Resection Of Retroperitoneal Sarcoma Research Articles

Compartment Surgery in Treatment Strategies for Retroperitoneal Sarcomas: A Single‐center Experience

Retroperitoneal sarcomas (RPS) are rare tumors and radical surgery is still the only curative treatment. We aim to estimate postoperative morbidity and mortality and identify significant prognostic factors for survival of patients with RPS and then evaluate the effect of en bloc resection on survival. This was a retrospective follow-up study of 91 patients with RPS who underwent surgery at the Section of Abdominopelvic Surgery of the National Cancer Institute-Brazil (INCA) between June 1992 and January 2008. Overall and 2-, 5-, and 10-year disease-free survival rates were calculated and univariate and Cox multivariate analyses were performed. The most common complaints were abdominal pain and mass. Resection was possible in 83.5% and curative resection in 55.3%. Six patients died within the postoperative period (mortality=6.6%) and 28 had complications (30.8%). Leiomyosarcomas and liposarcomas predominated, as well as G3. The median tumor diameter was 20.5cm. There were 124 organs resected in association, with only 42 proven invaded. The 5-year overall survival and disease-free survival rates were 32.0 and 36.8%, respectively. Cell differentiation, curative or palliative resection, blood transfusion, and re-resection were significant variables. Compartment surgery had no impact on survival, but it increased the duration of surgery, the need for blood transfusion, and overall morbidity. This study suggests that early diagnosis and curative resection of retroperitoneal sarcomas can improve long-term survival. Adjacent organs with evidence of direct invasion must be removed en bloc; others should be spared.

Effect of radiation therapy on survival of patients with surgically resected retroperitoneal sarcoma: A population-based matched pair study.

10082 Background: Although the only curative treatment for retroperitoneal sarcoma (RPS) is surgical resection, radiation therapy is frequently added. Currently no prospective randomized trial has measured the efficacy of neoadjuvant or adjuvant radiation therapy in patients with resected RPS. The purpose of this study was to determine the effect of radiation therapy on overall survival between propensity score-matched, surgically-resected RPS patients using the Surveillance, Epidemiology, and End Results (SEER) database. Methods: The study population consisted of SEER patients with histologically-confirmed RPS between the years of 1973 and 2006. Only patients who were coded with surgical resection were included. Exclusion criteria included multiple malignancies, distant metastasis, and unknown grade or stage. Univariate and multivariate logistic regression was used to identify covariates associated with radiation therapy. Cox regression analysis was used to determine associations of covariates with overall survival. Finally, propensity score methods were used to perform a survival analysis in patients who underwent surgery and radiation therapy matched with control patients who underwent surgery alone. Results: Prior to matching there were 762 patients (558 surgery only, 204 with radiation therapy). Factors independently associated with radiation therapy by multivariate analysis were: age (p=0.037), geographic region (p=0.041), grade (p=0.047), stage (p=0.003), and surgery type (p=0.01). Cox regression analysis demonstrated that age and tumor grade were independently associated with survival. Propensity scoring resulted in 317 pairs of patients who had surgery and radiation therapy matched with patients who had surgery alone. Survival analysis using both Kaplan Meier and matched pair analyses demonstrated no difference between patients receiving radiation therapy and those who did not (p=0.22). Conclusions: At present, patients with surgically-resected RPS entered into the SEER database who have received radiation therapy have not demonstrated a survival benefit as compared to surgery alone. No significant financial relationships to disclose.

Proton-Beam, Intensity-Modulated, and/or Intraoperative Electron Radiation Therapy Combined with Aggressive Anterior Surgical Resection for Retroperitoneal Sarcomas

