Kraybill W G, Callery M P, Heiken J P, Flye M W
Department of Surgery and Radiology, Washington University School of Medicine, St. Louis, Mo, USA.
Surgery. 1997 Jan;121(1):31-6. doi: 10.1016/s0039-6060(97)90179-3.
Tumors of the inferior vena cava (IVC) are rare tumors. Although often locally confined, juxtaposed vital structures usually limit the extent of resection. However, complete surgical resection has been shown to be the most important positive prognostic factor.
Four patients had resection of primary vena caval tumors. In two patients with locally extensive vena caval tumors the limits of conventional resection were extended by means of complete resection of the involved infrahepatic IVC, aorta, and both kidneys. The IVC and aorta were reconstructed with synthetic grafts, and the uninvolved kidney was autotransplanted for both patients.
Of the two patients treated with more extensive resection, one patient had no evidence of disease 26 months after operation, and the second patient died of recurrent disease 23 months after operation.
Primary tumors of the IVC may extend locally without distant metastasis. Radical surgical excision as the primary mode of treatment provides the best chance for prolonged survival in appropriately selected patients with tumors of the IVC. After surgical excision the patient with the leiomyosarcoma was treated with radiation therapy and the patient with rhabdomyosarcoma by chemotherapy. Although leiomyosarcomas of the IVC are rare tumors, the first patient is only the third reported case of the even rarer rhabdomyosarcoma of the IVC.
下腔静脉（IVC）肿瘤是罕见肿瘤。尽管通常局限于局部，但毗邻的重要结构往往限制了切除范围。然而，完整的手术切除已被证明是最重要的预后积极因素。
4例患者接受了原发性腔静脉肿瘤切除术。在2例局部广泛的腔静脉肿瘤患者中，通过完整切除受累的肝下下腔静脉、主动脉和双肾，扩大了传统切除的范围。用合成移植物重建下腔静脉和主动脉，对2例患者均将未受累的肾脏进行自体移植。
在接受更广泛切除的2例患者中，1例患者术后26个月无疾病证据，第2例患者术后23个月死于复发性疾病。
IVC原发性肿瘤可能局限于局部生长而无远处转移。根治性手术切除作为主要治疗方式，为适当选择的IVC肿瘤患者提供了延长生存期的最佳机会。手术切除后，平滑肌肉瘤患者接受了放射治疗，横纹肌肉瘤患者接受了化疗。尽管IVC平滑肌肉瘤是罕见肿瘤，但第一例患者是IVC更罕见的横纹肌肉瘤的第三例报道病例。
