Primary sarcomas of the heart.

Seventy-five primary sarcomas of the heart were classified by histologic appearance as angiosarcoma (26 cases), undifferentiated sarcoma (18 cases), osteosarcoma (9 cases), fibrosarcoma (6 cases), malignant fibrous histiocytoma (6 cases), leiomyosarcoma (4 cases), myxosarcoma (3 cases), synovial sarcoma (2 cases), and neurofibrosarcoma (1 case). The ages of the patients ranged from 1 to 75 years at the time of presentation (mean, 39 years). Angiosarcomas were predominantly right-sided and osteosarcomas left-sided. Forty patients treated surgically were examined, and survival correlated with clinical and histologic parameters. the survival rate was poor, with a mean of 11 months and median of 6 months. By univariate analysis, the survival rate was more favorable for patients with tumors located on the left side of the heart, without necrosis, with a low mitotic count, and without metastasis at diagnosis. Survival rates were better in patients receiving chemotherapy and radiation therapy. Age, gender, presence of differentiation, and histologic type did not affect prognosis. By multivariate analysis, a low level of mitotic activity and any therapy were the only significant factors affecting survival rate. Immunostaining with commercially available antisera was useful in the diagnosis of sarcoma but not in subclassification of 19 tumors so tested. Although the prognosis for patients with cardiac sarcomas is dismal, histologic grading is useful in predicting outcome, as has been shown for soft tissue sarcomas of other sites.     

指突状树突状细胞肉瘤诊治探讨

目的：探讨树突状细胞肉瘤的临床特征、诊断、治疗和预后。方法：对1例指突状树突状细胞肉瘤进行临床病理特点分析,CHOP（环磷酰胺、表阿霉素、长春地辛、地塞米松）方案化疗并观察疗效,复习文献总结国内树突状细胞肉瘤诊治情况。结果：临床表现为全身淋巴结肿大,颈部呈巨大肿块,两肺弥漫性微小结节,伴盗汗、轻度贫血。病理结果示淋巴结结构破坏,瘤细胞成梭形,形成束状,交错状排列。免疫组化S-100（＋）、Vimentin（＋）、Fascin（＋）、Lyso（＋）、Ki-67（＋）,部分肿瘤细胞CD68（＋）。经CHOP方案化疗后达到部分缓解。Langerhans细胞肉瘤罕见,呈高度恶性表现,常累及多个器官,目前国内仅3例报道。指突状树突状细胞肉瘤国内共报道6例,年龄19～41岁（中位33岁）,颈部淋巴结好发,手术后无病生存时间2～15月（中位10月）,18月总生存率40%（2/5）。滤泡树突状细胞肉瘤18例,年龄16～63岁（中位37岁）,发病部位1/3以上在结外,无病生存时间5～72月（中位11.5月）,18月总生存率100%（6/6）。结论：树突状细胞肉瘤诊断依赖病理和免疫组化;治疗尚需规范,可采取手术、放疗、化疗相结合的方案。     

Primary sarcoma of the breast

BACKGROUND AND OBJECTIVES: Primary sarcoma occurring in breast is rare and comprises 0.5-1% of all breast neoplasm. Majority of the series include both stromal and cystosarcoma phyllodes, only a few hundred cases of sarcomas other then cystosarcoma are reported. PATIENTS AND METHODS: We carried out a retrospective analysis of 19 patients with primary sarcoma of the breast treated between 1982 and 2002. RESULTS: Mean age of the patients was 38.6 years (12-70 years). Gradually progressive swelling was the commonest presenting feature. There were eight cases of angiosarcoma, four cases of spindle cell sarcoma, two each of pleomorphic sarcoma and stromal sarcoma, and one each of malignant fibrous histiocytoma, embryonal rhabdomyosarcoma, and sarcoma (NOS). Eight of these were high-grade (42%). Eight patients underwent either radical or modified mastectomy, three underwent wide excisions, and one underwent quadrantectomy. Ten (52.6%) patients received postoperative adjuvant radiation. Two patients received chemotherapy. After a mean follow-up time of 34.5 months (median 25 months), eight patients failed. Failure was local in five, opposite breast in one, and both local and distant in two. The disease free survival at 3-year was 39%. In univariate analysis only the margin of first surgery was found to be a significant predictor of survival (P = 0.05). CONCLUSIONS: Primary sarcomas of the breast are aggressive tumors. Surgical treatment should consist of at least simple mastectomy. All attempts should be made to achieve a negative margin as this appears to be the only factor influencing survival in these patients.     

