Myxoma differential diagnosis

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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] Ahmad Al Maradni, M.D. [3] Maria Fernanda Villarreal, M.D. [4]

Overview

Cardiac myxoma should be differentiated from other benign and malignant primary heart tumors including papillary fibroelastoma, lipoma, rhabdomyoma, and cardiac metastasis. Primarily, cardiac myxoma should be differentiated from other diseases that cause mitral valve obstruction and embolic events, such as thrombi or endocarditis.[1]

Differentiating Myxoma from other Diseases

Cardiac myxoma should be differentiated from other cardiac tumors that present as a cardiac mass. The following are the differentials:[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


  • Differential diagnosis of intracardiac masses requires histological analysis and a direct assessment of the specific location (endocardial, myocardial, and pericardial). The table below summarizes the findings that differentiate cardiac myxoma from other conditions that are also intracavitary cardiac masses and cause embolic events:
Disease Findings
Papillary fibroelastoma Is a rare benign primary tumor of the heart that typically involves one of the valves of the heart. Presents with syncope, chest pain, myocardial infarction, stroke and sudden cardiac death. They are the third most common type of primary tumor of the heart. There is a slightly increased male predilection.[49][50]
Lipoma Also known as "Lipomatous hypertrophy (LHIS)" is a very rare benign primary cardiac neoplasm. It is the proliferation of mature adipocytes. Lipomas are typically found in adult patients but can affect all ages. A common clinical feature is arrythmias.[51]
Rhabdomyoma Is the most common primary cardiac tumor in children. It is conformed from hamartomatous accumulation of the striated cardiomyocytes. Rhabdomyomas occur with more frequency in the myocardium of the left ventricle or in the interventricular septum. Is commonly associated with tuberous sclerosis, and congenital defects of the heart.[52]
Thrombus Intracardiac thrombi, can occur when there is an elevated risk of blood clots developing, such as: atrial fibrillation (atrial thrombi predominate), mitral stenosis, heart valve replacement and myocardial infarction (ventricular thrombus formation).[53]
Metastasis Secondary malignant tumor of the heart, arising by lymphatic or hematogenous spread of a primary neoplasm. Presents with dyspnea, congestive heart failure, hypotension and malignant pericardial effusion. Any primary malignancy may metastasize to the heart, however, lung cancer is among the most common.[54]

