Hospital Volume Threshold for the Treatment of Retroperitoneal Sarcoma.

BACKGROUND: Retroperitoneal sarcomas (RPS) are rare, histologically heterogeneous, and anatomically complex tumors. National Comprehensive Cancer Network guidelines recommend evaluation and management by multidisciplinary teams with experience in sarcoma. Our aim was to determine an appropriate hospital volume threshold for the treatment of RPS. PATIENTS AND METHODS: Patients undergoing resection of RPS were identified from the National Cancer Data Base (1998-2012). Multivariable modeling with restricted cubic splines was employed to examine the association between hospital volume and survival and identify possible hospital volume threshold. RESULTS: The study included 5,340 patients who underwent surgery at 909 different hospitals. Median annual volume was two cases per year. After adjustment, hospital volume was associated with improved survival (p=0.01), without cutoff. The cohort was then grouped into: Low-volume (≤5 cases/year), intermediate-volume (6-10 cases/year), and high-volume (>10 cases/year). The majority of patients were treated in low-volume hospitals (86%), compared to 9% in intermediate- and 5% in high-volume centers; 44% of patients were treated in hospitals that performed one case per year. Compared to low-volume, high-volume hospitals more often had patients with high-grade and larger tumors. Adjusted 90-day mortality was significantly lower in high- vs. low-volume hospitals (odds ratio(OR)=0.25, p=0.02). With adjustment, treatment in high- vs. low-volume hospitals was associated with lower odds of margin positivity (OR=0.58, p=0.001), and improved overall survival (hazard ratio(HR)=0.61, p=0.002). CONCLUSION: Treatment of RPS in high-volume centers is associated with significant reduction in short-term mortality and improved long-term survival. Hospital volume may be a surrogate for the infrastructure and support necessary for the optimal management of these complex malignancies.

Effect of intraoperative radiotherapy in the treatment of retroperitoneal sarcoma.

BACKGROUND: Current National Comprehensive Cancer Network guidelines for the treatment of retroperitoneal sarcomas (RPS) endorse surgical resection, but the role of radiotherapy (RT) is less clear. We investigate the utilization and benefits of intraoperative RT (IORT) in the treatment of RPS. METHODS: We queried the Surveillance, Epidemiology and End Results (SEER) database (1988-2013) for the utilization of IORT and perioperative external beam RT (EBRT) in patients who underwent surgical resection of RPS. Groups were defined as any IORT (aIORT), IORT alone (IORT-), IORT with EBRT (IORT+) and preoperative and/or postoperative EBRT without IORT (EBRT). Demographics, tumor characteristics, extent of disease, and survival were compared between groups. RESULTS: We identified 908 patients with RPS who underwent surgical resection with perioperative RT. Demographics of age, sex, and race were similar between groups. There was no difference in baseline tumor characteristics of mean size, tumor grade, or histological subtype between groups. A higher percentage of patients receiving aIORT had tumors >20 cm in size, and extension beyond local tissues. Liposarcoma and leiomyosarcoma were the most common subtypes overall and in each subgroup. Patients with liposarcoma undergoing IORT and EBRT (IORT+) demonstrated a survival benefit over both IORT alone (IORT-) and EBRT alone. CONCLUSION: IORT was used infrequently for RPS but generated equivalent outcomes compared to EBRT, despite being utilized more often for larger tumors and those with peri-tumoral soft-tissue invasion. Patients with the most common subtype (liposarcoma) may benefit from combination IORT with adjuvant EBRT versus other regimens.

[Operative therapy of abdominal and retroperitoneal sarcoma]. / Operative Therapie der abdominellen und retroperitonealen Sarkome.

Retroperitoneal soft tissue sarcomas are characterized by a high rate of local recurrence. Complete tumor resection is the only potentially curative therapeutic option. The concept of a systematic compartmental resection is to remove the tumor en bloc with a margin of uninvolved tissue and organs. This is frequently only achieved by multivisceral resection which often includes kidney, colon, pancreas and parts of the diaphragm or the psoas muscle. The adoption of such a policy of multivisceral organ resection improves the proportion of curative resections and, ultimately, results in lower local recurrence rates. The present article comprehensively describes the operative procedures, perioperative treatment and the oncological results of surgery for retroperitoneal sarcomas. The role of surgery in oncological treatment plans and the importance of specialized centres are outlined in detail.

Ovarian metastasis is associated with retroperitoneal lymph node relapses in women treated for colorectal peritoneal carcinomatosis.

