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.     

Retroperitoneal soft tissue sarcoma: effect of hyperthermic total abdominal perfusion

AIMS AND BACKGROUND: Soft tissue sarcomas (STS) of the retroperitoneum are rare tumors. Surgery remains the principal modality of therapy in the management of primary and recurrent retroperitoneal STS. However, little is known about the effect of regional chemotherapy using hyperthermic total abdominal perfusion (HTAP). We analyzed independent prognostic variables in 33 patients with STS in the retroperitoneum admitted from November 1990 through December 1996. METHODS AND STUDY DESIGN: Data regarding patients' age, gender, tumor size, histological tumor type, tumor location, type of operation (primary or secondary surgery), extent of surgical management (marginal or extended), use of HTAP, tumor grade, and tumor stage according to the TNM classification were examined by univariate and multivariate analyses. RESULTS: All 33 patients underwent complete resections (marginal or extended). Eleven of them received locoregional chemotherapy by HTAP. The overall cumulative 5-year survival rates in patients with stage IIA and advanced disease were 82% and 22%, respectively (log-rank test, P<0.01). Using Cox's proportional hazard model, tumor stage, use of HTAP and type of operation were found to have significant influence on overall survival (P<0.05). CONCLUSIONS: Our results showed that complete resection along with HTAP chemotherapy may improve survival in patients with retroperitoneal STS. These phase II data could be used to support the initiation of a phase III trial to test HTAP in patients submitted to complete resection of retroperitoneal STS.     

Retroperitoneal soft tissue sarcoma: an analysis of radiation and surgical treatment

PURPOSE: To evaluate the clinical outcomes of patients with localized retroperitoneal soft tissue sarcoma (STS) treated with complete surgical resection and radiation. METHODS AND MATERIALS: The medical records of 83 patients were reviewed retrospectively. Sixty patients presented with primary disease and the remaining 23 had recurrence after previous surgical resection. RESULTS: With a median follow-up of 47 months, the actuarial overall disease-specific survival (DSS), distant metastasis-free survival, and local control (LC) rates were 44%, 67%, and 40%, respectively. Of the 38 patients dying of disease, local disease progression was the sole site of recurrence for 16 patients and was a component of progression for another 11 patients. Multivariate analysis indicated that histologic grade was associated with the 5-year rates of DSS (low-grade, 92%; intermediate-grade, 51%; and high-grade, 41%, p = 0.006). Multivariate analysis also indicated an inferior 5-year LC rate for patients presenting with recurrent disease, positive or uncertain resection margins, and age greater than 65 years. The data did not suggest an improved local control with higher doses of external-beam radiation (EBRT) or with the specific use of intraoperative radiotherapy (IORT). Radiation-related complications (10% at 5 years) developed in 5 patients; all had received their EBRT postoperatively. CONCLUSIONS: Although preoperative radiation therapy and aggressive surgical resection is well tolerated in patients, local disease progression continues to be a significant component of disease death. In this small cohort of patients, the use of higher doses of EBRT or IORT did not result in clinically apparent improvements in outcomes.     

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。      

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。     

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.     

软组织肉瘤靶向治疗进展

化学治疗晚期软组织肉瘤疗效似已达到平台,靶向药物是进一步提高疗效的关键.尽管第1代靶向治疗药物开发的种类繁多,但仅有少数药物在前瞻性、随机的Ⅲ期临床试验中显示出生存优势.第2代靶向治疗药物多靶点抑制剂可同时阻断肿瘤多个信号传导,临床试验的初步结果表明,不良反应可以耐受,效果较好,治疗软组织肉瘤已显示出较好的应用前景.     

Palliative surgery of soft tissue sarcoma

Despite an increasing trend towards early diagnosis of breast cancer,patients still present with locally advanced disease. Also, in some patients chemotherapy will fail, and local and regional recurrence will occur. This article outlines options for palliative care for such patients.     