We sought to reduce local recurrence for retroperitoneal sarcomas by using a coordinated strategy of advanced radiation techniques and aggressive en-bloc surgical resection. Proton-beam radiation therapy (PBRT) and/or intensity-modulated radiation therapy (IMRT) were delivered to improve tumor target coverage and spare selected adjacent organs. Surgical resection of tumor and adjacent organs was performed to obtain a disease-free anterior margin. Intraoperative electron radiation therapy (IOERT) was delivered to any close posterior margin. Twenty patients had primary tumors and eight had recurrent tumors. Tumors were large (median size 9.75 cm), primarily liposarcomas and leiomyosarcomas (71%), and were mostly of intermediate or high grade (81%). PBRT and/or IMRT were delivered to all patients, preferably preoperatively (75%), to a median dose of 50 Gy. Surgical resection included up to five adjacent organs, most commonly the colon (n = 7) and kidney (n = 7). Margins were positive for disease, usually posteriorly, in 15 patients (54%). IOERT was delivered to the posterior margin in 12 patients (43%) to a median dose of 11 Gy. Surgical complications occurred in eight patients (28.6%), and radiation-related complications occurred in four patients (14%). After a median follow-up of 33 months, only two patients (10%) with primary disease experienced local recurrence, while three patients (37.5%) with recurrent disease experienced local recurrence. Aggressive resection of retroperitoneal sarcomas can achieve a disease-negative anterior margin. PBRT and/or IMRT with IOERT may possibly deliver sufficient radiation dose to the posterior margin to control microscopic residual disease. This strategy may minimize radiation-related morbidity and reduce local recurrence, especially in patients with primary disease.

Predictors of Survival After Resection of Retroperitoneal Sarcoma

To identify predictors of survival after resection of retroperitoneal sarcoma (RPS) and to evaluate the performance of the American Joint Committee on Cancer (AJCC) staging system for RPS. Previous studies of survival after RPS resection are restricted to at most several institutions, yet the current AJCC staging system for RPS is based entirely on these relatively small studies. Patients undergoing resection of primary RPS from 1988 to 2005 were identified from the Surveillance, Epidemiology, and End Results (SEER) database. Cox proportional hazards models were used to analyze survival and evaluate AJCC staging. In 1365 patient undergoing resection of primary RPS, the most prevalent histologies were liposarcoma (50%), leiomyosarcoma (26%), and malignant fibrous histiocytoma (11%). Median, 5-year, and 10-year survival after resection were 55 months, 47%, and 27%. Histological subtype (P < 0.001), histological grade (grade 3-4 vs. grade 1; HR, 2.42; P < 0.001), and tumor invasion of adjacent structures (HR, 1.37; P < 0.001) were associated with survival on multivariable analysis. However, tumor size had no prognostic value. Consequently, the AJCC T classification system demonstrated poor discriminatory ability (c = 0.50). The AJCC stage grouping system demonstrated moderate discriminatory ability (c = 0.66) but performed no better than a much simpler system that omits information on tumor size and lymph node metastasis (c = 0.67). Indicators of tumor aggressiveness (histological grade and invasion of adjacent structures) as well as histological subtype predict survival after RPS resection. Tumor size, however, does not impact survival. The AJCC staging system for RPS is in need of revision.

Complex diaphragm reconstruction using dermal collagen matrix after multivisceral resection of retroperitoneal sarcoma

Complex diaphragm reconstruction using dermal collagen matrix after multivisceral resection of retroperitoneal sarcoma

Bridging circuit for the resection of retroperitoneal sarcoma involving the aorta and the IVC - veno-venous to veno-arterial perfusion

A 50-year-old female underwent surgery for removal of a massive retroperitoneal sarcoma (RPS) involving the right hepatic lobe and the inferior vena cava (IVC), abdominal aorta, right lung, right hemi-diaphragm and pericardium. Resection of the RPS necessitated cross-clamping of the abdominal aorta, IVC and the hepatic artery. Cross-clamp time cannot be predicted prior to tumour resection and vascular re-construction. To prevent complications of prolonged cross-clamp time and distal hypo-perfusion, circulatory support was sought to facilitate the procedure. A perfusion circuit was designed to accommodate an easy and immediate redirection of blood flow from venovenous bypass (VVB) to veno-arterial bypass (VAB) without requiring a change of circuit and with minimum heparin administration. Furthermore, this circuit provides the added safety of an oxygenator and a heat-exchanger. Utilising the circuit enabled successful resection of the RPS. The patient was discharged from the intensive care unit (ICU) seven days later without any post-operative complications. This case report of a design of a perfusion circuit for the resection of RPS made use of a perfusion approach that had not previously been described and allowed for a reduction in the duration of ischaemic time and retroperitoneal bleeding.

