The management of retroperitoneal soft tissue sarcoma: a single institution experience with a review of the literature.

AIM: Ten percent of soft tissue sarcomas (STS) arise in the retroperitoneal tissues. The prognosis for patients with retroperitoneal sarcoma is poor with a 5-year survival rate between 12% and 70%. Stage at presentation, high histological grade, unresectable primary tumour and incomplete resection are associated with a less favourable outcome. METHODS: Complete follow-up data were available on 22 patients who underwent surgery for retroperitoneal STS in our institution between 1990 and 2000. Patient, tumour and treatment variables were analysed including use of adjuvant therapy and survival status. RESULTS: Eighteen patients underwent surgery for primary disease, four patients were treated for recurrent disease or metastases. Ten patients presented with pain, seven with an abdominal mass, other presentation included weight loss and haematuria. Thirteen patients presented with tumours larger than 10 cm. The tumours were seven liposarcomas, six leiomyosarcomas, three malignant fibrous histiocytomas, two rhabdomyosarcomas, two malignant schwannomas and two undifferentiated sarcomas. Six primary tumours were completely excised, five patients received radiotherapy and five received chemotherapy. Local recurrence rate was 45% and recurrence-free interval for 10 patients with recurrence was 11 months. Five patients received radiotherapy and five received chemotherapy. The median survival for patients with primary tumours was 36 months, and 5-year survival was 44%. Adjuvant therapy was not associated with higher survival rates. CONCLUSION: This study re-emphasizes the poor outcome of patients with retroperitoneal STS. Adjuvant radiotherapy and chemotherapy do not appear to be any proven benefit and the single most important prognostic factor is aggressive successful en bloc resection of the primary tumour. Our resection rate and 5-year survival rates are comparable with previous reported UK series although lower than large reports from North American centres. This might partly be explained by difficulty in data collection in a retrospective analysis, but may reflect inadequate subspecialization in UK centres. Copyright Harcourt Publishers Limited.     

Retroperitoneal soft tissue sarcoma: prognostic factors and therapeutic approaches

AIMS AND BACKGROUND: Retroperitoneal sarcomas are a rare group of malignant soft tissue tumors with a generally poor prognosis. The aim of the study was to assess clinical, pathological and treatment-related factors affecting prognosis in patients with retroperitoneal sarcomas. METHODS AND STUDY DESIGN: The hospital records of 73 patients who underwent surgical exploration at our unit for primary retroperitoneal sarcomas between 1984 and 2003 were reviewed. Factors influencing overall and disease-free survival were analyzed for all patients and for those who underwent complete surgical resection. RESULTS: The complete resectability rate was 69.8% (51/73). Operative mortality and morbidity rates were 2.7% and 21.9%, respectively. For patients who underwent complete resection, the 5-year survival rate was 58.3%, whereas it was 0% in cases of incomplete or no resection (P < 0.001). Local recurrence rate was 37.2%. Incomplete gross surgical resection and microscopic infiltration of margins were the most important independent predictors of a poor prognosis. CONCLUSIONS: The present study confirmed the importance of an aggressive surgical management for retroperitoneal sarcomas to offer these patients the best chance for long-term survival.     

Recurrent hepatocellular carcinoma after hepatic resection: prognostic factors and long-term outcome.

AIM: The prognosis of patients with recurrent hepatocellular carcinoma (HCC) after hepatic resection varies widely. This study analyzed long-term survival and prognostic factors of patients with recurrent HCC after hepatectomy. METHODS: From July 1991 to December 2000, 623 patients underwent hepatic resection for HCC. Of those, 347 (56.5%) patients had tumour recurrence, and 286 patients with follow-up time more than 24 months after recurrence were enrolled. Twenty-seven clinicopathologic factors underwent both univariate and multivariate analysis. RESULTS: Of these 286 patients, survival times after tumour recurrence were mean 672+/-619 days; median 468 days; and, range 10-3753 days. The overall 1-, 3-, 5-, and 10-year post-recurrence survival rates were 61.5, 33.4, 18.2, and 9.0%, respectively. Seventy (24.5%) patients were alive at the time of study, and 10 of the 34 patients who underwent re-resection were disease-free. By Cox regression analysis, multiple initial tumours (relative risk (RR) 1.428), recurrent multiple (RR 1.372), extrahepatic recurrence (RR 2.434), recurrent tumour size >2 cm (RR 1.926), post-hepatectomy period until recurrence <1 year (RR 1.769), and non-resectional treatment of recurrent tumours (RR 3.527) were independent prognostic factors for post-recurrent survival rates. CONCLUSIONS: In patients with recurrent HCC after hepatectomy, both initial and recurrent tumour factors influenced their prognosis. Early detection of recurrent tumours is important. Re-resection correlated with better post-recurrent survival rates.     

