Isolated limb perfusion for locally advanced angiosarcoma in extremities: A multi-centre study

BackgroundAngiosarcomas are rare and aggressive soft-tissue sarcomas. The only potential curative treatment is complete surgical excision. This study reports the outcome of isolated limb perfusion (ILP) with high-dose melphalan and tumour necrosis factor α for locally advanced angiosarcoma.Material and methodsAll patients who underwent an ILP for angiosarcomas between 1991 and 2016 in three tertiary referral centres were identified from prospectively maintained databases.ResultsA total of 39 patients were included, with a median follow-up of 18 months (interquartile range 6.1–60.8). Of these patients, 23 (58.9%) patients had a complete response (CR) after ILP, 10 (25.6%) had a partial response, 4 (10.3%) had stable disease and 2 (5.1%) patients had progressive disease immediately after ILP. A total of 22 patients developed local progression (56.4%), whereas nine (23.1%) developed distant metastases. The patients with CR had a significantly prolonged median local progression-free survival (PFS) (15.4 versus 7.3 months, p = 0.015) when compared with non-CR patients, and a trend towards better median overall survival (81.2 versus 14.5 months, p = 0.054). Six patients underwent multiple ILPs, whereby the CR rate of the first, second and third ILPs were 60%, 80% and 67%, respectively. Thirteen (33.3%) patients needed further surgical intervention, consisting of resection in eight patients (20.5%) and amputation in five patients (12.8%).ConclusionILP is an effective treatment option for patients with locally advanced angiosarcoma in the extremities, resulting in a high number of CRs, a high limb salvage rate and prolonged local PFS.     

Surgical treatment outcomes of primary hepatic sarcomas: A single-center experience

BACKGROUNDPrimary hepatic sarcoma is a rare tumor originated from mesenchymal tissue. There are various pathologic types of primary hepatic sarcoma and the treatment outcome of this tumor was usually disappointing. Unlike hepatocellular carcinoma, outcome of primary hepatic sarcoma is not well-known due to it’s rarity. However, with development of medical technology, surgical treatment may lead to better survival.AIMTo investigate the surgical outcomes of primary hepatic sarcoma, we gathered and analyzed the cases of a single institute.METHODSFrom August 2001 to September 2016, a total of nine patients were surgically treated for primary hepatic sarcoma after exclusion of cases with open and closure, early loss to follow-up and sarcomatoid hepatocellular carcinoma and sarcomatoid cholangiocellular carcinoma. Baseline characteristics, tumor characteristics such as tumor pathology, size and number, surgical and adjuvant treatments were reviewed. Tumor recurrence, and patient survival were analyzed with retrospective approach.RESULTSThe enrolled participants included five patients with angiosarcoma and four patients with undifferentiated sarcoma. All patients experienced tumor recurrence at a median of 52 post-operative days. Only two patients survived and the 5-year survival rate was 29.6%. One patient with angiosarcoma who received central hepatectomy for primary tumor and received radiofrequency ablation for recurrent tumor still lives for 11 years. One patient with undifferentiated sarcoma received Rt. lobectomy for primary tumor followed by chemotherapy and radiation therapy still lives around 30 mo even though she got additional operation for recurrent tumor. Two patients who received living donor liver transplantation due to angiosarcoma died. Only adjuvant therapy was associated with survival gain (P = 0.002).CONCLUSIONPatients with primary hepatic sarcoma may gain survival benefit with surgical resection followed by adjuvant therapy, even though the outcome remains relatively poor.     

Angiosarcoma in an area of lipodermatosclerosis

We present an unusual case in a post phlebitic lower limb that had previously developed an area of lipodermatosclerosis. An area within this broke down as a result of an angiosarcoma rather than the more probable: formation of a venous ulcer.     

Primary angiosarcomas of the anterior mediastinum:: A clinicopathologic and immunohistochemical study of 9 cases

We report 9 cases of primary angiosarcomas of the anterior mediastinum. Patient ages ranged from 25 to 62 years (mean, 40.7 years); 5 patients were male and 4 were female. Main presenting symptoms included chest pain, dyspnea, and cough. The tumor size ranged from 3 to 12.5 cm. Macroscopically, the lesions were ill-defined hemorrhagic masses. Histologically, the growth patterns ranged from large vascular spaces to capillary-like proliferations. These were either lined by bland or more pleomorphic endothelial cells. The mitotic activity was variable and corresponded to the degree of differentiation. A rim of thymic tissue was observed in 2 cases suggesting a thymic origin of the tumors. No teratomatous components were identified. Immunohistochemical studies showed that all 9 cases were reactive for vascular markers including factor VIII–related antigen, CD31, and CD34, and negative for cytokeratin CAM5.2. All cases were treated by complete resection and 3 patients received adjuvant chemotherapy. Follow-up information available for 6 patients revealed that 4 were alive and free of disease at intervals ranging from 6 to 36 months after diagnosis and 1 was alive with recurrence at 48 months. One patient had died of the disease 10 months after diagnosis. Primary angiosarcomas of the anterior mediastinum are rare tumors that need to be added to the differential diagnosis of primary anterior mediastinal neoplasms. Despite their histologic similarity to angiosarcomas at other sites, primary angiosarcomas of the anterior mediastinum appear to follow a more protracted clinical course than their counterparts in other organ systems.     

