Mark Fairweather

Variability in Patterns of Recurrence After Resection of Primary Retroperitoneal Sarcoma (RPS): A Report on 1007 Patients From the Multi-institutional Collaborative RPS Working Group

Background:Retroperitoneal sarcomas (RPS) are rare tumors composed of several well defined histologic subtypes. The aim of this study was to analyze patterns of recurrence and treatment variations in a large population of patients, treated at reference centers. Methods:All consecutive patients with primary RPS treated at 6 European and 2 North American institutions between January 2002 and December 2011 were included. Five, 8, and 10-year overall survival (OS) and crude cumulative incidence (CCI) of local recurrence (LR) and distant metastasis (DM) were calculated. Multivariate analyses for OS, CCI of LR, and DM were performed. Results:In all, 1007 patients were included. Median follow-up was 58 months (first and third quartile range 36–90). The 5, 8, and 10-year OS were 67% [95% confidence interval (CI), 63, 70), 56% (95% CI, 52, 61), and 46% (95% CI, 40, 53). The 5, 8, and 10-year CCI of LR and DM were 25.9 (95% CI, 23.1, 29.1), 31.3 (95% CI, 27.8, 35.1), 35% (95% CI, 30.5, 40.1), and 21% (95% CI, 18.4, 23.8%), 21.6 (95% CI, 19.0, 24.6), and 21.6 (95% CI, 19.0, 24.6), respectively. Tumour size, histologic subtype, malignancy grade, multifocality, and completeness of resection were significant predictors of outcome. Patterns of recurrence varied depending on histologic subtype. Different treatment policies at participating institutions influenced LR of well differentiated liposarcoma without impacting OS, whereas discrepancies in adjuvant systemic therapies did not impact LR, DM, or OS of leiomyosarcoma. Conclusions:Reference centers are critical to outcomes of RPS patients, as the management strategy requires specific expertise. Histologic subtype predicts patterns of recurrence and should inform management decision. A prospective international registry is under preparation, to further define our understanding of this disease.

Post‐relapse outcomes after primary extended resection of retroperitoneal sarcoma: A report from the Trans‐Atlantic RPS Working Group

Despite a radical surgical approach to primary retroperitoneal sarcoma (RPS), many patients experience locoregional and/or distant recurrence. The objective of this study was to analyze post‐relapse outcomes for patients with RPS who had initially undergone surgical resection of their primary tumor at a specialist center.

External validation of a multi-institutional retroperitoneal sarcoma nomogram

A multi‐institutional nomogram for predicting disease‐free survival (DFS) and overall survival (OS) in patients with primary retroperitoneal sarcoma (RPS) incorporating relevant prognostic factors not included in the American Joint Committee on Cancer staging system for soft tissue sarcoma has been reported. The authors validated this nomogram with an independent, transatlantic cohort.

Accuracy of EUS and CT imaging in preoperative gastric cancer staging

Neoadjuvant therapy is recommended for locally advanced gastric cancer patients (stage IB–IIIC). The objective of this study is to evaluate the accuracy of endoscopic ultrasound (EUS) and computed tomography (CT) in identifying patients with locally advanced gastric cancer.

Postoperative Morbidity After Radical Resection of Primary Retroperitoneal Sarcoma: A Report From the Transatlantic RPS Working Group

Andrea J. MacNeill, MD, y Alessandro Gronchi, MD,y Rosalba Miceli, PhD,z Sylvie Bonvalot, MD, PhD,§ Carol J. Swallow, MD, PhD, Peter Hohenberger, MD, Frits Van Coevorden, MD,jj Piotr Rutkowski, MD, Dario Callegaro, MD,y Andrew J. Hayes, MD, PhD,yy Charles Honoré, MD,zz Mark Fairweather, MD,§§ Amanda Cannell, BSc, Jens Jakob, MD, Rick L. Haas, MD,jjjj Milena Szacht, MD, Marco Fiore, MD,y Paolo G. Casali, MD, Raphael E. Pollock, MD, PhD,yyy Francesco Barretta, PhD,z Chandrajit P. Raut, MD, MSc,§§ and Dirk C. Strauss, MDyy

Current principles of surgery for retroperitoneal sarcomas

Surgery for primary retroperitoneal sarcomas (RPS) often requires a technically challenging, en bloc multivisceral resection to optimize outcomes. Surgery may also be appropriate for patients with localized recurrent RPS. Anatomic considerations and tumor biology driven by histologic subtype may guide the extent of resection in patients with RPS. This review provides an overview of the current surgical principles for primary and recurrent RPS.

