Cardiac Angiosarcoma With Spine Destruction as the First Symptom

Cardiac angiosarcoma with spine destruction is extremely rare, with only 1 reported case.1Shapiro S. Scott J. Kaufman K. Metastatic cardiac angiosarcoma of the cervical spine. Case report.Spine. 1999; 24: 1156-1158Crossref PubMed Scopus (13) Google Scholar Our hospital admitted a patient with cardiac angiosarcoma with spine destruction. The patient presented to the orthopedics department with back pain for 1 month. Magnetic resonance imaging, echocardiography, and computed tomography of the chest revealed a mass in the right atrium, and a diagnosis of angiosarcoma was made. The patient was given radiotherapy of the 10th thoracic vertebra and total body chemotherapy. The pain in the patient’s back had alleviated in the follow-up visit after 4 months. In addition, the patient's heart tumors had shrunk considerably. Cardiac angiosarcoma with spine destruction is extremely rare, with only 1 reported case.1Shapiro S. Scott J. Kaufman K. Metastatic cardiac angiosarcoma of the cervical spine. Case report.Spine. 1999; 24: 1156-1158Crossref PubMed Scopus (13) Google Scholar Our hospital admitted a patient with cardiac angiosarcoma with spine destruction. The patient presented to the orthopedics department with back pain for 1 month. Magnetic resonance imaging, echocardiography, and computed tomography of the chest revealed a mass in the right atrium, and a diagnosis of angiosarcoma was made. The patient was given radiotherapy of the 10th thoracic vertebra and total body chemotherapy. The pain in the patient’s back had alleviated in the follow-up visit after 4 months. In addition, the patient's heart tumors had shrunk considerably. Secondary spinal tumors rarely originate from the heart. We present surgical management strategies for a patient with cardiac angiosarcoma with spine destruction as the first symptom. This case was diagnosed and treated by multiple departments in our hospital, and the treatment effect is relatively good. A 28-year-old man presented to the orthopedics department with back pain for 1 month. No abnormalities were found in retrospective past medical history and in the physical examination except for back pain. Computed tomography (CT) showed bone destruction in the 10th thoracic vertebra, sacrum, and right ilium (Figure 1), and secondary malignant tumor lesions were considered. Thoracic needle biopsy was performed after the patient's condition was evaluated, and pathologic examination indicated hypervascular benign lesions. Nodular hyperplasia of bland spindle cells could be seen in the bone tissues. The cytoplasm was stained red or transparent; mitotic figures were not observed, and the stroma was rich in capillaries. Morphologic appearance and immunophenotype were insufficient for diagnosis of malignant disease because of limited biopsy tissue. CT showed a low-density mass in the right atrium and multiple nodules in both lungs (Figure 2). Cardiac magnetic resonance imaging (MRI) and echocardiography confirmed the existence of cardiac tumor. After consultation with physicians from the chest surgery department, partial lobectomy under thoracoscopy and biopsy were performed after communicating with the patient, and pathologic examination showed angiosarcoma. In combination with the patient’s medical history, he was diagnosed with multiple metastases of cardiac angiosarcoma. The patient was given radiotherapy of the 10th thoracic vertebra (Dt = 20 Gy/5 fractions) and total body chemotherapy (doxorubicin and ifosfamide). The pain in the patient’s back had alleviated in the follow-up visit after 4 months. In addition, the patient's heart tumors had shrunk considerably (Figure 3).Figure 2(A) Cross-sectional image showing left atrial mass. The arrow refers to the location of the cardiac lesion. (B) Lung computed tomography showing multiple pulmonary nodules. The arrow refers to the location of the nodule in the metastatic lesion in the lung.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 3Image comparison before and after treatment. (A) Cardiac magnetic resonance image before treatment. The arrow refers to the patient's visit as a cardiac tumor on MRI. (B) Magnetic resonance image at the follow-up review after 4 months. The arrow refers to the patient's cardiac tumor on the MRI four months after treatment.