Matrix metalloproteinase-1 expression in splenic angiosarcoma metastasizing to the serous membrane

Angiosarcoma involving the serous membrane may mimic mesothelioma; therefore, the term “pseudomesotheliomatous angiosarcoma” has been suggested for this entity. However, the pathogenesis of pseudomesotheliomatous angiosarcoma remains unclear. Here, we report an autopsy case of splenic angiosarcoma, which systemically metastasized to the serous membrane of both the peritoneum and pleura, closely resembling a mesothelioma. The spindle-shaped tumor cells exhibited marked invasion of the lymphatic vessels and invaded the serous membrane causing thickening of the fibrous tissues like desmoplastic mesothelioma. In the present case, immunohistochemical staining showed that the tumor expressed not only the endothelial cell markers, such as CD31, vascular endothelial growth factor receptor 3, and podoplanin (D2-40), but also matrix metalloproteinase-1 (also known as collagenase-1), which is known to increase the invasiveness of mesothelioma cells. MMP-1 expression was not observed in the other cases of angiosarcoma, examined. This tumor might systemically metastasize to the serous membrane via the lymphatic route and might generate the fibrous stroma aided by the matrix metalloproteinase-1.     

Primary cardiac angiosarcoma: A diagnostic challenge in a young man with recurrent pericardial effusions

Cardiac angiosarcomas are rare, rapidly progressive tumours that often present as diagnostic dilemmas resulting in delayed diagnosis. They should be considered in patients with recurrent pericardial effusions.A 33-year-old man presented for evaluation of a recurrent pericardial effusion. Infectious and rheumatological workups were negative. Pericardial fluid cytology and pericardial biopsy were unremarkable. Imaging, including echocardiogram and magnetic resonance imaging, were nondiagnostic.While awaiting surgical intervention, the patient developed respiratory failure requiring urgent intubation. Intraoperatively, he experienced significant hemorrhage from the myocardium. Hemostasis could not be achieved and the patient expired. Pathology reports revealed metastatic angiosarcoma.The present case illustrates a rare case of primary cardiac angiosarcoma posing a diagnostic dilemma in a young man. The authors present the challenges in diagnosis, and review the most current diagnostic and therapeutic strategies in the care of patients with this condition.     

Gemcitabine in soft tissue or bone sarcoma resistant to standard chemotherapy: a phase II study

Purpose: To assess the efficacy of gemcitabine in patients with a variety of sarcomas that have failed to respond or escaped Adriamycin- and ifosfamide-based chemotherapy. Patients and methods: A group of 18 symptomatic heavily pretreated patients with sarcomas of bone or soft tissue received one induction course of gemcitabine at a dose of 1000 mg/m² per week for 7 consecutive weeks, followed by 1 week rest. Response to the induction course was assessed by interview and by repeated ancillary tests. If no progression was observed, maintenance by gemcitabine 1000 mg/m² per week for 3 weeks every 28 days was given until failure was clinically or radiologically evident. Results: A total of 51 cycles of gemcitabine were given including 18 cycles of induction. A mean of 3.6 postinduction cycles were given to nine patients. The treatment was well tolerated by the patients. One partial response (leiomyosarcoma) and one minimal response (angiosarcoma) were observed, yielding a true objective response rate of 5.5%. An additional six patients achieved stabilization of disease (chondrosarcoma and osteosarcoma), yielding an overall progression-free rate of 44%. The median time to progression was more than 27 weeks. Clinical benefit response was observed only in those who also achieved a progression-free state. Conclusion: Gemcitabine was found to be effective in achieving stabilization and even a minimal response of soft tissue or bone sarcoma refractory to standard chemotherapy. Received: 10 June 1999 / Accepted: 7 September 1999     

Spinal epithelioid hemangioendothelioma with epithelioid angiosarcomatous areas

Epithelioid hemangioendothelioma is a distinctive vascular tumor rarely involving bones. We report a case of epithelioid hemangioendothelioma in the cervical spine with angiosarcomatous areas. A 50-year-old female presented with dizziness while walking. A mixed sclerotic and osteolytic or destructive lesion was radiographically disclosed in her upper cervical vertebrae. Histologically, a laminectomy specimen exhibited areas of ordinary epithelioid hemangioendothelioma together with foci of more atypical epithelioid cell proliferation, closely resembling epithelioid angiosarcoma. This phenomenon has been exceptionally described in cases with skeletal lesions, which are typically of low-grade malignancy.This case was presented at the Closed Meeting of the International Skeletal Society, St. Julians, Malta, October 3–5, 2004     

Angiosarcoma associated with chronic lymphedema (Stewart-Treves syndrome) of the leg: MR imaging

Magnetic resonance (MR) imaging findings of two patients with Stewart-Treves syndrome are presented. MR imaging showed edematous changes in the subcutaneous fat and skin masses that proved to be angiosarcomas. MR signal intensity of the tumor was low compared with fat on T1-weighted images and intermediate and heterogeneous on T2-weighted images. In one patient, administration of intravenous Gd-DTPA showed marked enhancement in the early phase, which persisted until the delayed phase. These finding on dynamic MR imaging may reflect the abundant vascular spaces seen in these tumors. Received: 20 August 1999 Revision requested: 28 October 2000 Revision received: 31 January 2000 Accepted: 8 February 2000     

Radiation-induced angiosarcoma of the breast: case report and self-criticism of therapeutic approach

Angiosarcoma (AS) of the breast is a rare and highly aggressive vascular cancer. It presents as a primitive or radioinduced form. The case of a 46-year-old woman who underwent quadrantectomy of the breast plus axillary lymph node dissection and radiotherapy postoperatively (QUART) for ductal infiltrant carcinoma is reported in the following. Ten years later, the patient underwent mastectomy with immediate reconstruction, for local recurrence that was diagnosed as an AS of the breast at final pathological examination. She did not receive any adjuvant treatment due to local post-operative complications related to breast reconstruction. We criticize our therapeutic approach and we recommend more attention about local recurrence suggesting that tru-cut needle biopsy of local recurrence of the breast after QUART, should be the correct diagnostic approach.