Abstract

BackgroundPrimary cardiac myxofibrosarcoma is a very rare cardiac malignancy. The majority of publications are limited to case reports. No pooled analyses of primary cardiac myxofibrosarcoma cases are available. Little clinical features and outcome patterns are acknowledged. The purpose of this study is to identify the clinical characteristics and prognostic factors of primary cardiac myxofibrosarcoma.Case presentationA case report of primary cardiac myxofibrosarcoma was presented, and a review of English language literatures of primary cardiac myxofibrosarcomas were performed electronically. Demographics, clinicopathologic data, therapy and follow-up were summarized. The median survival time and the mean survival time were calculated by Kaplan-Meier method. Survival distribution and overall survival were figured by log-rank test and cox proportional hazards models. We present a case, and retrospectively analyzed additional 30 patients derived from 24 isolated articles. The cohort consisted of 18 male and 13 female patients. The age was 41.87 ± 17.89 years. Some common features were found in clinical presentations, pathologic features, treatments and outcome patterns of primary cardiac myxofibrosarcoma. There were special features in echocardiography, histological and immunohistochemical examinations, which should be considered in diagnosis of primary cardiac myxofibrosarcoma. The median survival time/mean survival time (MST) was 14/32.66 months. The median survival time/mean survival time (MST) was 14/32.66 months. Compared to the other groups, the following groups had shorter survival characteristics, including age ≥ 40 years (14/11.79 months), female (14/26.26 months), mass diameter ≥ 40 mm (14/14.64 months), high-grade (2/11.81 months), and no post-treatment (14/28.09 months). Statistical analyses revealed that primary cardiac myxofibrosarcomas were more likely to present with local recurrences and dismal metastases. Tumors ≥ 40 mm in size (P = 0.055, HR = 6.79) or with high-grade (P = 0.063, HR = 11.45) had significantly worse prognosis.ConclusionsPrimary cardiac myxofibrosarcomas were more likely to present with local recurrences and dismal metastases. Echocardiography, together with histological method should be considered in ordinary diagnosis. Tumors ≥ 40 mm in size or with high-grade had significantly worse prognosis, which should be early diagnosed and treated with rational surgery.

Highlights

  • Primary cardiac myxofibrosarcoma is a very rare cardiac malignancy

  • Primary cardiac myxofibrosarcomas were more likely to present with local recurrences and dismal metastases

  • We presented a case report of primary cardiac myxofibrosarcoma, and retrospectively analyzed additional 30 patients with primary cardiac myxofibrosarcomas derived from 24 isolated articles in an effort to establish definite clinical presentations, pathologic features, treatments and outcome patterns of primary cardiac myxofibrosarcoma and to develop a rationale for diagnosis and prognostication of this disease

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Summary

Introduction

Primary cardiac myxofibrosarcoma is a very rare cardiac malignancy. The majority of publications are limited to case reports. Some common features were found in clinical presentations, pathologic features, treatments and outcome patterns of primary cardiac myxofibrosarcoma. Primary cardiac myxofibrosarcoma is even less, which has been only presented in a series of isolated case reports [3,4,5]. As there are still few series describing primary cardiac myxofibrosarcoma, and the majority of publications are limited to case reports, little characterizations are acknowledged. We presented a case report of primary cardiac myxofibrosarcoma, and retrospectively analyzed additional 30 patients with primary cardiac myxofibrosarcomas derived from 24 isolated articles in an effort to establish definite clinical presentations, pathologic features, treatments and outcome patterns of primary cardiac myxofibrosarcoma and to develop a rationale for diagnosis and prognostication of this disease

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