Castellanos Luis D, Tabbara Marina M, Livingstone Alan S, Salerno Tomas A, Gonzalez Javier, Ciancio Gaetano
Department of Surgery, Leonard M. Miller School of Medicine, University of Miami, Miami, FL, United States.
Miami Transplant Institute, Jackson Health System, Miami, FL, United States.
Front Oncol. 2024 Jan 4;13:1331896. doi: 10.3389/fonc.2023.1331896. eCollection 2023.
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.
下腔静脉平滑肌肉瘤(LMS)是一种罕见的腹膜后恶性肿瘤,其静脉延伸至右心房的情况更为罕见。这些肿瘤带来了独特的手术挑战,通常需要多学科团队协作的方法进行手术治疗。我们报告一例68岁男性,患有下腔静脉原发性平滑肌肉瘤,肿瘤血栓延伸至右心房,最初被认为无法手术切除。在进行了广泛的新辅助放化疗但肿瘤缩小效果甚微后,患者接受了肿瘤整块切除术,同时切除肾下下腔静脉、右肾和肾上腺,无需体外循环。该病例展示了采用新辅助放化疗和整块手术切除且无需体外循环的多模式方法,成功治疗了下腔静脉原发性平滑肌肉瘤伴右心房延伸的情况。这种策略可能为选定的这些罕见且侵袭性肿瘤患者提供治愈选择,提高他们的生存率和生活质量。