Long-term prognosis of primary retroperitoneal soft tissue sarcoma.

AIMS: To evaluate the result of treatment and long-term outcome of a population-based cohort of patients with retroperitoneal soft tissue sarcoma (RSTS). METHODS: Between 1 January 1989 and 1 January 1994, 143 patients diagnosed as having primary RSTS were selected from a national pathology database (PALGA) in the Netherlands. In this population-based group of patients, the result of surgery, overall survival (OS) and disease-free survival (DFS) were analysed as well as factors affecting OS and DFS. Median follow-up was 10.2 years. RESULTS: Operative treatment resulted in a complete tumour resection in 55% of the patients (n=78), low- and intermediate-grade tumours were more often completely resected than high-grade tumours (P=0.016). Five- and 10-year cumulative OS was 39% and 21%, respectively, while DFS was 22% and 17%, respectively. In a multivariate analysis low malignancy grade (P=0.017) and a complete tumour resection (P<0.001) were associated with better OS. CONCLUSIONS: Complete tumour resection and low malignancy grade were independent favourable prognosticators. However, these factors were related too, since surgical success was influenced by malignancy grade.     

Outcome after surgical resection of multiple recurrent retroperitoneal soft tissue sarcoma

IntroductionLocal recurrences (LR) and distant metastases (DM) are common in retroperitoneal soft tissue sarcoma (RPS). Longer time to recurrence and resection of the recurrent lesion have been identified as beneficial prognostic factors for overall survival (OS) upon first tumor relapse. However, prognostic factors concerning OS upon subsequent recurrences are scarcely defined. In this study, we aimed to identify prognostic factors for post-relapse outcome in multiple recurrent RPS.MethodsPatients undergoing resection of primary and recurrent RPS at the University Hospital Heidelberg were retrospectively analyzed. Multivariable Cox regression analyses were performed to identify predictors of overall, LR- and DM-free survival. Subgroup analyses were performed for liposarcoma and leiomyosarcoma patients.Results201 patients with primary disease, 101 patients with first, 66 patients with second and 43 patients with third LR as well as 75 patients with DM were analyzed. More than 12 months to recurrence and resection of recurrence were associated with improved OS after resection of first and second LR (5-year OS for first/second LR; resection: 64%/62%, no resection: 20%/46%). Gross macroscopic incomplete resection of first (p < 0.001), second (p = 0.001), and third recurrences (p < 0.001) was an independent prognostic factor for poor OS.ConclusionDevelopment of LR and DM is frequent in RPS. Once a tumor relapsed, patients benefit from tumor resection not only in case of first, but also in case of subsequent recurrences.     

术后放射治疗在原发肢体软组织肉瘤治疗中的作用

分析本院收治的原发于肢体的软组织肉瘤的治疗情况 ,评价术后放射治疗的作用。方法　本院共收治 15 1例 ,可供分析的 139例 ,分析影响生存和局控的因素及术后放射治疗的意义。生存率和局控率用Kaplan Meier方法计算 ,单因素分析用Logrank检验 ,多因素分析用Cox回归方法。结果　全组 5年生存率为 70 .2 % ,10年为 5 0 .4% ;5年无瘤生存率为 5 2 .9% ,10年为41.2 %。单因素分析对全组病例生存率有影响的因素 :肿瘤大小、年龄和治疗方式 (P值分别为0 .0 0 8,0 .0 0 7和 0 .0 40 )。多因素分析只有治疗方式对生存有影响 (P =0 .0 40 )。首次治疗方式对局控影响差异有极显著性 (P <0 .0 1)。手术方式对单纯手术组局控影响差异有极显著性 (P <0 .0 1) ;肿瘤 <5cm时 ,射野大小对术后放射治疗组局控影响差异有极显著性 (P <0 .0 1)。结论　术后放射治疗能提高局部控制率 ,初始射野应相对大 ,并采用缩野技术。对恶性度低、肿瘤 <5cm、手术切缘阴性患者第 1次术后可不做放射治疗。     