Retroperitoneal sarcoma--the continued challenge for surgery and oncology.

Retroperitoneal sarcomas (RS) are rare malignant tumours with an incidence rate of about 1-2 cases per million per year. Therefore only a few centres are able to acquire more experience in this field. Tumours are usually of large size, due to slow growth and uncommon symptoms. Different histologic types, grades and rare incidence make any comparison difficult. Radical excision including adjacent organs, called "en-block" resection, is the treatment of choice, however it is very often difficult to obtain adequate free margins around the tumour. Complete tumour excision remains a challenge even for an experienced surgeon. In the published series, resectability ranges from 38 to 100% with radicality rate between 8 and 95%. Local recurrence is very common (33-86%), with rare distant metastases (max. 33%), so local failure is usually the cause of death. It is well known that histological grading and completeness of surgery determine the chance of survival. Five-year survival rates after radical excision ranged from 62-92% in well-differentiated tumours, compared with 16-48% in nondifferentiated sarcomas. There is no evidence that adjuvant or neoadjuvant treatment affects the prognosis. Only the development of an international registry of retroperitoneal sarcoma and co-operative intergroup studies can help in evaluating treatment and in applying innovative multimodal therapies to these neoplasms.     

复发性腹膜后肉瘤的手术治疗探讨

背景与目的：腹膜后肉瘤是罕见的恶性肿瘤,彻底手术切除是最有效的治疗方法,但是其术后复发率很高。复发性腹膜后肉瘤通常在复查时被发现,治疗方法仍是手术切除,但由于其解剖层次不清,因此手术难度大,出血多。该研究旨在探讨复发性腹膜后肉瘤的诊断及手术方式。方法：回顾性分析2007年10月—2016年10月收治的25例复发性腹膜后肉瘤,根据手术情况分组后使用log-rank检验对完整切除组与部分切除或活检组两者进行单因素预后分析,并使用Kaplan-Meier法计算生存率。结果：全组25例完整切除16例,占64.0%(16/25),部分切除6例,占24.0%(6/25),未切除活检为3例,占12.0%(3/25)。完整切除中联合脏器切除8例,占完整切除的50.0%(8/16)。24例获随访,完整切除和部分切除两者5年生存率为56.3%(9/16)和20.0%(1/5),差异有统计学意义(P<0.05)。结论：手术切除是治疗复发性腹膜后肉瘤最有效的方法,联合脏器切除能提高肿瘤完整切除率。本病术后复发率高,术后需密切随访。     

Surgical cytoreduction in recurrent ovarian carcinoma in patients with complete response to paclitaxel-platinum.

AIM: The objective was to analyse the impact of secondary cytoreductive surgery in patients with recurrent ovarian carcinoma. METHODS: Retrospective review of 572 consecutive patients with primary ovarian carcinoma. Thirty-eight patients with intraabdominal/pelvic recurrence consisted the study group. Clinical variables affecting tumour resectability and survival were evaluated. RESULTS: Complete tumour resection was obtained in 42% of patients. A solitary tumour recurrence was independently associated with complete tumour resection (p=0.009). Median survival for patients with complete and incomplete tumour resection was 51.8 and 19.9 months. The parameter, residual tumour, was found independently correlated with survival after the relapse surgical procedure (p=0.02). However, including also the parameter, number of relapse tumour sites, in the multivariate analysis, the parameter, residual tumour, was no longer significantly associated with survival. CONCLUSIONS: Complete tumour resection following secondary cytoreductive surgery is associated with improved survival in selected groups of patients with recurrent ovarian cancer. However, other clinical factors than surgical cytoreduction are of considerable significance in determining the outcome of the salvage treatment.     

