Surgical Management of Retroperitoneal Soft Tissue Sarcomas: Role of Curative Resection

Retroperitoneal sarcomas are a rare group of malignant soft tissue tumors with a generally poor prognosis. However, factors affecting the recurrence and long-term survival are not well understood. The aim of this study was to assess clinical, pathological, and treatment-related factors affecting prognosis in patients with retroperitoneal sarcomas. The hospital records of 107 patients who underwent surgical exploration at our unit for primary or recurrent retroperitoneal sarcomas between 1984 and 2013 were reviewed. Of these patients, 92 had a primary tumor and 15 had a recurrent neoplasm. Study end points included factors affecting overall and recurrence-free survival for the 92 patients with primary disease. Mean follow-up was 79.7 ± 56.3 months. Only the patients undergoing surgery for primary sarcoma were included in this study. Overall 5-year survival was 71 per cent. Disease-free 5-year survival was 65 per cent. Only tumor grade affects overall and disease-free survival. This study confirmed the importance of an aggressive surgical management for retroperitoneal sarcomas to offer these patients the best chance of cure. In our series, only the tumor grade seems to be associated with worse outcome and higher rate of recurrence, regardless of the size of the tumor.

Download Full-text

Outcome Prediction in Primary Resected Retroperitoneal Soft Tissue Sarcoma: Histology-Specific Overall Survival and Disease-Free Survival Nomograms Built on Major Sarcoma Center Data Sets

Journal of Clinical Oncology ◽

10.1200/jco.2012.44.3747 ◽

2013 ◽

Vol 31 (13) ◽

pp. 1649-1655 ◽

Cited By ~ 165

Author(s):

Alessandro Gronchi ◽

Rosalba Miceli ◽

Elizabeth Shurell ◽

Fritz C. Eilber ◽

Frederick R. Eilber ◽

...

Keyword(s):

Overall Survival ◽

Soft Tissue ◽

Cox Model ◽

External Validation ◽

Soft Tissue Sarcomas ◽

Disease Free Survival ◽

Histologic Subtype ◽

Free Survival ◽

Quality Of Surgery ◽

Disease Free

Purpose Integration of numerous prognostic variables not included in the conventional staging of retroperitoneal soft tissue sarcomas (RPS) is essential in providing effective treatment. The purpose of this study was to build a specific nomogram for predicting postoperative overall survival (OS) and disease-free survival (DFS) in patients with primary RPS. Patients and Methods Data registered in three institutional prospective sarcoma databases were used. We included patients with primary localized RPS resected between 1999 and 2009. Univariate (Kaplan and Meier plots) and multivariate (Cox model) analyses were carried out. The a priori chosen prognostic covariates were age, tumor size, grade, histologic subtype, multifocality, quality of surgery, and radiation therapy. External validation was performed by applying the nomograms to the patients of an external cohort. The model's discriminative ability was estimated by means of the bootstrap-corrected Harrell C statistic. Results In all, 523 patients were identified at the three institutions (developing set). At a median follow-up of 45 months (interquartile range, 22 to 72 months), 171 deaths were recorded. Five- and 7-year OS rates were 56.8% (95% CI, 51.4% to 62.6%) and 46.7% (95% CI, 39.9% to 54.6%. Two hundred twenty-one patients had disease recurrence. Five- and 7-year DFS rates were 39.4% (95% CI, 34.5% to 45.0%) and 35.7% (95% CI, 30.3% to 42.1%). The validation set consisted of 135 patients who were identified at the fourth institution for external validation. The bootstrap-corrected Harrell C statistics for OS and DFS were 0.74 and 0.71 in the developing set and 0.68 and 0.69 in the validating set. Conclusion These nomograms accurately predict OS and DFS. They should be used for patient counseling in clinical practice and stratification in clinical trials.

Download Full-text

Sampling Modality Influences the Predictive Value of Grading in Adult Soft Tissue Extremity Sarcomas

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2012-0427-oa ◽

2013 ◽

Vol 137 (12) ◽

pp. 1774-1779 ◽

Cited By ~ 11

Author(s):

Hatim Khoja ◽

Anthony Griffin ◽

Brendan Dickson ◽

Jay Wunder ◽

Peter Ferguson ◽

...