Prognostic factors of retroperitoneal soft tissue sarcomas: analysis of 132 cases

Retroperitoneal sarcoma is a rare disease with poor prognosis. The aim of this study was to investigate the prognostic factors of the disease. Between January 1988 and December 2003, 132 patients with retroperitoneal sarcoma were surgically treated in our hospital, 79 of them were followed up for 1 - 122 months (median: 19 months). Their clinicopathological data including tumor size, histological subtype, grade and resection margin status, were studied. The Kaplan-Meier method and log-rank test were used to analyze the disease-specific survival rates after the resection. Among the 132 patients, 98 (74.2%) received macroscopic complete resection, 29 (22.0%) incomplete resection, and 5 (3.8%) surgical biopsy. In the 79 patients who were followed up, macroscopic clear resection of retroperitoneal sarcoma (n = 49) was associated with a significantly higher survival rate compared with unclear resection (n = 30, P < 0.001). The median survival period was 31 months (95% CI, 20.09 - 41.91; actuarial 1-year survival, 85.7%) in the patients with the tumor completely resected and 11 months (95% CI, 6.71 - 15.29; actuarial 1-year survival, 46.7%) in those with incomplete resection. Patients with high-grade sarcomas had a significantly shorter survival time (n = 39; median: 24, 95% CI: 5.71 - 42.29) than those with low-grade sarcomas (n = 40; median: 15; 95% CI: 8.80 - 21.20; P < 0.01). Moreover, compared with the patients with the tumor sized < 15 cm in diameter (n = 53), the survival rate was lower in those with a sarcoma sized > 15 cm (n = 26). (Median: 12, 95% CI: 8.26 - 15.74 vs median: 24, 95% CI: 17.25 - 30.75; P < 0.05). Furthermore, the survival of the patients with liposarcomas (n = 29, median: 29, 95% CI: 12.84 - 45.16), leiomyosarcomas (n = 14, median: 11, 95% CI: 6.11 - 15.89), and others (n = 36, median: 22, 95% CI: 14.95 - 29.05) varied significantly (P < 0.05). Completeness of resection, tumor volume, grade, and subtype are prognostic factors of retroperitoneal soft tissue sarcomas.

Retroperitoneal sarcomas in children: outcomes from an institution

Background Retroperitoneal sarcomas are uncommon in children and tend to present as large tumors with advanced locoregional involvement of abdominopelvic structures. Surgical control of these tumors remains to be a management challenge. We reviewed our institutional experience with retroperitoneal sarcomas in children. Materials and Methods In a retrospective review of charts dating between 1975 and 2005, we identified patients younger than 18 years who were diagnosed with a histologically confirmed retroperitoneal sarcoma. Patients were followed prospectively through clinic visits and mail correspondence. Standard statistical methods were used for comparative, risk, and survival analyses. Results Twenty-one children with a mean age at presentation of 9 ± 1 years were identified. There were more boys than girls (male/female ratio = 1.6). The most common presentations were abdominal mass/distention (76%) and pain (62%). The mean tumor size was 14.2 ± 1.4 cm, with locoregional involvement in 62% of the patients. The common histologic types were rhabdomyosarcoma (33%) and fibrosarcoma (33%). Seventy-six percent of the patients underwent primary or secondary resection, 71% received neoadjuvant and/or adjuvant chemotherapy therapy, and 38% received radiation therapy. Complete resection was achieved in 48% of the patients, including 3 who required inferior vena cava resection and reconstruction. The 5-year disease-specific survival rates for patients who underwent complete resection and those who underwent incomplete resection were 90% and 36% ( P = .018), respectively. For all patients, the mean survival time was 103 ± 16 months and the 5-year disease-specific survival rate was 62%. Survival was significantly better for patients with low-grade sarcomas than for those with high-grade sarcomas (90% vs 36%, P = .008). Among those who underwent an initial complete resection, 50% had a recurrence at a mean time of 88 ± 52 months (range = 3-261 months). The 30-day postoperative mortality and morbidity rates were 0% and 24%, respectively; in addition, 14% of the patients experienced long-term complications. Conclusions Resection of retroperitoneal sarcomas can be performed safely with minimal morbidity and mortality. Complete resection and low histologic grade are associated with significantly better disease-specific survival.

