ABSTRACT
BACKGROUND: Undifferentiated pleomorphic sarcoma is a very rare and aggressive type of primary cardiac tumors. Most cardiac sarcomas result in rapid growth and quick death. According to different sources the median survival is typically 6 to 12 months. We are presenting a case of primary cardiac sarcoma with 26 months disease free survival following cytoreductive surgery and chemotherapy. CASE PRESENTATION: A 48-year-old woman with progressing symptoms of dyspnea and palpitations for over 2 months was referred to a cardiologist. With the help of echocardiography and cardiovascular magnetic resonance cardiac sarcoma was suspected. Open biopsy and cytoreductive surgery were performed, complete resection of the tumor was not possible. Histology revealed undifferentiated pleomorphic sarcoma. Seven cycles of chemotherapy with Doxorubicine and Ifosfamide were completed. Cardiovascular magnetic resonance revealed a complete response - only signs of fibrosis without any signs of tumor were visible. Follow ups with echocardiography, cardiovascular magnetic resonance and chest, abdomen and pelvic computed tomography is performed every 3 months. Twenty-six months from initial diagnosis the patient is still free of recurrence of tumor with no compromises of the quality of life. CONCLUSION: Standard chemotherapy together with cytoreductive surgery can have a complete response effect in undifferentiated pleomorphic sarcoma with unusual long-term survival.
Subject(s)
Heart Neoplasms/therapy , Histiocytoma, Malignant Fibrous/therapy , Biopsy , Chemotherapy, Adjuvant , Combined Modality Therapy , Cytoreduction Surgical Procedures , Doxorubicin/administration & dosage , Female , Follow-Up Studies , Heart Neoplasms/diagnosis , Heart Neoplasms/pathology , Histiocytoma, Malignant Fibrous/diagnosis , Histiocytoma, Malignant Fibrous/pathology , Humans , Ifosfamide/administration & dosage , Middle Aged , Quality of Life , Treatment OutcomeABSTRACT
BACKGROUND AND OBJECTIVE: Left atrium (LA) is an important biomarker of adverse cardiovascular outcomes and cerebrovascular events. This study aimed to evaluate LA myocardial deformation using cardiac magnetic resonance feature tracking (CMR-FT) in patients with acute ST-segment elevation myocardial infarction (STEMI) and secondary mitral regurgitation (MR). Additionally, to assess interobserver and intraobserver variability of the technique. MATERIALS AND METHODS: Twenty patients with STEMI underwent CMR with a 1.5Tesla MRI scanner. According to the presence of MR patients were divided into two groups: MR(+) and MR(-). Total LA strain (És), passive LA strain (Ée), and active LA strain (Éa) were obtained. Additionally, total, passive and active strain rates (SRs, SRe, and SRa) were calculated. To assess interobserver agreement data analysis was performed by second independent observer. RESULTS: LA volumetric and functional parameters were similar in both groups. All LA strain values were significantly higher in patients with MR: És (27.67±10.25 for MR(-) vs. 32.80±6.95 for MR(+); P=0.01), Ée (15.29±7.30 for MR(-) vs. 19.22±6.04 for MR(+); P=0.01) and Éa (12.38±4.23 for MR(-) vs. 14.44±5.19 for MR(+); P=0.03). Only SRe significantly increased in patients with MR (-0.57±0.24 for MR(-) vs. -0.70±0.20 for MR(+); P=0.01). All LA deformation parameters demonstrated high interobserver and intraobserver agreement. CONCLUSIONS: Conventional volumetric and functional LA parameters do not detect early changes in LA performance in patients with STEMI and secondary MR. In contrast, LA reservoir, passive and active strain are significantly higher in patients with MR. Only peak early negative strain rate substantially increases during secondary MR. LA deformation parameters derived from conventional cine images using CMR-FT technique are highly reproducible.