Neoadjuvant chemotherapy for soft tissue sarcoma

Soft tissue sarcoma is a rare and very heterogeneous disease. The prognosis of these cancers is mainly influenced by histological grading size location and type of histology. Many aspects argue in favor of neoadjuvant chemotherapy but no singular trial could unambiguously prove an advantage of neoadjuvant therapy. This short review has a focus on neoadjuvant chemotherapy as induction therapy prior to chemoradiation or neoadjuvant chemotherapy alone.     

Surgical management of soft tissue sarcoma

From 1964 to 1978, 313 patients with soft tissue sarcoma were treated in our hospital by resection and followed over 5 years. 192 patients were males and 121 were females. The sex ratio was 1.5:1. In the 190 cases who developed postoperative recurrence, 55 were in the head and neck, 136 in the trunk and 122 in the limbs and 23 (7.4%) as metastasis in the lymph nodes. By pathology, the common types were fibrosarcoma (86 cases), neurofibrosarcoma (66 cases) and rhabdomyosarcoma (32 cases). All the 313 patients were treated by resection, the overall 5 and 10 year survival rates were 47.6% and 35.8%. The 5 year survival rate was 47.9% in the 117 patients treated by local resection, 62.7% in the 75 by extensive resection, 18.6% in the 43 by amputation and 48.7% in the 78 by combination of resection and irradiation. According to the UICC TNM staging system, the 5 year survival was 60% in stage I, 46.9% in stage II, 40% in stage III and 22.2% in stage IV. In this series, discussion of the treatment modalities and prognosis factors in emphasized.     

Radiation therapy for soft tissue sarcoma

Sarcomas are relatively uncommon tumors of mesenchymal origin. They may arise anywhere within the musculoskeletal system and are typically classified by clinical behavior into benign, borderline, or malignant. This article focuses primarily on the role of radiation for malignant soft tissue sarcoma and selected borderline tumors.     

Endobronchial metastases from soft tissue sarcoma

The majority of patients with pulmonary metastases from primary soft tissue sarcomas have peripheral lesions that can be removed by wedge excision. A subset of these patients, however, present with, or develop, clinically significant endobronchial metastases. These patients require a preoperative evaluation that includes pulmonary function testing and a detailed endoscopic evaluation of the bronchial tree. We described three of these patients from the National Cancer Institute and review the clinical histories of eight additional previously reported cases.     

Adjuvant chemotherapy of soft tissue sarcoma

As the adjuvant chemotherapy of osteosarcoma has been proved highly effective, that of soft tissue sarcoma has been expecting. However, there is still some question as to its effectiveness. The indication and methods were studied on literatures and our own cases. A survey of the literature regarding to the chemotherapeutic effect upon advanced soft tissue sarcoma shows response rate ranging 20 to 50 per cent. On the other hand, soft tissue sarcoma has a variety of histological type and malignancy and the sensitivity to chemotherapy varies considerably according to each tissue type. The literatures and our results indicate that most effective sarcoma is rhabdomyosarcoma, which is absolutely advisable to apply adjuvant chemotherapy. The same is the sarcoma with similar histological pattern. As to other type sarcomas, the therapy has to be applied according to their grade, stage, age and the effect of chemotherapy evaluated by advanced tumor. Most prevalent agents used for soft tissue sarcoma are adriamycin, cyclophosphamide, actinomycin-D, vincristine and DTIC. These agents usually used as combination called VAC, CYVADACT, CYVADIC, BCD.     

Pulmonary metastasectomy for soft tissue sarcoma

The American Cancer Society predicts 10,520 new cases and 3920 deaths from soft tissue sarcoma (STS) for 2010. STS disseminates primarily via the hematogenous route, although lymphatic spread does occur with certain subtypes. The lung is the most common metastatic site in most large series, accounting for up to 80% of metastases. The median overall survival for pulmonary metastatic disease with current multidisciplinary treatment is approximately 12 to 14 months. Pulmonary metastasectomy (PM) represents the only potentially curative treatment for patients with STS and lung metastases. This article discusses the management of STS using PM.