Primary cardiac myxofibrosarcoma: case report, literature review and pooled analysis

Abstract

Background: Primary 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 presentation: A 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.

Conclusions: Primary 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.

Keywords: Clinical features; Follow-up; Primary cardiac mycofibrosarcoma; Prognosis.

Fig. 1

Transthoracic echocardiogram revealed a mass measuring 50 × 35 mm in the left atrium (a). The mass was yellow-white with myxoid areas (b). Histological examination showed spindle-shaped cells with myxoid background (c)

Fig. 2

Flow diagram of identification of studies

Fig. 3

Overall survival comparison between patients with tumor size ≥ 40 mm versus < 40 mm. Months of clinical follow-up are plotted on the x-axes and percentage of surviving cases plotted on the y-axes

Fig. 4

Overall survival comparison between patients with high-grade tumors versus intermediate/low-grade tumors. Months of clinical follow-up are plotted on the x-axes and percentage of surviving cases plotted on the y-axes