Primary tumors of the heart
Encyclopedia
The primary tumors of the heart are tumor
s that arise from the normal tissues that make up the heart
. This is in contrast to secondary tumors of the heart, which are typically either metastatic from another part of the body, or infiltrate the heart via direct extension from the surrounding tissues.
. In surgical series, the myxoma makes up as much as 77% of all primary tumors of the heart. Less common tumours of the heart include lipoma
and cystic tumor of the atrioventricular nodal region.
s, angiosarcoma
s, myxosarcomas, fibrosarcoma
s, leiomyosarcoma
s, reticulum cell sarcomas, desmoplastic small round cell tumor
,and liposarcoma
s. The cardiac sarcomas may occur at any age, but are more commonly seen in individuals in their 20s to 40s. They occur equally in males and females.
s. Primary tumors of the valves of the heart are more likely to occur in males. While most primary tumors of the valves of the heart are not malignant, they are more likely to have symptoms related to the valve, including neurologic symptoms and (in a few cases) sudden cardiac death
.
from the tumor mass that may have lethal sequelae.
The malignant tumors of the heart have a worse prognosis. Cardiac sarcomas generally lead to death within 2 years of diagnosis, due to rapid infiltration of the myocardium of the heart and obstruction of the normal flow of blood within the heart.
Tumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
s that arise from the normal tissues that make up the heart
Heart
The heart is a myogenic muscular organ found in all animals with a circulatory system , that is responsible for pumping blood throughout the blood vessels by repeated, rhythmic contractions...
. This is in contrast to secondary tumors of the heart, which are typically either metastatic from another part of the body, or infiltrate the heart via direct extension from the surrounding tissues.
Types
Benign
The most common primary tumor of the heart is the myxomaMyxoma
A myxoma is a tumor of primitive connective tissue. It is the most common primary tumor of the heart in adults, but can also occur in other locations....
. In surgical series, the myxoma makes up as much as 77% of all primary tumors of the heart. Less common tumours of the heart include lipoma
Lipoma
A lipoma is a benign tumor composed of adipose tissue. It is the most common form of soft tissue tumor. Lipomas are soft to the touch, usually movable, and are generally painless. Many lipomas are small but can enlarge to sizes greater than six centimeters. Lipomas are commonly found in adults...
and cystic tumor of the atrioventricular nodal region.
Malignant
About 20 percent of primary tumors of the heart are malignant in nature. Malignant tumors of the heart include rhabdomyosarcomaRhabdomyosarcoma
A rhabdomyosarcoma is a type of cancer, specifically a sarcoma , in which the cancer cells are thought to arise from skeletal muscle progenitors. It can also be found attached to muscle tissue, wrapped around intestines, or in any anatomic location...
s, angiosarcoma
Angiosarcoma
Angiosarcoma is a malignant neoplasm of endothelial-type cells that line vessel walls. This may be in reference to blood or lymphatic vessels ....
s, myxosarcomas, fibrosarcoma
Fibrosarcoma
Fibrosarcoma is a malignant tumor derived from fibrous connective tissue and characterized by immature proliferating fibroblasts or undifferentiated anaplastic spindle cells.Usually in males ages 30 to 40. Originates in fibrous tissues of the bone. Invades long or flat bones such as femur, tibia,...
s, leiomyosarcoma
Leiomyosarcoma
Leiomyosarcoma , aka LMS, is a malignant cancer of smooth muscle....
s, reticulum cell sarcomas, desmoplastic small round cell tumor
Desmoplastic small round cell tumor
Desmoplastic small-round-cell tumor is classified as a soft tissue sarcoma. It is an aggressive and rare tumor that primarily occurs as masses in the abdomen. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large...
,and liposarcoma
Liposarcoma
Liposarcoma is a malignant tumor that arises in fat cells in deep soft tissue, such as that inside the thigh or in the retroperitoneum.They are typically large bulky tumors which tend to have multiple smaller satellites extending beyond the main confines of the tumor.Liposarcomas, like all...
s. The cardiac sarcomas may occur at any age, but are more commonly seen in individuals in their 20s to 40s. They occur equally in males and females.
Valvular
A subset of the primary tumors of the heart are tumors that are found on the valves of the heart. Tumors that affect the valves of the heart are found in an equal distribution among the four heart valves. The vast majority of these are papillary fibroelastomaPapillary fibroelastoma
A papillary fibroelastoma is a primary tumor of the heart that typically involves one of the valves of the heart. Papillary fibroelastomas, while considered generally rare, make up about 10 percent of all primary tumors of the heart...
s. Primary tumors of the valves of the heart are more likely to occur in males. While most primary tumors of the valves of the heart are not malignant, they are more likely to have symptoms related to the valve, including neurologic symptoms and (in a few cases) sudden cardiac death
Sudden Cardiac Death
Sudden cardiac death is natural death from cardiac causes, heralded by abrupt loss of consciousness within one hour of the onset of acute symptoms. Other forms of sudden death may be noncardiac in origin...
.
Prognosis
The vast majority of the tumors of the heart have a benign course and are not directly fatal. However, even the benign tumors of the heart can be lethal due to either direct extension into the electrical conduction system of the heart (causing complete heart block or a fatal dysrhythmia), or due to emboliEmbolism
In medicine, an embolism is the event of lodging of an embolus into a narrow capillary vessel of an arterial bed which causes a blockage in a distant part of the body.Embolization is...
from the tumor mass that may have lethal sequelae.
The malignant tumors of the heart have a worse prognosis. Cardiac sarcomas generally lead to death within 2 years of diagnosis, due to rapid infiltration of the myocardium of the heart and obstruction of the normal flow of blood within the heart.