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Cardiac tumors

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Varun Kumar, M.B.B.S. Syed Hassan A. Kazmi BSc, MD [3]

Overview

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

Overview

The primary tumors of the heart are tumors 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 from another part of the body, or infiltrate the heart via direct extension from the surrounding tissues.

History and Symptoms

Cardiac tumors present with a wide variety of symptoms. Many patients remain asymptomatic, and the tumor is an incidental finding. Some patients may present with symptoms of mimicking those of valve abnormalities such as mitral stenosis. Patients may also present with rhythm disturbances,endocarditis or even heart failure.

Physical Examination

A “tumor plop” (a sound related to movement of the tumor), abnormal heart sounds, or murmur may be heard. These sounds may change when the patient changes position.

Electrocardiogram

There are no specific ECG abnormalities associated with heart tumors. The ECG changes vary depending on the location of the tumor and aid in the diagnosis of tumors when used in conjunction with other diagnostic modalities.

Historical Perspective

As early as 1559, Dr. Realdo Colombo wrote about heart tumors, and the first successful surgery to remove a left atrial myxoma was performed in 1954 by Dr. Clarence Crafoord. After the discovery of cardiac tumors, new chemotherapeutic agents and treatments have been developed.

Classification

Based on their origin, cardiac tumors are classed as either primary or secondary. Primary cardiac tumors are classified as either benign, malignant, or intermediate.

Pathophysiology

Cardiac tumors’ exact pathogenesis remains an enigma. Some cardiac tumors are linked to genetic predispositions or are a part of syndromes.

  • Cardiac rhabdomyomas are linked to “Tuberous sclerosis,” an autosomal dominant disorder characterized by the development of several organ hamartomas.


  • Cardiac fibromas may be related with Gorlin syndrome, an autosomal dominant disorder characterized by congenital anomalies and a predisposition to tumorigenesis.




  • The majority of undifferentiated cardiac sarcomas share the same genetic profile as pulmonary artery intimal sarcomas, which are defined by recurrent mutations in the MDM2, PDGFRA, and CDKN2A genes.

Causes

Numerous genetic abnormalities are linked to cardiac tumors however no direct causes have been established as of yet. Some genetic disorders closely related with a high incidence of cardiac tumors are Tuberous Sclerosis, Gorlin Syndrome, and Carneys Triad.

Differentiating Cardiac Tumors from other Diseases

Common cardiac tumor symptoms include fever, weight loss, and other constitutional symptoms. Each tumor has unique traits that distinguish it from others.

Risk Factors

There are no recognized cardiac tumor risk factors. However, HIV increases the risk of Kaposi sarcoma of the heart. Certain disorders, including Gorlin syndrome, Tuberous Sclerosis, LAMB, NAME, and Paragangliomas, are linked to an elevated risk of cardiac tumors. The presence of other tumors also increases the risk of secondary tumors in heart

Screening

The USPSTF does not suggest screening for heart tumors routinely. Transthoracic echocardiography (TTE) is the preferred screening tool for cardiac cancers. Valvular lesions are frequently detected by Transesophageal echocardiography (TEE).

Natural History, Complications and Prognosis

Cardiac tumors can originate in numerous ways, resulting in a variety of clinical manifestations. Regardless of the type of heart tumor, cardiac symptoms depend mostly on tumor location and size. Patients with cardiac tumors can experience systemic constitutional symptoms, such as fever, tiredness, and joint issues. Depending on the location of tumors, particular symptoms may develop. The prognosis for benign cardiac tumors is good, but recurrence is possible, whereas the prognosis for malignant tumors is dismal.

Diagnosis

Multiple cardiovascular imaging techniques could be utilized to outline the anatomy of the cardiac mass and its potential functional consequences.

The primary purpose of cardiovascular imaging is to assess the location, extent, and vascularity of the tumor, as well as any hemodynamic consequences and myopericardial invasion.

All patients should receive two-dimensional transthoracic echocardiography (TTE). A biopsy provides the definitive diagnosis. The technique for a final diagnosis is carried out using percutaneous cardiac biopsy and transvenous cardiac biopsy, guided by echocardiography, mediastinoscopy, or thoracotomy.