PURPOSE: To analyze the patterns of recurrence and the prognostic impact of ovarian metastases (OM) in a population of women with colorectal peritoneal carcinomatosis (CRPC) treated with curative intent. METHODS: Data from all consecutive women with CRPC who underwent curatively intended complete cytoreductive surgery (CRS) plus intraperitoneal chemotherapy at our institution were retrieved from a prospective database. A bilateral oophorectomy or a complementary unilateral oophorectomy was systematically performed during CRS. RESULTS: From 1994 to 2009, among 105 women who underwent CRS plus intraperitoneal chemotherapy for CRPC, 62 (60 %) had OM. Women with and without OM had comparable peritoneal cancer index (PCI) scores (10 vs. 12, respectively, p = 0.09). After a median follow-up of 60 (range 5-145) months, median overall survival of women with OM did not differ statistically from that of women without OM (respectively, 36 and 40 months; p = 0.75). Relapses occurred in 82 % of the patients, distributed similarly between the two groups except for retroperitoneal lymph node recurrence, which occurred in 19 patients (18 %), including 18 with OM. The only predictive factor for a retroperitoneal relapse was a history of OM (p = 0.0012). CONCLUSIONS: Retroperitoneal lymph node recurrence seems to be linked to OM originating from colorectal cancer and could worsen the prognosis. A systematic lymphadenectomy could be evaluated in women with isolated OM or very limited peritoneal carcinomatosis to analyze the incidence of invaded lymph nodes and study its potential benefit on survival.

Primitive retroperitoneal tumors. Vascular involvement--a major prognostic factor.

Primitive retroperitoneal tumors, although very rare, arouse an increased interest, because of the poor prognosis, unsatisfactory surgical and complementary therapy results. Up to now, the very low number of cases has impeded the acquisition of a unitary view of these tumors, a unanimously accepted algorithm of diagnostic and treatment being absent. Randomized trials regarding the effects of different therapies have not been possible. The main factor that can fundamentally increase the survival of these patients is radical resection, some authors even recommending compartmental surgery. We found no significant statistical difference between the survival rates of the patients with different types of non-radical interventions, that shoud be therefore, as much as possible, avoided. Our study evidences that vascular involvement is the main limiting factor in achieving radicality. The involvement of large retroperitoneal vessels makes often impossible a radical intervention, usually because of the lack of an adequate material and human endowment for ample vascular resections followed by laborious reconstructions. That is why, in our study, vascular involvement was associated with a decreased survival rate for operated patients. Therefore, we underline the necessity both of a solid material base and of establishing multidisciplinary surgical teams for adequate vascular interventions in oncologic general surgery.

[Current state of neoadjuvant therapy of soft tissue sarcoma]. / Aktueller Stand der neoadjuvanten Therapie bei Weichteilsarkomen.

The treatment of soft tissue sarcoma is clinically challenging. Referral to an experienced center with an interdisciplinary team is strongly recommended. Neoadjuvant therapy, including irradiation and chemotherapy, has been applied to improve local control rates, eradicate micrometastases and assess chemosensitivity. However, the role of neoadjuvant therapy remains controversial, especially for systemic therapy, as the only available randomized trial failed to prove a benefit for survival. Nevertheless, on the basis of the current body of literature, neoadjuvant therapy can be considered on an individual basis for patients with high-risk tumors. Whenever possible, patients should be included in a clinical trial.

Neuroblastoma: treatment outcome after incomplete resection of primary tumors.

PURPOSE: For International Neuroblastoma Staging System (INSS) stages III or IV neuroblastoma (intermediate or high risk), complete excision of the primary tumor is not always feasible. Most current studies on the treatment outcome of these patients have reported on the complete excision status. The aim of this study is to review the treatment outcome after the incomplete resection. METHODS: The medical records of 37 patients that underwent incomplete resection between January 1986 and December 2005 were reviewed retrospectively. Incomplete resection was assessed by review of the operative notes and postoperative computerized tomography. Age, gender, tumor location, INSS stage, N-myc gene copy number, pre- and postoperative therapy, and treatment outcome were reviewed. The treatment outcome was evaluated according to the postoperative treatment protocol in the high-risk group. RESULTS: Intermediate-risk patients were treated with conventional chemotherapy, isotretinoin (ITT) and interleukin-2 (IL-2). High-risk patients were treated with peripheral blood stem cell transplantation (PBSCT), ITT, and IL-2 (N = 11). Before the introduction of PBSCT, the high-risk patients were also treated with the conventional chemotherapy (N = 19). Intermediate-risk patients (N = 5) currently have no evidence of disease (NED). For the high-risk patients (N = 32), 19 patients were treated with chemotherapy alone; 15 patients died of their disease while four patients currently have an NED status. Eight of 11 patients that underwent PBSCT are currently alive. CONCLUSIONS: For intermediate risk, conventional chemotherapy appears to be acceptable treatment. However, for high-risk patients, every effort should be made to control residual disease including the use of myeloablative chemotherapy, differentiating agents and immune-modulating agents.