Complications Associated with Neoadjuvant Radiotherapy in the Multidisciplinary Treatment of Retroperitoneal Sarcomas

Retroperitoneal sarcomas (RPS) remain a therapeutic challenge due to high local recurrence rates. Preoperative RT offers theoretical advantages in the multidisciplinary care of RPS. The purpose of our study was to evaluate our experience using preoperative radiotherapy (PRT) in the treatment of RPS. This is a single-institution review of patients with RPS treated with PRT from 1994 until 2004. Three radiation oncologists and four surgical oncologists were involved. Medical records, tumor registries, and death records were reviewed. Fourteen patients were included; nine were treated for primary presentation and five for recurrent disease. Histologic grade was grade I (n = 3), grade II (n = 3), and grade III (n = 8). Five patients received additional IORT. Radiotherapy complications were generally mild, including nausea (n = 3), diarrhea (n = 1), dehydration (n = 1), anemia (n = 1), and skin changes (n = 1); one required early cessation due to nausea. Thirteen patients had gross negative margins; while 7/13 had negative microscopic margins. Operative complications included anastomotic bleeding (n = 1), fluid collections (n = 2), ileus (n = 3), ascites (n = 2), temporary leg weakness (n = 1), and uncomplicated wound infections (n = 2). In patients with R0 or R1 resections, one and two year local control rates were 64 and 50%. Overall survival for all patients was 90% at 1 year and 74% at 2 years with median survival of 21 months. PRT and IORT can be administered effectively in carefully selected patients with resectable RPS. Larger multi-center studies are needed to delineate the role of PRT and IORT to improve local recurrence and survival rates in the treatment of RPS.

Retroperitoneale Sarkome: Diagnostik und Therapie

Due to the fact that there are no distinct anatomical compartments, retroperitoneal sarcomas are moreover diagnosed with evidence of large tumors and infiltration of adjacent organs. In spite of improvement of the diagnostic facilities and surgical techniques, quite frequently local recurrences with unfavourable prognosis turn up even after complete removal. It was the aim of this study to analyze diagnosis, therapy and long-term prognosis in patients with retroperitoneal sarcomas over a period of 10 years. Between January 1995 and January 2005, 379 patients underwent surgery for a primary retroperitoneal tumor at our clinic. Among the 67 (17.1 %) malignant lesions, a sarcoma was found in 35 patients. The present study is focused on the long-term prognosis of those 21 patients with a primary resected retroperitoneal sarcoma, recurrent sarcomas and exploratory laparotomies excluded. The median patient age at the time of surgery was 61 (25-86) years, 57.1 % were males. The duration of symptoms was 3 (1-36) months. A pR0-resection was achieved in 20/21 patients. Among the histopathological tumor types, liposarcomas (n = 10) and leiomyosarcomas (n = 6) were found most frequently. Local recurrence developed in 12 out of 21 patients at 13.5 (5-42) months after the first operation, and in 4 patients distant metastases were observed. The long-term survival of all 21 patients with primary resected retroperitoneal sarcoma was 24 (1-101) months. A more favourable prognosis was seen in patients with leiomyosarcoma as compared with liposarcoma. Retroperitoneal sarcomas are a heterogeneous entity, and they were found among our own patients in 6.1 % of all primary operatively treated retroperitoneal tumors. The strategy of treatment is complex and dependent on the stage, localization and histopathological characteristics. Long-term survival is limited as a result of the high rate of local recurrence.

Internal packing in the control of hemorrhage from large retroperitoneal veins

Background Hemorrhage from large retroperitoneal veins is usually controlled by suturing the venous tear. Infrequently, the extent and location of the tear and amount of hemorrhage preclude successful suturing. Methods In seven patients with severe hemorrhage from large retroperitoneal veins encountered in association with resection of retroperitoneal sarcoma (6) or repair of a ruptured abdominal aortic aneurysm (1), packing of the area with sufficient amounts of Surgicel (Ethicon, Johnson & Johnson, Somerville, NJ) and pressure for one half hour was used. Results All seven patients did not show any bleeding postoperatively and no clinical sequelae developed, with the exception of one patient who developed an abscess requiring drainage. Conclusion Internal packing with Surgicel appears to be reliable in controlling venous hemorrhage not manageable by the standard methods and may be preferable to roll gauze packing.

