原发性下腔静脉平滑肌肉瘤的诊治体会

目的 探讨原发性下腔静脉平滑肌肉瘤的临床特点和诊治方法.方法 回顾性分析2006年6月至2009年4月收治的7例原发性下腔静脉平滑肌肉瘤的临床资料,包括临床表现、手术方法、病理结果和预后.结果 3例完整切除肿瘤,其中2例下腔静脉管壁缺损应用人工血管进行修补;1例切除部分肿瘤,恢复肝静脉血流;3例行剖腹探查肿瘤活检,明确诊断后放弃手术.7例术后病理均为原发性下腔静脉平滑肌肉瘤,3例雌激素受体(estrogen receptor,ER)、孕激素受体(progesterone receptor,PR)呈阳性,4例呈阴性.无围手术期死亡病例,3例完整切除肿瘤者术后预防性口服华法林抗凝6个月,分别随访8、32、33个月,未发现肿瘤复发和血栓形成;1例部分切除者存活2个月,死于肝功能衰竭;3例只行肿瘤活检者均于7个月内死亡.结论 完整切除肿瘤和必要的下腔静脉重建是治疗原发性下腔静脉平滑肌肉瘤的惟一有效方法.     

A huge metastatic liver tumor from leiomyosarcoma of the inferior vena cava: report of a case

Leiomyosarcoma of the inferior vena cava (IVC) is a rare malignant tumor, and only a few cases of the resection of IVC leiomyosarcomas with synchronous liver metastases have been reported. This report describes a female patient who initially presented with a solitary, huge liver tumor and a retroperitoneal tumor. Following our preoperative diagnosis of primary liver cancer with a retroperitoneal lymph node metastasis, the patient underwent combined resection of both tumors. The surgical findings revealed that the retroperitoneal tumor originated from the IVC wall. The pathological and immunohistochemical findings revealed that both tumors were leiomyosarcomas. Although the liver tumor was much larger than the IVC tumor, we considered that the metastatic liver tumor arose from the IVC leiomyosarcoma. This was an instructive case because the metastatic liver tumor from the IVC leiomyosarcoma was so large as to be mistaken for a primary liver tumor.     

An unusual case of leiomyosarcoma of the inferior vena cava in a patient with a duplicated inferior vena cava

Leiomyosarcoma of primary vascular origin is a rare primary soft tissue tumour, which arises mainly from the inferior vena cava (IVC). Clinical symptoms depend upon the size and location of the tumour and presents usually with abdominal pain, palpable mass and weight loss. Complete surgical resection with clear surgical margin plays a central therapeutic role. The effect of chemotherapy and radiation therapy remains to be evaluated. We report a 64 year old Chinese female who presented with abdominal mass and pain associated with weight loss and was subsequently diagnosed with inferior vena cava leiomyosarcoma. She underwent successful surgical resection but unfortunately developed recurrence of tumour 12 month post-operative. She was also found to have a duplicated inferior vena cave which allowed reconstitution of venous return from the lower limbs after surgical resection of the IVC tumour. We discuss the surgical treatment and results of leiomyosarcoma of the IVC.     

A case of leiomyosarcoma of the inferior vena cava

Primary leiomyosarcoma of the inferior vena cava is rare and fatal. A 15-year-old female was admitted with the diagnosis of the right atrial myxoma associated with the Budd-Chiari syndrome. The tumor of the IVC was unresectable, although the right atrial tumor extended from the IVC was extirpated. Pathological examination of the tumor revealed a leiomyosarcoma. She was hemodynamically improved immediately after the operation. But she was expired due to the hepatic failure 9 days postoperatively. No autopsy was obtained. The literature regarding with leiomyosarcoma of the IVC was briefly reviewed.     