Staging | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | CT | MRI | Echocardiography | Other Imaging Findings | Other Diagnostic Studies

Treatment

The vast majority of cardiac tumors are treated surgically. Primary malignant cardiac tumors have a propensity for fast metastatic dissemination.

Systemic neoadjuvant therapy should be vigorously sought in hemodynamically stable patients with localized disease because it permits: (a) faster removal by lowering the size of the tumor, and (b) a reduction in the likelihood of systemic recurrence.

In certain instances, surgery may be an option for malignant tumors. Patients with a solitary heart problem and a controlled primary tumor are the best candidates. The tumor must be amenable to excision while preserving sufficient heart function to sustain life.

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1 | Case #2: Cardiac lymphoma

References

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Historical Perspective

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dheeraj Makkar, M.D.[2]

Overview

  • Realdo Colombo initially reported cardiac tumors in 1559, and Dr. Clarence Crafoord performed the first successful surgical resection of left atrial myxoma in 1954. New chemotherapy drugs and treatment have been developing after the discovery of cardiac tumors.

Historical Perspective

  • Cardiac Tumors were first described by Realdo Colombo in 1559.[1]
  • In 1936, Beck and Maurer were the first to resect right ventricular teratoma. They also resected a left ventricular lipoma in 1951.[2]
  • Dr. William Norris was the first to describe a “melanotic heart” with secondary metastases to the heart in 1820.[3]
  • In 1952, left atrial myxoma was successfully excised by Bahnson and Newman through a right atriotomy. Unfortunately, the patient succumbed to the procedure after 24 days.[4]

Landmark events in the development of treatment strategies

References

  1. 1.0 1.1 1.2 1.3 Chitwood WR (1992). “Clarence Crafoord and the first successful resection of a cardiac myxoma”. Ann Thorac Surg. 54 (5): 997–8. doi:10.1016/0003-4975(92)90676-u. PMID 1417305.
  2. Beck CS (1942). “AN INTRAPERICARDIAL TERATOMA AND A TUMOR OF THE HEART: BOTH REMOVED OPERATIVELY”. Ann Surg. 116 (2): 161–74. doi:10.1097/00000658-194208000-00001. PMC 1543811. PMID 17858078.
  3. Gibbs P, Cebon JS, Calafiore P, Robinson WA (1999). “Cardiac metastases from malignant melanoma”. Cancer. 85 (1): 78–84. PMID 9921977.
  4. BAHNSON HT, NEWMAN EV (1953). “Diagnosis and surgical removal of intracavitary myxoma of the right atrium”. Bull Johns Hopkins Hosp. 93 (3): 150–63. PMID 13094264.

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Classification

Primary cardiac tumors | Secondary cardiac tumors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dheeraj Makkar, M.D.[2]

Overview

  • Cardiac tumors are classified as either primary or secondary based on their origin.
  • Primary tumors can be categorized as benign, malignant, or intermediate.

Classification

In 2015, the World Health Organization (WHO) changed the classification of cardiac neoplasms, including benign tumors, tumor-like lesions, malignant tumors, and pericardial tumors. [1]

  • Now, cardiac tumors are categorized into primary and secondary.


 
 
 
Cardiac Tumors
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Primary
 
 
 
Secondary
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Malignant
 
 
 
 
Benign
 
 
 
 
Intermediate
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Low grade Sarcoma/Myxofibroma
 
 
 
High Grade sarcoma/Undifferentiated Pleomorphic Sarcoma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
CongenitalChildhood
 
 
Adult
 
 
 
 
 
 
 
 
 
 
 
Neoplastic benign
 
 
 
 
 
 
Non-neoplastic
 
 
 
 
 

Classification

Primary Cardiac Tumors

Secondary Cardiac Tumors

References

Template:WikiDoc Sources

  1. Travis WD, Brambilla E, Nicholson AG, Yatabe Y, Austin JHM, Beasley MB; et al. (2015). “The 2015 World Health Organization Classification of Lung Tumors: Impact of Genetic, Clinical and Radiologic Advances Since the 2004 Classification”. J Thorac Oncol. 10 (9): 1243–1260. doi:10.1097/JTO.0000000000000630. PMID 26291008.
Pathophysiology

Pathological Findings

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Varun Kumar, M.B.B.S. Dheeraj Makkar, M.D.[3]

Overview

The precise pathophysiology of cardiac tumors is unknown. Some cardiac tumors are associated with genetic predispositions or are components of genetic disorders.