Cutaneous Radiation-associated Breast Angiosarcoma: Radicality of Surgery Impacts Survival

Objective: Cutaneous radiation therapy (RT)-associated breast angiosarcoma (AS) is a rare consequence of breast RT associated with poor outcomes. Previous small case series have documented high recurrence rates and poor survival. We reviewed our experience and focused on the impact of conservative versus radical resections. Methods: Data for patients with RT-associated breast AS evaluated at our institution from 1993 to 2015 who underwent surgery were reviewed. Results: Seventy-six women were diagnosed with RT-associated breast AS at a median 85 months from surgery for invasive breast carcinoma or ductal carcinoma in situ. Thirty-eight underwent mastectomy/wide excision with partial skin resection (“conservative”) and 38 underwent resection of all or nearly all previously irradiated skin plus mastectomy (“radical”). The radical group (vs the conservative group) more often had multifocal disease (80% vs 56%, P = 0.04), chemotherapy for AS (58% vs 22%, P < 0.01), margin-negative resection (100% vs 73%, P < 0.01), reconstructive surgery (100% vs 13%, P < 0.01), and re-operation (16% vs 3%, P = 0.04). Five-year crude cumulative incidences of local recurrence and distant metastasis for radical versus conservative groups were 23% versus 76% (P < 0.01) and 18% versus 47% (P = 0.02), respectively. Five-year disease-specific survival (DSS) for radical versus conservative groups was 86% versus 46% (P < 0.01), respectively. On multivariable analysis, age, radicality of surgery, and margin were predictive of DSS. Conclusions: For patients with RT-associated breast AS, radical resection was associated with reduced recurrence rates and improved DSS. Although margin was predictive of DSS, multifocality calls into question the reliability of negative margin assessment.

Surgical management of biliary tract cancers

Biliary tract cancers (BTC) are aggressive gastrointestinal malignancies that are associated with a poor prognosis. This rare group of tumors includes cancers of the gallbladder, intrahepatic, and extrahepatic biliary tree. Chronic inflammatory processes such as cholelithiasis and chronic bacterial infection of the biliary system remain the most common underlying risk factor although most cases occur sporadically. The majority of patients present with advanced disease accompanied by nonspecific symptoms, making BTCs a therapeutic challenge for clinicians. In patients with localized, resectable disease, the only potentially curative treatment is a complete resection with negative microscopic margins. Liver transplantation has been used in selected patients with peri-hilar cholangiocarcinoma, with acceptable results. Recurrence rates remain high despite surgical treatment and 5-year survival rates range from 8-40% in patients treated with resection. In this review, we will summarize the current surgical management strategies for BTCs, focusing on the role of resection.

Acute gastrointestinal toxicity and bowel bag dose-volume parameters for preoperative radiation therapy for retroperitoneal sarcoma

PURPOSE Acute gastrointestinal (GI) toxicity has been studied in GI and gynecological (GYN) cancers, with volume receiving 15 Gy (V15) <830 mL, V25 <650 mL, and V45 <195 mL identified as dose constraints for the peritoneal space (bowel bag [BB]). There are no reported constraints derived from retroperitoneal sarcoma (RPS), and prospective trials for RPS have adopted some of the GI and GYN constraints. This study quantified GI toxicity during preoperative radiation therapy (RT) for RPS, assessed toxicity using published constraints, and evaluated predictors for toxicity. METHODS AND MATERIALS From 2003 to 2013, 56 patients with RPS underwent preoperative RT at 2 institutions. Toxicity was scored using Radiation Therapy Oncology Group criteria for upper and lower acute GI toxicity. BB was contoured on planning computed tomography scans per Radiation Therapy Oncology Group atlas guidelines with review by a radiologist. Relationships among toxicity, clinical factors, and BB dose were analyzed. RESULTS Three patients (5%) developed grade ≥3 acute GI toxicity: 2 grade 3 toxicities (anorexia and nausea) and 1 grade 5 toxicity (tumor-bowel fistula). Thirty-six patients (64%) had grade 2 toxicity (nausea, 55%; diarrhea, 23%; pain, 20%). Tumor size was the only significant clinical predictor of grade ≥2 acute GI toxicity. Larger mean BB volumes predicted for grade ≥2 toxicity (P = .001). On receiver operating characteristics analysis, V30 was the best discriminator for toxicity (P = .0001). Median BB V15 was 1375 mL; 75% of patients had V15 ≥830 mL. Median V25 was 1083 mL; 68% had V25 ≥650 mL. Median V45 was 575 mL; 82% had V45 ≥195 mL. V25 ≥650 mL was significantly associated with grade ≥2 toxicity (P = .01). CONCLUSIONS Among patients treated with preoperative RT for RPS, significant acute GI toxicity was very low despite BB dose exceeding established constraints for most cases. Acceptable dose constraints for RPS may be higher than those for GI or GYN cancers. Further assessment of dose-volume constraints for RPS is needed.

Surgical management of GIST and intra-abdominal visceral leiomyosarcomas

Intra‐abdominal visceral sarcomas are a rare group of mesenchymal tumors that require the use of distinct surgical principles and a multidisciplinary approach in order to optimize treatment. Surgery remains the only potentially curative treatment. Management and outcomes vary for the different sarcomas arising from viscera. This review will summarize the current treatment strategies for the two most common visceral sarcomas, leiomyosarcomas, and gastrointestinal stromal tumors. J. Surg. Oncol. 2015 111:562–569.