A case of leiomyosarcoma of the inferior vena cava

A case of primary leiomyosarcoma of the inferior vena cava was reported. The patient, a 74-year-old man, consulted our hospital with complaints of back pain and abdominal mass in right flank. Probe laparotomy revealed a tumor situated in the retroperitoneum and multiple metastatic nodules in the liver. Biopsy was performed and diagnosed as leiomyosarcoma. He was intensively treated with antineoplastic chemotherapy, but his condition gradually aggravated. He died 4 months after admission. The autopsy revealed a right retroperitoneal mass (17 X 12 X 18 cm in size, 1,340 g in weight) that showed a yellowish appearance. There was also a thumb-tip sized tumor with a stalk in the lumen of inferior vena cava. Both tumors grew in continuity with each other through the wall of inferior vena cava. The tumors were diagnosed as leiomyosarcoma, which derived from inferior vena cava.     

原发性下腔静脉平滑肌肉瘤的手术治疗

目的 探讨原发性下腔静脉平滑肌肉瘤的手术治疗.方法 回顾性分析山东大学附属省立医院血管外科2009年10月-2011年5月收治的5例原发性下腔静脉平滑肌肉瘤患者,均行手术治疗,采用肿瘤及受累下腔静脉切除、人工血管重建双肾静脉及远端下腔静脉血流的术式.结果 本组5例患者平均手术时间为166.6 min,术中平均失血量为1...     

Primary leiomyosarcoma of inferior vena cava: Case report

An intramural venous leiomyosarcoma is a rare, malignant tumour arising from the smooth muscle cells of the vessel wall and the inferior vena cava (IVC) is the most common location. The middle part of IVC is most often affected, often involving the kidneys. There is a strong prediction for women. Clinical symptoms depend upon the size and location of the tumour. Diagnosis is often not made until advanced stage, as the symptoms are non-specific and they present late in the disease course.     

Prosthetic replacement of the infrahepatic inferior vena cava for leiomyosarcoma

HYPOTHESIS: Resection of the infrahepatic inferior vena cava associated with prosthetic graft replacement for caval leiomyosarcoma is an acceptable procedure to obtain prolonged and good-quality survival. DESIGN: A consecutive sample clinical study with a mean follow-up of 40 months. SETTING: The surgical department of an academic tertiary center and an affiliated secondary care center. PATIENTS: Eleven patients, with a mean age of 51 years, who have primary leiomyosarcoma of the infrahepatic inferior vena cava. INTERVENTIONS: All of the patients underwent radical resection of the tumor en bloc with the affected segment of the vena cava. Reconstruction consisted of 10 cavocaval polytetrafluoroethylene grafts and 1 cavobiliac graft. An associated right nephrectomy was performed in 2 cases. The left renal vein was reimplanted in the graft in 3 cases. MAIN OUTCOME MEASURES: Cumulative disease-specific survival, disease-free survival, and graft patency rates expressed by standard life-table analysis. RESULTS: No patients died in the postoperative period. The cumulative (SE) disease-specific survival rate was 53% (21%) at 5 years. The cumulative (SE) disease-free survival rate was 44% (19%) at 5 years. The cumulative (SE) graft patency rate was 67% (22%) at 5 years. CONCLUSION: Radical resection followed by prosthetic graft reconstruction is a valuable method for treating primary leiomyosarcoma of the infrahepatic inferior vena cava.     

原发性下腔静脉平滑肌肉瘤1例

<正>患者女,55岁,因"无明显诱因出现腹部包块2年余"入院。查体:下腹部触及约15cm×15cm×20cm肿物,活动性欠佳,无明显压痛、反跳痛及肌紧张。CT:右侧腹膜后见团块状浅分叶型软组织密度影,约18.1cm×10.4cm×8.6cm,CT值约9~70HU,边缘尚清,增强扫描病灶呈不均匀延迟强化,内见     

Acute Budd-Chiari syndrome secondary to leiomyosarcoma of the inferior vena cava

The authors report a case of leiomyosarcoma of the inferior vena cava, responsible for an acute Budd-Chiari syndrome. The diagnosis, suggested by lower limb edema, ascites, and renal failure, was confirmed by sonography, CT scan, and pathological examination. A mesoatrial shunt and right atrial thrombectomy were performed under extracorporeal circulation. The inferior vena cava was ligated because the tumor was considered to be unresectable.