Keyword(s):

Soft Tissue ◽

Core Needle Biopsy ◽

Needle Biopsy ◽

Spindle Cell ◽

Soft Tissue Sarcomas ◽

Disease Free Survival ◽

Incisional Biopsy ◽

Free Survival ◽

Core Needle ◽

Disease Free

Context.—Histologic grade is one of the best predictors of outcome in adult soft tissue sarcomas. Current grading systems were validated on resection specimens; however, there has been a trend toward the use of biopsies to diagnosis these tumors. Objectives.—To determine whether the grade of an extremity soft tissue sarcoma determined on tissue obtained by either core needle biopsy or incisional biopsy is predictive of metastasis- or disease-free survival, and whether either sampling modality is superior. Design.—One hundred three core needle biopsies and 107 incisional biopsies of nonmetastatic spindle cell sarcomas of the extremities were retrieved from the archives. All cases had a minimum 2-year follow-up. Patient data and outcome and tumor characteristics were recorded. Tumors were reviewed and evaluated using the French Federation of Cancer Centers Sarcoma Group grading system. Kaplan-Meier survival curves were generated to correlate tumor grade with metastasis- and disease-free survival for both groups. Results.—Patient and tumor characteristics were similar between groups except that more tumors were grade 3 and superficial in the incisional biopsy group. Grade determined on core needle biopsy was not predictive of either metastasis-free survival (P = .59) or disease-free survival (P = .50). In contrast, grade determined on incisional biopsy was predictive of both metastasis-free survival (P < .001) and disease-free survival (P = .001). Conclusions.—Biopsy, particularly core needle biopsy, represents a convenient diagnostic tool, particularly in the context of neoadjuvant therapy. However, based on these results incisional biopsy is recommended if grading is to be used to predict prognosis in spindle cell soft tissue sarcomas of the extremities.

Download Full-text

Clinical Outcomes of Intraoperative Radiation Therapy for Extremity Sarcomas

Sarcoma ◽

10.1155/srcm/2006/91671 ◽

2006 ◽

Vol 2006 ◽

pp. 1-6 ◽

Cited By ~ 5

Author(s):

Quy N. H. Tran ◽

Anne C. Kim ◽

Alexander R. Gottschalk ◽

William M. Wara ◽

Theodore L. Phillips ◽

...

Keyword(s):

Radiation Therapy ◽

Soft Tissue ◽

Soft Tissue Sarcomas ◽

Disease Free Survival ◽

Intraoperative Radiation Therapy ◽

Intraoperative Radiation ◽

Free Survival ◽

Control Disease ◽

Metastatic Sites ◽

Disease Free

Purpose.Radiation of extremity lesions, a key component of limb-sparing therapy, presents particular challenges, with significant risks of toxicities. We sought to explore the efficacy of intraoperative radiation therapy (IORT) in the treatment of soft tissue sarcomas of the extremities.Patients.Between 1995 and 2001, 17 patients received IORT for soft tissue sarcomas of the extremities. Indications for IORT included recurrent tumors in a previously radiated field or tumors adjacent to critical structures.Results. Gross total resections were achieved in all 17 patients. Two patients experienced locoregional relapses, six patients recurred at metastatic sites, and one patient died without recurrence. Thirty-six month estimates for locoregional control, disease free survival, and overall survival were 86%, 50%, and 78%, respectively. IORT was extremely well tolerated, with no toxicities referable to IORT.Conclusions. For patients with soft tissue sarcomas of the extremities, IORT used as a boost to EBRT provides excellent local control, with limited acute toxicities.

Download Full-text

Adjuvant Treatment of High-risk Adult Soft Tissue Sarcomas: A Survey by the Italian Sarcoma Group

Tumori Journal ◽

10.1177/030089160609200202 ◽

2006 ◽

Vol 92 (2) ◽

pp. 92-97 ◽

Cited By ~ 5

Author(s):

Sergio Frustaci ◽

Massimiliano Berretta ◽

Alessandro Comandone ◽

Ettore Bidoli ◽

Antonino De Paoli ◽

...