  • Cardiac rhabdomyomas are associated with Tuberous sclerosis, an autosomal dominant condition characterized by the development of various organ hamartomas.
  • Cardiac fibromas may be associated with Gorlin syndrome, an autosomal dominant condition marked by congenital abnormalities and a propensity for neoplasia.
  • Most cardiac undifferentiated sarcomas share the same genetic profile as pulmonary artery intimal sarcomas, characterized by recurrent mutations in the MDM2, PDGFRA, and CDKN2A genes.
  • There have been reports of papillary fibroelastomas following cardiac instrumentation and thoracic irradiation.

Pathophysiology

Pathophysiology of Cardiac Tumors
Tumor Macroscopic appearance Microscopic appearance Remarks
Myxoma(Images B, C) Two Types Solid(round with a smooth, lustrous, undulating surface)/Villous(frequently fragile papillary surfaces) Cells have eosinophilic cytoplasm and an oval or round nucleus. Embolization is frequent.[1]
Cardiac Lipoma Isolated lumps that are yellow, soft, smooth, well-circumscribed, and encapsulated Fat cells exhibit minimal to no atypia, fibrous capsule They can cause arrhythmias or conduction system disorders and heart failure symptoms.[2]
Papillary fibroelastoma(Images F,G) Round, whitish appearance and soft consistency, sea anemone-like” appearance[3] Thin avascular papillary fronds that frequently display complicated branching Embolization is frequent.
  • Insufficiency or blockage of the valves could also be seen.
  • Symptoms of heart failure and a new murmur on physical examination are also common.
Rhabdomyomas Nodular, unencapsulated, whitish, or grey, range in size from millimeters to several centimeters Large vacuolated cells with transparent cytoplasm due to glycogen deposition, spider cells also present Heart block or ventricular tachyarrhythmias, ventricular tachyarrhythmias may develop[4]
  • May spontaneously resolve after birth, excision if symptomatic
Cardiac Fibromas(Image D) Solitary well-defined formation, Monomorphic fibroblasts exhibiting minimal or no atypia *Resembles fibromatosis or uterine leiomyomas May manifest like heart failure, ventricular tachyarrhythmia, Can present as Sudden Cardiac Death[5]
  • Second most common cardiac tumor in children, with a male predilection
  • Whorled appearance on transverse section
Angiosarcoma Darkish brown or black hemorrhagic aggregates with invading margins Vascular conduits, infiltrating cardiac muscle, anastomosing, uneven, *Pleomorphic lining cells displaying numerous mitotic figures[6] Most commonly involves right atrium
Secondary Cardiac Tumors(Image A) Direct tumor infiltration from the mediastinum or hematogenous spread[7] Same as underlying Malignancy Pericardial effusion is one of the most frequent findings in individuals with secondary tumors.
  • May also present as heart failure or valvular disease

Papillary fibroelastoma

  • Primary tumor of the heart that typically involves one of the valves of the heart
  • Papillary fibroelastoma is generally considered benign, and represents 10% of all primary tumors of the heart
  • Papillary fibroelastoma are the third most common type of primary tumor of the heart, behind cardiac myxomas and cardiac fibromas.[8]
  • The pathogenesis of papillary fibroelastoma is characterised by the mechanical effects of the tumor and the transient occlusion of the left main coronary artery (by the tumor), while a heart attack or sudden cardiac death may be due to embolisation of a portion of the tumor into a coronary artery.[9]

