Myxoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2] Maria Fernanda Villarreal, M.D. [3]Cafer Zorkun, M.D., Ph.D. [4]Ahmad Al Maradni, M.D. [5]

Overview

Cardiac myxoma is a benign intracavitary endocardial mass that represents the most common primary tumor of the heart. Myxoma cells are characterized by undifferentiated mesenchymal cells, which potentially differentiate into many tissues such as blood vessels, glandular structures, and bones. The primary distribution of cardiac myxoma is the left atrium (75%) of the heart; regularly, they tend to be located in the fossa ovalis and endocardium of the atrial septum.

Pathogenesis

Pathogenesis

Genetics

Associated Conditions

Gross Pathology

Microscopic Pathology

  • The Gamna-Bodies which consist of fibrosis and deposition of iron pigments are a characteristic finding of myxoma tumors.[17]
Black arrow (top): Endothelium Black arrow (bottom): Hemosiderin macrophage. Source: Case courtesy by Nephron, via Wikimedia Commons
Gamna Bodies: A peculiar form of fibrosis with deposition of iron pigment, identical to that seen in the spleens of patients with sickle cell anemia, is not uncommon in myxoma. Image courtesy of Professor Peter Anderson DVM Ph.D. and published with permission © PEIR, the University of Alabama at Birmingham, Department of Pathology
Cardiac myxoma: Common features at the interface with the atrial septum include lymphoid aggregates, smooth muscle bundles, and thick-walled vessels which angiographically may look like neovascularization. Image courtesy of Professor Peter Anderson DVM Ph.D. and published with permission © PEIR, the University of Alabama at Birmingham, Department of Pathology
Cardiac myxoma: The extramedullary hematopoiesis seen here is present in about 7 percent of cardiac myxomas. Image courtesy of Professor Peter Anderson DVM Ph.D. and published with permission © PEIR, the University of Alabama at Birmingham, Department of Pathology
Cardiac myxoma: Glandular structures are seen in less than 5 percent of cases. In this example, they were limited to the base of the myxoma. Image courtesy of Professor Peter Anderson DVM Ph.D. and published with permission © PEIR, the University of Alabama at Birmingham, Department of Pathology

Immunohistochemistry

  • Another immunohistochemical marker, survivin (an apoptosis inhibitor) has been detected to play an important role in the development and growth of cardiac myxomas.[22]
Cardiac Myxoma Summary
Features Description
General aspects
  • Isolated cells with irregular cellular borders, mild or no atypia, absence of mitosis
  • Myxoma requires the presence of lepidic cells
Genetics
  • PRKAR1A gene plays an important role in cardiac development and myxomagenesis
Gross Pathology
Micropathology
Inmunohistochemistry