Primary malignant fibrous histiocytoma of the lung. A clinicopathologic and ultrastructural study of five cases

Primary sarcomas of the lung are rare malignant neoplasms which have usually been classified as fibrosarcomas or as leiomyosarcomas. Only five cases of primary malignant fibrous histiocytoma (MFH) of the lung have previously been reported in the literature. The authors reviewed 10 cases of primary pulmonary sarcoma of the lung seen at their institution during the last 12 years, and five of these cases met the morphologic criteria for the diagnosis of MFH. The morphology in these cases is identical to MFH of other body locations, and shows a combination of fibroblast-like and histiocyte-like cells at both the light and electron microscopic level. By electron microscopic examination there was no evidence for anaplastic carcinoma, rhabdomyosarcoma, or leiomyosarcoma. Of the three patients with adequate follow-up, one patient is alive without evidence of disease at 10 years, one died at 5 years without evidence of recurrent sarcoma, and one died at 7 months with liver metastasis. Two other patients are alive without recurrence at 8 months and 1 year, respectively, following surgery. Because MFH is primarily a tumor of the soft tissues of the extremities, patients with MFH of the lung must be carefully evaluated to rule out a metastatic origin. The reported prognosis in patients with primary MFH of the lung is poor, but early surgical resection does offer the possibility of a cure.     

Sarcoma of the oral and maxillofacial soft tissue in adults.

AIMS: Sarcoma occurring in oral and maxillofacial soft tissue is rare. This study was carried out to evaluate the prevalence of oral soft tissue sarcoma and to record its natural history and survival. METHODS: Retrospective analysis of the patients with histologically proven oral and maxillofacial soft tissue sarcoma treated at the Regional Cancer Centre (RCC), Trivandrum, betweeen 1990-1998 was carried out. RESULTS: During this period, ten cases of oral and maxillofacial sarcomas were registered. Three lesions were located on the cheek mucosa, two on the tongue and two on the mandibular alveolus, while there was one lesion each in the parotid region, maxilla and face (NOS). Mean age at presentation was 31.3+/-14.1 years (range 15-54 years). Seven of the patients (70%) were males. There were three cases of rhabdomyosarcoma (RMS), three cases of spindle cell sarcoma and one case each of angiosarcoma, haemangioendothelioma, malignant schwannoma and malignant fibrous histiocytoma (MFH). All the patients were treated with surgery as a primary modality. Median follow-up time was 30 months (range 5-94 months). An overall srvival of 87.5% at 5 years was observed; however, 5-year disease free survival was 60.0% (95% CI 19.5-85.2). CONCLUSIONS: Soft tissue sarcomas are of comparatively less frequent occurrence in oral and maxillofacial soft tissue than in other tissues. A good survival rate can be achieved by multimodality treatment.     

11例原发性肾肉瘤临床治疗分析

目的:探讨成人原发性肾肉瘤的临床表现、病理特点和预后影响因素.方法:收集1970年1月～2000年12月收治的成人肾脏肿瘤752例,对其中11例原发性肾肉瘤进行回顾性分析.结果:11例中8例以肿块、消瘦为首发症状:11例术后病理确诊为:恶性纤维组织细胞瘤5例,平滑肌肉瘤3例,脂肪肉瘤2例,低分化肉瘤1例.术后无残余瘤组平均生存期为5.8年,残余瘤组术后平均生存10.6个月;恶性纤维组织细胞瘤平均生存期为6个月、平滑肌肉瘤和脂肪肉瘤分别为3.5和10.2年.结论:肿块、消瘦是肾肉瘤的最常见临床症状,病理类型及术后有无残余瘤是决定预后的重要因素.     

