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原发性下腔静脉平滑肌肉瘤12例回顾性分析

Retrospective analysis of management for primary leiomyosarcoma of inferior vena cava

摘要:

目的 探讨原发性下腔静脉平滑肌肉瘤治疗策略及预后.方法 回顾性分析2006年1月至2014年9月北京大学人民医院血管外科收治的12例经病理检查证实的下腔静脉平滑肌肉瘤患者的临床资料,其中男性4例,女性8例,平均年龄(54±9)岁.根据肿瘤累及的下腔静脉水平,分为肝上段5例,肾上段7例.8例患者接受手术切除(手术组),4例仅接受剖腹探查肿瘤活检(保守组).结果 手术组中,根治切除6例,姑息切除2例;下腔静脉缩缝成形6例,下腔静脉补片成形1例,人工血管原位置换1例.4例肝上段肿瘤在完全体外循环(2例)或右心房灌注插管(2例)技术辅助下完成手术切除.围手术期发生并发症4例,其中2例早期死亡.保守组4例患者术后8个月内均死亡.手术组非早期死亡的6例患者随访期内均存活,平均生存时间(54 ±40)个月;术后复发2例,总复发率2/6.根治切除的1例患者获得101个月的无复发生存.结论 外科治疗可能是下腔静脉平滑肌肉瘤患者唯一有望获得长期生存的治疗手段.右心房插管灌注或心肺转流的应用可以使一部分肝上段下腔静脉平滑肌肉瘤患者获得手术机会、争取更长的生存期.

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abstracts:

Objective To study the treatment strategy and survival of patients with primary leiomyosarcoma of inferior vena cava (PIVCLMS).Methods Clinical data of 12 cases with PIVCLMS admitted in Peking University People's Hospital from January 2006 to September 2014 were reviewed retrospectively.All cases were confirmed by pathology examination.Among them,there were 4 male and 8 female patients with a mean age of (54 ± 9) years old.Tumors arose from the inferior vena cava (IVC) upper segment in 5 patients,from the middle in other 7 patients.Cardiac extension was observed in 4 cases.Tumor resection was undertaken in 8 patients,the other 4 patients were inoperable.The series was analyzed to identify clinical outcome of surgical strategy and protective factors for patient survival.Results In tumor resection group,6 patients had radical resection and 2 underwent palliative resection.As for IVC reconstruction,caval wall resection with a direct suture was carried out in 6 patients or with prosthetic patch in 1 patient.The other 1 patient underwent a segment caval resection and prosthetic graft replacement in situ.In 4 cases of suprahepatic PIVCLMS cardiopulmonary bypass or perfusion by right atrial intubation was performed to assist bleeding control and maintain circulation stabilization,among them 1 patient survived for more than 101 months with no tumor recurrence or metastasis.Among the patients submitted to tumor resection 2 early postoperative deaths occurred,and another 2 patients had complications.All 4 patients submitted to non-resective operation (only neoplasm biopsy) died of PIVCLMS within 8 months.Except for 2 cases of early death,mean survival after tumor resection was (54 ± 40) months.Two patients presented local recurrence and hepatic metastasis at follow-up of 16 months and 68 months.Conclusions Tumor resection is the only therapy for PIVCLMS with an expectation for long-term survival.The applicant of cardiopulmonary bypass makes some inoperable indicated to tumor resection.

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