Gross Pathology

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




References

  1. Pinede L, Duhaut P, Loire R (2001). “Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases”. Medicine (Baltimore). 80 (3): 159–72. doi:10.1097/00005792-200105000-00002. PMID 11388092.
  2. Benvenuti LA, Mansur AJ, Lopes DO, Campos RV (1996). “Primary lipomatous tumors of the cardiac valves”. South Med J. 89 (10): 1018–20. doi:10.1097/00007611-199610000-00020. PMID 8865802.
  3. Burke A, Tavora F (2016). “The 2015 WHO Classification of Tumors of the Heart and Pericardium”. J Thorac Oncol. 11 (4): 441–52. doi:10.1016/j.jtho.2015.11.009. PMID 26725181.
  4. Basso C, Valente M, Poletti A, Casarotto D, Thiene G (1997). “Surgical pathology of primary cardiac and pericardial tumors”. Eur J Cardiothorac Surg. 12 (5): 730–7, discussion 737-8. doi:10.1016/s1010-7940(97)00246-7. PMID 9458144.
  5. Pacini D, Careddu L, Pantaleo A, Berretta P, Leone O, Marinelli G; et al. (2012). “Primary benign cardiac tumours: long-term results”. Eur J Cardiothorac Surg. 41 (4): 812–9. doi:10.1093/ejcts/ezr067. PMID 22219403.
  6. Orlandi A, Ferlosio A, Roselli M, Chiariello L, Spagnoli LG (2010). “Cardiac sarcomas: an update”. J Thorac Oncol. 5 (9): 1483–9. doi:10.1097/JTO.0b013e3181e59a91. PMID 20651614.
  7. Fred HL, van Dijk HA (2017). “Cardiac Metastases from Malignant Melanoma: The “Charcoal Heart. Methodist Debakey Cardiovasc J. 13 (4): 254–255. doi:10.14797/mdcj-13-4-254. PMC 5935287. PMID 29744020.
  8. Matsumoto N, Sato Y, Kusama J, Matsuo S, Kinukawa N, Kunimasa T, Ichiyama I, Takahashi H, Kimura S, Orime Y, Saito S. (2007). “Multiple papillary fibroelastomas of the aortic valve: case report”. Int J Cardiol. 122 (1): e1–3. PMID 17196273.
  9. Takada A, Saito K, Ro A, Tokudome S, Murai T. (2000). “Papillary fibroelastoma of the aortic valve: a sudden death case of coronary embolism with myocardial infarction”. Forensic Sci Int. 113 (1–3): 209–14. PMID 10978627.

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Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dheeraj Makkar, M.D.[2]

Overview

  • Numerous genetic disorders are associated with cardiac tumors however, till now no direct causes have been identified.

Causes

  • Many genetic conditions are strongly associated with a large number of heart tumors. Some of them are as follows:[1]
Cardiac tumors genetic causes
Tumor Syndrome Gene Associated with syndrome
Rhabdomyoma Tuberous Sclerosis TSC1 or hamartin

TSC2 or tuberin

Fibroma Gorlin Syndrome PATCHED gene
Myxoma Carney Complex Inactivating mutation of PRKARA1A

Activating pathogenic variant of PRKACA gene

Paraganglioma Carney triad No known gene associations
Paraganglioma Hereditary Paraganglioma Mutation of succinate dehydrogenase subunit

SDHAF2 gene, and possibly MAX gene but these mutations are rare

Paraganglioma Von Hippel Lindau VHL gene
Paraganglioma Neurofibromatosis type 1 NF1 gene


References

  1. Lee E, Mahani MG, Lu JC, Dorfman AL, Srinivasan A, Agarwal PP (2018). “Primary cardiac tumors associated with genetic syndromes: a comprehensive review”. Pediatr Radiol. 48 (2): 156–164. doi:10.1007/s00247-017-4027-2. PMID 29214333.

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Differentiating Cardiac Tumors from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

  • The cardiac tumors have some common features like fever, weight loss, and other constitutional symptoms. Each tumor has its peculiar characteristics that sets it apart from other tumors.

Differenting Cardiac Tumors from Other Diseases

Primary Cardiac Tumors
Benign Malignant
Myxoma Rhabdomyosarcoma
Rhabdomyoma Angiosarcoma
Fibroelastoma Fibrosarcoma
Cardiac Lipoma Lymphoma
Cardiac Hemangioma
Cardiac Fibroma
Atrioventricular tumors
Paragangliomas

Differential Diagnosis of Cardiac Mass

Cardiac tumors present as a cardiac mass on imaging. The following are the differentials of cardiac mass:[2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43][44][45][46][47][48]