Chemotherapy and hormone therapy for uterine sarcomas

Uterine sarcomas are relatively rare mesenchymal malignant neoplasms with poor prognosis, accounting for 8%of all uterine malignant neoplasms. There are only a few moderately active cytotoxic agents for this entity, and therefore, chemotherapy for uterine sarcomas is palliative in most cases. According to traditional classification systems, uterine sarcomas encompass carcinosarcoma(CS), leiomyosarcoma(LMS), and endometrial stromal cell sarcoma(ESS). For carcinosarcoma, ifosfamide, cisplatin, and paclitaxel are reported to be moderately effective single agents. The combination of ifosfamide and cisplatin appeared to improve progression-free survival, but the severe toxicity it induced was not negligible. Paclitaxel and ifosfamide were the only chemotherapy regimen which slightly improved both progression-free and overall survival. For leiomyosarcoma and undifferentiated endometrial sarcoma(formerly named high-grade ESS), doxorubicin, ifosfamide, and gemcitabine are moderately effective single agents. There are several reports showing the effectiveness of gemcitabine plus docetaxel. For endometrial stromal sarcoma(formerly named low-grade ESS), progestins and aromatase inhibitors have been proven beneficial.     

肢体软组织肉瘤34例外科治疗体会

目的探讨肢体软组织肉瘤的诊断与手术疗效。方法回顾性分析外科手术治疗并经病理证实的34例肢体软组织肉瘤患者的诊治资料。结果术后病理:韧带样瘤型纤维瘤病4例,纤维肉瘤5例,浅表型纤维瘤病1例,隆突性皮肤纤维肉瘤4例,恶性纤维组织细胞瘤4例,脂肪肉瘤5例,平滑肌肉瘤5例,恶性周围神经鞘膜瘤2例,滑膜肉瘤2例,原始神经外胚层瘤2例。28例获术后随访1～4 a,4例(14.3%)复发,复发时间12～26个月,平均20个月。结论外科手术是肢体软组织肉瘤最重要的治疗手段,合理应用综合治疗和个体化治疗可提高切除率、降低复发率。     

原发性心脏肉瘤8例临床病理分析

目的:分析原发性心脏肉瘤的临床病理学特征及其预后与分级之间的关系。方法:回顾性分析8例原发性心脏肉瘤的临床资料、组织学形态及免疫组化结果,并进行随访。所有肉瘤均按照FNCLCC分级系统进行分级。结果:心脏肉瘤8例,年龄30~60岁（平均年龄45岁）,男女比例为1∶3,临床表现无特异性,发生部位:左心房4例,左心室1例,右心房1例,右房、右室及部分右室流出道1例,双侧心房1例。肿瘤直径从17 mm到93 mm。具体组织学类型:未分化多形性肉瘤3例,血管肉瘤、上皮样血管内皮瘤、平滑肌肉瘤、胚胎性横纹肌肉瘤、去分化脂肪肉瘤各1例。所有肿瘤被分级为1级（2例）、2级（1例）、3级（5例）。除2例失访外,其余6例均获得随访结果,随访时间4~84个月(中位时间40个月）,其中2例低级别（1级）肿瘤患者均未见复发及转移,4例中、高级别（2、3级）肿瘤患者在术后4~30个月死亡。结论:心脏肉瘤罕见,其中未分化多形性肉瘤与血管肉瘤多见,患者的总体预后差,与肿瘤的分级有关,由于本组样本量少,无法做统计学分析,需要更大的样本量来判断两者有无统计学相关性。     

Prostatic embryonal rhabdomyosarcoma in adults. A clinicopathologic review.

Embryonal rhabdomyosarcoma of the prostate is a rare, highly malignant tumor that occurs predominantly in male infants and children, in whom it is the most common prostatic sarcoma. Six cases occurring in adults have been published, and the authors report three additional cases. The natural history is characterized by rapid growth, with the typical formation of large pelvic or abdominal masses, often leading renal failure due to bilateral ureteric obstruction. The tumor eventually disseminates widely, mainly to the lungs, bone, liver, and serosal surfaces, and unlike most other sarcomas, regional lymph node metastases are common. Combined modality therapy has resulted in marked improvement in survival rates and reduced surgical morbidity for children with these tumors. However, in adults the prognosis remains poor, with all patients dying of disseminated disease within 16 months of histologic diagnosis (mean survival, 8 months).     

