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{{Primary tumors of the heart}}
{{Primary tumors of the heart}}
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{{CMG}}; '''Associate Editor(s)-In-Chief:''' {{CZ}}; [[Varun Kumar]], M.B.B.S.
{{CMG}}; '''Associate Editor(s)-In-Chief:''' {{CZ}}; [[Varun Kumar]], M.B.B.S. {{DMakkar}}
 
==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.
 
*[[Cardiac myxomas]] may be associated with the autosomal dominant condition [[Carney complex]].
 
*[[Histiocytoid cardiomyopathy]] is sometimes inherited, however the underlying genetic disorders remain unknown.
 
*Most cardiac undifferentiated [[sarcomas]] share the same genetic profile as pulmonary artery intimal sarcomas, characterized by recurrent [[mutation]]s in the MDM2, PDGFRA, and CDKN2A genes.
*There have been reports of [[papillary fibroelastomas]] following cardiac instrumentation and thoracic irradiation.
 
==Pathophysiology==
==Pathophysiology==
{| class="wikitable"
|+ 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.<ref name="pmid11388092">{{cite journal| author=Pinede L, Duhaut P, Loire R| title=Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. | journal=Medicine (Baltimore) | year= 2001 | volume= 80 | issue= 3 | pages= 159-72 | pmid=11388092 | doi=10.1097/00005792-200105000-00002 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11388092  }} </ref>
|-
| [[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.<ref name="pmid8865802">{{cite journal| author=Benvenuti LA, Mansur AJ, Lopes DO, Campos RV| title=Primary lipomatous tumors of the cardiac valves. | journal=South Med J | year= 1996 | volume= 89 | issue= 10 | pages= 1018-20 | pmid=8865802 | doi=10.1097/00007611-199610000-00020 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8865802  }} </ref>
|-
| [[Papillary fibroelastoma]](Images F,G)|| Round, whitish appearance and soft consistency, sea anemone-like" appearance<ref name="pmid26725181">{{cite journal| author=Burke A, Tavora F| title=The 2015 WHO Classification of Tumors of the Heart and Pericardium. | journal=J Thorac Oncol | year= 2016 | volume= 11 | issue= 4 | pages= 441-52 | pmid=26725181 | doi=10.1016/j.jtho.2015.11.009 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26725181  }} </ref> || 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<ref name="pmid9458144">{{cite journal| author=Basso C, Valente M, Poletti A, Casarotto D, Thiene G| title=Surgical pathology of primary cardiac and pericardial tumors. | journal=Eur J Cardiothorac Surg | year= 1997 | volume= 12 | issue= 5 | pages= 730-7; discussion 737-8 | pmid=9458144 | doi=10.1016/s1010-7940(97)00246-7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9458144  }} </ref>
*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<ref name="pmid22219403">{{cite journal| author=Pacini D, Careddu L, Pantaleo A, Berretta P, Leone O, Marinelli G | display-authors=etal| title=Primary benign cardiac tumours: long-term results. | journal=Eur J Cardiothorac Surg | year= 2012 | volume= 41 | issue= 4 | pages= 812-9 | pmid=22219403 | doi=10.1093/ejcts/ezr067 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22219403  }} </ref>
*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<ref name="pmid20651614">{{cite journal| author=Orlandi A, Ferlosio A, Roselli M, Chiariello L, Spagnoli LG| title=Cardiac sarcomas: an update. | journal=J Thorac Oncol | year= 2010 | volume= 5 | issue= 9 | pages= 1483-9 | pmid=20651614 | doi=10.1097/JTO.0b013e3181e59a91 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20651614  }} </ref> || Most commonly involves right atrium
|-
| Secondary Cardiac Tumors(Image A) || Direct tumor infiltration from the mediastinum or hematogenous spread<ref name="pmid29744020">{{cite journal| author=Fred HL, van Dijk HA| title=Cardiac Metastases from Malignant Melanoma: The "Charcoal Heart". | journal=Methodist Debakey Cardiovasc J | year= 2017 | volume= 13 | issue= 4 | pages= 254-255 | pmid=29744020 | doi=10.14797/mdcj-13-4-254 | pmc=5935287 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29744020  }} </ref> || 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 tumors of the heart|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 [[myxoma|cardiac myxomas]] and cardiac fibromas.<ref name="Matsumoto-2007">{{cite journal | author=Matsumoto N, Sato Y, Kusama J, Matsuo S, Kinukawa N, Kunimasa T, Ichiyama I, Takahashi H, Kimura S, Orime Y, Saito S. | title=Multiple papillary fibroelastomas of the aortic valve: case report. | journal=Int J Cardiol | year=2007 | volume=122 | issue=1 | pages=e1-3 | id=PMID 17196273}}</ref>
:*The pathogenesis of papillary fibroelastoma is characterised by the mechanical effects of the tumor and the transient occlusion of the [[left coronary artery|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.<ref name="Takada-2000">{{cite journal | author=Takada A, Saito K, Ro A, Tokudome S, Murai T. | title= Papillary fibroelastoma of the aortic valve: a sudden death case of coronary embolism with myocardial infarction.| journal=Forensic Sci Int | year=2000 | volume=113 | issue=1-3 | pages=209-14 | id=PMID 10978627}}</ref>
===Gross Pathology===
===Gross Pathology===
Image shown below is courtesy of Professor Peter Anderson DVM PhD and published with permission. [http://www.peir.net © PEIR, University of Alabama at Birmingham, Department of Pathology]
Image shown below is courtesy of Professor Peter Anderson DVM PhD and published with permission. [http://www.peir.net © PEIR, University of Alabama at Birmingham, Department of Pathology]
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Image:Cardiac tumor 1.jpg|HEART: Metastatic Tumor: Gross very unusual large metastatic carcinoid in [[right atrium]]
Image:Cardiac tumor 1.jpg|'''Image A''' HEART: Metastatic Tumor: Gross very unusual large metastatic carcinoid in [[right atrium]]  
Image:Cardiac tumor 2.jpg|Cardiac Myxoma A gelatinous tumor is attached by a narrow pedicle to the atrial septum. The myxoma has an irregular surface and nearly fills the left atrium.  
Image:Cardiac tumor 2.jpg|'''Image B''' Cardiac Myxoma A gelatinous tumor is attached by a narrow pedicle to the atrial septum. The myxoma has an irregular surface and nearly fills the left atrium.  
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Image:Cardiac tumor 3.jpg|Cardiac Myxoma There was a calcified right atrial mass on the X ray of a 47-year-old man. Resection demonstrated a smooth-surfaced tumor. The gritty material seen microscopically on cut section was calcified and ossified myxoma.
Image:Cardiac tumor 3.jpg|'''Image C''' Cardiac Myxoma There was a calcified right atrial mass on the X ray of a 47-year-old man. Resection demonstrated a smooth-surfaced tumor. The gritty material seen microscopically on cut section was calcified and ossified myxoma.
Image:Cardiac tumor 4.jpg|Cardiac Fibroma Cut surface of the tumor shown in figure 6-2. The left ventricular (LV) cavity is present behind the mass. The patient was a 4-month-old child who died suddenly without a previous medical history.  
Image:Cardiac tumor 4.jpg|'''Image D''' Cardiac Fibroma Cut surface of the tumor shown in figure 6-2. The left ventricular (LV) cavity is present behind the mass. The patient was a 4-month-old child who died suddenly without a previous medical history.  
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Image:Cardiac tumor 5.jpg|This tumor was resected from the right atrium of a 1-year-old boy with pericardial effusions. Note areas of hemorrhage and dilated vessels. The patient was well 49 months postoperatively.  
Image:Cardiac tumor 5.jpg|'''Image E''' This tumor was resected from the right atrium of a 1-year-old boy with pericardial effusions. Note areas of hemorrhage and dilated vessels. The patient was well 49 months postoperatively.  
Image:Papillary fibroelastoma.jpg|Papillary fibroelastomas are often on the arterial surface and may project into the coronary ostium, causing ostial occlusion. This tumor is in the noncoronary sinus.
Image:Papillary fibroelastoma.jpg| '''Image F''' Papillary fibroelastomas are often on the arterial surface and may project into the coronary ostium, causing ostial occlusion. This tumor is in the noncoronary sinus.
Image:Papillary fibroelastoma 001.jpg| '''Image G''' Papillary Fibroelastoma: When located on the mitral valve, these tumors are usually on the anterior leaflet of the atrial surface.
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==References==
==References==
{{reflist|2}}
{{reflist|2}}
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Latest revision as of 05:16, 7 July 2022