Retroperitoneal soft tissue sarcoma

The objective of the current study was to define the optimal treatment for patients with retroperitoneal soft tissue sarcomas (RPS). The authors conducted a review of the pertinent literature and found that the majority of patients had locally advanced RPS at the time of diagnosis. Surgery was the mainstay of treatment, and macroscopic total excision was feasible in approximately 50-67% of patients. The 5-year probability of local control was approximately 50%, and the likelihood of developing distant metastasis was approximately 20-30%. The 5-year survival rate was approximately 50%, and deaths due to recurrent RPS continued to occur after 5 years. Postoperative radiotherapy (RT) decreased the likelihood of local recurrence, although it demonstrated no impact on survival. Adjuvant chemotherapy had no proven efficacy. The factor that was found to have the greatest impact on the prognosis for patients with RPS was the ability to achieve a macroscopic total resection. The authors concluded that RPS usually is advanced at the time of diagnosis. The optimal treatment was complete resection. Most patients experienced recurrent disease after surgery, and the most common site of failure was local. Adjuvant RT reduced the risk of local recurrence, but its impact on survival was questionable.     

Retroperitoneal sarcoma in a series of 51 adults.

Fifty-one patients with retroperitoneal sarcoma underwent surgery at the University Clinics of Hamburg from 1970 to 1988. Malignant schwannoma, liposarcoma and malignant fibrous histiocytoma were the most common histological types. High grade sarcomas (G3) predominated (42%), while 26% were graded G2 and 33% G1. Complete resection was possible in 59% of cases. Reoperation for local recurrence and debulking in case of not completely resectable disease was common. Regional lymph node metastasis was found in 20% of cases. The peritoneum was the preferred location of metastatic spread. All adjuvant chemotherapies were followed by tumour recurrences. After adjuvant radiotherapy three of six patients remained free of disease. In cases of residual tumour we observed few partial responses on chemo- and radiotherapy, and all patients died because of the disease. The mean survival time was 60 months, the 5- and 10-year survival rates were 35% and 15%, respectively. Factors influencing prognosis in univariate analysis included grade, size, resectability, presence of metastatic spread, histological type, microscopic local growth (infiltrative vs intact pseudocapsule of fibrous tissue) and local recurrence. With multivariate analysis grade, size and lymph node metastasis retained their prognostic significance.     

49例胃癌根治术后残胃复发的外科治疗和预后分析

目的：探讨胃癌根治术后残胃复发癌的可切除性和预后影响因素，方法：对1986～2002年间在我院接受手术治疗的胃癌根治术后残胃复发的49例患者进行回顾性研究。结果：本组病例的根治性切除率为36．7％(18／49)。合并腹膜复发者无1例获得根治性切除。非吻合口处残胃复发的根治切除率为45．5％(10／22)，吻合口处残胃复发者为29．6％(8／27)。获得根治切除患者的5年生存率为26．7％，未获得根治切除者为0。未合并腹膜复发患者的5年生存率为15．6％，合并腹膜复发者为0，结论：残胃复发癌不能被根治切除的主要原因是同时合并腹膜复发．或肿瘤复发侵犯周围重要脏器，吻合口处的复发肿瘤的根治性切除率有减低的趋势．复发肿瘤能否被根治切除、是否合并腹膜复发直接影响患者的预后。     

Squamous cell carcinoma of the nasopharynx--an analysis of treatment results in 149 consecutive patients

The purpose of this study was to evaluate the outcome of primary treatment and treatment of recurrences in patients with nasopharyngeal carcinoma. The material included 149 consecutive patients seen at the Aarhus University Hospital from 1963 to 1991 (49 females and 100 males). The stage distribution was: Stage I -9%, II-3%, III-28%, and IV-60%. Primary treatment was delivered with curative intent in 145 patients (97%). Persistent or recurrent disease after primary radical treatment was observed in 82 of the patients; 54% at the T-level, 40% at the N-level, and 33% at the M-level. A curative salvage attempt was carried out in 14 patients only, all with nodal recurrence: surgery in 8 patients (4 controlled) and radiotherapy in 6 patients (2 controlled). The 5-year local tumour control, locoregional tumour control, disease-specific survival rate and the overall survival rate for the patients treated with curative intent were 66%, 53%, 50% and 43%, respectively. Most of the patients (88%) had poorly differentiated tumours and these patients had the best prognosis. A major complication in three patients was radiation-induced myelopathy due to high-dose radiation delivered to the brain stem. Significant positive prognostic factors for treatment outcome in univariate analyses were early T-classification, small clinical stage, poor differentiation and low age. The Cox multivariate analysis showed that early T-categories, low N-categories and poor differentiation were independent, positive prognostic factors. Nasopharyngeal carcinoma is curable with primary radiotherapy; patients with poorly differentiated tumours have the best prognosis. Only a few patients were salvaged after recurrence. The factor most essential for success is primary control of the disease at the T- and N-levels.     

