Fibrosarcoma Complications: • arrhythmia, • congestive heart failure, • thromboembolism, • decrease in ventricular function, • Metastasis.