Goldstein D J, Oz M C, Rose E A, Fisher P, Michler R E
Department of Cardiothoracic Surgery, College of Physicians & Surgeons and Columbia Presbyterian Medical Center, New York, N.Y., USA.
J Heart Lung Transplant. 1995 Mar-Apr;14(2):382-6.
Primary cardiac tumors are rare clinical entities. Benign tumors are often amenable to surgical excision, whereas malignant tumors are seldom resectable. Five patients have been reported to undergo orthotopic heart transplantation for inoperable primary cardiac tumors. We review the clinical course of these five patients and present our institutional experience with three patients who underwent orthotopic heart transplantation as primary therapy for unresectable cardiac tumors.
Retrospective chart review and telephone interview, when possible, were used to gather historical and clinical course data.
Of the eight patients who underwent orthotopic heart transplantation for primary cardiac neoplasms, four had malignant tumors (three sarcomas, one lymphoma) and four had locally invasive neoplasms (three fibromas, one pheochromocytoma). For those patients in whom cardiectomy resulted in surgical margins free of tumor (six of eight), orthotopic heart transplantation provided long-term survival (range 14 to 78 months) without tumor recurrence despite therapeutic immunosuppression. The only death in this group, at 6.2 years after operation, was unrelated to tumor recurrence. The patients with tumor identified at the surgical margins died of metastatic disease at 14 and 15 months after the operation in spite of adjuvant chemotherapy.
An awareness by clinicians of the presenting clinical picture of these tumors is warranted in view of the potential for cure by resection or transplantation. Patients with benign primary cardiac tumors appear to benefit from the complete resection afforded by cardiectomy and transplantation. The role of transplantation for patients with malignant tumors remains unclear. Additional studies and continued follow-up with serial echocardiography is necessary to further ascertain the role of heart transplantation in the management of patients with primary cardiac tumors.
原发性心脏肿瘤是临床罕见疾病。良性肿瘤通常可通过手术切除，而恶性肿瘤很少能切除。据报道，有5例患者因无法手术切除的原发性心脏肿瘤接受了原位心脏移植。我们回顾了这5例患者的临床病程，并介绍了我们机构对3例因无法切除的心脏肿瘤而接受原位心脏移植作为主要治疗方法的患者的经验。
尽可能通过回顾性病历审查和电话访谈收集病史和临床病程数据。
在8例因原发性心脏肿瘤接受原位心脏移植的患者中，4例患有恶性肿瘤（3例肉瘤，1例淋巴瘤），4例患有局部浸润性肿瘤（3例纤维瘤，1例嗜铬细胞瘤）。对于那些心脏切除术后手术切缘无肿瘤的患者（8例中的6例），尽管进行了免疫抑制治疗，原位心脏移植仍提供了长期生存（14至78个月）且无肿瘤复发。该组中唯一的死亡发生在术后6.2年，与肿瘤复发无关。手术切缘发现有肿瘤的患者尽管接受了辅助化疗，但仍在术后14个月和15个月死于转移性疾病。
鉴于手术切除或移植有可能治愈这些肿瘤，临床医生有必要了解这些肿瘤的临床表现。原发性心脏良性肿瘤患者似乎从心脏切除和移植所提供的完全切除中受益。移植对恶性肿瘤患者的作用仍不清楚。需要进一步的研究和通过连续超声心动图进行持续随访，以进一步确定心脏移植在原发性心脏肿瘤患者管理中的作用。
