Ruptured angiosarcoma of the liver treated by emergency catheter-directed embolization.

Angiosarcoma is a rare primary malignant neoplasm of the liver with a poor prognosis. Here, we report a case of a patient with a ruptured hepatic angiosarcoma which was treated by emergency catheter-directed embolization, followed by left-sided hemihepatectomy.

Clinical results of surgery for retroperitoneal sarcoma with major blood vessel involvement.

PURPOSE: The study was conducted to evaluate the clinical results of resection for retroperitoneal soft tissue sarcoma (STS) with vascular involvement. METHODS: The study group consisted of consecutive patients (mean age, 52 years) who underwent surgery for retroperitoneal STS with vascular involvement. The procedures were performed between 1988 and 2004. Vessel involvement by STS was classified as type I, artery and vein; type II, only artery; type III, only vein; and type IV, neither artery nor vein (excluded from the analysis). Patient data were prospectively gathered in a computerized database and retrospectively analyzed. RESULTS: Of 141 patients with retroperitoneal STS, 25 (17.7%) underwent surgery for tumors with vascular involvement. The most common vascular involvement pattern was vein only (type III) at 64%. Arterial and vein (type I) and arterial only (type II) involvement were observed in 16% and 20% of the cases, respectively. STS originating from the vessel wall (primary vessel involvement) was seen in eight patients, and 17 patients had secondary vascular involvement. Resection and vascular repair were done in 22 patients (no vascular repair in three patients due to ligation of the external iliac vein in one patient, and debulking procedures in two). All patients with arterial involvement (type I and II) had arterial reconstruction consisting of aortic replacement (Dacron, n = 3; and expanded polytetrafluoroethylene [ePTFE], n = 2), iliac repair (Dacron, n = 3), and truncal reimplantation (n = 1). The inferior vena cava (6 ePTFE tube grafts, 3 ePTFE patches, 2 venoplasties), iliac vein (1 ePTFE bypass, 1 Dacron bypass, 1 venous patch), and superior mesenteric vein (1 anastomosis, 1 Dacron bypass) were restored in 80% of the patients (n = 16) with either arterial and venous or only venous involvement (type I and type III setting). Morbidity was 36% (hemorrhage, others), and mortality was 4%. At a median follow-up of 19.3 months (interquartile range, 12.8 to 49.9 months) the arterial patency rate was 88.9%, and the venous patency rate was 93.8% (primary and secondary). Thrombosis developed in one arterial and venous (type I) iliac reconstruction due to a perforated sigmoid diverticulitis 12 months after surgery. The local control rate was 82.4%. The 2-year and 5-year survival rates were 90% and 66.7% after complete resection with tumor-free resection margins (n = 10 patients, median survival not reached at latest follow-up). The median survival was 21 months in patients with complete resection but positive resection margins (n = 7) and 8 months in patients with incomplete tumor clearance (n = 8, persistent local disease or metastasis). CONCLUSIONS: Patency rates and an acceptable surgical risk underline the value of en bloc resection of retroperitoneal STS together with involvement of blood vessels. The oncologic outcome is positive, especially after complete resection with tumor-free resection margins. A classification of vascular involvement can be used to plan resection and vascular replacement as well as to compare results among reports in a standardized fashion.

Results of limb-sparing surgery with vascular replacement for soft tissue sarcoma in the lower extremity.

OBJECTIVE: To evaluate limb-salvage surgery with vascular resection for lower extremity soft tissue sarcomas (STS) in adult patients and to classify blood vessel involvement. METHODS: Subjects were consecutive patients (median age, 56 years) who underwent vascular replacement during surgery of STS in the lower limb between January 1988 and December 2003. Blood vessel involvement by STS was classified as follows: type I, artery and vein; type II, artery only; type III, vein only; and type IV, neither artery nor vein (excluded from the analysis). Patient data were prospectively gathered in a computerized database. RESULTS: Twenty-one (9.9%) of 213 patients underwent vascular resections for lower limb STS. Besides 17 type I tumors (81.0%), 3 (14.3%) type II and 1 (4.7%) type III STS were diagnosed. Arterial reconstruction was performed for all type I and II tumors. Venous replacement in type I and III tumors was performed in 66.7% of patients. Autologous vein (n = 8) and synthetic (Dacron and expanded polytetrafluoroethylene; n = 12) bypasses were used with comparable frequency for arterial repair, whereas expanded polytetrafluoroethylene prostheses were implanted in veins. Morbidity was 57.2% (hematoma, thrombosis, and infection), and mortality was 5% (embolism). At a median follow-up of 34 months, the primary and secondary patency rates of arterial (venous) reconstructions were 58.3% (54.9%) and 78.3% (54.9%). Limb salvage was achieved in 94.1% of all cases. The 5-year local control rate and survival rate were 80.4% and 52%, respectively. We observed a 5-year metastasis-free survival rate of 37.7% and found vessel infiltration and higher tumor grade (low-grade vs intermediate grade and high grade tumors) to be negative prognostic factors at univariate and multivariate analysis. CONCLUSIONS: Long-term bypass patency rates, the high percentage of limb salvage, and the oncologic outcome underline the efficacy of en bloc resection of STS involving major vessels in the lower limb. Disease-specific morbidity must be anticipated. The classification of vascular involvement (type I to IV) is useful for surgical management.

Malignant vascular tumors: clinical presentation, surgical therapy, and long-term prognosis.

BACKGROUND: The aim of this study was to analyze the presentation of, surgery for, and prognosis of malignant vascular tumors (MVTs). METHODS: This was an observational single-center study. Patients who underwent operation for MVTs between 1988 and 2004 were included. Data were gathered prospectively in a computerized registry. RESULTS: Of 568 adult patients with soft tissue malignancies, 43 (7.6%) were treated for MVTs. Twenty-four men and 19 women (median age, 55.3 years) were referred for 30 primary tumors and 13 recurrences. Symptoms were observed in 90.7% of the cases (swelling [37.2%], pain [34.9%], extrusion [11.6%], hemorrhage [7%], weight loss [4.7%], loss of energy [4.7%], impaired function [4.7%], and others [30.2%]). Tumors were located in the extremities (n = 16), trunk (n = 3), abdomen (n = 15), retroperitoneum (n = 7), and thyroid gland (n = 2). Twenty-two (51.2%) angiosarcomas, nine (20.9%) malignant hemangiopericytomas, eight (18.6%) malignant epithelioid hemangioendotheliomas, and four (9.3%) lymphangiosarcomas were seen. The median overall survival after surgery was 21.4 months, with 2-, 5-, and 10-year overall survival rates of 41.5%, 38.3%, and 18.8%, respectively. MVTs of the extremities and trunk and localized disease indicated a better prognosis than abdominal or retroperitoneal MVTs (univariate and multivariate analyses: P = .0122 and P = .0287) and metastasized stages (univariate and multivariate analyses: P = .0187 and P = .0287). CONCLUSIONS: A considerable number of patients with soft tissue malignancies undergo surgery for MVT. Various symptoms and a multilocular occurrence are typical. The course of MVTs is aggressive. Tumor site and stage are important prognostic factors. Surgery is potentially curative, especially for localized disease of the extremities and trunk.