Temporary Arterial and Venous Bypass for Resection of Retroperitoneal Sarcomas

Retroperitoneal sarcomas that simultaneously affect large abdominal vessels are rare. Tumour resection and vascular clamping may take a long-time, and possibly have significant local and systemic consequences. For this reason, temporary arterial and venous bypass may be useful. Three patients underwent operation with utilization of this technique, which made it possible to resect tumour masses with a shorter duration of ischaemia, while maintaining venous return. These patients remain free of disease after an average follow-up of 37 months, without any arterial graft complications. Two of the patients progressed to symptomatic occlusion of the venous graft after the perioperative period.

Phase I Trial of Preoperative Concurrent Doxorubicin and Radiation Therapy, Surgical Resection, and Intraoperative Electron-Beam Radiation Therapy for Patients With Localized Retroperitoneal Sarcoma

The primary objective of this phase I trial was to define the maximum-tolerated dose of external-beam radiation with concurrent fixed-dose continuous-infusion doxorubicin followed by surgical resection and electron-beam intraoperative radiation therapy (EB-IORT) for patients with localized, potentially resectable retroperitoneal sarcomas (RPS). Thirty-five patients with radiographically resectable primary or recurrent intermediate- or high-grade RPS were treated. Doxorubicin was administered each week for 4 or 5 weeks as an initial bolus (4 mg/m2) followed by a 4-day continuous infusion (4 mg/m2/d). Concurrent radiation therapy was administered in escalating doses of 18.0, 30.6, 36.0, 41.4, 46.8, or 50.4 Gy in 1.8-Gy fractions. Radiographic restaging was performed 4 to 8 weeks after chemoradiation, and patients with localized disease underwent surgical resection with EB-IORT (15 Gy). Chemoradiation was completed as outpatient therapy in 31 patients (89%); four patients required hospital admission during chemoradiation or in the postchemoradiation preoperative period. At the highest radiation dose of 50.4 Gy, two (18%) of 11 patients had grade 3 or 4 nausea. Twenty-nine patients (83%) underwent laparotomy; six patients had interval disease progression and did not undergo surgery. Grossly complete resection (R0 or R1) was performed in 26 (90%) of 29 patients who had surgery. EB-IORT was feasible and successfully administered to 22 patients who had R0 or R1 resections. Preoperative chemoradiation, surgical resection, and EB-IORT are feasible for patients with RPS. Preoperative external-beam radiation can be administered to a total dose of 50.4 Gy with continuous-infusion doxorubicin.

Aggressive resection of retroperitoneal sarcoma is superior to simple excision: an animal study using a novel model for retroperitoneal sarcoma.

Retroperitoneal sarcomas constitute a difficult management problem. The need for, and extent of, aggressive surgery continues to be debated. The aim of our study was to compare the impact of radical en bloc resection of retroperitoneal sarcoma with complete resection of the tumor alone in a rat model. Under laparoscopic guidance, a fibrosarcoma cell line suspension was injected into the left paranephric space of a rat, resulting in the development of a macroscopic retroperitoneal tumor. Ten days after inoculation, 50 rats were randomized into three groups: (1) local resection, (2) radical resection, and (3) follow-up only. Groups 1 and 2 were further randomized for sacrifice 1 month after surgery or were followed up for 2 months. Local recurrence: 46% of group 1, while none in group 2 developed local recurrence during the same follow-up period (P = 0.02). Survival: 33% of group 1 were alive after 2 months, as compared with 54.5% of group 2. (P = 0.04). All rats in the control group died within <25 days. Our results suggest that aggressive en bloc resection of retroperitoneal sarcomas with adjacent viscera, even when macroscopically uninvolved with disease, has an advantage over complete local resection alone.

Retroperitoneal sarcomas: combined modality treatment approaches.

Retroperitoneal soft tissue sarcomas are rare tumors estimated to account for 15%of all patients with soft tissue sarcoma seen in referral populations. The standard of care for patients with localized, resectable retroperitoneal sarcomas is surgical resection with gross and microscopically negative margins. However, owing to the large size and locally advanced nature of these tumors, this goal is difficult to achieve in most patients. As a result, the disease is characterized by a high propensity for local recurrence and a grade-specific risk for distant metastasis. Over the past decade, there has been considerable research into combined modality treatment of these tumors. The present report outlines current concepts relating to the diagnosis, staging, and management of retroperitoneal sarcomas. Emphasis is placed on evolving combined modality treatment approaches and current investigational strategies.

