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Inferior vena cava (IVC) leiomyosarcoma is a rare and aggressive malignancy arising from smooth muscle cells of the IVC wall, accounting for less than 1 in 100,000 adult malignancies. It carries a poor prognosis, with high recurrence rates and 5-year disease-free survival reported as low as 6%. Clinical presentation is often vague, including abdominal or back pain, lower extremity edema, and weight loss, making imaging pivotal for diagnosis. Contrast-enhanced CT (CECT) is the modality of choice, but diagnosis remains challenging due to overlap with more common pathologies such as renal cell carcinoma, hepatocellular carcinoma, adrenal tumors, and retroperitoneal lymphadenopathy. This pictorial review presents a curated series of cases highlighting the spectrum of imaging appearances of IVC leiomyosarcoma. By showcasing both typical and atypical imaging patterns, this review aims to broaden radiologists' diagnostic awareness and improve confidence in identifying this elusive tumor. Special emphasis is placed on 3D CT imaging techniques, including optimal acquisition protocols and post-processing methods, to enhance lesion visualization and vascular mapping. Early and accurate recognition is crucial, as aggressive surgical resection remains the mainstay of treatment. Understanding key imaging clues can facilitate timely diagnosis and intervention, ultimately impacting patient outcomes in this rare but life-threatening disease.
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http://dx.doi.org/10.1007/s00261-025-05175-5 | DOI Listing |
Discov Oncol
September 2025
Department of Vascular Surgery, The Affiliated People's Hospital of Ningbo University, Ningbo, Zhejiang, China.
Background: Retroperitoneal leiomyosarcoma represents a poor-prognosis malignancy for which current clinical treatment options remain limited. This study may further help bridge the gap in available therapeutic modalities.
Case Presentation: This case report describes a 75-year-old female patient who was admitted to the hospital with right lower abdominal pain and imaging studies suggesting a retroperitoneal mass and consideration of leiomyosarcoma.
Ann Surg Oncol
August 2025
Division of Abdominal Organ Transplant and Hepatobiliary Surgery, Department of Surgery, Columbia University Vagelos College of Physicians and Surgeons, New York, NY, USA.
Background: Leiomyosarcomas of the inferior vena cava (IVC) pose significant surgical challenges, particularly when tumors extend to the hepatic veins (HVs) and the heart. Such cases often necessitate innovative surgical strategies involving complex vascular and organ reconstruction. PATIENT AND METHODS: The patient is a 61-year-old female with a large expansile IVC leiomyosarcoma extending into the right atrium and involving the atrial septum.
View Article and Find Full Text PDFAbdom Radiol (NY)
August 2025
Johns Hopkins Hospital, Baltimore, United States.
Inferior vena cava (IVC) leiomyosarcoma is a rare and aggressive malignancy arising from smooth muscle cells of the IVC wall, accounting for less than 1 in 100,000 adult malignancies. It carries a poor prognosis, with high recurrence rates and 5-year disease-free survival reported as low as 6%. Clinical presentation is often vague, including abdominal or back pain, lower extremity edema, and weight loss, making imaging pivotal for diagnosis.
View Article and Find Full Text PDFCureus
July 2025
Radiodiagnosis, Alluri Sitarama Raju Academy of Medical Sciences College and Hospital, Eluru, IND.
Primary sarcomas of renal origin are among the rarest tumors to present, and definitive diagnosis is based primarily on histopathology since there are no characteristic clinical features. Renal sarcomas are known for their aggressive growth and late presentation, resulting in a particularly poor prognosis. This article reports a case of primary leiomyosarcoma of renal origin in a 38-year-old female patient who presented with complaints of pain and a palpable abdominal mass.
View Article and Find Full Text PDFBMC Cancer
July 2025
Department of Urology, Peking University Third Hospital, Beijing, 100191, PR China.
Background: Retroperitoneal vascular leiomyosarcoma (RVLMS), which originates from vascular wall smooth muscle cells, typically requires inferior vena cava (IVC) reconstruction during radical surgery.
Methods: A retrospective cohort study was conducted on 24 patients who underwent primary resection of RVLMS from June 2015 to November 2023 in one institution. The patient details, operative management, and follow-up data were assessed.