75例腹膜后软组织肉瘤的临床分析

目的　分析腹膜后软组织肉瘤的预后和放射治疗作用。方法　 75例中 ,5 0例为首程治疗 ,2 5例为局部复发后再治疗。 5 0例初治患者均接受手术治疗 ,其中 13例接受术后放射治疗。 2 5例外院术后复发再治疗患者有 16例接受手术 ,其中 3例接受术后放射治疗 ,另 9例接受单纯放射治疗。结果　全组、初程和复发再治疗患者的 5年总生存率分别为 39%、4 0 %和 37%。全组患者手术完整切除、部分切除和未切除的 5年生存率分别为 5 2 .7%、36 .6 %和 0 % ;完整切除和部分切除或未切除的生存率间差异有显著性意义 (P值分别为 0 .0 0 7、0 .0 0 0 ) ,但部分切除与未切除的 5年生存率间差异无显著性意义 (P =0 .196 )。手术完整切除的 4 1例患者 ,未放射治疗和放射治疗的 5年局部复发率分别为4 7%和 6 0 % (P =0 .880 ) ,5年生存率分别为 5 1%和 6 0 % (P =0 .780 )。手术部分切除和未切除的 2 5例患者 ,未放射治疗和放射治疗的 5年生存率分别为 17%和 4 4 % (P =0 .15 1)。结论　腹膜后软组织肉瘤的治疗以手术为主 ,手术完整切除可显著提高生存率。常规体外放射治疗的作用尚不肯定     

Surgical resection and radiotherapy for primary retroperitoneal soft tissue sarcoma.

METHODS AND MATERIALS: Forty-five patients were consecutively treated for primary retroperitoneal soft tissue sarcoma with surgery in combination with radiation therapy in the same institution. The median follow-up time was 53 months (7-108). RESULTS: Seventeen (38%) patients had clear microscopic margins (R0 resection), 26 patients (58%) had gross complete surgical excision (R1 resection) and two patients (4%) had a macroscopic residual disease (R2 resection). External radiotherapy doses ranged from 40.8 to 59.4 Gy (mean and median: 49 Gy). Seventeen patients underwent intraoperative radiation therapy (IORT). Moreover, 11 patients received chemotherapy. The overall 1-, 2-, and 5-year survival for all 45 patients were 93, 85 and 60%, respectively. The 1-, 2-, and 5-year locoregional relapse-free rate for the whole group was 91, 70 and 40%, respectively. In univariate analysis, quality of surgery was the only variable to show a significant effect for overall survival (P=0.0386) and for local control (P=0.0059). Tumor size and tumor grade had no statistically significant effect. For the patients receiving IORT+external beam radiation therapy, no difference was observed for survival or locoregional control. The most frequent acute side effects treatment complications were radiation-induced nausea or vomiting (42%) and moderate enteritis (30%). Significant late morbidity was observed for two patients. CONCLUSIONS: This study confirms the feasibility of external postoperative radiotherapy with an acceptable level of toxicity. However, the high rate of local relapses (especially in field of radiation) does not demonstrate the usefulness of radiotherapy at the level of dose used and further preferably randomized studies should be planned.     

腓肠肌肌皮瓣在修复膝关节周围软组织肉瘤手术组织缺损中的应用

目的探讨应用腓肠肌肌皮瓣修复膝关节周围软组织肉瘤广泛切除术软组织缺损的临床疗效。方法回顾性分析2009年3月至2012年5月有完整随访资料的膝关节周围软组织肉瘤患者的临床资料。所有病例均行软组织肉瘤广泛切除手术,并依据不同部位及缺损范围设计应用腓肠肌肌皮瓣修复软组织肉瘤广泛切除后的软组织缺损。结果全组12例,手术过程顺利,腓肠肌肌皮瓣均顺利成活。术后随访3—39个月,平均15个月,全组病例无复发,其中1例滑膜肉瘤术后8个月出现肺转移,均无死亡。移植肌皮瓣质地好,皮肤色泽与受区相似,其中4例蒂部外观略显臃肿,其余外形及功能恢复均良好,膝关节屈伸无受限。肌皮瓣供区小腿运动功能无影响,供区瘢痕位置隐蔽,无明显挛缩。结论腓肠肌肌皮瓣血管恒定,术中操作简单,成活率高,是修复膝关节周围软组织肉瘤广泛切除术后软组织缺损的良好选择。     

Neo-adjuvant chemotherapy for primary high-grade extremity soft tissue sarcoma.

BACKGROUND: The purpose of this study was to retrospectively analyze the relationship between neo-adjuvant chemotherapy (NAC) and outcome in patients with high-grade extremity sarcomas. PATIENTS AND METHODS: Inclusion criteria were high-grade, deep, >5 cm extremity soft tissue sarcomas. Patients diagnosed between 1990 and 2001 were treated with surgery only (n=282) or NAC containing doxorubicin/ifosfamide/mesna (AIM) (n=74). The stratified Cox proportional hazards model was used to test the effect of NAC on disease-specific survival and recurrence while adjusting for known prognostic factors. RESULTS: NAC was associated with improved disease-specific survival for this cohort of patients (P=0.02). This overall improvement appears to be driven by the benefit of NAC on disease-specific survival for patient with tumors >10 cm. The 3-year disease-specific survival for tumors >10 cm was 0.62 (95% CI: 0.53-0.71) for patients not receiving NAC and 0.83 (95% CI: 0.72-0.95) for patients receiving NAC. CONCLUSION: NAC with AIM was associated with a significant improvement in disease-specific survival in patients with high-grade extremity soft tissue sarcomas >10 cm. These data emphasize the need for further prospective clinical studies of neo-adjuvant or adjuvant chemotherapy for patients with large high-grade extremity sarcomas.     

