Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Hospital, Baltimore, MD, USA.
Bone and Soft Tissue Pathology, The Johns Hopkins Hospital, 1800 Orleans Street, Zayed Tower Suite 7107, Baltimore, MD, 21287, USA.
J Cardiothorac Surg. 2024 Mar 28;19(1):161. doi: 10.1186/s13019-024-02555-8.
Primary cardiac angiosarcomas are very rare and present aggressively with high rates of metastasis. Given the poor prognosis, particularly once disease has spread, early diagnosis and multidisciplinary treatment is essential.
We present the case of a 46-year-old male who presented with chest pain, intermittent fevers, and dyspnea. Workup with computed tomography scan and transesophageal echocardiography demonstrated a right atrial pseudoaneurysm. Given the concern for rupture, the patient was taken to the operating room, where resection of the pseudoaneurysm and repair using a bovine pericardial patch was performed. Histopathology report initially demonstrated perivascular lymphocyte infiltrate. Six weeks later, the patient represented with chest pain and new word finding difficulty. Workup revealed multiple solid lung, pericardial, brain, and bone nodules. Eventual biopsy of a cardiophrenic nodule demonstrated angiosarcoma, and rereview of the original pathology slides confirmed the diagnosis of primary cardiac angiosarcoma.
Primary cardiac angiosarcomas are often misdiagnosed given the rarity of these tumors, but early diagnosis and initiation of treatment is essential. The unique presentation of our case demonstrates that clinical suspicion for cardiac angiosarcoma should be maintained for spontaneous pseudoaneurysm originating from the right atrium.
原发性心脏血管肉瘤非常罕见,侵袭性强,转移率高。鉴于预后不良,特别是一旦疾病扩散,早期诊断和多学科治疗至关重要。
我们报告了一例 46 岁男性患者,其表现为胸痛、间歇性发热和呼吸困难。计算机断层扫描和经食管超声心动图检查显示右心房假性动脉瘤。由于担心破裂,患者被送往手术室,在那里进行了假性动脉瘤切除和使用牛心包补片修复。组织病理学报告最初显示血管周围淋巴细胞浸润。六周后,患者出现胸痛和新的单词发现困难。检查显示多个肺部、心包、脑部和骨骼结节。最终对心膈角结节进行活检显示为血管肉瘤,重新审查原始病理切片证实了原发性心脏血管肉瘤的诊断。
由于这些肿瘤罕见,原发性心脏血管肉瘤常常被误诊,但早期诊断和开始治疗至关重要。我们的病例的独特表现表明,对于起源于右心房的自发性假性动脉瘤,应保持对心脏血管肉瘤的临床怀疑。