Unusual primary tumors of the heart

Our plain-English summary of Unusual primary tumors of the heart, by T.J. Vander Salm (Seminars in Thoracic and Cardiovascular Surgery, 2000).

The study

• Author: T.J. Vander Salm
• Journal: Seminars in Thoracic and Cardiovascular Surgery
• Published: 2000;12(2):89-100
• Type of article: Review
• Cited by: 174 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Primary tumors of the heart, with the exception of atrial myxomas, occur rarely; tumors metastatic to or directly invasive of the heart are far more common. About 75% of primary tumors are benign, and 75% of these are atrial myxomas. The benign tumors include rhabdomyomas, fibromas, papillary fibroelastomas, hemangiomas, pericardial cysts, lipomas, hamartomas, teratomas, mesotheliomas, and paragangliomas or pheochromocytomas. The last 3 may also be malignant. The malignant tumors consist of various sarcomas: myxosarcoma, liposarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, osteosarcoma, synovial sarcoma, rhabdomyosarcoma, undifferentiated sarcoma, reticulum cell sarcoma, neurofibrosarcoma, and malignant fibrous histiocytoma. Cardiac tumors produce a large variety of symptoms through any of 4 mechanisms. Their mass can obstruct intracardiac blood flow or interfere with valve function. Local invasion can lead to arrhythmias or pericardial effusions with tamponade. Bits of tumor can embolize, causing systemic deficits when the tumors are on the left side of the heart. Finally, the tumors may cause systemic or constitutional symptoms. Some tumors, of course, produce no symptoms and become evident as incidental findings. The most useful diagnostic tool is the echocardiogram, which in almost all cases precisely locates the tumor and defines its extent. The echocardiographic appearance may also allow quite accurate prediction of the tumor type and whether it is malignant or benign. Magnetic resonance imaging serves as the next most important test where the density of T1 and T2 images may allow tumor cell type identification. With few exceptions, these tumors require operative excision. Most benign tumors can be resected completely; a few, because of their large size, cannot be, and only tumor debulking may be possible. Heart transplantation should be considered for these patients. Many of the malignant tumors cannot be resected completely, either because of the extent of local spread and invasion or because of the frequent distant metastases. Transplantation may also be an option for those with extensive local disease. The long-term results for resected benign tumors are excellent; the long-term results for sarcomas are very poor, and there are few survivors. For patients with unresectable sarcomas, radiation and chemotherapy may be used, but without great expectation of successful results.

In plain English

This article was a narrative review by a cardiac surgeon at the University of Massachusetts Medical School. It covered the primary tumors of the heart other than atrial myxoma, the most common type. It described how each tumor type presents, how it is diagnosed and how it is treated, drawing on published reports. Apart from one observation of a heart angiosarcoma at the author's own facility, it reported no new patient data, and it did not discuss asbestos. The author stated that about 75% to 80% of primary heart tumors are benign. Mesothelioma was described as arising mostly from the pericardium, the sac around the heart, where it is usually malignant. Mesothelioma of the atrioventricular (AV) node of the heart often behaves in a benign fashion. Citing one earlier series, the author reported that only 1 of 27 patients with pericardial mesothelioma stayed alive without evidence of disease, and 24 of the 27 died of the tumor. For heart sarcomas, cited median survival was 24 months with complete removal and 10 months with incomplete removal. The author concluded that long-term results for sarcomas are very poor.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Narrative review of published reports on unusual primary heart tumors (all except atrial myxoma), organized by tumor type. It includes one brief observation from the author's facility but no original study data.
• Who was studied: No study population. The review covered the benign primary heart tumors other than atrial myxoma and the primary malignant heart tumors, as described in the published literature.
• Main results:
  • The review stated that about 75% to 80% of all primary heart tumors are benign and the rest malignant, based on cited sources.
  • Mesothelioma of the atrioventricular (AV) node was described as often found at autopsy and behaving in a benign fashion, although it can cause heart block.
  • Almost all pericardial mesotheliomas were described as malignant, while those of the AV node often are not.
  • For pericardial mesothelioma, the review stated that an echocardiogram shows a thickened pericardium in about 20% of cases and a pericardial effusion (fluid around the heart) in about 90% of cases, citing an earlier report.
  • The review stated that pericardial mesothelioma should be completely removed if possible, but usually it cannot be.
  • In 1 earlier series cited by the author, 1 of 27 patients with pericardial mesothelioma stayed alive without evidence of disease, and 24 of the 27 died because of the tumor. The author added that most patients survive only a few months.
  • For primary heart sarcomas, cited median survival was 24 months with complete surgical removal and 10 months with incomplete removal.
  • The author described one observation at the author's own facility: a heart angiosarcoma that progressed from involving only the right side of the heart to extensive involvement of the left atrium, over a 2-week period according to the same sentence.
• The authors' conclusion: The author concluded that long-term results after removal of benign tumors are excellent, while long-term results for sarcomas are very poor with few survivors.
• Limitations the authors state:
  • The author noted that reported tumor frequencies depend on the population seen and vary between adult, pediatric and autopsy series, and with the chance variation of small series.

Topics

Medical terms in this study, explained in our Lexicon: Pericardial, Mesothelioma, Sarcoma, Synovial Sarcoma, Pericardial Effusion, Echocardiogram, Unresectable, Chemotherapy.

Related studies

• Primary tumors and cysts of the heart and pericardium — H.A. McAllister (1979)
• Surgery for primary intrapericardial tumors in adults — S. Taguchi, R. Yozu (2013)
• Surgical pathology of primary cardiac and pericardial tumors — C. Basso, M. Valente, et al. (1997)
• Primary cardiac tumors: experience at the University of Minnesota — J.E. Molina, J.E. Edwards, et al. (1990)
• Cardiac tumors that cause arrhythmias — K.F. Kusano, T. Ohe (2002)
• Primary epithelial tumour in the right atrium of the heart and inferior vena cava in NZR/gd inbred rats; pathology of 18 cases — C.M. Goodall, G.S. Christie, et al. (1975)

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