PDF(Pubmed)
Abstract：
Leiomyosarcomas (LMS) of the inferior vena cava (IVC) are a rare form of retroperitoneal malignancy, and their venous extension to the right atrium is an even rarer event. These tumors pose a unique surgical challenge and often require a multidisciplinary team-based approach for their surgical treatment. We present a case of a 68-year-old man with primary LMS of the IVC with a tumor thrombus extending into the right atrium that was initially deemed inoperable. After extensive neoadjuvant chemo-radiation with minimal tumor effect, the patient underwent en bloc surgical resection of the tumor along with removal of the infrarenal IVC and right kidney and adrenal without the need for cardiopulmonary bypass. This case demonstrates the successful management of a primary LMS of the IVC with right atrial extension using a multimodal approach of neoadjuvant chemo-radiation and en bloc surgical resection without cardiopulmonary bypass. This strategy may offer a curative option for selected patients with these rare and aggressive tumors, improving their survival and quality of life.
摘要：
下腔静脉（IVC）的平滑肌肉瘤（LMS）是一种罕见的腹膜后恶性肿瘤，他们的静脉延伸到右心房是一个更罕见的事件。这些肿瘤构成了独特的手术挑战，通常需要基于多学科团队的手术治疗方法。我们介绍了一例68岁的IVC原发性LMS患者，其肿瘤血栓延伸到右心房，最初被认为无法手术。在广泛的新辅助化疗放疗后，肿瘤效应最小，患者接受了肿瘤的整体手术切除,同时切除了肾下IVC,右肾和肾上腺,不需要进行体外循环.此病例证明了使用新辅助化学放射的多模态方法和无体外循环的整块手术切除成功地治疗了具有右心房扩展的IVC的原发性LMS。这种策略可能为这些罕见和侵袭性肿瘤的选定患者提供治疗选择，提高他们的生存和生活质量。
