Retroperitoneal soft tissue sarcomas (original) (raw)
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European Journal of Surgical Oncology (EJSO), 2001
Aim: Ten percent of soft tissue sarcomas (STS) arise in the retroperitoneal tissues. The prognosis for patients with retroperitoneal sarcoma is poor with a 5-year survival rate between 12% and 70%. Stage at presentation, high histological grade, unresectable primary tumour and incomplete resection are associated with a less favourable outcome. Methods: Complete follow-up data were available on 22 patients who underwent surgery for retroperitoneal STS in our institution between 1990 and 2000. Patient, tumour and treatment variables were analysed including use of adjuvant therapy and survival status. Results: Eighteen patients underwent surgery for primary disease, four patients were treated for recurrent disease or metastases. Ten patients presented with pain, seven with an abdominal mass, other presentation included weight loss and haematuria. Thirteen patients presented with tumours larger than 10 cm. The tumours were seven liposarcomas, six leiomyosarcomas, three malignant fibrous histiocytomas, two rhabdomyosarcomas, two malignant schwannomas and two undifferentiated sarcomas. Six primary tumours were completely excised, five patients received radiotherapy and five received chemotherapy. Local recurrence rate was 45% and recurrence-free interval for 10 patients with recurrence was 11 months. Five patients received radiotherapy and five received chemotherapy. The median survival for patients with primary tumours was 36 months, and 5-year survival was 44%. Adjuvant therapy was not associated with higher survival rates. Conclusion: This study re-emphasizes the poor outcome of patients with retroperitoneal STS. Adjuvant radiotherapy and chemotherapy do not appear to be any proven benefit and the single most important prognostic factor is aggressive successful en bloc resection of the primary tumour. Our resection rate and 5-year survival rates are comparable with previous reported UK series although lower than large reports from North American centres. This might partly be explained by difficulty in data collection in a retrospective analysis, but may reflect inadequate subspecialization in UK centres.
Operative Management of Primary Retroperitoneal Sarcomas
Annals of Surgery, 2004
Objective: To review our recent experience with primary retroperitoneal sarcomas, determine prognostic factors for disease recurrence and patient survival, and compare them to our previous results. Background: Medical therapies have shown little efficacy in the management of retroperitoneal sarcomas, making total surgical extirpation the best chance for patient cure. Methods: The case histories of all patients operated upon for retroperitoneal sarcomas between January 1983 and December 1995 were retrospectively reviewed. Results: Ninety-seven patients underwent attempted surgical resection of a primary retroperitoneal sarcoma. There were 54 (56%) men and 43 (44%) women, with a mean age of 59 years. Seventy-six (78%) patients underwent gross total resection, 13 (14%) had residual disease, and 8 (8%) underwent biopsy only with an actuarial 1-year survival of 88%, 51%, and 47%, respectively (P ϭ 0.001). The actuarial 5-and 10-year survivals for patients who underwent gross total resection were 51% and 36%, respectively. Thirty-three patients (43%) developed locoregional recurrence, and 20 patients (26%) developed distant metastases at a median time of 12 months. The cumulative probability at 5 years was 44% for locoregional recurrence and 29% for distant metastases. On univariate analysis, factors associated with improved survival were complete resection of the tumor (P ϭ 0.001), nonmetastatic disease at presentation (P ϭ 0.01), low-grade tumors (P ϭ 0.02), liposarcomas (P ϭ 0.003), and no disease recurrence (P ϭ 0.0001). Contrary to previous reports, the histologic subtype (P ϭ 0.04) was the only significant factor predicting survival on multivariate analysis. Conclusions: Compared with our earlier experience, the rates of complete resection and overall survival have improved. Local control continues to be a significant problem in the management of retroperitoneal sarcomas. Because new surgical options for this problem are limited, further outcome improvement requires novel adjuvant therapies.
