Surgical Implications in Malignant Cardiac Disease

doi:10.1016/S0003-4975(10)60494-8

The Annals of Thoracic Surgery

Volume 36, Issue 4, October 1983, Pages 484-491

https://doi.org/10.1016/S0003-4975(10)60494-8 Get rights and content

Abstract

Despite the rarity of primary malignant tumors of the heart (0.0017 to 0.03% of large postmortem series) and the infrequency of clinical signs and symptoms (0 to 50%) of the more common metastatic cardiac tumors, many cardiothoracic surgeons at some time will encounter a patient with one of these two conditions. A review of the medical literature yielded 28 cases of primary cardiac tumors, 10 of secondary tumors, and 12 of carcinoid heart disease treated surgically and followed sufficiently for retrospective evaluation. We summarized those cases and made the following conclusions. Primary malignant tumors of the heart are occasionally resectable, although cure is unlikely; survival may be enhanced by postoperative irradiation but probably not by postoperative chemotherapy. For patients with secondary malignant tumors of the heart, surgical intervention is rarely of benefit except for establishing a tissue diagnosis, effecting artificial cardiac pacing, decompressing symptomatic pericardial effusions, or reducing an obstructive tumor mass. Patients with carcinoid heart disease affecting the valves may derive benefit from valve replacement or repair. Thus, in selected patients with malignant cardiac disease, surgical intervention may be feasible and should always be among the therapeutic options considered.

References (74)

LA Longino et al.
Primary cardiac tumors in infancy
J Pediatr
(1953)
G de Saint-Florent et al.
A primary malignant tumor of the mitral valve with surgical removal
J Thorac Cardiovasc Surg
(1969)
JM Matloff et al.
Rhabdomyosarcoma of the left atrium: physiologic responses to surgical therapy
J Thorac Cardiovasc Surg
(1971)
JG Scannell et al.
Primary tumors of the heart: a surgical problem
J Thorac Surg
(1958)
E Dong et al.
Primary cardiac sarcoma
Am J Cardiol
(1962)
S Goldstein et al.
Right ventricular fibrosarcoma causing pulmonic stenosis
Am J Cardiol
(1966)
MM DiGilio et al.
Myxosarcoma of the pulmonary valve
Chest
(1972)
WRC Murphy et al.
Recurrent myxosarcoma of the left atrium
Chest
(1975)
G Sanoudos et al.
Primary cardiac sarcoma
J Thorac Cardiovasc Surg
(1972)
J Yashar et al.
Primary osteogenic sarcoma of the heart
Ann Thorac Surg
(1979)

EL Forker et al.

Primary sarcoma of the mitral valve

Am Heart J

(1963)

H Winer et al.

Primary cardiac chondromyxosarcoma—clinical and echocardiographic manifestations: a case report

J Thorac Cardiovasc Surg

(1977)

C Gabelman et al.

Surgical treatment of recurrent primary malignant tumor of the left atrium

J Thorac Cardiovasc Surg

(1979)

JR Torstveit et al.

Primary plasmacytoma of the atrium: report of a case with successful surgical management

J Thorac Cardiovasc Surg

(1977)

ET Gelfand et al.

Melanotic malignant schwannoma of the right atrium

J Thorac Cardiovasc Surg

(1977)

G Kubac et al.

Malignant granular cell myoblastoma with metastatic cardiac involvement: case report and echocardiogram

Am Heart J

(1980)

JR Hankins et al.

Pericardial window for malignant pericardial effusions

Ann Thorac Surg

(1980)

CP Bailey et al.

Extending operability in lung cancer involving the heart and great vessels

Ann Thorac Surg

(1971)

GL Hammond et al.

Chondrosarcoma simulating malignant atrial myxoma

J Thorac Cardiovasc Surg

(1976)

GJ Magovern et al.

The surgical resection and chemotherapy of metastatic osteogenic sarcoma of the right ventricle

Ann Thorac Surg

(1980)

C Mavroudis et al.

Diagnosis and operative treatment of intracavitary liposarcoma of the right ventricle

J Thorac Cardiovasc Surg

(1981)

JR Utley et al.

Acute obstruction of tricuspid valve by Wilms' tumor

J Thorac Cardiovasc Surg

(1973)

ED Vaughan et al.

Nephroblastoma with right atrial extension: preoperative diagnosis and management

J Urol

(1977)

DA Ehrich et al.

Intracavitary cardiac extension of hepatoma

Ann Thorac Surg

(1975)

TW Boland et al.

Chondrosarcoma: a case report with left atrial involvement and systemic embolization

J Thorac Cardiovasc Surg

(1977)

MAH Gardner et al.

