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

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.
• 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.

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