Importance of preoperative diagnosis for management of patients with suspected retroperitoneal sarcoma

Soft tissue sarcoma is an umbrella term which encompasses over 60 histological tumour types. Approximately 15% of soft tissue sarcomas arise in the retroperitoneum. This complex group of tumours poses unique management challenges due to their often large size, histological heterogeneity and complexity of anatomical relationships. This review discusses the management of retroperitoneal tumours including the need for preoperative diagnosis, the evidence for neoadjuvant radiotherapy, the role of multivisceral resection and the importance of a multidisciplinary team approach.     

Treatment of Ewing's sarcoma in the cooperative Ewing's sarcoma study group

Multidisciplinary treatment approaches have greatly improved the prognosis of patients with Ewing's sarcoma. Four- or five-drug combination chemotherapy regimens are combined with radiation and/or surgery for local control. Currently, in the Cooperative Ewing's Sancoma Study (CESS) trials, approximately 60% of the protocol patients with Ewing's sarcoma show a relapse-free 10-year survival. For improvement of local control, preoperative radiotherapy and/or brachytherapy may be helpful. The early detection of blood and/or bone marrow contamination by molecular diagnosis can lead to a new clinical staging system. A myeloablative regimen consisting of simultaneous total-body irradiation and chemotherapy may improve the prognosis of patients with disseminated disease.     

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探讨累及髂动静脉的盆腔腹膜后肿瘤手术切除时,髂血的处理方法,以提高切除率。方法总结１９９４年７月至１９９９年６手术切除累及髂动静脉的盆腔腹膜后肿瘤１４例,合并髂动静脉切除及重建的经验。其中合并右侧髂动静脉发除与重建６例。合并左侧血管切除与重建８例。结果随访３个月至５年,平均随访３．２年。３例肿瘤复发再切除,无一例死亡。     

Retrospective review of the outcome of post-chemotherapy retroperitoneal lymph node dissection in the management of metastatic testicular cancer: Local experience

Objective: Post-chemotherapy retroperitoneal lymph node dissection (RPLND) for metastatic testicular cancer is an uncommon surgical procedure in Hong Kong. Therefore, in the present article, we review the perioperative and long-term morbidity, as well as the survival outcome for RPLND carried out in a urological centre. Method: This is a retrospective study of patients suffering from metastatic testicular cancer with post-chemotherapy residual mass subjected to RPLND between 1998 and 2008. Patient and tumour parameters including initial presentation, chemotherapy regimens received prior to RPLND, perioperative outcomes, pathology and long-term results were retrieved from hospital notes and reviewed. Results: Seven patients who underwent post-chemotherapy RPLND were included in the present review. The mean follow-up time was 63.7 months (range 6–136 months), and no recurrence or mortality was reported in this series. Early perioperative morbidity included pulmonary oedema (one), chylous ascites (one) and wound dehiscence (one). Other than ejaculatory dysfunction in all of the patients, there was no major-long term complication reported in our series. Conclusion: This local series confirmed the survival benefit and association of minimal long-term morbidity with post-chemotherapy RPLND.     

The importance of the margin of resection and external radiation in non-lipomatous retroperitoneal sarcoma

BackgroundPrior studies evaluating the impact of adjuvant or neoadjuvant radiation on clinical outcomes of patients with non-lipomatous retroperitoneal sarcoma have been underpowered.MethodsWe queried the National Cancer Database to identify patients undergoing surgical resection of retroperitoneal sarcoma with non-lipomatous histology from 2004 to 2016. Multivariable logistic regression and Cox proportional hazards modelling with patients stratified by tumor size were used to identify factors associated with overall survival.Results3,394 patients met inclusion criteria. 592 had small (<5 cm), 1,186 had intermediate (5–10 cm), and 1,616 had large (>10 cm) tumors. Use of either neoadjuvant or adjuvant radiotherapy was associated with improved survival for patients with intermediate (neoadjuvant HR 0.67, CI [0.46, 0.98]; adjuvant HR 0.61, CI [0.50, 0.76]) and large (neoadjuvant HR 0.50, CI [0.37, 0.68]; adjuvant HR 0.56, CI [0.47, 0.69]) tumors, while adjuvant radiation therapy was associated with a survival benefit for small-sized tumors (HR 0.67, CI [0.46, 0.99]).ConclusionsRadiation therapy is associated with an overall survival benefit in patients presenting undergoing resection of non-lipomatous retroperitoneal sarcoma.     

