Department of Cardiology, Medical Academy, Lithuanian University of Health Sciences, Kaunas, Lithuania.
Department of Oncology and Hematology, Medical Academy, Lithuanian University of Health Sciences, Kaunas, Lithuania.
BMC Cancer. 2019 May 31;19(1):529. doi: 10.1186/s12885-019-5705-2.
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.
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.
Standard chemotherapy together with cytoreductive surgery can have a complete response effect in undifferentiated pleomorphic sarcoma with unusual long-term survival.
未分化多形性肉瘤是一种非常罕见且侵袭性很强的原发性心脏肿瘤。大多数心脏肉瘤会导致快速生长和快速死亡。根据不同的来源,中位生存期通常为 6 到 12 个月。我们报告了一例原发性心脏肉瘤患者,在接受减瘤手术和化疗后无疾病生存 26 个月。
一名 48 岁女性,因呼吸困难和心悸症状逐渐加重超过 2 个月而被转介给心脏病专家。在超声心动图和心血管磁共振的帮助下,怀疑患有心脏肉瘤。进行了开放性活检和减瘤手术,但无法完全切除肿瘤。组织学显示为未分化多形性肉瘤。完成了 7 个周期的多柔比星和异环磷酰胺化疗。心血管磁共振显示完全缓解——仅可见纤维化迹象,无肿瘤迹象。每 3 个月进行一次超声心动图、心血管磁共振以及胸部、腹部和盆腔计算机断层扫描随访。从最初诊断开始的 26 个月后,患者仍然没有肿瘤复发,生活质量没有受到影响。
标准化疗联合减瘤手术可使未分化多形性肉瘤获得完全缓解效果,并带来异常长的生存时间。