References

  1. Bulkley BH, Hutchins GM (1979). "Atrial myxomas: a fifty year review". Am. Heart J. 97 (5): 639–43. PMID 433739.
  2. 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.
  3. Roscher AA, Kato NS, Quan H, Padmanabhan M (1996). "Intra-atrial myxomas, clinical-pathologic correlation based on two case studies including historical review". J Cardiovasc Surg (Torino). 37 (6 Suppl 1): 131–7. PMID 10064365.
  4. Acebo E, Val-Bernal JF, Gómez-Román JJ (2001). "Prichard's structures of the fossa ovalis are not histogenetically related to cardiac myxoma". Histopathology. 39 (5): 529–35. PMID 11737312.
  5. 5.0 5.1 5.2 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.
  6. Orlandi A, Ciucci A, Ferlosio A, Genta R, Spagnoli LG, Gabbiani G (2006). "Cardiac myxoma cells exhibit embryonic endocardial stem cell features". J. Pathol. 209 (2): 231–9. doi:10.1002/path.1959. PMID 16508920.
  7. Amano J, Kono T, Wada Y, Zhang T, Koide N, Fujimori M, Ito K (2003). "Cardiac myxoma: its origin and tumor characteristics". Ann Thorac Cardiovasc Surg. 9 (4): 215–21. PMID 13129418.
  8. 8.0 8.1 8.2 Reynen K (1995). "Cardiac myxomas". N. Engl. J. Med. 333 (24): 1610–7. doi:10.1056/NEJM199512143332407. PMID 7477198.
  9. 9.0 9.1 9.2 9.3 Sun Y, Chen X, Sun J, Wen X, Liu X, Zhang Y, Hoffman AR, Hu JF, Gao Y (2015). "A Novel Inherited Mutation in PRKAR1A Abrogates PreRNA Splicing in a Carney Complex Family". Can J Cardiol. 31 (11): 1393–401. doi:10.1016/j.cjca.2015.05.018. PMID 26416542.
  10. Correa R, Salpea P, Stratakis CA (October 2015). "Carney complex: an update". Eur. J. Endocrinol. 173 (4): M85–97. doi:10.1530/EJE-15-0209. PMC 4553126. PMID 26130139.
  11. Stratakis, Constantine A.; Kirschner, Lawrence S.; Carney, J. Aidan (2001). "Clinical and Molecular Features of the Carney Complex: Diagnostic Criteria and Recommendations for Patient Evaluation". The Journal of Clinical Endocrinology & Metabolism. 86 (9): 4041–4046. doi:10.1210/jcem.86.9.7903. ISSN 0021-972X.
  12. Stratakis, Constantine A.; Kirschner, Lawrence S.; Carney, J. Aidan (2001). "Clinical and Molecular Features of the Carney Complex: Diagnostic Criteria and Recommendations for Patient Evaluation". The Journal of Clinical Endocrinology & Metabolism. 86 (9): 4041–4046. doi:10.1210/jcem.86.9.7903. ISSN 0021-972X.
  13. Carney JA, Gordon H, Carpenter PC, Shenoy BV, Go VL. The complex of myxomas, spotty pigmentation, and endocrine overactivity. Medicine (Baltimore). 1985;64(4):270-83.
  14. Iglesias C, Torrelo A, Colmenero I, Mediero IG, Zambrano A, Requenca L. Isolated multiple congential epithelioid blue naevus. British Journal of Dermatology 2005;152:391-393.
  15. Gaissmaier et al. (letter and response) Carney Complex. Circulation 1999;100 (25); e150 http://circ.ahajournals.org/cgi/reprint/100/25/e150
  16. Reynen, K. (1995). "Cardiac Myxomas". New England Journal of Medicine. 333 (24): 1610–1617. doi:10.1056/NEJM199512143332407. PMID 7477198.
  17. 17.0 17.1 17.2 17.3 17.4 17.5 Di Vito A, Mignogna C, Donato G (2015). "The mysterious pathways of cardiac myxomas: a review of histogenesis, pathogenesis and pathology". Histopathology. 66 (3): 321–32. doi:10.1111/his.12531. PMID 25297937.
  18. He DK, Zhang YF, Liang Y, Ye SX, Wang C, Kang B; et al. (2015). "Risk factors for embolism in cardiac myxoma: a retrospective analysis". Med Sci Monit. 21: 1146–54. doi:10.12659/MSM.893855. PMC 4418206. PMID 25900256.
  19. Yoon DH, Roberts W (2002). "Sex distribution in cardiac myxomas". Am. J. Cardiol. 90 (5): 563–5. PMID 12208428.
  20. Vaideeswar P, Butany JW (2008). "Benign cardiac tumors of the pluripotent mesenchyme". Semin Diagn Pathol. 25 (1): 20–8. PMID 18350919.
  21. 21.0 21.1 Acebo E, Val-Bernal JF, Gómez-Roman JJ (2001). "Thrombomodulin, calretinin and c-kit (CD117) expression in cardiac myxoma". Histol. Histopathol. 16 (4): 1031–6. PMID 11642722.
  22. Lin YS, Jung SM, Wu HH, Shiu TF, Tzai FC, Chu JJ, Lin PJ, Chu PH (2011). "Survivin expression in cardiac myxoma". Chang Gung Med J. 34 (4): 360–6. PMID 21880190.


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