Management of primary liver sarcomas

BACKGROUND: Primary hepatic sarcoma is a rare entity. The objectives of the study were to define treatment and long-term outcome and to identify prognostic factors. METHODS: Between January 1981 and December 2004, 30 patients with primary sarcoma of the liver and 5 patients with primary carcinosarcoma of the liver were treated. Patient demographics, tumor characteristics, treatment, and actuarial survival were analyzed. RESULTS: Of the 30 patients with primary hepatic sarcoma (10 epithelioid hemangioendothelioma, 5 embryonal sarcoma, 5 angiosarcoma, 3 solitary fibrous tumor, and 7 other types), 11 underwent R0-resection and had a 5-year disease-specific survival of 64%. Of these 11 patients, 4 had low-grade sarcoma and have not developed tumor recurrence. In the group of 7 patients with high-grade sarcomas who underwent R0-resection, both patients with angiosarcoma died within 11 months, whereas the 5 patients with embryonal sarcoma had a 5-year disease-free and disease-specific survival of 80%. Six of the 10 patients with an epithelioid hemangioendothelioma were managed without surgery, as they had diffuse, slowly progressing, or stable lesions; these patients had a 5-year disease-specific survival of 67%. Of the remaining 13 patients in whom R0-resection was not performed, there were no 3-year survivors. The prognosis for patients with primary carcinosarcoma of the liver was poor, with all but 1 patient dying within a year and no 3-year survivors. CONCLUSIONS: The outcome of patients with primary hepatic sarcoma depends primarily on tumor histology and the ability to achieve complete tumor resection. Improvements in outcome will require the development of more effective systemic therapies.     

Sarcoma metastatic to the brain: a series of 35 cases and considerations from 27 years of experience

The authors report their 27-year experience regarding 35 cases of supratentorial brain metastasis from sarcoma treated in a single institution: these included ten osteosarcomas, seven leiomyosarcomas, five Ewing sarcomas, four malignant fibrous histiocytomas, three alveolar soft-part sarcomas (ASPS), two rhabdomyosarcomas, one liposarcoma, and three unclassified sarcomas. The first 15 cases of the series have already been described in a previous publication. Median survival after craniotomy was 9.8 months (range: 4–24). In patients with preoperative Karnofsky performance score (KPS) > 60 it was 12.8 months (range: 6.5–24 months) versus 5.4 months for those patients with a KPS ≤ 60 (P = 0.01). Eight patients had more than one lesion, six of which were treated in the last ten years. Of the three patients with ASPS, the first two were alive at 15 and 20 months (before being lost to follow-up) whereas the third patient is alive at 24 month follow-up. The authors conclude that surgery is more effective in treating selected patients with sarcoma metastatic to the brain, and that patients with metastasis from ASPS have good prognosis when submitted to surgical treatment. The complete removal of all brain metastases “en bloc” and a KPS > 60 are associated with the best prognosis. Finally, it seems that surgical indications for multiple brain metastases from sarcoma have increased during the last ten years.     

单中心高度恶性软组织肉瘤的诊疗经验

背景与目的：高度恶性软组织肉瘤是一类转移率高、预后差的恶性肿瘤。该研究总结单中心高度恶性软组织肉瘤的治疗经验,以指导制定这类肿瘤患者的个体化治疗方案。方法：对2000年7月—2014年7月在北京大学人民医院骨与软组织治疗中心接受手术及辅助治疗的473例高度恶性软组织肉瘤患者的临床资料进行回顾性分析,随访这些患者复发、转移及生存情况,分析影响预后的因素。结果：共有400例（84.6%）患者接受了保肢治疗。103例患者出现局部复发,148例患者出现肺、骨等远处转移,419例（88.6%）患者接受广泛性切除。370例患者接受术后化疗,153例患者接受局部放疗。平均随访时间为46.9个月（8.0~127.0个月）。随访期间114例患者死亡。3、5及10年总生存率分别为82.6%、69.0%及58.7%。统计学分析显示,肿瘤的组织学类型、辅助化疗及转移情况是影响患者预后的危险因素。结论：对高风险的肿瘤患者应进行积极的辅助治疗,包括通过化疗降低转移风险和局部放疗降低复发率,以期提高这些患者的生存率。     

