Cardiac tumors pathophysiology
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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
| Tumor | Macroscopic appearance | Microscopic appearance | Remarks |
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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. |
| 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.
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| 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
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| Cardiac Fibromas | Solitary well-defined formation,
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| Cardiac Sarcomas
Angiosarcoma ||Darkish brown or black hemorrhagic aggregates with invading margins || Vascular conduits, infiltrating cardiac muscle, anastomosing, uneven,
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| Secondary Cardiac Tumors | Direct tumor infiltration from the mediastinum or hematogenous spread | same as Underlying Malignancy | Pericardial effusion is one of the most frequent findings in individuals with secondary tumors.
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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
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HEART: Metastatic Tumor: Gross very unusual large metastatic carcinoid in right atrium
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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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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.
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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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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.
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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.
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Papillary Fibroelastoma: When located on the mitral valve, these tumors are usually on the anterior leaflet of the atrial surface.
References
- ↑ 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.