Background: Primary cardiac tumours are rare, and diagnosis may be difficult, as symptoms and cardiac imaging may mimic other cardiac diseases. The intimal sarcoma is the least commonly reported cardiac tumour with only few cases reported worldwide. In this case report, we present a case of an intimal sarcoma with a highly aggressive disease course.
Case Summary: A 60-year-old male with a history of prior aortoplasty due to congenital aortic stenosis, mechanical aortic valve replacement, and aortic stenting due to aortic dilatation presented with night sweats, malaise, and dyspnoea. Initial imaging (including transthoracic and transoesophageal echocardiography and emergency computed tomography) revealed masses suspected to be thrombi in the left atrium and ventricle. However, a positron emission tomography/computed tomography scan revealed that the masses were suspicious for malignancy. The patient underwent non-radical tumour resection and insertion of biological valve prostheses. Subsequent tissue analysis and pathology assessment revealed an intimal sarcoma. There were no curative treatment options, and the patient succumbed to his illness <3 months after surgery.
Discussion: This case report presents a case of a highly aggressive intimal sarcoma. As complete tumour resection is of great importance when it comes to life expectancy in cardiac sarcomas, early diagnosis using non-invasive and invasive imaging modalities is essential to start early treatment and to improve outcomes in this patient group.
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http://dx.doi.org/10.1093/ehjcr/ytad004 | DOI Listing |
Cardiovasc Pathol
January 2025
Section Cytopathology, Institute of Pathology, University Medical Center Hamburg-Eppendorf UKE, D-20246 Hamburg, Germany; Airway Research Center North (ARCN), German Center for Lung Research (DZL), Giessen, Germany.
Intimal sarcoma of blood vessels is a rare, aggressive tumor originating from vascular endothelial cells. This report presents a 22-year-old male diagnosed with an intimal sarcoma of the lower pulmonary vein, detailing diagnosis, treatment, and prognosis information. Additionally, this report explores the application application of Endobronchial Ultrasound-Guided Fine-Needle Aspiration (EBUS-FNA) alongside with Rapid Remote Online Evaluation (ROLE) for identifying a mass-like lesion in the pulmonary vein.
View Article and Find Full Text PDFIntern Med
January 2025
Department of Respiratory Medicine, National Hospital Organization Iwakuni Clinical Center, Japan.
Aortic intimal sarcoma is a rare disease with no established treatment and a poor prognosis. A 70-year-old man who underwent surgery for a mass shadow extending from the ascending aorta to the left common carotid artery on contrast-enhanced computed tomography was diagnosed with intimal sarcoma and underwent postoperative radiotherapy (66 Gy/33 Fr). Three brain metastases were identified after 1.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
September 2024
Department of Cardiovascular Surgery, Kanazawa University, Kanazawa, Japan.
Pulmonary artery intimal sarcoma has very poor prognosis, for which radical resection may be difficult. A 68-year-old man with pulmonary artery intimal sarcoma underwent bilateral pulmonary artery resection and replacement. At 31 months postoperatively, recurrence occurred at the anastomotic site of the right pulmonary artery.
View Article and Find Full Text PDFPathol Int
January 2025
Department of Surgical Pathology, Kagoshima University Hospital, Kagoshima, Japan.
A male in his seventies presented with lung cancer in the right lower lobe. The surgically resected specimen revealed a pleomorphic carcinoma featuring an adenocarcinoma component with lepidic, acinar, and papillary patterns, alongside a spindle cell component spreading along the pulmonary artery wall, resembling intimal sarcoma. The spindle tumor cells were positive for keratins, TTF-1, napsin A, and vimentin, but negative for p40, CK14, desmin, alpha-smooth muscle actin, CDK4, and MDM2.
View Article and Find Full Text PDFESMO Open
January 2025
Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea; Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea. Electronic address:
Background: Intimal sarcoma is a rare and aggressive soft-tissue sarcoma with limited treatment options. We explored genomic profiles of intimal sarcoma to uncover therapeutic implications.
Materials And Methods: We analyzed tumor tissues from patients with intimal sarcoma who visited the Seoul National University Hospital (SNUH) using whole-exome, whole-transcriptome, and clinical next-generation sequencing (NGS), integrated with intimal sarcoma NGS data from two public cohorts.
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