■血管周围上皮样细胞肿瘤(PEComas)是罕见的软组织肿瘤,通常发生在子宫内,皮肤,和肝脏,不太常见于腹膜后,结肠,和纵隔。
■一名36岁男性患者,有纵隔PEComa病史,原发性高血压,和目前未接受抗凝治疗的心耳结扎后房颤状态,有1周的发烧史,发冷,生产性咳嗽,胸痛,劳累时呼吸困难,食欲不振,一般的弱点。生命体征,体检,实验室数据,心电图,胸部X光片基本不明显。一种利用经胸超声心动图的多模态成像方法,经食管超声心动图(TEE),心脏磁共振成像(cMRI),和胸部的计算机断层扫描血管造影,腹部,骨盆显示局部40mm×53mm球形双叶血管化后纵隔肿块,起源于左右心房的顶部,并向上延伸到主肺动脉,向下延伸到下腔静脉。根据肿块的大小和与重要结构的接近程度以及肿瘤复发,该病例是在肿瘤板房中出现的,结果是手术切除肿块,然后让病人随访肿瘤和放射肿瘤,以进行可能的化疗和放疗,分别。
■血管周围上皮样细胞肿瘤很少见,纵隔PEComas更罕见,保证涉及TEE和cMRI的多模态成像方法以及涉及麻醉师的多学科方法,心脏病学家,心胸外科医生,医学肿瘤学家,病理学家,放射科医生,和放射肿瘤学家。
UNASSIGNED: Perivascular epithelioid cell tumours (PEComas) are rare soft tissue neoplasms that commonly occur in the uterus, skin, and liver and less commonly in the retroperitoneum, colon, and mediastinum.
UNASSIGNED: A 36-year-old male patient with a history of mediastinal PEComa status post resection, essential hypertension, and atrial fibrillation status post appendage ligation currently not on anticoagulation presented with a 1-week history of fevers, chills, productive cough, chest pain, dyspnoea on exertion, loss of appetite, and general weakness. Vital signs, physical exam, laboratory data, electrocardiogram, and chest radiograph were grossly unremarkable. A multimodality imaging approach utilizing transthoracic echocardiogram, transoesophageal echocardiogram (TEE), cardiac magnetic resonance imaging (cMRI), and computed tomography angiography of the chest, abdomen, and pelvis revealed a local 40 mm × 53 mm globular bilobed vascularized scar-free posterior mediastinal mass arising from the roof of the left and right atria and extending superiorly to the main pulmonary artery and inferiorly to the inferior vena cava. Based on the mass\' size and proximity to vital structures and tumour recurrence, the case was presented during tumour board rounds, and the outcome was to surgically resect the mass and then have the patient follow up with medical oncology and radiation oncology for possible chemotherapy and radiation, respectively.
UNASSIGNED: Perivascular epithelioid cell tumours are rare, and mediastinal PEComas are even rarer, warranting a multimodality imaging approach involving TEE and cMRI and a multidisciplinary approach involving anaesthesiologists, cardiologists, cardiothoracic surgeons, medical oncologists, pathologists, radiologists, and radiation oncologists.