Outcomes for soft-tissue sarcoma in 8249 cases from a large state cancer registry

BACKGROUND AND OBJECTIVES: To date, outcome reports for soft-tissue sarcoma (STS) have largely been limited to single or paired institutional series. To more accurately elucidate population-based outcomes and prognostic factors associated with STS, a large cancer registry was examined. METHODS: STS arising in the Florida Cancer Data System were examined (1981-2004). RESULTS: A total of 8249 patients were identified, the calculated annual incidence of sarcoma being approximately 38 cases per million in 2003. The tumor histologies among these patients were leiomyosarcoma and gastrointestinal stromal tumor (LMS/GIST) (43.5%), malignant fibrous histiocytoma (MFH) (31.5%), liposarcoma (19.0%), and fibrosarcoma (6.0%). Tumors were situated in the extremities (30.7%), truncal or visceral locations (50.4%), retroperitoneum (11.7%), and head or neck (7.2%). Thirty-three percent of lesions were over 10 cm in greatest dimension, while 50.2% were classified as high grade. Median overall survival was 25 months. Superior survival was observed for liposarcomas and fibrosarcomas as compared to MFH and LMS/GIST (P < 0.001). Retroperitoneal and truncal sarcomas had a more ominous prognosis than did other sites (P < 0.001). Multivariate analysis of pretreatment variables demonstrated that increasing age, male gender, non-Caucasian race, advanced stage, and a truncal or retroperitoneal location were each independently associated with lower survival. Histological subtype was also an independent predictor of outcome. Surgical resection and radiation therapy were the only treatment variables shown to improve survival. CONCLUSIONS: Histological subtype, tumor site, and stage are independent prognostic factors in STS. Surgical resection and radiotherapy are unique among treatment modalities in association with a significant survival benefit.     

Long-term outcome of idiopathic retroperitoneal fibrosis treated with surgical and/or medical approaches.

BACKGROUND: Retroperitoneal fibrosis is a severe disease that affects the ureters, causing renal insufficiency in three-quarters of patients. The optimal treatment is far from being established. METHODS: Seventeen patients with idiopathic retroperitoneal fibrosis and ureteral entrapment followed in our unit for at least 1 year were selected for this study. At presentation 13 patients had renal insufficiency. All patients received steroids, associated with ureterolysis in five (group 1), with azathioprine in six (group 2) and with tamoxifen in six (group 3). Four patients of group 2 and five of group 3 received ureteral stenting or nephrostomy. There were no significant differences among the three groups or the clinical and biochemical characteristics at presentation. RESULTS: All patients of groups 1 and 2 entered remission after therapy. One patient from group 3 did not respond to therapy. During a mean follow-up of 56 +/- 41 months, three patients (two from group 1, one from group 2, 18%) had a recurrence of the disease, which fully responded to retreatment in all three cases. At the last observation, all patients were alive; three patients (18%) had renal insufficiency, of them one from group 1 had to start dialysis 6 years after ureterolysis, one patient from group 2 and one from group 3 had serum creatinine of 1.5 mg/dl. Renal survival was 100% at 5 years and 80% at 10 years. CONCLUSIONS: In most patients, each of the three different therapeutic approaches restored renal function and significantly reduced the fibrotic mass in the short-term and maintained stable serum creatinine in the long-term.     

Long-term survival by repeat resection for metastases from primary retroperitoneal leiomyosarcoma: A case report

BackgoundRetroperitoneal (RP) leiomyosarcoma (LMS) is a rare type of cancer, accounting for 0.1% of all malignancies. The gold-standard treatment for sarcoma is complete resection, and a 50% 5-year overall survival (OS) rate can be achieved by curative surgery. The survival benefits of radiotherapy and systemic chemotherapy for recurrence are not as good as those of surgical resection. To the best of our knowledge, there are a few reports that aggressive radical surgery significantly prolonged the survival period as our case. This case was reported in accordance with the SCARE 2020 Guideline (Ref).Case presentationAn 84-year-old woman was referred to our hospital for treatment of a primary RP tumour. At the age of 52-year-old, she underwent complete resection of an RP mass in 1991. Twenty-four years after the primary resection, metachronous recurrences occurred within the soft tissues, which were repeatedly resected. From 2015–2019, liver resections were performed thrice, and the patient survived with no signs of recurrence 1 year after the last surgery.ConclusionLong-term survival of 29 years was achieved after undergoing over 20 surgical resections. Herein, we report the long-term survival of a patient who underwent repeated aggressive surgical resections for RP LMS recurrence anda literature review.     

Long-term outcome after multivisceral and tumor/vascular resection in patients with soft tissue sarcoma

Background/Aims  Soft tissue sarcomas (STS) are rare tumors. General treatment is difficult while multimodality treatment strategies are more and more common. In these strategies, surgical resection of the primary tumor is essential to achieve local control of the tumor. In certain cases, complex resections (CR) including multivisceral and/or vascular resection are needed to achieve resection with tumor-free margins. In this study, we evaluated retrospectively the overall prognosis, morbidity, and mortality of patients treated for STS at our university hospital. Patients/Methods  Between 1992 and 2000, 24 of 154 patients with STS received multivisceral resection and four of 154 underwent vascular resection. To determine the influence of CR on overall prognosis, we compared n = 19 patients after CR with a matched control group after simple tumor resection (SR). To determine surgical morbidity and mortality the whole study group was used (n = 154, SR n = 126, CR n = 28). Results  The median follow up for all patients was 6.89 years (mean 5.64 years SD 4.3) with no difference between the groups (CR vs SR: 5.4 SD 4.8 vs 5.9 SD 3.9 years; p = 0.711). Patients receiving CR had a similar overall prognosis (mean survival 9.9 years), morbidity (10.7%) and mortality (0%) compared to patients with SR (mean survival 8.5 years; morbidity 10.3%; mortality 3.96%). Conclusions  Multivisceral resection and/or vascular resection with tumor-free margins can be achieved with the same overall prognosis, same morbidity and mortality as SR. This has to be taken into account when evaluating the treatment strategy in patients with STS.     