Surgical management of retroperitoneal sarcomas associated with external and intraoperative electron beam radiotherapy.

AIMS: To report outcomes of adults with retroperitoneal sarcoma (RS) treated by surgery, external beam radiotherapy (EBRT) and intraoperative electron beam radiotherapy (IORT). METHODS: From July 1988 to February 2001; 24 patients with primary and recurrent RS were diagnosed and treated. The median dose and energy of IORT delivered was 15 Gy/9meV. EBRT dose varies between 45-50 Gy. RESULTS: There were five primary and 19 recurrent tumours. One primary and five recurrent tumours underwent R0 resection. There were 12 liposarcomas and 19 grade I tumours; 13 patients developed local recurrence and three developed distant metastases.Twenty-two patients received IORT associated with EBRT: 11 developed recurrences. Six patients developed Neurotoxicity (4 grade II and 2 grade III). Disease free survival and overall survival at 5 years was 28 and 56% respectively. CONCLUSIONS: EBRT with IORT treatment is a promising technique for local control. Lower recurrence rates are associated with radical (R0) surgical procedures.     

Prognosis following locally recurrent soft-tissue sarcoma. A staging system based on primary and recurrent tumour characteristics

We have shown that the clinical growth rate of local recurrence from soft-tissue sarcoma could be expressed as a growthrate index (GRI) which was predictive for metastasis, and which was able to identify 2 equal populations of good (80% 2-year MFS) and poor survivors (33%). We now report the associations between characteristics of the primary and GRI, and combine primary and locally recurrent tumour characteristics in a staging system. We studied 460 adult patients with soft-tissue sarcomas of the extremities and trunk wall who were diagnosed and treated between 1964 and 1990, of whom 134 developed local recurrences and 151 metastases. The association of primary tumour size, histologic malignancy grade, depth, spontaneous necrosis, intratumoral vascular invasion and S-phase fraction with local recurrence, GRI and metastasis were examined. High GRI was associated with primary tumours that were larger, deeper, more malignant, underwent spontaneous tumour necrosis, demonstrated intravascular invasion and had a higher S-phase fraction. The same factors were also strongly associated with the incidence of metastasis. A multivariate analysis found GRI and primary tumour necrosis to be the strongest and most significant prognostic factors. GRI and tumour necrosis were combined in a staging system that identified groups with good survival (79 to 94% 2-year MFS), intermediate survival (61% 2-year MFS) and exceptionally poor survival (6% 2-year MFS). These findings validate our earlier assertion that high GRI reflects highly malignant tumours. A staging system composed of primary tumour necrosis and GRI can identify patients who may be suitable candidates for trials of adjuvant chemotherapy. © 1995 Wiley-Liss, Inc.     

Surgical management of soft tissue sarcoma in patients over 80 years

AIMS: To report outcome on patients over 80years of age with soft tissue sarcoma (STS), with respect to surgical treatment, co-morbidity, complications and survival. METHODS: From a prospective database of 3400 patients with STS presenting over a 13-year period, all patients over 80years of age were identified and reviewed, with respect to tumour characteristics morbidity, mortality and outcome. RESULTS: 128 patients over 80years were treated for STS with 63 referred for treatment of primary disease, of whom 50 underwent resectional surgery. The remaining 65 patients were treated for recurrent or incompletely excised disease. Of the 50 patients treated primarily with surgery, 56% of tumours where high grade and 56% were greater than 10cm in diameter. The overall complication rate was 34%, with a 30-day mortality of 4%. Two- and 5-year survival rates were 56% and 46%, with a local recurrence rate of 22% at a mean follow-up of 22months. CONCLUSION: This patient group presented with poor prognosis tumours that were associated with poor outcomes in the medium to long term. Age need not be considered a contra-indication to radical surgery with curative intent.     