References

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  2. ↑ Mankad R, Herrmann J (December 2016). "Cardiac tumors: echo assessment". Echo Res Pract. 3 (4): R65–R77. doi:10.1530/ERP-16-0035. PMC 5292983. PMID 27600455.
  3. ↑ Zaragoza-Macias E, Zaragosa-Macias E, Chen MA, Gill EA (February 2012). "Real time three-dimensional echocardiography evaluation of intracardiac masses". Echocardiography. 29 (2): 207–19. doi:10.1111/j.1540-8175.2011.01627.x. PMID 22283202.
  4. ↑ Larrieu AJ, Jamieson WR, Tyers GF, Burr LH, Munro AI, Miyagishima RT, Gerein AN, Allen P (March 1982). "Primary cardiac tumors: experience with 25 cases". J. Thorac. Cardiovasc. Surg. 83 (3): 339–48. PMID 7062746.
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  7. ↑ Sarjeant JM, Butany J, Cusimano RJ (2003). "Cancer of the heart: epidemiology and management of primary tumors and metastases". Am J Cardiovasc Drugs. 3 (6): 407–21. doi:10.2165/00129784-200303060-00004. PMID 14728061.
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  13. ↑ Carney JA, Gordon H, Carpenter PC, Shenoy BV, Go VL (July 1985). "The complex of myxomas, spotty pigmentation, and endocrine overactivity". Medicine (Baltimore). 64 (4): 270–83. PMID 4010501.
  14. ↑ McAllister HA, Hall RJ, Cooley DA (February 1999). "Tumors of the heart and pericardium". Curr Probl Cardiol. 24 (2): 57–116. PMID 10028128.
  15. ↑ Klarich KW, Enriquez-Sarano M, Gura GM, Edwards WD, Tajik AJ, Seward JB (September 1997). "Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathologic correlation". J. Am. Coll. Cardiol. 30 (3): 784–90. PMID 9283541.
  16. ↑ Tamin SS, Maleszewski JJ, Scott CG, Khan SK, Edwards WD, Bruce CJ, Oh JK, Pellikka PA, Klarich KW (June 2015). "Prognostic and Bioepidemiologic Implications of Papillary Fibroelastomas". J. Am. Coll. Cardiol. 65 (22): 2420–9. doi:10.1016/j.jacc.2015.03.569. PMID 26046736.
  17. ↑ Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ (September 2003). "Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases". Am. Heart J. 146 (3): 404–10. doi:10.1016/S0002-8703(03)00249-7. PMID 12947356.
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  19. ↑ Smythe JF, Dyck JD, Smallhorn JF, Freedom RM (November 1990). "Natural history of cardiac rhabdomyoma in infancy and childhood". Am. J. Cardiol. 66 (17): 1247–9. PMID 2239731.
  20. ↑ Jacobs JP, Konstantakos AK, Holland FW, Herskowitz K, Ferrer PL, Perryman RA (November 1994). "Surgical treatment for cardiac rhabdomyomas in children". Ann. Thorac. Surg. 58 (5): 1552–5. PMID 7979700.
  21. ↑ Elbardissi AW, Dearani JA, Daly RC, Mullany CJ, Orszulak TA, Puga FJ, Schaff HV (September 2008). "Survival after resection of primary cardiac tumors: a 48-year experience". Circulation. 118 (14 Suppl): S7–15. doi:10.1161/CIRCULATIONAHA.107.783126. PMID 18824772.
  22. ↑ Basu S, Folliguet T, Anselmo M, Greengart A, Sabado M, Cunningham JN, Jacobowitz IJ (April 1994). "Lipomatous hypertrophy of the interatrial septum". Cardiovasc Surg. 2 (2): 229–31. PMID 8049952.
  23. ↑ Simpson L, Kumar SK, Okuno SH, Schaff HV, Porrata LF, Buckner JC, Moynihan TJ (June 2008). "Malignant primary cardiac tumors: review of a single institution experience". Cancer. 112 (11): 2440–6. doi:10.1002/cncr.23459. PMID 18428209.
  24. ↑ Vander Salm TJ (April 2000). "Unusual primary tumors of the heart". Semin. Thorac. Cardiovasc. Surg. 12 (2): 89–100. PMID 10807431.
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  28. ↑ Grebenc ML, Rosado de Christenson ML, Burke AP, Green CE, Galvin JR (2000). "Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation". Radiographics. 20 (4): 1073–103, quiz 1110–1, 1112. doi:10.1148/radiographics.20.4.g00jl081073. PMID 10903697.
  29. ↑ Grinda JM, Couetil JP, Chauvaud S, D'Attellis N, Berrebi A, Fabiani JN, Deloche A, Carpentier A (January 1999). "Cardiac valve papillary fibroelastoma: surgical excision for revealed or potential embolization". J. Thorac. Cardiovasc. Surg. 117 (1): 106–10. PMID 9869763.
  30. ↑ Webb DW, Thomas RD, Osborne JP (March 1993). "Cardiac rhabdomyomas and their association with tuberous sclerosis". Arch. Dis. Child. 68 (3): 367–70. PMC 1793857. PMID 8466239.
  31. ↑ Yoshitake I, Hata M, Sezai A, Niino T, Unosawa S, Shimura K, Kasamaki Y, Minami K (September 2009). "Cardiac angiosarcoma with cardiac tamponade diagnosed as a ruptured aneurysm of the sinus valsalva". Jpn. J. Clin. Oncol. 39 (9): 612–5. doi:10.1093/jjco/hyp044. PMID 19493870.
  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.
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  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.
  49. ↑ Papillary fibroelastoma of the heart. Dr Craig Hacking et al http://radiopaedia.org/articles/papillary-fibroelastoma-of-the-heart Accessed on November 24, 2015
  50. ↑ Papillary fibroelastoma .Wikipedia https://en.wikipedia.org/wiki/Papillary_fibroelastoma Accessed on November 24, 2015
  51. ↑ Lipoma. Dr Craig Hacking et al . Radiopedia. http://radiopaedia.org/articles/cardiac-lipoma Accessed on November 24, 2015
  52. ↑ Rhabdomyoma. Wikipedia.https://en.wikipedia.org/wiki/Rhabdomyoma Accessed on November 24, 2015
  53. ↑ Intracardiac thrombi. Dr Craig Hacking et al. Radiopedia.http://radiopaedia.org/articles/intracardiac-thrombi Accessed on November 24, 2015
  54. ↑ Secondary cardiac neoplasm. Radiopedia.http://radiopaedia.org/articles/secondary-cardiac-neoplasms Accessed on November 24, 2015


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