Akishima Shinji, Imai Akito, Enomoto Yoshiharu, Shigeta Osamu
Department of Cardiovascular Surgery, Ibaraki Prefectural Central Hospital, 6528 Koibuchi, Kasama-shi, Ibaraki, 309-1793, Japan.
Surg Case Rep. 2019 Jun 17;5(1):97. doi: 10.1186/s40792-019-0658-x.
Undifferentiated pleomorphic sarcoma (UPS) as a primary cardiac tumor is rare, with extremely poor prognosis owing to high recurrence and invasion. We encountered a patient who presented with a primary cardiac tumor incarcerating the mitral valve and who was in a shock state.
A 41-year-old man was transported emergently to our hospital owing to acute respiratory distress and hemoptysis. He was diagnosed with acute left heart failure caused by a large mass in the left atrium (LA) that obstructed cardiac blood flow, as revealed by imaging study findings, and he underwent an emergency open-heart surgery for tumor resection. He was pathologically diagnosed with UPS invading the muscle layer of the LA. However, after receiving combined therapy for local recurrence and distant metastasis, including proton beam radiotherapy and chemotherapy with molecularly targeted drugs, he could return to work for 2 more years after surgery.
In this study, we reported the case of a patient who was in a state of shock state owing to the presence of UPS in the LA. The patient underwent an emergency surgery and received combined therapy. He survived for 2 more years after an initial diagnosis, without active local recurrence and distant metastasis.
未分化多形性肉瘤(UPS)作为原发性心脏肿瘤较为罕见,因其高复发率和侵袭性,预后极差。我们遇到一名患有原发性心脏肿瘤并嵌顿二尖瓣且处于休克状态的患者。
一名41岁男性因急性呼吸窘迫和咯血被紧急送往我院。影像学检查结果显示,他被诊断为由左心房(LA)内一个巨大肿块阻塞心脏血流导致的急性左心衰竭,随后他接受了紧急心脏手术以切除肿瘤。病理诊断为UPS侵犯LA肌层。然而,在接受包括质子束放疗和分子靶向药物化疗在内的局部复发和远处转移联合治疗后,他术后还能重返工作岗位2年。
在本研究中,我们报告了一例因LA内存在UPS而处于休克状态的患者病例。该患者接受了紧急手术并接受了联合治疗。初次诊断后他存活了2年多,无局部复发和远处转移。