Leiomyosarcoma of inferior vena cava

A case of leiomyosarcoma of the inferior vena cava is presented and pertinent clinical features of 57 reported cases in the English literature are reviewed.     

Leiomyosarcoma of the inferior vena cava

Leiomyosarcoma of the inferior vena cava is a rare tumor of mesenchymal origin most commonly found in women. Clinical signs are non-specific. Imagery with ultrasound, CT, or MRI may strongly suggest the diagnosis, but it can only be confirmed by histologic examination of tissue obtained pre or intra-operatively. The tumor is slow growing but nonetheless carries a bad prognosis; it may grow to a large size before directly invading adjacent structures. Systemic spread is a late occurrence. Radical surgical resection is the only treatment which offers any hope for prolonged survival. Standard vascular surgical techniques are usually sufficient. Progress in the techniques of hepatectomy and liver transplantation have allowed the experienced surgeon to undertake the removal of retrohepatic lesions once considered unresectable. High-lying lesions adjacent to the hepatic veins or with thrombus extending into the proximal vena cava may require extracorporeal circulation with or without profound hypothermic circulatory arrest. The efficacy of chemotherapy, whether pre-operative for inaccessible tumors or post-operative for incompletely resected or recurrent tumor, is poorly defined and very limited.     

Leiomyosarcoma of the inferior vena cava

Leiomyosarcoma is a rare tumor of soft tissues. The inferior vena cava is affected infrequently, a literature review showing only 66 cases reported previously, 29 of these patients undergoing radical surgery. The case described was diagnosed during operation for gallstones, and the lesion was excised radically using an original technique. Experience of this case and opinions of various authors who have treated similar cases suggest that the factors determining choice of therapy are: the caval segment involved, an effective collateral circulation, longitudinal or circumferential extension of the affected vascular wall, the presence or absence of neoplastic or hepatic thromboses.     

Leiomyosarcoma of the inferior vena cava. Three cases]

We report three new cases of leiomyosarcoma of the inferior vena cava observed between 1985 and 1990. Two of them developed in women and one in a man. The age of the patients were 56, 29 and 57 years. The three tumors have been removed completely. One patient received postoperative radio- and chemotherapy and is well 68 months after excision. One patient developed liver metastasis 18 months after resection despite chemotherapy and died after 3 years. The last one refused postoperative radio- and chemotherapy, developed massive local recurrence and pulmonary metastasis three months postoperatively, and is still alive with disease after 8 months. The diagnostic modalities of these rare tumors as well as the problems created by their surgical resection and the need for a complementary treatment are discussed.     

Leiomyosarcoma of the inferior vena cava.

The thirty-second case of leiomyosarcoma of the inferior vena cava and the twelfth resectable patient in the series is presented. A review of the literature demonstrates a marked female preponderance (5 to 1). The most common presenting symptom is right abdominal pain and a palpable mass. The different surgical problems generated by the involved segment of the cava are discussed. Noteworthy is the high incidence of Budd-Chiari syndrome owing to hepatic vein obstruction with involvement of the upper third of the cava in the postmortem cases. An argument is developed for debulking the tumor for palliation when it is not completely removable.     

Leiomyosarcoma of the infra-renal inferior vena cava

Leiomyosarcoma of the inferior vena cava (IVC) is a rare slow-growing retroperitoneal tumor. Two percent of leiomyosarcomas are vascular in origin, and tumors of the IVC account for the majority of the cases. The diagnosis is frequently delayed, because affected patients remain asymptomatic for a long period. It has an extremely poor prognosis, with 5-year actuarial malignancy-free survival rates of 30% to 50% after a wide surgical resection. The authors present the case of a patient with IVC leiomyosarcoma who underwent en bloc resection of the tumor along with the involved segment of the infrarenal IVC without caval reconstruction. Complete surgical resection offers the only potential of long-term survival, but survival of unresected patients is generally measured in months. Palliative resections may temporarily improve symptoms but do not offer long-term survival.     

Leiomyosarcoma of renal vein and inferior vena cava

A case of leiomyosarcoma of the right renal vein and inferior vena cava causing total obstruction of the vena cava is reported. This rare condition was treated successfully after two previous operations had deemed it unresectable. Treatment consisted of a right nephrectomy and resection of the vena cava and ligation of the left renal vein. Because of the slow growth of the well-differentiated leiomyosarcoma, there was no problem with venous return from the left kidney, and the patient recovered promptly without evidence of renal function impairment. Postoperatively, she received chemotherapy consisting of doxorubicin (Adriamycin) and vincristine. She is well and free of tumor over four years postoperatively. The good result, in this case, is attributed to complete surgical excision and multimodal therapy.     

Leiomyosarcoma of the inferior vena cava (case report)

The occur of resecable retroperitoneal tumours invading into inferiér vena cava is rare. The authors report in detail a case of fibrosarcoma of the inferior vena cava In article is presented case of 49-year-old female presented with echographic and computed tomographic evidence of solid formation below porta hepatis and ower head of pankreas, behinde duodenum, growed into inferior vena cava with trombus. Treatment of the abdominal and retroperitoneal tumours closely related to major blood vessels must be interdisciplinary, considering diagnostics, operability estimation and additional measures. The radical resection rate for involving important vesel may bee improved with vascular technique.     

下腔静脉平滑肌肉瘤1例

[病例简介]女,66岁.无明显诱因上腹部持续性胀痛1个月,上腹部皮肤发热入院.无恶心、呕吐及放射痛,无尿频、尿急、尿痛及血尿.查体：腹部饱满,未见肠型及蠕动波,右上腹部压痛,无明显反跳痛,肝脾肋下未触及,肝、肾区无叩痛,无移动性浊音,肠鸣音正常,双下肢无水肿.[第一段]     

Leiomyosarcoma of the inferior vena cava: a case report

Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a rare vascular tumour, with about 200 cases reported since 1871. The Authors report a case of leiomyosarcoma of the suprarenal inferior vena cava preoperatively diagnosed by ultrasonography and computed tomography. A surgical resection and venous wall reconstruction was performed. Complete surgical resection with a tumour-free margin (1 cm) is the treatment of choice. Neoadjuvant therapy may be given to downsize the tumour and increase resectability rate. When complete resection is not possible, debulking combined with radiation therapy provides good palliation.     

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??Objective:To evaluate the clinical characteristics of leiomyosarcoma of inferior vena cava and to summarize the experience on the treatment of leiomyosarcoma of the inferior vena cava. Methods:The clinical data of 14 patients with leiomyosarcoma of inferior vena cava admitted between 1986 and 2006 in the General Hospital of PLA was analyzed retrospectively. Results:The diagnosis of the 12 patients was certained by pathology.Eleven cases were resected successfully with necessary reconstruction of inferior vena cava and only one patient was performed laparotomy.Two patients were not operated because of distant metastases. Conclusion:The en bloc resection with necessary reconstruction of inferior vena cava is an effective method to treat leiomyosarcoma of inferior vena cava.     

下腔静脉平滑肌肉瘤的诊断及外科治疗

目的 了解下腔静脉平滑肌肉瘤的临床特点，总结下腔静脉平滑肌肉瘤的诊治经验。方法 对1986—2006年中国人民解放军总医院普外科收治的14例下腔静脉平滑肌肉瘤病人的临床资料进行回顾性分析。结果 12例病人实施手术，其中11例行肿瘤及相应的下腔静脉切除和必要的下腔静脉重建，1例仅行剖腹探查肿瘤活检；2例因远处转移而未手术。结论 肿瘤整块切除及必要的下腔静脉重建是治疗下腔静脉平滑肌肉瘤的有效方法。