Commentary: The Unrelenting Challenge of Primary Cardiac Sarcoma

10584 Background: Primary cardiac sarcoma (PCS) is the most common primary cardiac malignancy, but is a rare primary site of sarcoma. We present 21 cases from a tertiary care center to better understand this uncommon malignancy. Methods: A cancer center-based registry and pathology database were searched to identify pts diagnosed with PCS from 1992-2013 at University of Michigan. Kaplan-meier method was used to estimate survival. Cox proportional hazard model was used to associate variables to occurrence of metastases (mets) or death. Results: Atotal of 21 pts (F12, 9M) with PCS were identified, median age 36 (range 11-74). The most common presenting symptoms included dyspnea (16) and chest pain (6; 5 with associated pericardial effusion). Histologies included: angiosarcoma (9), leiomyosarcoma (4), undifferentiated pleomorphic (3), spindle cell (2), fibrosarcoma (1), rhabdomyosarcoma (1) and synovial (1). Sites of origin were R atrium (7), R ventricle (2), L atrium (10) and pericardium (2). Ten pts presented with mets; most common sites were lung (8), liver (2), brain (2), pancreas (2) and bone (2). Surgery was attempted in 12 pts, achieving 1 R0 resection. Pts received a median of 1 (0-7) systemic therapies. Median overall (OS) was 12.6 mos (range 3-79) from diagnosis. Pts without prior surgery were more likely to have mets or death (p=0.038). Brain mets were common, occurring in 7 of 21 pts after a median of 7 mos (range 1-75) from diagnosis. Median OS after diagnosis of brain mets was 8 mos. Of the 7 pts who developed brain metastasis, 5 had PCS originating in the left heart. Of the 2 pts with PCS in the right heart, one was evaluated for and had a right to left shunt. The likelihood of developing brain mets did not correlate with age, chemotherapy, or surgery. Conclusions: PCS portends an extremely poor prognosis, marked by inability to achieve complete resection and a high incidence of disseminated disease at diagnosis. Metastatic disease to the brain was much more common in PCS (33%) as compared to STS of any origin (approximately 1-8%), particularly in pts with PCS originating in the left heart. Clinicians should have a low threshold for brain imaging evaluation of PCS pts.

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Respiratory Failure in an Adolescent with Primary Cardiac Sarcoma

Case Reports in Cardiology ◽

10.1155/2015/592385 ◽

2015 ◽

Vol 2015 ◽

pp. 1-4

Author(s):

Daniel Angeli ◽

Stephen J. Angeli

Keyword(s):

Heart Failure ◽

Respiratory Failure ◽

Lung Disease ◽

Adult Population ◽

Correct Diagnosis ◽

Adolescent And Young Adult ◽

Cardiac Tumors ◽

Intravenous Contrast ◽

Tertiary Center ◽

Primary Cardiac Sarcoma

We report a case of progressive respiratory failure secondary to primary cardiac sarcoma masquerading as primary lung disease. An 18-year-old female presented to our hospital emergency department with progressive cough, dyspnea, and hemoptysis. She was treated for primary lung infection without improvement and had respiratory failure with endotracheal intubation by the third hospital day. An “intermediate” plasma brain natriuretic protein (BNP) of 216 pg/mL did not raise concerns about a heart failure diagnosis and may have delayed the correct diagnosis. Computed tomography of the chest with intravenous contrast was performed on the fifth hospital day and revealed a cardiac mass. A transthoracic echocardiogram confirmed a large left atrial mass that was obstructing mitral inflow. She was transferred to a tertiary center for emergency cardiac surgery. Primary cardiac tumors are a rare and treatable cause of heart failure in adolescent and young adult patients. Presentation can be confused with primary lung disease and must be suspected early. Plasma BNP cutoff levels used in the adult population should not be extrapolated to adolescents, as levels, both normal and abnormal, are significantly lower in this group of patients.

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