Retroperitoneal soft tissue sarcomas: patterns of recurrence in 167 patients treated at a single institution

BACKGROUND: The objective of this study was to assess long-term prognosis and patterns of failure in patients with retroperitoneal soft tissue sarcoma who underwent surgery with curative intent at a single institution. Several series of patients with retroperitoneal sarcoma were reported, providing widely variable data regarding local and distant failure. METHODS: Overall, 167 consecutive patients who underwent surgery for retroperitoneal soft tissue sarcoma over a 20 year span at a single referral center were reviewed retrospectively. Eighty-two patients presented with primary disease, whereas 85 patients had recurrent locoregional tumors. Surgical resection was considered macroscopically complete in 147 of 167 patients (88%). RESULTS: Overall survival at 10 years after definitive surgery was approximately 27%, and the disease-free survival was approximately 16%. Only a minority of patients developed distant metastases. The risk of recurrence was correlated with whether patients underwent surgery for primary disease or for recurrent disease; the 10 year disease-free survival rate was 27% in the former group and 4.6% in the latter group. Histotype and malignancy grade were other prognostic factors, with the former found to be predictive of the pattern of failure and the latter predictive of overall survival. CONCLUSIONS: Local recurrence after primary surgery and high-grade malignancy were associated with the worst survival. Histologic subtype appeared to influence the pattern of recurrence, which mainly was local for patients with liposarcoma but was both local and distant for patients with the other histotypes.     

Radiotherapy and hyperthermia with curative intent in recurrent high risk soft tissue sarcomas

Purpose: Radiotherapy before or after resection is one of the pillars of treatment for localised high risk soft tissue sarcomas. Treatment intensification has been described with concurrent chemotherapy and hyperthermia. The aim of this study is to assess local control after multimodal treatment, focussing on the treatment of local recurrences after surgery only.

Patients and methods: Of 42 patients treated in a prospective protocol with radiotherapy and hyperthermia, nine were treated for isolated local recurrences without metastatic spread. Most patients were treated with trimodal therapy including chemotherapy with ifosfamide and underwent resection whenever possible. Median follow-up was 1.4?years.

Results: The treatment was well tolerated. Estimated disease free survival, distant metastases free survival and local control for the whole cohort after 1.5?years were 66, 73 and 88%, respectively. Neoadjuvant vs. adjuvant treatment influenced local control with a trend to statistical significance. Resection status did not influence local control. The cohort of patients treated for local recurrence after surgery alone had a significantly impaired local control compared to multimodal treatment at primary diagnosis (100 vs. 52%, p?Conclusions: With multimodal therapy including radiotherapy and hyperthermia local tumour control is achievable even in locally recurrent tumours. The clear-cut difference of the treatment of local recurrence in contrast to primary diagnosis might either reflect difficulties in diagnosis and treatment of local recurrences or biological aggressiveness of recurrent tumours. However, we recommend to consider multimodal treatment at primary diagnosis of high risk soft tissue sarcomas.     

Prognostic factors in retroperitoneal sarcomas: ploidy of DNA as a predictor of clinical outcome.

BACKGROUND AND OBJECTIVES: Radical surgery is the best mode of treatment of retroperitoneal sarcomas (RS); however, common recurrences are unpredictable. METHODS: For the better understanding of outcomes and possibilities of treatment retrospective analysis of different factors, including DNA content, was performed based on 70 patients. RESULTS: Leiomyosarcoma and liposarcoma were most common histologic types of classified sarcomas. Different kinds of resection were successfully performed in 51 patients (73%) and 35 of their available DNA specimens were analyzed. The actuarial 5- and 10-year survival rates in the resection group were 53% and 40%, respectively, with the median survival of 57 months. Patients with diploid resected tumors had a better 10-year survival rate (58%), than those patients with aneuploid tumors (25%,)--P<0.005. Those patients with low-grade sarcomas had a significantly longer survival than those with high-grade sarcomas (10-year survival rate: 44% compared to 29%). In the univariate analysis, adjuvant therapy, type of histology, type of surgery, location of tumor, and S-phase fraction had no influence on survival. In the multivariate analysis (Cox), only ploidy was an independent prognostic variable. Relative risk of death was over three times higher for aneuploid than for diploid tumors. CONCLUSION: Tumor ploidy should be analyzed in every case of retroperitoneal sarcoma for better assessment of prognosis and possible indication for adjuvant therapy.     

