Bartholomew Vanessa, Schammel Christine M G, Devane A Michael, Schammel David P, Trocha Steven D
University of South Carolina School of Medicine Greenville, Greenville, SC, USA.
Pathology Associates, Greenville, SC, USA.
Am Surg. 2025 Aug;91(8):1316-1322. doi: 10.1177/00031348251333096. Epub 2025 Apr 14.
Leiomyosarcomas of the inferior vena cava (IVC) are the most common primary malignancy of the IVC; however, these lesions are extremely rare. These lesions are slow-growing, often asymptomatic and, present late in the disease course portending a poor prognosis. Surgical resection is considered the standard of care and only potential curative treatment. Progression free survival rates after surgical resection have been estimated to be 31.4% after five years and 7.4% after 10 years. To date, there have been a total of 418 cases documented within small and large single center institutional case series. Here, we present a case series from a single institution and a comprehensive literature review to investigate the presentation, treatment, and outcomes of this rare lesion. A surgically relevant designation of tumor location to include involvement of the renal veins is proposed.
下腔静脉平滑肌肉瘤是下腔静脉最常见的原发性恶性肿瘤；然而，这些病变极为罕见。这些病变生长缓慢，通常无症状，且在疾病进程后期才出现，预后较差。手术切除被认为是标准治疗方法，也是唯一可能治愈的治疗手段。手术切除后的无进展生存率估计5年后为31.4%，10年后为7.4%。迄今为止，在大小不等的单中心机构病例系列中总共记录了418例病例。在此，我们展示来自单一机构的病例系列以及全面的文献综述，以研究这种罕见病变的表现、治疗及结果。我们提出一种与手术相关的肿瘤位置命名法，包括肾静脉受累情况。