Use of adjuvant,preoperative chemo-radiotherapy in patients with locally advanced rectal cancer

Background: The objective of this study was to perform a non‐randomised prospective examination of the efficacy of adjuvant, preoperative chemo‐radiotherapy in patients with locally advanced rectal cancer. Methods: Between 1996 and 2001, patients presenting with biopsy‐proven, locally advanced, rectal cancers within 12 cm of the anal verge were referred for a long course of adjuvant chemo‐radiotherapy prior to their surgery. Locally advanced lesions were defined by either: (i) endoanal ultrasound showing at least full thickness penetration of the rectal wall (i.e. T3, T4); (ii) abdominal computed tomography scan showing infiltration of adjacent structures, or; (iii) clinical examination demonstrating a fixed lesion. All patients were followed through the hospital colorectal unit. A Kaplan?Meier survival analysis was used to determine survival and local recurrence rates. Results: There were 60 patients with a mean age of 61.5 years (range 33?77 years) with a sex distribution of males to females of 1.7?1.0. Curative resections were performed in 81% of these patients. The remainder (n = 12) were found to have either metastatic disease at operation (n = 5), inoperable disease (n = 2), or had positive resection margins on histology (n = 7). The mean follow up was 2.1 years (maximum 5.1 years). The overall 2‐year survival rate was 86.1% (95% CI ±5.4%). In patients undergoing curative resections, the overall 2‐year survival rate was 91.4% (95% CI ±4.8%), and the 2‐year disease free survival rate was 85.1% (95% CI ±6.2%). The 2‐year local recurrence rate was 7.5%. Conclusions: The use of adjuvant, preoperative, chemo‐radiotherapy in patients with locally advanced rectal cancer is associated with high short‐term survival and a low recurrence rate.     

Initial results of a trial of preoperative external-beam radiation therapy and postoperative brachytherapy for retroperitoneal sarcoma

Background Surgical resection alone does not cure the majority of patients with retroperitoneal sarcoma (RPS). We evaluated the effects of preoperative external-beam radiotherapy (XRT) and postoperative brachytherapy (BT) combined with complete surgical resection. Methods Fifty-five patients with primary or locally recurrent RPS judged to be resectable were entered onto a trial of combined therapy and observed prospectively. Forty-six patients underwent complete gross resection with curative intent. Of these, 41 patients completed preoperative XRT and 23 patients received BT. Outcome measures were treatment toxicity, overall survival, and disease-free survival (DFS). Results Preoperative XRT was very well tolerated and was associated with Radiation Therapy Oncology Group acute toxicity scores of ≤2 in all patients. Acute postoperative and BT-related toxicity resulted in modified RTOG scores of ≥3 in 39.1% (18 of 46) of patients. Late toxicity was associated with death in 4.3% (2 of 46) and with life-threatening illness in 2.2% (1 of 46) of patients, all of whom had been treated with BT to the upper abdomen. The 2-year overall survival and DFS for resected RPS were 88% and 80%, respectively. Significantly better 2-year DFS was achieved in patients with primary RPS and in those with low-grade tumors (93% and 95%, respectively). Conclusions The initial results of combined therapy are promising. Although preoperative XRT was very well tolerated, BT to the upper abdomen was associated with substantial toxicity. Our current protocol includes selective application of BT to the lower abdomen only. Presented at the 54th Annual Cancer Symposium of the Society of Surgical Oncology, Washington, DC, March 15–18, 2001.     

Clinical outcome of retroperitoneal lymph node dissection after induction chemotherapy for metastatic non-seminomatous germ cell tumors

BACKGROUND: Retroperitoneal lymph node dissection (RPLND) following induction chemotherapy has been considered a critical component in the comprehensive management of advanced non-seminomatous germ cell tumors (NSGCT). The objectives of the present study were to review the clinical outcome of patients who underwent RPLND and to evaluate the probability of necrosis alone, based on some readily available clinical data for these patients. METHODS: Forty-seven consecutive patients with NSGCT were treated with first-line chemotherapy at our institution between January 1993 and September 2002. Twenty-four of these patients, who underwent RPLND with normal values of tumor markers after induction chemotherapy, were included in the study. The cause-specific survival rate was calculated using the Kaplan-Meier method. Various predictive factors for the histology were analyzed using multivariate analysis. RESULTS: The pathological findings at resection were necrosis alone in 62.5% of cases, teratoma in 25.0%, and viable cancer in 12.5%. The cause-specific 3-year survival rate of patients who underwent complete and incomplete resection was 100% and 50.0%, respectively. Among several clinical factors, prechemotherapy tumor size less than 50 mm was found to be an independent predictor of necrosis alone (hazard ratio = 4.45, P= 0.04). CONCLUSION: Metastatic tumor size before chemotherapy appears to be one of the most important factors for the prediction of necrosis alone in the resected specimens of RPLND. The prognosis of patients might be influenced by the degree to which resection has been completed.