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Histologic findings were as follows: Pathologic examination of the 10th thoracic vertebra puncture sample, spindle cells tested in immunohistochemistry: caldesmon (+), smooth muscle actin (+), P63 (focal weak +), calponin (−), desmin (−), cytokeratin (−), S100 (−), brachyury (−), epithelial membrane antigen (−), progesterone receptor (−), SSTR2 (−), glial fibrillary acidic protein (−), erythroblast transformation-specific related gene (−), CD34 (−), STAT6 (−), CD68 (KP1) (+), SOX10 (−), ALK (−), CD10 (−), retinoblastoma (+), neuron-specific enolase (−), α-inhibin (−), Ki67 (positive rate about 3%). Pathologic examination of the biopsy specimen of the left lower lobectomy: angiosarcoma (the maximum diameter of tumors was 0.6 cm); no tumor was found at the incisal edge of the anastomotic nail. Tumor cells tested in immunohistochemistry: erythroblast transformation-specific related gene (+), CD31 (+), CD34 (+), Ki67 (positive rate about 60%), FLI1 (+), cytokeratin (−), vimentin (+), epithelial membrane antigen (−), MDM2 (−). Metastasis of cardiac malignant tumor to bone is uncommon; however, misdiagnosis of these lesions can have devastating consequences, such as continued metastasis of the tumor or paralysis caused by compression of nerves by spinal tumors. We present the rare case of a cardiac angiosarcoma that started with back pain without any symptoms in the heart. As the most common primary cardiac tumor, cardiac angiosarcoma accounts for 20% of primary cardiac malignant tumors.2Endara S.A. Davalos G.A. Ponton P.M. et al.Cardiac failure due to left atrial angiosarcoma.Sarcoma J. 2018; 2: 5-8Google Scholar Cardiac angiosarcoma is found when the tumor has progressed and involved important cardiac structures or distal metastases have developed because its symptoms are hidden and the available noninvasive imaging examination methods have a lack of specificity.3Lin C.T. Ducis K. Tucker S. Tranmer B. Metastatic cardiac angiosarcoma to the lung, spine, and brain: a case report and review of the literature.World Neurosurg. 2017; 107: 1049.e9-1049.e12Crossref PubMed Scopus (6) Google Scholar This case was found because of the back pain caused by the metastases of cardiac angiosarcoma to the 10th thoracic vertebra. The metastases of cardiac angiosarcoma mainly include lung metastasis, liver metastasis, and brain metastasis. Common imaging examinations include echocardiography, enhanced CT, and MRI. Among them, MRI has become the "gold standard" for imaging evaluation of cardiac angiosarcoma because of its excellent contrast resolution, multiplanar imaging capability, and wide field of view. Whereas echocardiography is effective for initial identification of anatomic location, MRI is a more advantageous modality for delineating tumor expanse, spread, and blood supply preoperatively.4Lindsey J. Stacey R.B. Cardiac magnetic resonance in cardiac angiosarcoma.Echocardiography. 2017; 34: 1077-1081Crossref PubMed Scopus (11) Google Scholar,5Valeviciene N. Mataciunas M. Tamosiunas A. et al.Primary heart angiosarcoma detected by magnetic resonance imaging.Acta Radiol. 2006; 47: 675-679Crossref PubMed Scopus (4) Google Scholar The final diagnosis depends on the biopsy under thoracoscopy.6Ge Y. Ro J.Y. Kim D. et al.Clinicopathologic and immunohistochemical characteristics of adult primary cardiac angiosarcomas: analysis of 10 cases.Ann Diagn Pathol. 2011; 15: 262-267Crossref PubMed Scopus (33) Google Scholar Surgical resection combined with adjuvant chemotherapy is the preferred treatment currently. Chemotherapy will be the primary treatment if there are multiple metastases of tumors, and doxorubicin and ifosfamide are currently the first-line chemotherapy regimens for advanced metastatic angiosarcoma.7Wang M. Fu G. Jiang H. Zeng K. Hua P. Multimodality treatment for cardiac angiosarcoma.Intern Med. 2014; 53: 1949-1953Crossref PubMed Scopus (12) Google Scholar The prognosis of cardiac angiosarcoma is extremely unsatisfactory owing to several factors, including aggressive tumor biology, poor response to adjuvant therapy, and lack of targeted therapy. The prognosis is poor with a mean survival of 3 months to 1 year, even with complete radical resection.8Moeri-Schimmel R. Pras E. Desar I. Krol S. Braam P. Primary sarcoma of the heart: case report and literature review.J Cardiothorac Surg. 2020; 15: 104Crossref PubMed Scopus (19) Google Scholar This work was funded by the Wuhan University Zhongnan Hospital.