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<p>Leiomyosarcoma of the inferior vena cava (IVC) is a rare and aggressive malignancy, with poor prognosis and no universally accepted treatment protocol. We present the case of a 69-year-old male with an IVC leiomyosarcoma, which extended from the common iliac veins to the right renal vein. Initial imaging showed a retroperitoneal mass with suspected IVC involvement. Histopathological examination confirmed leiomyosarcoma, and the patient underwent four chemotherapy cycles with doxorubicin, followed by surgical resection. A multidisciplinary approach, including general, vascular, and urologic surgeons, was employed. The tumor was excised en bloc with the IVC and right kidney. No prosthetic IVC reconstruction was necessary due to the lack of hemodynamic changes after clamping. Postoperative recovery was uneventful except for mild renal impairment. Molecular profiling of the tumor using next-generation sequencing (NGS) identified mutations in TP53, RB1, KMT2C, TSC2, and other genes associated with chromatin stability and tumor suppression. The patient experienced a local recurrence at four months and was treated with chemotherapy. This case underscores the importance of personalized treatment strategies, including surgical decision-making, chemotherapy, and molecular characterization, in managing rare tumors like IVC leiomyosarcoma. Further research is needed to explore potential oncogenic targets and improve prognosis.</p>

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Multidisciplinary management and molecular characterization of inferior vena cava leiomyosarcoma: a case report

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年11月25日

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  • Alessia Pini,
  • Maria Abbondanza Pantaleo,
  • Annalisa Astolfi,
  • Stefano Ancetti,
  • Martina Sofia Rossi,
  • Maria Giulia Pirini,
  • Livia Gozzellino,
  • Alice Costa,
  • Maria Concetta Nigro,
  • Luca Boschi,
  • Riccardo Schiavina,
  • Gilberto Poggioli,
  • Mauro Gargiulo

摘要

Leiomyosarcoma of the inferior vena cava (IVC) is a rare and aggressive malignancy, with poor prognosis and no universally accepted treatment protocol. We present the case of a 69-year-old male with an IVC leiomyosarcoma, which extended from the common iliac veins to the right renal vein. Initial imaging showed a retroperitoneal mass with suspected IVC involvement. Histopathological examination confirmed leiomyosarcoma, and the patient underwent four chemotherapy cycles with doxorubicin, followed by surgical resection. A multidisciplinary approach, including general, vascular, and urologic surgeons, was employed. The tumor was excised en bloc with the IVC and right kidney. No prosthetic IVC reconstruction was necessary due to the lack of hemodynamic changes after clamping. Postoperative recovery was uneventful except for mild renal impairment. Molecular profiling of the tumor using next-generation sequencing (NGS) identified mutations in TP53, RB1, KMT2C, TSC2, and other genes associated with chromatin stability and tumor suppression. The patient experienced a local recurrence at four months and was treated with chemotherapy. This case underscores the importance of personalized treatment strategies, including surgical decision-making, chemotherapy, and molecular characterization, in managing rare tumors like IVC leiomyosarcoma. Further research is needed to explore potential oncogenic targets and improve prognosis.