Primary cardiac leiomyosarcoma: An analysis of clinical characteristics and outcome patterns

Background Primary cardiac leiomyosarcoma is an extremely rare entity. There is no accumulated knowledge about the clinical characteristics and therapeutic strategies for this tumor. This study aimed to systematically review the available literature to investigate the clinicoradiologic and clinicopa...

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Bibliographic Details
Published in:Asian Cardiovascular and Thoracic Annals 2015-06, Vol.23 (5), p.623-630
Main Authors: Wang, Ji-Gang, Cui, Li, Jiang, Tao, Li, Yu-Jun, Wei, Zhi-Min
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Aged, 80 and over
Chemoradiotherapy, Adjuvant
Child
Child, Preschool
Female
Heart Neoplasms - mortality
Heart Neoplasms - therapy
Humans
Infant
Leiomyosarcoma - diagnostic imaging
Leiomyosarcoma - mortality
Leiomyosarcoma - pathology
Leiomyosarcoma - therapy
Magnetic Resonance Imaging
Male
Middle Aged
Prognosis
Risk Factors
Tomography, X-Ray Computed
Treatment Outcome
Ultrasonography
Young Adult
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Summary:Background Primary cardiac leiomyosarcoma is an extremely rare entity. There is no accumulated knowledge about the clinical characteristics and therapeutic strategies for this tumor. This study aimed to systematically review the available literature to investigate the clinicoradiologic and clinicopathologic characteristics, treatment, and outcomes in patients with primary cardiac leiomyosarcoma. Methods We identified 79 instances derived from 72 unique reports and carefully analyzed all clinical presentations, imaging, pathology, treatments, and outcomes. Results The mean age at onset was 48 years. A symptom of obstruction was the most frequent complaint at diagnosis. Routine imaging methods such as echocardiography, computed tomography, and magnetic resonance imaging had high sensitivity in diagnosis. The tumor has a poorer life expectancy than its counterparts in other sites. The 5-year overall survival and recurrence-free survival rates were 25.4% and 14.7%, respectively. Overall survival was affected by age, surgery, and adjunctive chemotherapy or radiotherapy. Conclusions Our findings suggest that primary cardiac leiomyosarcomas tend to be biologically more aggressive compared to their counterparts in other sites. However, complete resection and adjunctive chemotherapy or radiotherapy may help to increase the life expectancy.
ISSN:0218-4923
1816-5370
DOI:10.1177/0218492315574197