The role of radiation therapy in resectable retroperitoneal sarcomas

A 29 year old female presented to her primary care physician soon after she noted a palpable mass in her left lower abdomen. She was otherwise asymptomatic. An abdominal and pelvic CT revealed a 12]10]9 cm heterogenous mass arising from the psoas and extending into the pelvis (Fig. 1a). There was no evidence of ureteral or bowel obstruction. Chest CT revealed no evidence of metastases. She had no other signicant medical or surgical history. Exploratory laparotomy with complete gross total excision was performed. The mass was noted to be adherent to the ureter and iliac vessels. A section of the external iliac artery was resected with the mass, and repaired with an interposition graft. Surgical clips were placed to identify regions of tumor adherence. Pathological evaluation revealed a grade III liposarcoma, with close margins in several locations along the posterior aspect of the mass. She recovered from surgery without signicant di$culty. Adjuvant radiotherapy was recommended and she agreed to proceed. A treatment planning CT was obtained with the patient immobilized in a prone alpha cradle with a false table top (belly board) to allow displacement of the bowel [1]. A region along the psoas and iliac vessels was identied, in collaboration with the surgical oncologist, that was felt to be at highest risk for local recurrence. Three dimensional treatment planning was utilized to develop a 6 eld plan of axial beams that localized dose to the region identied (Fig. 1b and c). A total dose of 54Gy was delivered in 1.8Gy fractions. She experienced occasional diarrhea and mild nausea, but no other signicant acute toxicity. Treatment was completed in 7/98. She remains without evidence of local recurrence or late toxicity. She is without evidence of distant metastases as well.

Nephrectomy during operative management of retroperitoneal sarcoma.

Complete resection of a retroperitoneal sarcoma often requires removal of adjacent organs. In this study we evaluated the role of nephrectomy during operation for retroperitoneal sarcoma. Between July 1982 and July 1995, 75 of the 371 (20%) patients who underwent resection of retroperitoneal sarcoma at MSKCC underwent concommitant nephrectomy. Data concerning the reasons for nephrectomy, degree of sarcomatous renal involvement, and survival were retrospectively analyzed. Fifty-four patients (72%) underwent nephrectomy during the initial resection, and 21 (28%) during a resection of a recurrent or persistent tumor. The most common reason for nephrectomy was total encasement by sarcoma (n = 40; 53%), followed by dense adherence of the tumor to the kidney (n = 21; 28%), and the direct invasion of the kidney by tumor (n = 2; 3%). Pathology demonstrated an absence of kidney invasion in the majority of cases (55 of 75; 73%). Renal capsular invasion was present in 11 of 75 (15%), renal parenchymal invasion in 7 of 75 (9%), and renal vein invasion in 2 of 75 (3%) of cases. There were no significant differences in survival based on degree of sarcoma involvement of the kidney, tumor grade, or whether the resection was for primary or recurrent disease. The 53 patients who underwent a complete gross resection of all tumor had a significantly improved long-term survival compared to the 20 patients who did not (50% versus 20% DFS at 5 years, respectively; p < 0.001). Decisions for concomitant nephrectomy during resection of retroperitoneal sarcoma should be based on whether this maneuver will provide a complete resection of all gross tumor, in which case the long-term disease-free survival of 50% is comparable to the reported 5-year survival of all patients with retroperitoneal sarcoma who are completely resected.

Retroperitoneal sarcoma treated by surgery

A retrospective analysis of extensive surgery with resection of retroperitoneal sarcoma and total or partial resection of adjacent organs in 15 patients is presented. Dominating symptoms were a palpable mass (80%). The tumor was felt in all patients. In 9 of 10 patients, ultrasonography showed a solid tumor with retroperitoneal location. In 14 patients (93%) the tumor was removed, in 11 of them adjacent organs also. Five of the patients were subjected to more than one operation because of tumor recurrence. Two patients died postoperatively. Six patients are alive without tumor recurrence; and three, with recurrence 18 to 62 months after the first operation. Four patients died 5--24 months after primary operation. In seven patients (50%), the tumor recurred locally. Surgery for recurrent tumor was beneficial. Complete surgical removal provides the most effective therapeutic approach. Routine second-look operation within a year after primary surgery may be a way to improve the results. Adjuvant cytostatic therapy could be considered.