Primary sarcomas of the kidney. A clinicopathologic and DNA flow cytometric study of 17 cases

Primary sarcomas of the kidney in adults are rare. In the handful of published reports of all soft tissue sarcomas, DNA ploidy has correlated with histologic grade and outcome. This report presents the clinicopathologic and flow cytometric features of 17 cases of primary renal sarcoma (seven men and ten women, ages 28-69 years). Presenting symptoms included abdominal and back pain and hematuria. Stages at diagnosis were I, in three patients; II, five patients; III, two patients; and IV, two patients. Eight tumors were leiomyosarcoma, two malignant fibrous histiocytoma, one hemangiopericytoma, one fibrosarcoma, and five unclassified. Tumors measured 5.5 to 23 cm, seven contained marked nuclear pleomorphism, seven were extensively necrotic, and mitotic rate was 1 to 33 per 10 high-power fields. Seven tumors showed aneuploidy and five were diploid. Thirteen patients were dead of disease after a mean of 23 months and two were alive with known metastases at 29 and 33 months, respectively. Ploidy pattern and outcome or time to death were not correlated, but aneuploidy correlated with histologic grade, marked nuclear pleomorphism (P less than 0.05), extensive necrosis (p less than 0.01), and high mitotic rate (0.05 less than P less than 0.10). The authors conclude that although DNA ploidy does correlate with histologic grade, for primary renal sarcomas, whose prognosis in this series was extremely poor, it does not correlate with outcome.     

Cardiac Sarcomas: An Update

Primary cardiac sarcomas are rare and represent 20% of all primary cardiac tumors. Symptoms depend on the chambers and the cardiac structures involved. Transthoracic echocardiography is commonly used to identify a cardiac mass. The diagnosis of cardiac sarcoma requires adequate sampling and the careful use of ancillary diagnostic techniques. In the most recent histologic classification, angiosarcoma is the most common malignant tumor of the heart with recognizable differentiation. Undifferentiated sarcomas account for one-third of all cardiac sarcomas and have been incorporated in the malignant fibrous histiocytoma/pleomorphic sarcoma subgroup. Elective cardiac sarcoma therapy includes complete surgical excision when possible, followed by radio and chemotherapeutic regimen, the latter preferably containing anthracyclines, ifosfamide, or taxanes. Prognosis of cardiac sarcomas is very poor, with mean survival ranging from 9.6 to 16.5 months. A less-aggressive course seems related to the left atrium location, a low histologic grading with scarce cellular pleomorphism and low-mitotic activity, absence of necrosis, myxoid tumor appearance, and absence of metastasis at diagnosis.     

Results of surgery and multimodal therapy for patients with soft tissue sarcoma invading to vascular structures

BACKGROUND: The aim of this study was to analyze the impact of resection and reconstruction of major vessels on the limb salvage rate, local disease free survival, and overall survival for patients with soft tissue sarcomas invading to neurovascular bundles. METHODS: Twenty patients were treated in a 7-year period by one surgical team. Preoperative therapy consisted of isolated limb perfusion (n = 6), systemic chemotherapy (n = 4), systemic chemotherapy combined with regional hyperthermia (n = 2), and external beam irradiation (n = 1). All patients underwent resection of the sarcoma monobloc together with the neurovascular bundle invaded. Vessels were replaced by an autologous vein transplant or an allograft, and, in six patients, a myocutaneous flap or skin graft had to be used for soft tissue coverage. RESULTS: Histologic examination revealed negative histologic margins (R0-resection) and infiltration of the neurovascular bundle in all patients. In four patients, a local recurrence was observed, and, in three of them, reresection with negative margins was achieved. The mean local recurrence free survival was 54 months (confidence interval [CI], 42-66 months), and the mean overall survival was 48 months (CI, 32-57 months). Limb salvage was achieved in 19 of 20 patients. Eleven patients developed distant metastases after a mean survival time of 30 months. CONCLUSIONS: Extended sarcoma resection, including vessel replacement after preoperative multimodal therapy, provides long term local control and limb salvage. Amputation of extremity sarcoma can hardly be justified, even in cases of tumor invasion to neurovascular bundles. However, efforts to achieve better control over systemic spread are required for long term disease free survival.     