Challenges in the Surgical Treatment of Retroperitoneal Sarcomas
Indian Journal of Surgery, 2015
The objective of this study is to assess long-term prognosis and patterns of failure in patients with retroperitoneal sarcoma who underwent surgery with curative intent at a single institution. We also provide a thorough review of the literature including several series and widely variable data regarding local and distant failure. During a 25-year period , 45 consecutive patients who underwent surgery for retroperitoneal sarcoma at a single referral center were reviewed retrospectively. We recorded and analyzed the presenting symptoms, type of surgical excision, and the 5-year survival rate as well as the local recurrence rate of patients with complete tumor resection. Overall survival is significantly diminished in patients in whom resection margins are not adequate. In our series, 5-year recurrence rate for patients with complete and incomplete resection of the tumor was 30.76 and 68.42 %, respectively. The 5-year survival rate was 51.12 %, and the 5-year survival rate according to type of resection was 76.93 % for complete and 15.79 % for incomplete resections. Local recurrence after first surgery and high-grade malignancy are associated with poor survival. The role of radiation therapy and chemotherapy in the treatment of retroperitoneal sarcoma (RPS) should be investigated further. Complete en bloc resection of the tumor mass and adjacent structures is the single most important factor that improves survival.
Outcomes in a series of 103 retroperitoneal sarcomas
European Journal of Surgical Oncology (EJSO), 2006
To report the effect on outcome of selection in patients receiving intra-operative electron beam radiation (IOERT) and external beam radiation therapy (EBRT). Methods: One hundred and three patients treated for primary RS were studied. Median follow-up was 27 months. Clinical presentation, tumor characteristics, and treatment methods were analyzed to determine impact on survival and recurrence and if selection was occurring. Results: Mean age was 55 AE 17 years. Mean tumor size was 15 AE 6 cm and 88 were high-grade. Complete gross tumor resection (CR) occurred in 62 patients and improved survival vs. both debulking ( p ¼ 0.0005) and biopsy ( p < 0.0001). The 5-and 10-year survival rates were 62% and 52% for those with CR vs. 29% and 20% after incomplete resection. Among the 62 CR patients, there was selection to receive additional EBRT AE IOERT in patients with high-grade tumors ( p ¼ 0.005) and/or microscopically positive margins ( p ¼ 0.011). In these high-risk patients there was a trend for IOERT to further augment survival vs. EBRT alone and to increase the time to both local and distant recurrences ( p ¼ 0.036). Conclusions: Complete gross resection is the primary form of curative treatment for retroperitoneal sarcomas. Selection led to patients with high-risk tumors receiving additional radiation therapy. There appears to be a beneficial effect of IOERT plus EBRT in these high-risk patients after complete tumor resection.
Current diagnosis and management of retroperitoneal sarcoma
Cancer control : journal of the Moffitt Cancer Center, 2011
Retroperitoneal sarcomas are rare neoplasms that often present with multivisceral involvement. Treatment for these tumors requires careful decision making requiring a combination of surgery, chemotherapy, and radiation therapy. We reviewed the scientific literature pertaining to the diagnosis and management of retroperitoneal sarcomas. We also identify recent developments in treatment and discuss future trends in the care of patients with this disease. Retroperitoneal tumors often present as large, locally advanced lesions. Evaluation of these tumors requires careful consideration of a multimodality approach. Retrospective data and historical prospective series have demonstrated the survival benefit of radical resection for these tumors with en bloc resection of involved structures. Compartmental resections in the retroperitoneum along with debulking of high-grade disease and regional therapy are controversial approaches with significant morbidity that can lead to long-term survival...
Surgical management of primary retroperitoneal sarcoma
British Journal of Surgery, 2010
Background Local recurrence after surgical resection is the main cause of disease-related mortality in patients with primary retroperitoneal sarcoma (RPS). This study analysed predictors of local recurrence and disease-specific survival. Methods A prospective database was reviewed to identify patients who underwent surgery for primary RPS between 1990 and 2009. Patient demographics, operative outcomes and tumour variables were correlated with local recurrence and disease-specific survival. Multivariable analysis was performed to evaluate predictors for local recurrence and disease-free survival. Results Macroscopic clearance was achieved in 170 of 200 patients. The median weight of tumours was 4·0 kg and median maximum diameter 27 cm. Resection of adjacent organs was required in 126 patients. The postoperative mortality rate was 3·0 per cent. Seventy-five patients developed local recurrence during follow-up. At 5 years the local recurrence-free survival rate was 54·6 per cent and th...