Site of Tumor Malignant Potential Type of Tumor Tissue of Origin Age of Presentation Location Morphology Signs and Symptoms MRI Findings
Systemic Manifestations Cardiac Manifestations Embolic Manifestations
Primary Cardiac Tumor Primary Benign Myxoma
  • Between third to sixth decade of life
  • 1-15 cm in diameter
  • Pedunculated
  • Polypoid
  • Smooth and lobulated
  • Villous and pappillary appearance associated with embolization
Rhabdomyoma
  • Striated Muscle
  • 1-3 cm in size
  • Yellow-gray color
  • Firm
  • Circumscribed lobulated
  • Majority multiple if associated with tuberous sclerosis (of those with no association, 50% are single)
  • Multiple masses isointense to muscle tissue on T1 images
  • Hyperintense on T2 images
Fibroma
  • Children (1/3rd in infants)
  • Solitary
  • Solitary mass
  • Low intensity on T2 weighted image
Fibroelastoma
  • < 1 cm in diameter
  • Solitary
  • Papillary
  • Flower-like appearance with multiple attachments to valve
  • Short pedicle
  • T1 and T2 weighted images show uniform intermediate signal intensity similar to myocardium
  • Homogeneous late gadolinium contrast enhancement
Hemangioma
  • < 1 year to 70 years
  • < 2 % of primary cardiac tumors
  • Polypoid
  • Encapsulated
  • Intermediate density on T1 images
  • Hypointense on T2 images
  • Multicystic enhancing lesion
  • Involvement of epicardium or pericardium
Lipoma
  • Between fourth to sixth decade of life
  • Epicardial or intramural lesion
  • High intensity on T1 weighted image
  • Drop out on fat saturation images
Paraganglioma
  • Average age of presentation is 11-13 years
    • 3-8 cm
    • Well-defined
    • Broad base
    • Encapsulated
    • Heterogeneous
    • Hypervascular
    Atrioventricular Node Tumor
    • Average age of diagnosis is 38 years
    • Female to male ratio 3:1
    • Hypointense cardiac mass on standard imaging
    • Hyperintensity on late gadolinium enhancement (LGE) images with heterogeneous contrast enhancement
    Lipomatous hypertrophy of the interatrial septum
    • > 60 years
    Primary Malignant Fibrosarcoma
    • 20 to 80 years
    • Sessile or pedunculated protruding masses in ventricular cavities
    • Soft
    • Lobulated
    • Gelatinous
    • Heterogenous or isointense to myocardium on T1 weighted images
    Angiosarcoma
    • 30 to 50 years
    • Arterial phase enhancement
    Rhabdomyosarcoma
    • Most common primary sarcoma of children
    • Average age of presentation is 20 years
    • Multiple
    • Three types:
      • Embryonic
      • Pleomorphic
      • Alveolar
    • Intermediate-to-hypointensity compared with muscle on T1 images
    • Hyperintense on T2-weighted imaging with heterogeneous contrast enhancement
    Lymphoma
    • 10 to 90 years
    • Males > females
    • Right sided mostly
    • Majority solitary (1/3rd multiple)
    • Hypointense on T1-weighted images and hyperintense on T2-weighted images
    Secondary Cardiac Tumor Metastastatic Malignant Metastasis
    • Any age
    • Multiple

