Cardiac Metastasis of Sacral Chordoma Pier Luigi Stef ano, MD, Giuseppe Barletta, MD, FESC, Valentina Andrei, MD, Alfredo Giuseppe Cerillo, MD, Gabriella Nesi, MD, Giuseppe Pilato, MD, Silvia Pradella, MD, PhD, Raffaella Santi, MD, PhD, and Maria Riccarda Del Bene, MD, PhD Department of Cardiac Surgery, Department of Diagnostic Cardiology, Pathology Section, Department of Health Sciences, Department of Radiodiagnostics, and Pathology Unit, Careggi University Hospital, Florence, Italy Chordoma is a rare tumor, usually diagnosed when the disease is advanced. Despite its slow growth, it is locally aggressive and has a poor long-term prognosis. Surgery is the mainstay treatment. Although cardiac metastases are very rare, the heart is frequently involved in systemic neoplastic diseases. This report describes a typical case of metastatic chordoma: the age at first diagnosis, the site of the primary tumor, and the slow growth of the cardiac metastasis were all typical features. Surgical excision of the mass from the right ventricular outflow tract is described together with echocardiographic, radiologic, and histopathologic characteristics of the metastatic chordoma. (Ann Thorac Surg 2021;111:e319-21) Ó 2021 by The Society of Thoracic Surgeons C hordomas are rare primary bone tumors of the axial skeleton. The incidence is approximately 0.08 tumors per 100,000, and men in their 50s to 60s are mainly affected. 1 Although benign neoplasms, chordomas are locally aggressive, with high rates of recurrences and a clinical course similar to that of a malignant cancer with a poor long-term prognosis. Metastases occur in 3% to 48% of cases: lungs, lymph nodes, skin, bones, and liver are the most common sites of metastasis. Six cases of cardiac metastases from chordoma have been reported. 2,3 We describe a case of asymptomatic right ventricular metastasis of a sacral chordoma that manifested 10 years after the first diagnosis. A 69-year-old man who had received a diagnosis of sacral chordoma and who had been treated surgically 10 years earlier was referred to our outpatient echocardiographic clinic to evaluate the hemodynamic impact of a solid mass that had been documented in the right ventricular outflow tract (RVOT) by chest computed tomography for the past 4 years (Figure 1A). The patient was asymp- tomatic and in good general condition. He had been receiving imatinib for 6 years because of multiple liver, bilateral lung, lymph node, and muscle metastases after focal radiation therapy (dose, 25 Gy). Physical examina- tion and the electrocardiogram were normal. Echocardi- ography was unremarkable except for the nodular and well-defined solid mass occupying almost the whole RVOT close to the pulmonic valve (Supplemental Figure 1A, 1B). The mass was isoechogenic and finely structured, with scant and fine vascularity seen on low-power contrast-enhanced 2-dimensional imaging (not shown). Color flow Doppler imaging showed some acceleration of RVOT flow. For further evaluation, a cardiac magnetic resonance (CMR) study was performed (Figures 1B to 1D). This study further characterized the mass and demonstrated slow and prolonged contrast enhancement after gadolinium injection. As a multidisciplinary decision, surgical removal of the mass was planned because of the high risk of adverse events. The operation was performed through a median ster- notomy, with the patient on cardiopulmonary bypass with cardioplegic arrest (Figure 2). The patient was easily weaned from cardiopulmonary bypass, and apart from some paroxysms of atrial fibrillation requiring pharma- cologic cardioversion, his postoperative course was un- eventful. He was discharged to cardiac rehabilitation with oral anticoagulant therapy. On pathologic study, gross examination demonstrated the specimen, which consisted of a solid, gray-to-tan mass, with myxoid areas and a glistening cut surface. The diagnosis of chordoma was established by histologic examination (Figure 3). Comment Although primary tumors of the heart are extremely rare (0.0017% to 0.28% of all neoplasm in an autoptic series), the incidence of cardiac metastasis is not so uncommon, ranging from 2.3% to 18.3%. 4 Chordomas account for 1% to 4% of all malignant tu- mors of the bone. They originate from remnant noto- chordal mesenchymal cells situated in the axial skeleton, which is the characteristic localization, with relatively even distribution among cranial, spinal, and sacral sites. The age at diagnosis (mean, 58 years) seems to influence Accepted for publication Jul 22, 2020. Address correspondence to Dr Barletta, Department of Diagnostic Car- diology, Careggi University Hospital, Largo Brambilla 3, 50134, Florence, Italy; email: [email protected]. The Supplemental Figure can be viewed in the online version of this article [https://doi.org/10.1016/j.athoracsur. 2020.07.093] on http://www.annalsthoracicsurgery.org. Ó 2021 by The Society of Thoracic Surgeons 0003-4975/$36.00 Published by Elsevier Inc. https://doi.org/10.1016/j.athoracsur.2020.07.093
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