The relationship between prognosis and CEA-dt after hepatic resection in patients with colorectal carcinomas.

AIMS: CEA-doubling time (CEA-dt) was calculated by measuring serum CEA at two voluntary points. As CEA-dt is correlated with tumour doubling time the growth rate of liver metastasis could be determined. We investigated the relationship between CEA-dt and prognosis to determine the indications for resection of liver metastasis. METHODS: We examined 334 patients diagnosed with resected liver metastasis of colorectal carcinoma. Patients were divided into three categories based on CEA-dt; Group A, CEA-dt <30 days; Group B, 30 days /=s80 days. Clinicopathological parameters, the 3-year or 5-year survival rate and the rate of recurrence were compared among the three groups. RESULTS: In Group A, the survival time after hepatic resection was significantly shorter compared to the other groups. Furthermore, multiple liver metastasis showed slightly reduced CEA-dt levels compared with solitary metastasis, but even in patients with solitary liver metastasis, the rate of survival was poor. In 70% of Group A patients, recurrent tumour was recognized within 1 year of hepatic resection. CONCLUSION: When surgery for liver metastasis of colorectal cancer is considered. Group A patients should be recognized as having a poor prognosis and a high rate of recurrence after hepatic resection, and CEA-dt should be employed as a prognostic factor.     

134 例原发性腹膜后软组织肉瘤的临床诊治分析

目的:探讨原发性腹膜后软组织肉瘤的临床特点及诊治方法。方法:收集中国医学科学院肿瘤医院1999年1 月至2010年7 月所收治的134 例原发性腹膜后软组织肉瘤的临床资料，回顾性分析其临床特点和治疗方式对患者生存期的影响。结果:腹部肿块、腹痛、腹胀为最常见的临床表现。本组患者总的5 年生存率为37.8% ，行根治性切除术患者的5 年生存率为46.5% ，行姑息性切除术患者的5 年生存率为6.7% ，仅行剖腹探查患者的3 年生存率为0。结论:根治性手术切除是原发性腹膜后软组织肉瘤治疗的首选方法，辅助性化疗、放疗的作用有待进一步研究。      

Intraoperative electron-beam therapy for primary and recurrent retroperitoneal soft-tissue sarcoma

PURPOSE: This study assesses the long-term outcome of patients with retroperitoneal soft-tissue sarcomas treated by maximal resection in combination with intraoperative electron-beam therapy (IOERT) and postoperative external-beam radiotherapy. METHODS AND MATERIALS: From 1991 to 2004, 67 patients were treated with curative intent for primary (n = 26) or recurrent (n = 41) retroperitoneal soft-tissue sarcoma. All patients underwent maximal resection in combination with IOERT (mean dose, 15 Gy), 45 patients underwent additional postoperative EBRT, and 20 patients were previously irradiated. RESULTS: The 5-year actuarial overall survival (OS), disease-free survival, local control (LC), and freedom from metastatic disease of all patients was 64%, 28%, 40%, and 50%, respectively. The 5-year LC inside the IOERT field was 72%. For patients who completed IOERT and EBRT after R0-resection 5-year and 10-year OS was 80%, and 5-year and 10-year LC was 100%. Only 1 of the 21 patients after R0-resection and only 8 of 34 patients after R1-resection compared with 9 of 12 patients after R2-resection experienced inside IOERT-field relapse. Grade II or higher late complications were seen in 21% of the patients, but only 2 patients required surgical intervention because of late complications. CONCLUSION: In selected patients, IOERT results in excellent local control and survival, with acceptable morbidity.     

The impact of primary tumour location in patients undergoing hepatic resection for colorectal liver metastasis

Background

Primary tumour location has long been debated as a prognostic factor in colorectal cancer patients with liver metastases (CRLM) undergoing liver resection. This retrospective study was conducted to clarify the prognostic value of tumour location after radical hepatectomy for CRLM and its underlying causes.