Keyword(s):

Overall Survival ◽

High Risk ◽

Soft Tissue ◽

Survival Rates ◽

Soft Tissue Sarcomas ◽

Disease Free Survival ◽

Free Survival ◽

Disease Free ◽

Overall Survival Rates

Aims and Background After the first adjuvant study on adult soft tissue sarcomas was concluded, the participating institutions continued to select and treat patients according to that protocol. The aim of this study was to test the protocol reproducibility when applied as a standard practice. Methods A call for retrospective data was launched in June 1999 (self-referral of consecutive unregistered patients); thereafter, a prospective follow-up was performed. The treatment regimen consisted of epirubicin (60 mg/m2 days 1 and 2), ifosfamide (3 g/m2/die for 3 days) and equimolar doses of 6-mercapto-ethansulfonate (MESNA), with 300 μg G-CSF administered subcutaneously from day +8 until recovery, every 3 weeks for a total of 5 cycles. Results From November 1996 to June 1999, 55 high-risk, adult patients were treated. The average median dose intensity was 89% of the planned program. Grade 3-4 toxicities were leukopenia (49%), thrombocytopenia (14%), transfusion requiring anemia in 7 patients (16%), and alopecia in all patients (100%). After a median follow-up of 70 months, 23 patients (41.8%) relapsed and 19 died. Median disease-free, local disease-free and overall survival rates have not yet been reached. The disease-free survival rates at 2 and 4 years were 73% and 57%, respectively; the corresponding overall survival rates were 91% and 70%, respectively. Conclusions The feasibility and reproducibility of the original protocol were confirmed, since disease-specific overall survival and disease-free survival rates at the same period of observation and with the same prolonged follow-up did not differ.

Download Full-text

Prediction of long-term survival using expression of FOXA1, a determinant of estrogen response domains in breast cancer

Journal of Clinical Oncology ◽

10.1200/jco.2006.24.18_suppl.539 ◽

2006 ◽

Vol 24 (18_suppl) ◽

pp. 539-539

Author(s):

S. Badve ◽

D. Turbin ◽

A. Morimiya ◽

T. Nielsen ◽

C. Perou ◽

...

Keyword(s):

Breast Cancer ◽

Disease Free Survival ◽

Tumor Grade ◽

Nodal Status ◽

Breast Cancers ◽

Luminal A ◽

Term Survival ◽

Free Survival ◽

Disease Free ◽

Foxa1 Expression

539 Background: Recent studies have demonstrated cofactor dependency of estrogen receptor alpha (ERα) in defining specific pattern of estrogen (E2) response in breast cancer. FOXA1, also called HNF3α, a forkhead family transcription factor, has emerged as a major cofactor that is essential for optimum transcription of ∼50% of ERα:E2 responsive genes. FOXA1 is expressed in breast cancer cells and in cDNA microarray cluster analysis segregates with genes that characterize the luminal A subtype such as ERα, GATA3, and XBP-1. Detailed expression analysis of FOXA1 in human breast tumors has not been previously performed and it is not known whether it is an independent prognostic factor in breast cancer. Methods: A tissue microarray comprising tumors from 438 patients with 20 years follow-up was analyzed for FOXA1 expression using goat-anti-human FOXA1 antibody by immunohistochemistry. Percentage (P) and intensity (I) of expression was analyzed to generate numerical score (S= P × I). FOXA1 expression was correlated with tumor grade, nodal status, disease free survival, and expression of ER, PR, GATA3, HER2, p27kip1, phospho-AKT, and luminal A subtype (defined as ER &/or PR+, bcl-2+ and Her-2-neg). Results: FOXA1 expression (score greater than 100) was seen in 187 of 438 breast cancers and correlated with greater likelihood of survival at 20 year (p=0.0114). A significant positive correlation was observed between FOXA1 expression and expression of ERα (p= 0.000001), GATA3 (p= 0.000001), PR (p= 0.00001), phospho-AKT (p= 0.00001). Similarly, a significant correlation was noted with luminal A subtype. An inverse correlation was noted with tumor grade and mdm-2 expression (p=0.00001). HER2 or p27kip1 expression and nodal status showed no correlation with FOXA1. Conclusions: FOXA1 is good prognostic marker predicting disease free survival in patients with breast cancer. Since its expression correlates well with breast cancer of luminal A subtype, it can also be used to identify these good prognosis tumors from the rest of the ER positive breast cancers in archival paraffin embedded breast tissues. No significant financial relationships to disclose.

Download Full-text

What Factors Influence the Outcome of Surgically Treated Soft Tissue Sarcomas of the Hand and Wrist?

Hand ◽

10.1177/1558944716672197 ◽

2016 ◽

Vol 12 (5) ◽

pp. 493-500 ◽

Cited By ~ 4

Author(s):

Matthew T. Houdek ◽

Brian E. Walczak ◽

Benjamin K. Wilke ◽

Sanjeev Kakar ◽

Peter S. Rose ◽

...

