■原发性心脏内膜肉瘤极为罕见,预后不佳。他们的管理代表了一个复杂的临床挑战,因为完全手术切除是唯一可靠的治愈可能性,但仅在50%的患者中可能。在不可切除的疾病中,蒽环类药物是最有效的治疗方法,但帕唑帕尼可用于不适合接受蒽环类药物治疗的患者。
■一名38岁男子因原发性心脏内膜肉瘤而出现急性右心衰竭症状。心脏手术后明确诊断。多模式心脏成像显示二尖瓣和肺静脉侵犯的疾病早期复发,患者被认为无法手术。由于慢性肾脏疾病和以前的心力衰竭症状,他开始接受一线帕唑帕尼姑息治疗。化疗11个月后,有良好的临床耐受性,没有疾病进展的证据,发生在13个月后。
■此案例突出了多模态成像方法对心脏肿块的价值。最重要的是,它报道了一名年轻的原发性心脏内膜肉瘤患者的成功治疗,该患者开始接受姑息帕唑帕尼治疗,无进展生存期明显高于文献报道。当蒽环类化疗有禁忌症时,这一发现可能支持帕唑帕尼作为一线治疗的良好选择。
UNASSIGNED: Primary intimal sarcomas of the heart are extremely rare and have a dismal prognosis. Their management represents a complex clinical challenge since complete surgical resection is the only reliable possibility of cure but is only possible in 50% of patients. In non-resectable disease, anthracycline-based therapy is the most effective treatment, but pazopanib may be used in patients unfit to receive anthracyclines.
UNASSIGNED: A 38-year-old man presented with acute right heart failure symptoms due to a primary intimal sarcoma of the heart. A definite diagnosis was made after cardiac surgery. Multi-modality cardiac imaging showed early recurrence of disease with mitral valve and pulmonary veins\' invasion, and the patient was deemed inoperable. Due to chronic kidney disease and previous heart failure symptoms, he was started on first-line pazopanib palliative treatment. After 11 months of chemotherapy, there was good clinical tolerance and no evidence of disease progression, which occurred after 13 months.
UNASSIGNED: This case highlights the value of a multi-modality imaging approach for cardiac masses. Most importantly, it reports the successful treatment of a young patient with a primary intimal sarcoma of the heart who was started on palliative pazopanib, with a significantly higher progression-free survival than is reported in the literature. This finding may support pazopanib as a good alternative as first-line treatment when there is contraindication for anthracycline-based chemotherapy.