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.     

Pathology of soft tissue sarcoma

The evaluation and treatment of soft tissue sarcomas has never been more demanding than it is today. The pathologist plays a central role in this process and is an integral member of the multidisciplinary sarcoma treatment team. This article provides a brief summary of the role of the soft tissue pathologist and includes sections on methods of diagnosis, frozen section, classification of sarcomas, expert consultation, molecular pathology, grading, assessment of treatment response, and tumor banking.     

软组织肉瘤的放射治疗

放疗是软组织肉瘤重要治疗手段之一,术前或术后放疗可降低肿瘤局部复发风险,局部扩大切除结合术前或术后放疗能达到与截肢术同等的长期生存率并且保存肢体功能,近些年来随着放疗技术的进步,软组织肉瘤放疗的正常组织损伤风险正在减少。     

Histology‐specific nomogram for primary retroperitoneal soft tissue sarcoma

BACKGROUND:

This study was conducted to develop a histology‐specific nomogram to predict postoperative overall survival (OS) at 5 and 10 years in primary retroperitoneal soft tissue sarcoma (STS).

METHODS:

Data registered at a single institution (National Cancer Institute, Milan, Italy) prospective sarcoma database were used. In the present analysis, patients with primary localized retroperitoneal STS resected with curative intent between 1985 and 2007 were included. A parametric piecewise exponential survival multivariate model was used for nomogram development, and internal validation was performed with standard methodologies. Known prognostic variables, such as age, tumor burden, histologic variant (as reviewed by a sarcoma pathologist), grade, and surgical margins were considered as putative predictors.

RESULTS:

Among the 192 patients analyzed, within 10 years from surgery, 114 patients were alive, with a median follow‐up time of 55 months (interquartile range, 25‐104 months). Among the investigated factors, only histologic subtype did not reach significance at the 10% level. The relative hazard increased while increasing tumor size up to about 25 cm, and decreased thereafter.

CONCLUSIONS:

A histology‐specific nomogram for retroperitoneal STS is now available. It can be used for better assessing the risk of the single patient and then making individualized decisions within the specific subset of retroperitoneal sarcomas. Cross‐check external validation should be performed. Cancer 2010. © 2010 American Cancer Society.     

Tolerance of tissue transfers to adjuvant radiation therapy in primary soft tissue sarcoma of the extremity

PURPOSE: Treatment of extremity sarcomas occasionally requires tissue transfer in the form of pedicle flaps, free flaps, or skin grafts to repair surgical defects. These tissues are often subject to radiation (RT) and are therefore at risk for wound breakdown requiring reoperation. This study reviews a single center's experience with tissue transfer and postoperative RT. METODS AND MATERIALS: Between 1983 and 2000, 43 adult patients (>16 years old) with primary high-grade soft tissue extremity sarcomas underwent limb-sparing surgery and reconstruction of their surgical defects, followed by adjuvant RT. The reconstructions were as follows: pedicle flaps (n = 14), free flaps (n = 10), skin grafts (n = 4), or a combination (n = 15). Postoperative external beam radiation therapy (EBRT) (median dose: 63 Gy) alone was given to 27 patients (63%). Adjuvant brachytherapy (BRT) was given to 16 patients (37%); BRT alone (median dose: 45 Gy) was given to 12 patients and combined with EBRT for 4 patients (EBRT: 45 Gy; BRT: 20 Gy). Comorbid conditions such as diabetes, hypertension, tobacco use, and obesity (calculated using body mass index >or=30) were present in 30 patients (70%). Tumor characteristics were as follows: 26 were >5 cm in size, 37 were deep, and 30 were in the lower extremity. The median follow-up time, calculated from the date of operation, was 32 months.Five of 43 patients suffered wound complications necessitating reoperation; however, 3 patients developed complications before initiation of RT and were therefore excluded from the analysis. Two of 43 patients (5%) required reoperation for wound complications after RT; 1 of these patients ultimately required amputation for necrosis. The 5-year overall wound reoperation rate was 6% (95% confidence interval: 0-14%). The influence of patient and tumor characteristics, as well as the type of RT, on the wound reoperation rates is as follows: BRT vs. EBRT (17% vs. 0%, p = 0.06); upper vs. lower extremity (0% vs. 8%, p = 0.41); 5 cm (8% vs. 4%, p = 0.9); comorbidity vs. no comorbidity (3% vs. 13%, p = 0.8); age 50 (8% vs. 4%, p = 0.8). CONCLUSION: Based on this review, most tissue transfers (95%) tolerated subsequent adjuvant radiation therapy well. Although more wound complications necessitating reoperation were seen in patients who received BRT, whether this is because of the inherent susceptibility of flaps and skin grafts to breakdown in the immediate postoperative period vs. the direct result of BRT needs further investigation.     

