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Heart tumor, adult

Orpha number ORPHA874
Prevalence of rare diseases Unknown
Inheritance
  • Sporadic
Age of onset Adulthood
ICD 10 code -
MIM number -
Synonym(s) -

Summary

Primary tumors of the heart in adult age can be either benign or malignant. Great variability of incidence was found with values ranging from 1 per 56 000 and 1 per 360. Benign tumors occur more frequently than malignant ones with a ratio of 3:1. The most common benign tumors are in order of frequency: mixoma, lipoma, papillary fibroelastoma, rhabdomyoma, fibroma, hemangioma, teratoma, mesothelioma of the atrio-ventricular node, granular cell tumor, neurofibroma, lymphangioma. The most common malignant tumors are: angiosarcoma, rhabdomyosarcoma, mesothelioma, fibrosarcoma, malignant lymphoma, extra-skeletal osteosarcoma, thymoma, neurogenic sarcoma, leiomyosarcoma, liposarcoma, synovial sarcoma. The types of tumors found in the heart are similar to those found in the terminal parts of striated muscles. Symptoms strictly depend on the degree of involvement of heart structures. Pleural pericardial pain, dyspnea, angina may be described; systemic manifestations such as embolic phenomena, cachexia, and syncope can be observed. Clinical signs are usually: clubbing of the fingers, fever, rush, pericarditis, arrhythmias, conduction anomalies. Myocardial infarction, cardiac failure, reduction of atrial cavities and valvular dysfunction or obstruction may occur. Available diagnostic techniques are: echocardiography, computed tomography, magnetic resonance and radionuclide imaging. Treatment is strictly surgical including cardiac transplantation, though malignant tumors need further therapeutic support consisting in chemotherapy, radiation therapy. *Author: Dr V. R. Petretta (August 2003)*.

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