1 / 73

Cardiac tumors

Cardiac tumors. Dr Shreetal Rajan Nair Senior Resident, Department of Cardiology, Calicut Medical College. History . 1559 : Columbus , a noted pathologist – first to report 1934 : first report of a primary sarcoma of the heart diagnosed in a living patient by Barnes

doyle
Download Presentation

Cardiac tumors

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Cardiac tumors Dr ShreetalRajan Nair Senior Resident, Department of Cardiology, Calicut Medical College

  2. History • 1559 : Columbus , a noted pathologist – first to report • 1934 : first report of a primary sarcoma of the heart diagnosed in a living patient by Barnes • 1942 : surgical resection of intrapericardialteratoma by Beck • 1951 : The first successful excision of a cardiac tumor performed by Maurer • 1952 : Goldberg diegnosedintracavitarymyxoma by angiocardiography • 1968 : Shattenberg used echo to diagnose cardiac tumors

  3. Classification Primary benign (75%) malignant (25%) Myxoma – 50% Rhabdomyoma – 20% Fibroma Papillary fibroelastoma Lipoma Angiosarcoma Rhabdomyosarcoma Leiomyosarcoma Lymphomas secondary Breast lungs

  4. Incidence • All age groups - 0.002% to 0.03% • Pediatric - 0.027% to 0.08% • Adults - upto 0.02% • By comparison, metastatic involvement is 20 times more common

  5. Incidence of benign tumors Shapiro LM. Cardiac tumours: diagnosis and management. Heart 2001; 85: 218 - 22.

  6. LITERATURE

  7. Tumors of the heart - • Constitutional or systemic • Embolic • Cardiac • Phenomena secondary to metastatic diseases. Specific signs and symptoms generally are determined by the location of the tumor, size, friability , mobilityin the heart and depends least on the histopathology

  8. Tumors of the heart- clinical presentations • Primary tumors most commonly involve the endocardium, followed by myocardium and then epicardium • Metastatic involvement of heart is reverse that of primary tumors • Endocardium- obstruction to flow of blood through heart • Myocardium - rhythm abnormalities • Epicardium – pericardial effusion, cardiac tamponade

  9. Approach to a cardiac tumor Differential diagnosis • PUO • IE/CONNECTIVE TISSUE DISEASES • VALVULAR LESIONS • CCF • CONDUCTION ABNORMALITIES • EMBOLISM • SYNCOPE • PERICARDIAL EFFUSION

  10. Diagnostic evaluation • Confirm the diagnosis • locate the lesion within the heart • Whether benign or malignant

  11. Echocardiography  •  Simple, noninvasive technique for initial evaluation. • Images myocardium and the cardiac chambers Identify the presence of a mass and its mobility. • Information about any obstruction to the circulation & whether the tumor could be a source of emboli

  12. Echocardiography - TEE vs TTE Transthoracic echocardiography – the initial diagnostic tool Transesophageal echocardiography (TEE)- more informative. This is due to the proximity of the esophagus to the heart, the lack of intervening lung and bone, and the ability to use high-frequency imaging transducers that afford superior spatial resolution

  13. CARDIAC MRI VS CT • Both provide noninvasive, high resolution images of the heart, but MRI generally is preferred. • cMRI -detailed anatomic images T1- and T2-weighted sequences help to identify the chemical microenvironment within a tumor • CT -useful when calcification is present and MRI is not available or is contraindicated.

  14. CORONARY ANGIOGRAPHY •  Mapping the blood supply of tumors arising from the epicardial surfaces • vital to the success of excising such tumors. • Significant involvement of coronary arteries - resection and grafting of such arteries

  15. Role of Transvenous biopsy • Limited data are available on the risks and benefits • considered reasonable if potential benefits outweigh risks and there is diagnostic dilemma

  16. Benign tumors • Around 75 percent of primary cardiac tumors are benign • In adults, most common- myxomas (50%) • Other common benign lesions- papillary fibroelastomas and lipomas. • In children, the most common- rhabdomyomas and fibromas

  17. Myxomas • Most common primary cardiac neoplasm. • Histology- scattered cells within a mucopolysaccharidestroma which originate from multipotentmesenchymal cells capable of neural and endothelial differentiation . Hallmark histological feature is myxoma (lepidic) cell • Produce VEGF-induction of angiogenesis and the early stages of tumor growth & IL 6 -responsible for the constitutional features.

  18. Myxoma ( lepidic ) cells

  19. Myxomas • Typically pedunculated and gelatinous in consistency; smooth, villous, or friable. • Calcification , necrosis and cystic changes seen • Mostly (90%) solitary • About 35 % of are friable or villous, these tend to present with emboli. • Larger tumors -smooth surface and associated with cardiovascular symptoms.

  20. Myxomas • The cardiovascular manifestations depend upon the anatomic location of the tumor. • Mostly seen in females • 40-60 years of age • 80% -originate in the left atrium, most of the remainder is found in the right atrium • constitutional symptoms (eg, weight loss, fever) and laboratory abnormalities

  21. Syndrome Myxoma Characteristics Syndromic associations • Younger than 40 years • Biatrial ,ventricular , valves • all first degree relatives should be screened • Recurrent – 30% • No gender predilection • LAMB (lentigines, atrialmyxomas,mucocutaneousmyxomas, and blue nevi), • NAME(nevi, atrialmyxomas, myxoidneurofibroma, and ephelides • Carney syndrome (atrial, cutaneous and mammary myxomas, lentigines, blue nevi, endocrine disorders and testicular tumours)

  22. Myxoma Carney syndrome Histological features Mutations in : • PRKAR1A (protein kinase A regulatory subunit 1A ) • MYH8 - myosin isoform • PAS • Vimentin • S100 and NSE - POSITIVITY

