Rationale: Intimal sarcoma of inferior vena cava (IVC) is a rare soft tissue sarcoma with no typical symptoms and specific imaging features in the early stage, and there is a lack of standardized treatment and methods.
Patient Concerns: A 54-year-old female patient presented to Fenghua District People's Hospital with a post-active cough and hemoptysis and was subsequently referred to our hospital.
Diagnoses: The patient was pathologically diagnosed as intimal sarcoma of IVC complicating multiple intrapulmonary metastases. Chest CT revealed left lung malignant tumor with multiple intrapulmonary metastases; while enhanced upper abdominal CT showed cancer embolus of IVC with extension to right atrium and bilateral renal veins. Besides, hematoxylin and eosin staining suggested intimal sarcoma of veins. Immunohistochemical staining showed positivity for PD-L1, Ki-67, CD31, Desmin and ERG.
Interventions: The patient initially received GT chemotherapy (gemcitabine injection + docetaxel). Then, immunotherapy (tislelizumab) was added based on the results of genetic testing (TP53 gene mutation).
Outcomes: The disease was stabilized after receiving the treatment.
Lessons: Given the lack of characteristic clinical manifestations in patients with intimal sarcoma of IVC, imaging examination combined with immunohistochemical index were helpful for diagnosis of intimal sarcoma of IVC. Furthermore, the combination of tislelizumab and GT chemotherapy was feasible in such patients with positive PD-L1 expression and TP53 mutation.
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http://dx.doi.org/10.1097/MD.0000000000038056 | 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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