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| Myxoma || 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.
| Myxoma || 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.
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|-
| 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.
| 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.
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|-
| Papillary fibroelastoma|| Round, whitish appearance and soft consistency, sea anemone-like" appearance || 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.
| Papillary fibroelastoma|| Round, whitish appearance and soft consistency, sea anemone-like" appearance || 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 * may spontaneously resolve after birth, excision if symptomatic
| 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  
*May spontaneously resolve after birth, excision if symptomatic
|-
|-
| Example || Example || Example || Example
| Example || Example || Example || Example

Revision as of 19:52, 16 June 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.

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

Pathophysiology of Cardiac Tumors
Tumor Macroscopic appearance Microscopic appearance Remarks
Myxoma 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.
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.
Papillary fibroelastoma Round, whitish appearance and soft consistency, sea anemone-like" appearance 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
  • May spontaneously resolve after birth, excision if symptomatic
Example Example Example Example
Example Example Example Example
Example Example Example Example

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.[1]
  • 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.[2]

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. 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.
  2. 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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