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Cardiac tumors Microchapters

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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. 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


  • Image A HEART: Metastatic Tumor: Gross very unusual large metastatic carcinoid in right atrium

    Image A HEART: Metastatic Tumor: Gross very unusual large metastatic carcinoid in right atrium

  • Image B Cardiac Myxoma A gelatinous tumor is attached by a narrow pedicle to the atrial septum. The myxoma has an irregular surface and nearly fills the left atrium.

    Image B Cardiac Myxoma A gelatinous tumor is attached by a narrow pedicle to the atrial septum. The myxoma has an irregular surface and nearly fills the left atrium.


  • Image C Cardiac Myxoma There was a calcified right atrial mass on the X ray of a 47-year-old man. Resection demonstrated a smooth-surfaced tumor. The gritty material seen microscopically on cut section was calcified and ossified myxoma.

    Image C Cardiac Myxoma There was a calcified right atrial mass on the X ray of a 47-year-old man. Resection demonstrated a smooth-surfaced tumor. The gritty material seen microscopically on cut section was calcified and ossified myxoma.

  • Image D Cardiac Fibroma Cut surface of the tumor shown in figure 6-2. The left ventricular (LV) cavity is present behind the mass. The patient was a 4-month-old child who died suddenly without a previous medical history.

    Image D Cardiac Fibroma Cut surface of the tumor shown in figure 6-2. The left ventricular (LV) cavity is present behind the mass. The patient was a 4-month-old child who died suddenly without a previous medical history.


  • Image E This tumor was resected from the right atrium of a 1-year-old boy with pericardial effusions. Note areas of hemorrhage and dilated vessels. The patient was well 49 months postoperatively.

    Image E This tumor was resected from the right atrium of a 1-year-old boy with pericardial effusions. Note areas of hemorrhage and dilated vessels. The patient was well 49 months postoperatively.

  • Image F Papillary fibroelastomas are often on the arterial surface and may project into the coronary ostium, causing ostial occlusion. This tumor is in the noncoronary sinus.

    Image F Papillary fibroelastomas are often on the arterial surface and may project into the coronary ostium, causing ostial occlusion. This tumor is in the noncoronary sinus.

  • Image G Papillary Fibroelastoma: When located on the mitral valve, these tumors are usually on the anterior leaflet of the atrial surface.

    Image G Papillary Fibroelastoma: When located on the mitral valve, these tumors are usually on the anterior leaflet of the atrial surface.

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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