Pulmonary metastatic chondrosarcoma with intracardiac extension

Ann Thorac Surg

(1979)

FJ Schoen et al.

Porcine heterograft valve replacement in carcinoid heart disease

J Thorac Cardiovasc Surg

(1981)

PW Wright et al.

Carcinoid heart disease: report of a case treated by open heart surgery

Am J Cardiol

(1963)

C Carpena et al.

Carcinoid heart disease: surgery for tricuspid and pulmonary valve lesions

Am J Cardiol

(1973)

N Hendel et al.

Carcinoid heart disease: eight-year survival following tricuspid valve replacement and pulmonary valvotomy

Ann Thorac Surg

(1980)

R Straus et al.

Primary tumor of the heart

Arch Pathol

(1945)

HS Benjamin

Primary fibromyxoma of the heart

Arch Pathol

(1939)

NA Silverman

Primary cardiac tumors

Ann Surg

(1980)

RW Prichard

Tumors of the heart: review of the subject and report of one hundred and fifty cases

Arch Pathol

(1951)

WB Lockwood et al.

The changing prevalence of secondary cardiac neoplasms as related to cancer therapy

Cancer

(1980)

LH Smith

Secondary tumors of the heart

Rev Surg

(1976)

HA McAllister et al.

Tumors of the cardiovascular system

Cited by (76)