  23. Treatment • Prompt resection-due to the risk of embolization or cardiovascular complications, including sudden death • Result of surgical resection is good ( <5% mortality) • Cardiac autotransplantation

  24. Papillary fibroelastomas • Second most common primary cardiac tumor in adults • Their appearance is compared to pom-pom or sea anemones

  25. Clinical features • Symptoms -caused by embolization of the tumor/thrombus. • most common clinical presentation- stroke /TIA. • Commonly attached to the aortic valve • 30%- incidentally diagnosed by echocardiography, at cardiac surgery, or at autopsy

  26. TREATMENT • SURGERY – 1. patients who have had embolic events or complications directly related to tumor mobility (eg, coronary ostial occlusion) 2.Those with highly mobile or large (≥1 cm) tumors • Recurrence not reported

  27. Lipomas • Lipomas &fibrolipomas - characterized by a predominance of benign fatty cells. • About half of these tumors occur in the subendocardialregion.Others in the myocardial and subepicardial regions • They may also occur on valves

  28. Lipoma

  29. Lipomas • Symptoms- generally related to local tissue encroachment (arrhythmias, conduction block, sudden death) valvular tumors – insufficiency & symptoms of heart failure • Diagnosis - echocardiography and the distinctive fat pattern on MRI. • Require resection.

  30. Lipomatous hypertrophy of the interatrial septum • Exaggerated growth of normal fat existing within the septum and is not a true tumor. • Developmental disorder caused by expansion of adipose tissue trapped in the interatrial septum during embryogenesis • The septal hypertrophy may be as much as 2 cm in thickness and is seen primarily in older patients and in those who are obese

  31. Lipomatous hypertrophy of the interatrial septum • Associated with the presence of CAD in proportion to the degree of atrial septal thickness • Indistinguishable from lipoma except that the former occurs in the atrial septum with a typical distribution (generally sparing the fossaovalis). • In the absence of symptoms of atrial arrhythmias, heart block or rare vena caval obstruction, they do not require resection

  32. Pericardial lipomas • Incidental finding and clinically insignificant. • Rarely assumes gigantic proportions and its appearance on a chest radiograph may be mistaken for a huge pericardial effusion or massive cardiomegaly • Benign pericardial lipomas can infiltrate the myocardium. If the ventricular septum is invaded, communication between the pericardial space and the right ventricular cavity may result.

  33. Rhabdomyomas • Develop almost exclusively in children, mostly <1 year of age • 80 to 90% are associated with tuberous sclerosis • Usually found in the ventricular walls/AV valves. • Most regress spontaneously • Resection is usually not required unless symptomatic • Symptoms –due to obstruction of blood flow through the heart or consist of rhythm disturbances • Present with features of preexcitation on the ECG

  34. Rhabdomyositis • A rare form of cardiomyopathy in infants • Tumor nodules are not grossly apparent • Microscopically, the cardiac muscle fibers and conduction system are diffusely involved with rhabdomyomatoushistologic changes • Recurrent atrial tachycardia and sudden death from intractable ventricular tachycardia

  35. Why rhabdomyomas regress? • High expression of ubiquitin which starts expressing from 32 week onwards • Absent mitotic activity • Ubiquitin is responsible for the degradation of rhabdomyoma cells and hence tumor regression • Characteristic spider cells are formed in the process.

  36. Spider cells in rhabdomyoma

  37. Fibromas • Second most common pediatric cardiac tumor and can also occur in adults • Histologically similar to fibromas arising elsewhere in the body. • Usually arise in the ventricular muscle and may become quite large. • Do not regress spontaneously. • Arise approximately 5 times more frequently in the LV than RV

  38. Fibromas • Most common symptom, due to obstruction of blood flow or interference with valvular function. • Myocardial dysfunction and conduction disturbances • Echocardiography, supplemented with CT/MRI confirms the diagnosis. • Symptomatic tumors should be resected. Complete resection of very large tumors may require cardiac transplantation.

  39. Fibromas

  40. Teratomas • Arise within the pericardium, but do not originate from cardiac structures • Although generally benign,can have serious mechanical consequences by causing tamponade or through direct pressure on the heart. • Risk of death in-utero or immediately after birth

  41. Teratomas • Treatment therefore requires either fetal tumor excision, or caesarean section and immediate operation on the newborn • Have a single blood supply and are not invasive, properly timed tumor surgery is straightforward and successful

  42. Purkinje cell tumors/hamartomas • Consist of small, flat sheets of cells most frequently located in the left ventricle, and on the endocardial and epicardial surfaces • Undetectable by echocardiographic or radiologic techniques. • Tumors of young children and present with incessant ventricular tachycardia • ECGs often demonstrate a bundle branch pattern (right bundle branch block when the tumor is in the left ventricle). Electrophysiologic studies can localize the tumors, facilitating surgical excision.

  43. Paragangliomas • Neuroendocrine tumors that can be hormonally active or inactive. • In tumors not producing catecholamines, symptoms are due to cardiac compression or tamponade. • In contrast, cardiac paragangliomas that are hormonally active primarily produce norepinephrine and may cause systemic symptoms (eg, headache, sweating, tachycardia, hypertension) • Hormonally inactive tumors are more frequent in the pericardium

  44. Paragangliomas • May be localized with echocardiography. • Extremely vascular • Coronary angiography -to plan the operative resection

  45. Paragangliomas • Paragangliomas (benign/malignant) within the pericardium parasitize the cardiac blood supply and hence are very difficult to excise • All intrapericardialparagangliomas require resection • Cardiopulmonary bypass and even circulatory arrest may be required due to the high degree of vascularity, or to moderate the extreme hypertension possible from tumor manipulation or hormonally active tumors • If complete resection is not possible, cardiac transplantation may be required

More Related