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'''Associate Editor-In-Chief:''' {{CZ}}
'''Associate Editor-In-Chief:''' {{CZ}}


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==[[Primary tumors of the heart overview|Overview]]==
 
==Overview==
 
The '''primary tumors of the heart''' are [[tumor]]s that arise from the normal tissues that make up the [[heart]].  This is in contrast to secondary tumors of the heart, which are typically either [[metastatic cancer|metastatic]] from another part of the body, or infiltrate the heart via direct extension from the surrounding tissues.
 
The most common primary tumor of the heart is the [[myxoma]].  In surgical series, the myxoma makes up as much as 77% of all primary tumors of the heart.<ref name="MolinaEdwards">{{cite journal | author=Molina JE, Edwards JE, Ward HB | title=Primary cardiac tumors: Experience at the University of Minnesota | journal=Thorac Cardiovasc Surg | year=1990 | pages=183-91 | volume=38 Suppl 2| id=PMID 2237900}}</ref>  Other benign tumors of the heart include [[rhabdomyoma]]s, [[fibroma]]s, [[hemangioma]]s, [[lipoma]]s, [[paraganglioma]]s, myocytic hamartomas, and [[papillary fibroelastoma]]s<ref name="Edwards-1953134">{{cite journal | author=Edwards FH, Hale D, Cohen A, Thompson L, Pezzella AT, Virmani R. | title=Primary cardiac valve tumors | journal=Ann Thorac Surg | year=1991 | volume=52 | issue=5 | pages=1127-31 | id=PMID 1953134}}</ref>.
 
About 20 percent of primary tumors of the heart are malignant in nature.<ref name="MolinaEdwards"/>  Malignant tumors of the heart include [[rhabdomyosarcoma]]s, [[angiosarcoma]]s, myxosarcomas, [[fibrosarcoma]]s, [[leiomyosarcoma]]s, reticulum cell sarcomas, and [[liposarcoma]]s.  The cardiac sarcomas may occur at any age, but are more commonly seen in individuals in their 20s to 40s.  They occur equally in males and females.
 
A subset of the primary tumors of the heart are tumors that are found on the valves of the heart.  Tumors that affect the valves of the heart are found in an equal distribution among the four heart valves.<ref name="Edwards-1953134"/>  The vast majority of these are papillary fibroelastomas.  Primary tumors of the valves of the heart are more likely to occur in males.  While most primary tumors of the valves of the heart are not malignant, they are more likely to have symptoms related to the valve, including neurologic symptoms and (in a few cases) [[sudden cardiac death]].


==Epidemiology and Demographics==
==Epidemiology and Demographics==

Revision as of 22:55, 5 September 2011

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Overview

Epidemiology and Demographics

Overview

  • Prevalence in autopsy series: 1/300 to 1/5900
  • 80% of primary tumors are benign:
  • 1/2 (40%) are myxomas
  • 1/2 (40% ) are of other types

Benign Tumors of the Heart

The most common primary tumor of the heart is the myxoma. In surgical series, the myxoma makes up as much as 77% of all primary tumors of the heart.[1] Other benign tumors of the heart include rhabdomyomas, fibromas, hemangiomas, lipomas, paragangliomas, myocytic hamartomas, and papillary fibroelastomas[2].

Primary Malignant Tumors of the Heart

About 20 percent of primary tumors of the heart are malignant in nature.[1] Malignant tumors of the heart include rhabdomyosarcomas, angiosarcomas, myxosarcomas, fibrosarcomas, leiomyosarcomas, reticulum cell sarcomas, and liposarcomas. The cardiac sarcomas may occur at any age, but are more commonly seen in individuals in their 20s to 40s. They occur equally in males and females.

A subset of the primary tumors of the heart are tumors that are found on the valves of the heart. Tumors that affect the valves of the heart are found in an equal distribution among the four heart valves.[2] The vast majority of these are papillary fibroelastomas. Primary tumors of the valves of the heart are more likely to occur in males. While most primary tumors of the valves of the heart are not malignant, they are more likely to have symptoms related to the valve, including neurologic symptoms and (in a few cases) sudden cardiac death.

Secondary (metastatic) Malignant Tumors of the Heart

  • 20 to 40x more common than primary cardiac tumors
  • typically carcinomas, melanomas, and leukemias/lymphomas
  • discrete tumors found in 10 to 12% of all autopsies
  • any involvement of heart by a malignancy found in 20% of all autopsies

Pericardium

  • frequently bloody effusion containing tumor cells
  • tamponade
  • may also cause constriction from tumor infiltration of pericardial sac

Pathophysiology

  • Emboli into coronary arteries
  • Retrograde flow through cardiac lymphatics
  • Direct extension from mediastinum
  • Emboli or spread via great veins => endocardial metastases

Diagnosis

Electrocardiographic Findings

Replacement of electrically active tissue by inert tissue such as a tumor or Chagas disease can result in Q waves. Pericardial metastases can result in atrial fibrillation or atrial flutter.

CT

Labeled images below are courtesy of RadsWiki and copylefted.

  • Atrial Myxoma


  • Cardiac Rhabdomyosarcoma

MRI

MRI: Cardiac myxoma


Pathological Findings

Image shown below is courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology




Prognosis

The vast majority of the tumors of the heart have a benign course and are not directly fatal. However, even the benign tumors of the heart can be lethal due to either direct extension into the electrical conduction system of the heart (causing complete heart block or a fatal dysrhythmia), or due to emboli from the tumor mass that may have lethal sequelae.

The malignant tumors of the heart have a worse prognosis. Cardiac sarcomas generally lead to death within 2 years of diagnosis, due to rapid infiltration of the myocardium of the heart and obstruction of the normal flow of blood within the heart.[3][4]

References

  1. 1.0 1.1 Molina JE, Edwards JE, Ward HB (1990). "Primary cardiac tumors: Experience at the University of Minnesota". Thorac Cardiovasc Surg. 38 Suppl 2: 183–91. PMID 2237900.
  2. 2.0 2.1 Edwards FH, Hale D, Cohen A, Thompson L, Pezzella AT, Virmani R. (1991). "Primary cardiac valve tumors". Ann Thorac Surg. 52 (5): 1127–31. PMID 1953134.
  3. Burke AP, Cowan D, Virmani R (1992). "Primary sarcomas of the heart". Cancer. 69 (2): 387–95. PMID 1728367.
  4. Burke AP, Virmani R (1991). "Osteosarcomas of the heart". Am J Surg Pathol. 15 (3): 289–95. PMID 1705103.

See also

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