The Value of Surgery for Retroperitoneal Sarcoma
Sarcoma, 2009
Introduction. Retroperitoneal sarcomas are uncommon large malignant tumors. Methods. Forty-one consecutive patients with localized retroperitoneal sarcoma were retrospectively studied. Results. Median age was 58 years (range 20-91 years). Median tumor size was 17.5 cm (range 4-41 cm). Only 2 tumors were <5 cm. Most were liposarcoma (44%) and high-grade (59%). 59% were stage 3 and the rest was stage 1. Median followup was 10 months (range 1-106 months). Thirty-eight patients had an initial complete resection; 15 (37%) developed recurrent sarcoma and 12 (80%) had a second complete resection. Patients with an initial complete resection had a 5-year survival of 46%. For all patients, tumor grade affected overall survival (P = .006). Complete surgical resection improved overall survival for high-grade tumors (P = .03). Conclusions. Tumor grade/stage and complete surgical resection for high-grade tumors are important prognostic variables. Radiation therapy or chemotherapy had no significant impact on overall or recurrence-free survival. Complete surgical resection is the treatment of choice for patients with initial and locally recurrent retroperitoneal sarcoma.
Patient outcome after complete surgery for retroperitoneal sarcoma
Anticancer research, 2013
Aggressive surgery has been suggested for improving local tumor control in patients with retroperitoneal sarcoma (RS). This study aimed at investigating local disease-free and overall survival after complete surgery, in patients with RS. Retrospective data of patients submitted to complete surgery for RS were extracted from a prospectively-maintained database. Forty-three out of 78 patients (55%) presented with primary RS. Infiltrated organs were resected in 42 patients (54%). Patients presenting with recurrent (hazard ratio (HR)=5.57, p=0.002) and high-grade (HR 3.47, p=0.041) tumors were at higher risk of local recurrence. Microscopically-involved tumor resection margins (HR=3.47, p=0.04) and recurrent tumor at presentation (HR=2.49, p=0.008) were independent predictors of poor survival. Patients presenting with primary RS had longer local disease-free survival and overall survival than those with recurrent tumor after complete surgery. Complete surgery remains the standard-of-car...
Indian Journal of Surgical Oncology, 2020
Retroperitoneal sarcomas (RPS) are rare heterogeneous tumors arising in the retroperitoneum with unique biological and behavioral patterns that are thought to be closely linked to histology. The aim of the study was to audit our results and analyze various clinico-pathological factors including surgical excision, histology, and their implications on the recurrences and survival outcomes in RPS. Retrospective analysis of patients treated at a tertiary referral center in India from March 2008 to July 2017 was performed. The clinico-pathological variables were analyzed for their association with tumor recurrence and survival with special emphasis on histological subtype. The primary outcome was overall survival (OS). One hundred consecutive patients operated for RPS were analyzed. Of these, 27 were operated for recurrent tumors. Liposarcomas (LPS) and leiomyosarcomas (LMS) constituted 50% (n = 50) and 30% (n = 30) of patients respectively. Complete tumor excision was achieved in 83%, with 43% patients undergoing adjacent organ resection. At a median follow-up of 25.3 months, the median disease-free survival (DFS) and overall survival (OS) were 30 months and 87.8 months respectively. On multivariate analysis, tumor grade was the only factor to significantly affect survival (p = 0.001 for DFS and 0.005 for OS). There was no difference in survival outcomes between infiltrative and adhesive tumors with respect to adjacent organ invasion (p = 0.361 for OS). Tumor grade remains an important prognostic factor affecting disease-free and overall survival in retroperitoneal sarcomas irrespective of tumor size, site, and histology.
Retroperitoneal sarcomas: The yale experience and a review of the literature
Journal of Surgical Oncology, 1986
Between 1964 and 1978, 23 patients were referred for radiation therapy for retroperitoneal sarcoma. Three patients had complete excision and negative resection margins and all survived for 5 years without recurrent disease. One patient had complete resection but positive margins, but had a local recurrence 3 1/2 years after radiation. One patient had partial excision with gross residual, and this patient survived only 17 months after an incomplete radiation treatment schedule. Ten cases had biopsy only and were then referred. We found that there were only four cases that survived longer than 1 year, and their average dose of radiation was 4,400 rads, as compared to only 2,691 rads for the remaining six. We conclude that for incompletely resected or only biopsied retroperitoneal sarcoma, doses of 4,000-5,000 rads should be given and then a re-evaluation for complete resection should be done with CT scan and re-exploration. For completely resected cases, we feel that postoperative radiation adds to the local control. We have reviewed the literature and presented the data in support of our conclusions.