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.     

Sarcomas and malignant phyllodes tumours of the breast--a retrospective study

BackgroundAlthough most breast cancers are adenocarcinomas of the mammary gland, primary breast sarcomas may also arise from mammary gland mesenchymal tissue. The annual incidence of primary breast sarcoma is low and has been estimated at 45 new cases per 10 million women. These tumours are at high risk of recurrence and are known to have poor prognosis. Phyllodes tumours represent a specific subset of these breast soft tissue tumours. They are composed of a connective tissue stroma and epithelial elements. Pathological presentation ranges from grade I to malignant phyllodes tumours (grade III) where the stromal component clearly exhibits a sarcoma pattern.Materials and MethodsSAPHYR (SArcoma and PHYllode Retrospective) is a retrospective study of the experience of Leon Bérard Cancer Centre (Lyon, France) from 1966 to August 2004. SAPHYR aims to describe the characteristics of primary breast sarcomas and to define potential survival factors to be evaluated in future prospective studies.ResultsWe included 70 patients. Half of them presented at least one recurrence (35/70). Median disease-free-survival (DFS) was 1.15 years. At 3 years, median overall survival had not been reached and more than 61% of the patients were alive. Quality of surgical resection was significantly (p = 0.036) different whether patients were in the R0 group (72%) or not (38%). No survival difference was found between malignant phyllodes (grade III) and other primary breast sarcomas (angiosarcomas excluded). Histology revealed three significantly (p = 0.0003) different prognostic groups: phyllodes grade I and II (DFS = 57%), angiosarcomas (DFS = 7%) and phyllodes grade III and other primary breast sarcomas (DFS = 45%).DiscussionPhyllodes tumours and primary breast sarcomas are totally different from epithelial breast cancers and should be considered as a distinct group of rare tumours. The first goal of treatment is to achieve negative margins (R0). We propose to treat the patients according to the clinical practice guidelines in use for soft tissue sarcomas and address them to a reference centre for sarcoma. Treating rare tumours in the same place should permit us to standardise pathological data and to include patients into multicentric radiotherapy or chemotherapy protocols to improve overall survival. As further prospective studies are needed, European oncology groups must join their forces to create a prospective Rare Cancer Network.     

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.     

Doxorubicin and cisplatin chemotherapy in high-grade spindle cell sarcomas of the bone, other than osteosarcoma or malignant fibrous histiocytoma: a European Osteosarcoma Intergroup Study

There are limited data that define the role of chemotherapy in the treatment of high-grade spindle cell sarcomas of bone, other than osteosarcoma or malignant fibrous histiocytoma (MFH-B). This prospective study evaluates the effect of doxorubicin and cisplatin on these tumours. Thirty-seven patients, age 65 years, with spindle cell sarcoma of bone, except osteosarcoma or MFH-B, were included. Chemotherapy consisted of doxorubicin and cisplatin every 3 weeks for six cycles. Resection was performed after three cycles. In 15 patients with metastases, response assessment showed three complete responses (CR), four stable disease (SD), five progression; three were not evaluable. Median time to progression was 30 months (95% Confidence Interval (CI), 8-51 months) for the operable non-metastatic patients; median survival 41 months (95% CI, 16-82 months). Median time to progression in the metastatic group was 10 months (95% CI, 0-18 months) and median survival was 14 months (95% CI, 4-45 months). This study suggests a limited role for doxorubicin and cisplatin in metastatic high-grade spindle cell sarcoma of bone, other than osteosarcoma or MFH-B cases.