      References

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      32. Parissis H, Akbar MT, Young V (October 2010). “Primary leiomyosarcoma of the right atrium: a case report and literature update”. J Cardiothorac Surg. 5: 80. doi:10.1186/1749-8090-5-80. PMC 2964688. PMID 20939891.
      33. Gulati G, Sharma S, Kothari SS, Juneja R, Saxena A, Talwar KK (2004). “Comparison of echo and MRI in the imaging evaluation of intracardiac masses”. Cardiovasc Intervent Radiol. 27 (5): 459–69. doi:10.1007/s00270-004-0123-4. PMID 15383848.
      34. Narin B, Arman A, Arslan D, Simşek M, Narin A (February 2010). “Assessment of cardiac masses: magnetic resonance imaging versus transthoracic echocardiography”. Anadolu Kardiyol Derg. 10 (1): 69–74. PMID 20150010.
      35. “academic.oup.com”.
      36. Ismail I, Al-Khafaji K, Mutyala M, Aggarwal S, Cotter W, Hakim H, Khosla S, Arora R (2015). “Cardiac lipoma”. J Community Hosp Intern Med Perspect. 5 (5): 28449. doi:10.3402/jchimp.v5.28449. PMC 4612478. PMID 26486106.
      37. D’Souza J, Shah R, Abbass A, Burt JR, Goud A, Dahagam C (January 2017). “Invasive Cardiac Lipoma: a case report and review of literature”. BMC Cardiovasc Disord. 17 (1): 28. doi:10.1186/s12872-016-0465-2. PMC 5237479. PMID 28088193.
      38. Yadav, Pradeep K.; Baquero, Giselle A.; Malysz, Jozef; Kelleman, John; Gilchrist, Ian C. (2014). “Cardiac Paraganglioma”. Circulation: Cardiovascular Interventions. 7 (6): 851–856. doi:10.1161/CIRCINTERVENTIONS.114.001856. ISSN 1941-7640.
      39. Tahir M, Noor SJ, Herle A, Downing S (2009). “Right atrial paraganglioma: a rare primary cardiac neoplasm as a cause of chest pain”. Tex Heart Inst J. 36 (6): 594–7. PMC 2801953. PMID 20069088.
      40. Hamilton BH, Francis IR, Gross BH, Korobkin M, Shapiro B, Shulkin BL, Deeb CM, Orringer MB (January 1997). “Intrapericardial paragangliomas (pheochromocytomas): imaging features”. AJR Am J Roentgenol. 168 (1): 109–13. doi:10.2214/ajr.168.1.8976931. PMID 8976931.
      41. Shih, Wei-Jen; McCullough, Scott; Smith, Mary (1993). “Diagnostic imagings for primary cardiac fibrosarcoma”. International Journal of Cardiology. 39 (2): 157–161. doi:10.1016/0167-5273(93)90028-F. ISSN 0167-5273.
      42. Arai T, Kurashima C, Wada S, Chida K, Ohkawa S (November 1998). “Histological evidence for cell proliferation activity in cystic tumor (endodermal heterotopia) of the atrioventricular node”. Pathol. Int. 48 (11): 917–23. PMID 9832064.
      43. Wolf PL, Bing R (November 1965). “The smallest tumor which causes sudden death”. JAMA. 194 (6): 674–5. PMID 5897246.
      44. Burke AP, Anderson PG, Virmani R, James TN, Herrera GA, Ceballos R (October 1990). “Tumor of the atrioventricular nodal region. A clinical and immunohistochemical study”. Arch. Pathol. Lab. Med. 114 (10): 1057–62. PMID 2222148.
      45. Burke A, Tavora F (April 2016). “The 2015 WHO Classification of Tumors of the Heart and Pericardium”. J Thorac Oncol. 11 (4): 441–52. doi:10.1016/j.jtho.2015.11.009. PMID 26725181.
      46. Tran, Thao T; Starnes, Vaughn; Wang, Xuedong; Getzen, James; Ross, Brian D (2009). “Cardiovascular magnetics resonance diagnosis of cystic tumor of the atrioventricular node”. Journal of Cardiovascular Magnetic Resonance. 11 (1): 13. doi:10.1186/1532-429X-11-13. ISSN 1532-429X.
      47. Tatli, Servet; O’Gara, Patrick Thomas; Lambert, Jarvis; Kwong, Raymond; Byrne, John Gerald; Yucel, E. Kent (2004). “MRI of Atypical Lipomatous Hypertrophy of the Interatrial Septum”. American Journal of Roentgenology. 182 (3): 598–600. doi:10.2214/ajr.182.3.1820598. ISSN 0361-803X.
      48. Saboo, Sachin S.; Krajewski, Katherine M.; Zukotynski, Katherine; Howard, Stephanie; Jagannathan, Jyothi P.; Hornick, Jason L.; Ramaiya, Nikhil (2012). “Imaging Features of Primary and Secondary Adult Rhabdomyosarcoma”. American Journal of Roentgenology. 199 (6): W694–W703. doi:10.2214/AJR.11.8213. ISSN 0361-803X.

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      Epidemiology and Demographics

      Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Varun Kumar, M.B.B.S. Dheeraj Makkar, M.D.[3]

      Overview

      The rates of primary and secondary cardiac tumors were 0.056 and 1.23 percent, respectively, as determined by autopsy. Lung, esophageal, and lymphoma metastases are the three most prevalent malignant neoplasms impacting the heart. Nearly 90% of primary cardiac tumors are benign. Extracardiac tumors are 20–40 times greater probable than primary cardiac tumors to cause secondary heart involvement. A primary intracardiac tumor may be responsible for less than 1 percent of cardiovascular fatalities.