Hyperthermia in soft tissue sarcoma

Patients with high-risk soft tissue sarcomas (STS)–FNCLCC grade 2–3, size >5 cm, deep to the fascia—are at risk for developing local recurrence and distant metastasis despite surgical tumor resection. Therefore, the management of high–risk STS requires a multidisciplinary approach. Besides surgery, radiotherapy, and chemotherapy, regional hyperthermia (RHT) has the potential to become the fourth standard treatment modality for the treatment of these patients. RHT means non-invasive selective heating of the tumor area to temperatures within the range of 40–43°C for 60 min by the use of an electromagnetic heating device. Thereby RHT is always applied in addition to radiotherapy or chemotherapy or both but is not effective as a single treatment. Beside direct cytotoxicity, RHT in combination with chemotherapy enhances the drug cytotoxicity mainly by increased chemical reaction and intratumoral drug accumulation. For the neoadjuvant setting, RHT in combination with a doxorubicin- and ifosfamide-based chemotherapy has been shown to dramatically improve the tumor response rate but also prevents from early disease progression as compared to chemotherapy alone. The addition of RHT to a multimodal treatment of high-risk STS consisting of surgery, radiotherapy, and chemotherapy either in the neoadjuvant setting but also after incomplete or marginal tumor resection has been shown to significantly improve local recurrence- and disease-free survival. Based on these results and in conjunction with the low RHT-related toxicity, RHT combined with preoperative or postoperative chemotherapy should be considered as an additional standard treatment option for the multidisciplinary treatment of locally advanced high-grade STS.     

软组织肉瘤的放射治疗

软组织肉瘤(soft tissue sarcomas,STS) 是起源于结缔组织的软组织恶性肿瘤,具有多种不同类型。手术是 STS 主要治疗方法,放疗也是其重要的治疗方式并且是综合治疗早期选择之一。对 STS 进行放疗已经超过 50 年历史,术前和术后放疗对于局部控制都有疗效,只是不良反应不同。软组织肉瘤放疗技术包括远距离放疗(适形放疗、调强放疗、立体定向放疗等) 、近距离放疗(组织间插植放疗、腔内后装放疗、术中放疗等) 等。放疗技术的进步,提高了放疗的精准性和确定性,降低了对病灶周围正常组织的损伤。本文主要针对 STS 放疗技术以及适用原则进行综述。      

Primary soft tissue sarcoma of the breast

Opinion statement Primary soft tissue sarcoma (STS) of the breast is a rare and heterogeneous disease. The rarity of this tumor limits most studies to small retrospective case reviews and case reports. The optimal treatment of primary STS of the breast can best be determined through multidisciplinary discussions prior to the initiation of therapy. Whether chemotherapy is indicated is primarily determined by tumor size. There is evidence that tumors larger than 5 cm are associated with an elevated risk of systemic failure and a poor prognosis [1-3]. Negative surgical margins are more important for local recurrence and overall survival than is the extent of surgical resection [1]. Thus, neoadjuvant chemotherapy should be considered in order to shrink the tumor and help obtain negative surgical margins. After surgical resection, patients with chemosensitive tumors should undergo additional adjuvant chemotherapy to treat micrometastatic disease. Patients with tumors less than 5 cm that are easily resectable should undergo complete resection to the extent required to provide negative surgical margins. Radiation therapy should be used to improve local control in cases in which the tumor is larger than 5 cm and in cases with positive surgical margins [4-6]. The appropriate treatment of primary STS of the breast requires a multidisciplinary approach necessitating experienced surgeons, pathologists, radiotherapists, and medical oncologists.     

Screening populations at high risk for soft tissue sarcoma and surveillance following soft tissue sarcoma resection

Soft tissue sarcomas (STS) are a rare and diverse group of tumors that affect both adult and pediatric populations. This review discusses current screening recommendations for populations at increased risk for STS, including those with genetic predispositions. We also review surveillance guidelines for those at risk for recurrence following curative-intent surgery.