Keyword(s):

Overall Survival ◽

Soft Tissue ◽

Local Recurrence ◽

Soft Tissue Sarcomas ◽

Disease Free Survival ◽

Disease Status ◽

Distant Metastases ◽

Factors Affecting ◽

Disease Free

Background: Soft tissue sarcomas (STS) of the hand are exceedingly rare. The aim of this study was to review our institution’s experience with STS of the hand to identify factors affecting outcomes and survivorship. Methods: We retrospectively reviewed the records of 46 hand STS treated with definitive surgery at our institution between 1992 and 2013. Pertinent demographics as well as information regarding the surgical procedure, and disease status at latest follow-up were reviewed. Mean age at diagnosis was 38 years with a mean follow-up of 5 years. Results: The most common tumor subtypes were epithelioid (n = 10) and synovial sarcoma (n = 8). Sixty-one percent were superficial in location. Thirty-three patients had had a nononcologic resection prior to definitive surgical treatment at our institution. Ultimately, negative margins were obtained in all cases. Local recurrence was observed in 5 patients and distant metastases in 14 patients. Tumor sizes ≥2 cm, American Joint Committee on Cancer (AJCC) grade, and depth of the tumor were found to adversely affect the outcome in terms of disease-free and overall survival. Reexcision of an inadvertently excised tumor at an outside institution did not adversely affect the outcome. The 10-year overall and disease-free survival was 72% and 63%. Conclusions: Local recurrence after a wide excision was observed infrequently; however, distant disease was relatively common. Tumors with a size ≥2 cm were associated with a worse disease-free and overall survival, highlighting the aggressive nature of these tumors.

Download Full-text

Predictors of Disease-Free and Overall Survival in Retroperitoneal Sarcomas: A Modern 16-Year Multi-Institutional Study from the United States Sarcoma Collaboration (USSC)

Sarcoma ◽

10.1155/2019/5395131 ◽

2019 ◽

Vol 2019 ◽

pp. 1-8 ◽

Cited By ~ 3

Author(s):

Patrick B. Schwartz ◽

Kara Vande Walle ◽

Emily R. Winslow ◽

Cecilia G. Ethun ◽

Thuy B. Tran ◽

...

Keyword(s):

United States ◽

Overall Survival ◽

Soft Tissue Sarcomas ◽

Disease Free Survival ◽

The United States ◽

Cox Proportional Hazards Model ◽

Study Cohort ◽

Free Survival ◽

Retroperitoneal Sarcomas ◽

Disease Free

Background. Retroperitoneal sarcomas (RPS) comprise approximately 15% of all soft-tissue sarcomas and frequently associated with significant morbidity and as little as 30% 5-year survival. Here, we provide a large, contemporary, and multi-institutional experience to determine which tumor, patient, and treatment characteristics are associated with long-term outcomes in RPS. Methods. 571 patients with primary RPS were identified from the United States Sarcoma Collaboration (USSC). RPS patients who underwent resection from January 2000 to April 2016 were included with patient, tumor, and treatment-specific variables investigated as independent predictors of survival. Survival analyses for disease-free and overall survival were conducted using Kaplan–Meier and Cox proportional hazards model methods. Results. The study cohort was 55% female, with a median age of 58.9 years (IQR: 48.6–70.0). The most common tumor histiotypes were liposarcoma (34%) and leiomyosarcoma (28%). Median follow-up was 30.6 months (IQR: 11.2–60.4). Median disease-free survival was 35.3 months (95% CI: 27.6–43.0), with multivariate predictors of poorer disease-free survival including higher grade tumors, nodal-positive disease, and multivisceral resection. Median overall survival was 81.6 months (95% CI: 66.3–96.8). Multivariate predictors of shorter overall survival included higher grade tumors, nodal-positive and multifocal disease, systemic chemotherapy, and grossly positive margins (R2) following resection. Conclusions. The strongest predictors of disease-free and overall survival are tumor-specific characteristics, while surgical factors are less impactful. Nonsurgical therapies are not associated with improved outcomes despite persistent interest and utilization. Complete macroscopic resection (R0/R1) remains a persistent potentially modifiable risk factor associated with improved overall survival in patients with retroperitoneal sarcomas.

Download Full-text