      Epidemiology and Demographics

      Prevalence

      • 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

      Primary Malignant Tumors of the Heart

      About 20 percent of primary tumors of the heart are malignant in nature.[1]

      Tumors of the Valve

      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.
        • The primary tumors of the valves of the heart are mainly benign.
        • The symptoms related to the valve, include 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

      References

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      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.
      Risk Factors

      Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dheeraj Makkar, M.D.[2]

      Overview

      There are no direct risk factors for cardiac tumors. Most of them are sporadic but some are associated with certain syndromes and can be congenital.

      Risk Factors

      Factors affecting Prognosis

      • There are definitive risk factors influencing the prognosis of patients afflicted with cardiac tumors.
        • The independent risk factors associated with poor prognosis of cardiac tumors include, conservative management, malignant nature of tumor, and ”biatrial” tumors.[4]
        • It is also important to note that features like age, sex, NYHA grading, leg edema, and peripheral embolism do not affect the longevity of patients with cardiac tumors. [4]

      References

      1. Ghosn J, Taiwo B, Seedat S, Autran B, Katlama C (2018). “HIV”. Lancet. 392 (10148): 685–697. doi:10.1016/S0140-6736(18)31311-4. PMID 30049419.
      2. Lee E, Mahani MG, Lu JC, Dorfman AL, Srinivasan A, Agarwal PP (2018). “Primary cardiac tumors associated with genetic syndromes: a comprehensive review”. Pediatr Radiol. 48 (2): 156–164. doi:10.1007/s00247-017-4027-2. PMID 29214333.
      3. 3.0 3.1 Reynen K, Köckeritz U, Strasser RH (2004). “Metastases to the heart”. Ann Oncol. 15 (3): 375–81. doi:10.1093/annonc/mdh086. PMID 14998838.
      4. 4.0 4.1 Maimaitiaili A, Zhang WM, Guo YZ, Elmaik D, Tang NH, Zhang ZG (2019). “Survival of Patients with Primary Cardiac Tumors in an 11-Year Single Center Study”. Heart Surg Forum. 22 (2): E097–E102. doi:10.1532/hsf.2271. PMID 31013217.

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      Screening

      Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dheeraj Makkar, M.D.[2]

      Overview

      There are no screening guidelines by USPSTF for cardiac tumors.

      Screening

      • According to the USPSTF, routine screening for cardiac tumors is not recommended.
      • Transthoracic echocardiography (TTE) continues to be the method of choice for screening for cardiac malignancies. Transesophageal echocardiogram (TEE) is commonly used for valvular lesions.[1]
      • Emerging technologies such as contrast echocardiography and three-dimensional echocardiogram may substantially improve the diagnostic accuracy of echocardiography.[2]
      • The diagnostic accuracy of echocardiography is 80%. After initial diagnosis on TTE, cardiac CT/MRI are commonly used to stage and further evaluate the tumors.[3]
      • In patients undergoing myxoma resection, routine echocardiography screening must be performed one year following removal of myxoma and then every five years.[1]
      • Pediatric patients with tuberous sclerosis require monitoring because of the risk of rhabdomyoma.
        • At the initial diagnosis, electrocardiograms and echocardiography must be performed on all kids with tuberous sclerosis.
        • Periodic monitoring of electrocardiograms are advised every three to five years due to the lifetime risk of arrhythmia regardless of the presence early-onset cardiac rhabdomyoma. [4]

      References

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      1. 1.0 1.1 Lamba G, Frishman WH (2012). “Cardiac and pericardial tumors”. Cardiol Rev. 20 (5): 237–52. doi:10.1097/CRD.0b013e31825603e7. PMID 22447042.
      2. Mankad R, Herrmann J (2016). “Cardiac tumors: echo assessment”. Echo Res Pract. 3 (4): R65–R77. doi:10.1530/ERP-16-0035. PMC 5292983. PMID 27600455.
      3. Nomoto N, Tani T, Konda T, Kim K, Kitai T, Ota M; et al. (2017). “Primary and metastatic cardiac tumors: echocardiographic diagnosis, treatment and prognosis in a 15-years single center study”. J Cardiothorac Surg. 12 (1): 103. doi:10.1186/s13019-017-0672-7. PMC 5704631. PMID 29183343.
      4. Hinton RB, Prakash A, Romp RL, Krueger DA, Knilans TK, International Tuberous Sclerosis Consensus Group (2014). “Cardiovascular manifestations of tuberous sclerosis complex and summary of the revised diagnostic criteria and surveillance and management recommendations from the International Tuberous Sclerosis Consensus Group”. J Am Heart Assoc. 3 (6): e001493. doi:10.1161/JAHA.114.001493. PMC 4338742. PMID 25424575.
      Natural History, Complications and Prognosis

      Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Cafer Zorkun, M.D., Ph.D. [2] Dheeraj Makkar, M.D.[3]

      Overview

      Patients with heart tumors may develop systemic constitutional symptoms like fever, fatigue, and joint problems. Depending on the location of tumors, specific symptoms may manifest. The prognosis for benign cardiac tumors is favorable, but there is a potential of recurrence, but the prognosis for malignant tumors is poor.

      Natural History

      Cardiac tumors can arise in a multitude of ways, resulting in diverse clinical presentations. Irrespective of cardiac tumor type, cardiac symptoms primarily depend on tumor position and extent.[1]

      • Patients may develop systemic symptoms including fever, fatigue, and joint pains but can be asymptomatic as well. Myxomas were asymptomatic in 72% cases in a study by Dias et al.[2]
      • Tumors can impede blood flow, causing hemodynamic abnormalities, poor cardiac output, or heart failure.
      • Malignant tumors may especially cause weight loss, anorexia, and exhaustion.
      • In addition, valvular disease can lead to stenosis or regurgitation.[3]
      • Local infiltration of the cardiac conduction system could result in arrhythmias.
      • Non-specific features like pericardial effusion and compression of nearby structures, such as the coronary arteries, might develop.
      • Clinically, the tumor type (benign or malignant), anatomical location, time duration of receiving treatment and available treatment options are the most critical clinical criteria in determining a cardiac tumor patient’s natural history of disease.
      • The relapse rate of benign cardiac tumors is minimal. In some familial types of benign tumors, such as familial myxomas, however, incidence rates of tumor recurrence can reach as high as 22%.[4] In certain syndromes like Tuberous Sclerosis the rhabdomyomas disappear and have a good prognosis.
      • In contrast, malignant tumors often have a poorer prognosis and are difficult to treat due to their more invasive behavior, metastatic origin, and high recurrence rate. Malignant tumors have a much greater mortality rate. Most of the patients succumb to the tumor within an year even with treatment[4]

      Complications

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

      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.[5][6]

      References

      1. Paraskevaidis IA, Michalakeas CA, Papadopoulos CH, Anastasiou-Nana M (2011). “Cardiac tumors”. ISRN Oncol. 2011: 208929. doi:10.5402/2011/208929. PMC 3195386. PMID 22091416.
      2. Dias RR, Fernandes F, Ramires FJ, Mady C, Albuquerque CP, Jatene FB (2014). “Mortality and embolic potential of cardiac tumors”. Arq Bras Cardiol. 103 (1): 13–8. doi:10.5935/abc.20140096. PMC 4126756. PMID 25029470.
      3. Avakian SD, Takada JY, Mansur Ade P (2012). “Giant obstructive left atrial myxoma resembling mitral valve stenosis”. Clinics (Sao Paulo). 67 (7): 853–4. doi:10.6061/clinics/2012(07)25. PMC 3400890. PMID 22892935.
      4. 4.0 4.1 Palaskas N, Thompson K, Gladish G, Agha AM, Hassan S, Iliescu C; et al. (2018). “Evaluation and Management of Cardiac Tumors”. Curr Treat Options Cardiovasc Med. 20 (4): 29. doi:10.1007/s11936-018-0625-z. PMID 29556752.
      5. Burke AP, Cowan D, Virmani R (1992). “Primary sarcomas of the heart”. Cancer. 69 (2): 387–95. PMID 1728367.
      6. Burke AP, Virmani R (1991). “Osteosarcomas of the heart”. Am J Surg Pathol. 15 (3): 289–95. PMID 1705103.

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      Diagnosis

      Diagnosis

      Staging | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | CT | MRI | Echocardiography | Other Imaging Findings | Other Diagnostic Studies

      Treatment

      Treatment

      Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

      Case Studies

      Case Studies

      Case #1 | Case #2: Cardiac lymphoma

      Related Chapters


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