Multifocality in retroperitoneal sarcoma: a prognostic factor critical to surgical decision-making.

OBJECTIVE: To evaluate the significance of multifocality on overall survival (OS) in patients with retroperitoneal sarcoma (RPS) and establish a data-derived, prognostically and therapeutically useful definition of sarcomatosis. SUMMARY BACKGROUND DATA: The incidence, clinical features, and prognostic significance of multifocality in RPS is unknown. No current standardized definition for sarcomatosis is available. METHODS: We conducted a retrospective analysis of 393 patients with primary or recurrent nonmetastatic RPS treated at a comprehensive cancer center between 1996 and 2006. Baseline and treatment variables were compared in patients with unifocal and multifocal disease. A multivariate model was used to evaluate the association of multifocality and OS and identify additional prognostic factors in patients with multifocal disease. RESULTS: The median follow-up time for all patients was 69 months; 79 patients (20%) presented with multifocal disease. The 5-year OS rate was less in the multifocal group compared with the unifocal group (31% vs. 60%, respectively; P < 0.0001). After multivariate analysis, multifocality remained an independent predictor of worse OS {hazard ratio (HR) 1.7 (95% confidence interval (CI), 1.2-2.5); P = 0.004}. Additionally, patients with more tumors had significantly worse prognosis (>7 tumors, HR 2.1 (95% CI, 1.1-3.9); P = 0.03), with a 5-year OS rate of 7%. CONCLUSIONS: Multifocal RPS is associated with worse OS in patients with either primary or recurrent RPS; Patients with >7 tumors had the worst prognosis. This criterion can be used to define sarcomatosis, thereby identifying patients whose survival will ultimately depend on effective systemic therapy.

Cardiopulmonary bypass and cell-saver technique in combined oncologic and cardiovascular surgery.

The purpose of this study was to work out an adequate operative technique for patients with malignant tumors who also need open heart surgery or procedures on major blood vessels. We had 8 such patients. In 6 of them, a tumor (3 cases hypernephroid cancer and 3 cases retroperitoneal sarcoma) had grown through the inferior vena cava (IVC) up to the right atrium. Two patients had lung cancer together with severe coronary artery disease. All of these patients were operated on using a heart-lung machine (HLM) and cell saver (CS). In 6 patients the intravascular portion of the tumor was extracted as much as possible through a right atrium approach (in 3 cases a nephrectomy was performed). Two patients had a one-stage coronary artery bypass graft (CABG) and a lobectomy. All of the patients had uneventful postoperative periods and were alive when checked on 1 year after the procedures. During cytological investigation after each operation, tumor cells were found only on the internal surface of the HLM arterial filters with 20 microns holes. We suggest that special cardiovascular devices such as the HLM and CS might be used in borderline situations in oncology without increasing the risk of hematogenous tumor dissemination.

Surgical resection following interleukin 2 therapy for metastatic renal cell carcinoma prolongs remission.

Records of 399 patients with metastatic renal cell carcinoma treated with interleukin 2 with or without lymphokine-activated killer cell immunotherapy enrolled in 14 separate clinical trials from multiple institutions were reviewed to determine whether patients with a partial response to interleukin 2 therapy would benefit from surgical resection of residual tumor. Sixty-two patients demonstrated objective responses (15.5%), 18 (4.5%) complete and 44 (11.0%) partial. Eleven patients underwent resection of residual tumor in the lung, kidney, retroperitoneum, or pelvis so that they had "surgically no evidence of disease" (SNED). Of these, 10 had partial responses, and one patient with progressive disease had a complete response. Comparison of response duration showed no difference between the complete response and SNED groups, but there was a significant difference between each of these groups and the partial response group. At this writing, all 11 patients in the SNED group remained alive without evidence of disease (median follow-up, 21 months). In contrast, only 14 patients (76%) with complete responses and 15 patients (35%) with partial responses remained free of disease progression. Enhanced survival of the complete response and SNED groups compared with the partial response group borders on significance and awaits longer follow-up. These data suggest that surgical resection, if technically feasible, may benefit patients who show a partial response to interleukin 2 treatment for metastatic renal cell carcinoma.