Cardiac tumors pathophysiology: Difference between revisions
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{{Primary tumors of the heart}} | {{Primary tumors of the heart}} | ||
{{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== | ==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" | ||
:[[Cardiac tumors | |+ 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=== | |||
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] | |||
<div align="left"> | |||
<gallery heights="175" widths="175"> | |||
Image:Cardiac tumor 1.jpg|'''Image A''' HEART: Metastatic Tumor: Gross very unusual large metastatic carcinoid in [[right 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. | |||
</gallery> | |||
</div> | |||
<div align="left"> | |||
<gallery heights="175" widths="175"> | |||
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|'''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. | |||
</gallery> | |||
</div> | |||
<div align="left"> | |||
<gallery heights="175" widths="175"> | |||
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| '''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. | |||
</gallery> | |||
</div> | |||
==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 pathophysiology On the Web |
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Risk calculators and risk factors for Cardiac tumors pathophysiology |
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.
- 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 mutations in the MDM2, PDGFRA, and CDKN2A genes.
- There have been reports of papillary fibroelastomas following cardiac instrumentation and thoracic irradiation.
Pathophysiology
| 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.
|
| 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]
|
| 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]
|
| 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.
|
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 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 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 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.
References
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.