New curative approach using embolization followed by moderate-dose radiotherapy after surgical failure for large right heart metastasis
2022, Clinical and Translational Radiation Oncology
Citation Excerpt :
When the heart is the only site of metastasis, with limited tumor invasion, surgery is an option if resection is technically feasible in selected patients with good performance status and long life expectancy [8]. Postoperative survival after CM resection varies in the literature: from hours to 26 months in 10 case reports between 1971 and 1981 [4], and 2–34 months in 4 patients in 2012 [8]. Two-thirds of primary malignant cardiac tumors are sarcomas.
Cardiac metastasis is a rare fatal event. An intracavitary right tumor mainly in the ventricle is difficult to manage. Literature reports suggest that cardiac surgery in oligometastatic patients could offer median survival of 1 year. We investigated salvage treatment comprising transcoronary tumor embolization followed 15 days later by cardiac radiotherapy (40.5 Gy/15 fractions).
We report two cases of severe right cardiac metastasis with a history of abdominal cancer managed by this salvage treatment following residual cardiac mass after previous cardiac surgery.
Both symptomatic patients improved progressively and were locally controlled for at least 1 year without toxicity.
Postoperative chemotherapy and radiation improve survival following cardiac sarcoma resection
2021, Journal of Thoracic and Cardiovascular Surgery
Citation Excerpt :
In general, patients diagnosed with cardiac sarcoma in the United States tend to be more commonly male and white and can present in early or old age. This study corroborates a combination of several smaller reports that the median survival for patients with this disease is less than a year and that surgical resection does indeed provide a survival advantage and is warranted as the current standard of care for appropriate candidates based on a pragmatic and selective approach given high rates of mortality.6-8,10,19-21 The “appropriate candidate” can be difficult to define due to the rarity of the disease and the infrequency of treatment at any one institution or by any one surgeon.
Cardiac sarcoma represents a rare and aggressive form of cancer with a paucity of data to produce outcome-driven evidence-based guidelines. Current surgical management consists of resection with postoperative therapy (chemotherapy, radiation, or both) offered on a selective, individualized basis. This study was designed to determine whether postoperative therapy was associated with improved overall survival after resection.
The National Cancer Database was used to identify patients with cardiac sarcoma between 2004 and 2015. Patient characteristics were stratified by treatment (surgical, nonsurgical, and none), and treatment was analyzed by stage. Overall survival, assessed with Kaplan–Meier methodology, was compared between patients who received postoperative therapy and those who did not following resection. Multivariable survival modeling using a Weibull model identified risk factors associated with survival while controlling for confounders.
The study included 617 patients diagnosed with cardiac sarcoma. Only 24% (149/617) of patients were diagnosed with early-stage disease. Angiosarcoma represented 48% (298/617) of cases and was the most commonly identified histologic subtype. 60% (372/617) underwent surgical resection and 58% (216/372) of those patients were treated with postoperative therapy. Following surgery, median survival was more than doubled for patients treated with postoperative therapy (19 months vs 8 months, P = .026). However, 5-year overall survival was similar between the groups. Multivariable analysis confirmed an improvement in survival with postoperative therapy (hazard ratio, 0.68; 95% confidence interval, 0.51-0.91, P = .009).
Postoperative therapy is associated with better median survival following resection of cardiac sarcoma. However, at 5 years, the difference in overall survival is not statistically significant.
Diseases of the Pleura and Mediastinum
2019, Abeloff’s Clinical Oncology
Malignant pleural mesothelioma is a rare disease. Ninety percent of cases are attributed to asbestos exposure. Surgical options include extrapleural pneumonectomy or pleurectomy and decortication. The role of chemotherapy and radiation in resectable disease is controversial. For unresectable or metastatic disease, the standard treatment is the combination of a platinum drug and an antifolate drug.
Thymoma is the most common mediastinal tumor. Fifty percent of patients are symptomatic at diagnosis, and thymomas can be associated with paraneoplastic syndromes such as myasthenia gravis. Surgical resection is the standard of care. Postoperative radiation therapy should be considered for patients with stage IIB disease, close surgical margins, World Health Organization (WHO) grade B type, and tumor adherent to the pericardium. Chemotherapy may be helpful for patients with unresectable or metastatic disease. Sunitinib and PD-1/PD-L1 agents are being studied for thymoma and thymic carcinoma. Thymic carcinoids exhibit a spectrum of pathologic and clinical aggressiveness. When possible, the primary treatment is surgical resection. Radiation therapy can palliate symptomatic local and distant metastases. More aggressive atypical carcinoid tumors can be sensitive to the same chemotherapy used for other neuroendocrine cancers. When they bear octreotide receptors, somatostatin analogues or radiolabeled somatostatin analogues may be considered. Mammalian target of rapamycin (mTOR) inhibitors are promising.
The most common causes of malignant pleural effusions are lung cancer, breast cancer, lymphoma, pleural mesothelioma, and cancer of unknown primary. Common symptoms include shortness of breath and cough. Management strategies include thoracentesis, indwelling pleural catheters, and pleurodesis.
Cardiac Tumors
2016, Clinical Cardio-oncology
Prognostic analysis for survival after resections of localized primary cardiac sarcomas: A single-institution experience
2014, Annals of Thoracic Surgery
Citation Excerpt :
The surgical strategy has evolved over the past four decades. In the 1970s, gross excisions were attempted in 27% to 50% of cases, and the surgical treatment was considered palliative with a median survival of only several months [10, 11, 15]. The early result of complete resection was not encouraging, with a median survival of 6 months reported by Burke and colleagues [16].
Primary cardiac sarcomas are rare diseases with a poor prognosis. This study aims to provide a prognostic analysis after different levels of resections of cardiac sarcomas.
Twenty-nine patients undergoing resections of primary cardiac sarcomas at the Zhongshan Hospital from September 1995 to July 2012 were retrospectively reviewed.
There were 15 women and 14 men. The mean age was 41.0 years. The most common histologic type was angiosarcoma (28%). The median survival for the entire cohort was 17 months (range, 5 to 216 months). Patients with microscopically negative margin (R0) resections had a better median survival than those with microscopically positive margin (R1) resections (58 months versus 11 months; p < 0.001). The median survival after an R1 resection was not different from that after a partial resection (12 months; p = 0.81). The median local recurrence-free survival after an R0 resection was longer than that after an R1 resection (36 months versus 6 months; p < 0.001). Five patients who underwent R0 resections and repeated resections of local recurrences or metastases had the longest median survival of 72 months. None of the patients with R0 resections received adjuvant therapy. Multimodality treatment after R1 and partial resections slightly increased the survival.
For nonmetastatic and localized primary cardiac sarcoma, an R0 surgical resection of cardiac sarcomas should be performed. Aggressive surgical treatment or radiation therapy for local recurrence or metastasis prolongs the survival. Multimodality treatment is recommended after incomplete resections of cardiac sarcomas. The role of adjuvant chemotherapy after R0 resections is unclear.
Diseases of the Pleura and Mediastinum
2014, Abeloff's Clinical Oncology: Fifth Edition

View all citing articles on Scopus

View full text

Surgical Implications in Malignant Cardiac Disease

Abstract

J Pediatr

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Thorac Surg

Am J Cardiol

Am J Cardiol

Chest

Chest

J Thorac Cardiovasc Surg

Ann Thorac Surg

Am Heart J

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

Am Heart J

Ann Thorac Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Urol

Ann Thorac Surg

J Thorac Cardiovasc Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

Am J Cardiol

Am J Cardiol

Ann Thorac Surg

Primary tumor of the heart

Arch Pathol

Primary fibromyxoma of the heart

Arch Pathol

Primary cardiac tumors

Ann Surg

Tumors of the heart: review of the subject and report of one hundred and fifty cases

Arch Pathol

The changing prevalence of secondary cardiac neoplasms as related to cancer therapy

Cancer

Secondary tumors of the heart

Rev Surg

Tumors of the cardiovascular system