Duodenal metastasis of alveolar soft part sarcoma

Aveolar soft part sarcoma is a rare tumor responsible for about 1% of all soft tissue sarcomas, affecting mostly adolescents and young adults. ASPS has curious patterns of metastatic spread, with seldom lymph node involvement. Lung, bone and brain are the most common metastatic places. Small bowel metastasis are infrequent, having found reported only one case of duodenal metastasis with polypous appearance. We describe a case of duodenal metastasis presenting as abdominal mass five years after initial diagnosis of alveolar soft part sarcoma.     

Contrast‐Enhanced Sonography for Monitoring Neoadjuvant Chemotherapy in Soft Tissue Sarcomas

Neoadjuvant chemotherapy is a mainstay in treating soft tissue sarcomas. Soft tissue sarcomas can show an increase in size and central necrosis, with a decrease in the viable tumor, as an initial response to neoadjuvant chemotherapy. Thus, the maximum tumor diameter may not reliably assess the response to this therapy. Contrast‐enhanced sonography may address this limitation. We evaluated 4 patients with soft tissue sarcomas by contrast‐enhanced sonography, performed concomitantly with conventional imaging (computed tomography, magnetic resonance imaging, or positron emission tomography). Quantitative analysis was also performed on 1 sarcoma. A viable, enhancing tumor versus tumor necrosis was nearly identical on contrast‐enhanced sonography and conventional imaging. Preliminary results demonstrate potential for contrast‐enhanced sonographic monitoring of soft tissue sarcomas during neoadjuvant chemotherapy.     

Konsensus Diagnose und Therapie von Weichteilsarkomen

Soft tissue sarcomas are heterogeneous tumours and relatively uncommon. There have been advances over the past years concerning pathology, clinical behaviour, diagnosis strategies and the treatment. To summarize these advances as well as making it public is one of the goals of the following consensus guidelines. But why do we need special guidelines for Austria? There are international guidelines published by the European Society of Medical Oncology (ESMO) and the National Comprehensive Cancer Network (NCCN). The cause is that we need an explanation of the matrix the ESMO and the NCCN gave according to our clinical practice, the local requirements and facilities in Austria. The following recommendations were drawn up following a consensus meeting of sarcoma specialists from the three high volume centres located at the medical universities in Austria. All fields of involved physicians from diagnosis to therapy worked together to know that soft tissue sarcomas are an interdisciplinary challenge and multimodal treatment is essential. For this reason, these guidelines not only explain but also give the state of the art and clear recommendations. One of the most important guidelines is that any patient with a suspected soft tissue sarcoma should be referred to one of the three university centres and managed by a specialist sarcoma multidisciplinary team. We hope that the consensus is helpful for the clinical practice and improves the quality of care for patients with soft tissue sarcomas in Austria.     

Ewing's sarcoma family of tumors: an overview from diagnosis to survivorship

The Ewing's sarcoma family of tumors (ESFT) is a malignant primary bone tumor often involving soft tissue that affects not only children but also young adults. Since 1992, with the addition of ifosfamide and etoposide to standard chemotherapy for primary tumors, much improvement has been made in the treatment of ESFT, with a primary focus on children. Though often recognized as a childhood cancer, it can affect individuals into the middle years of their lives, but little is known about the outcomes of adults with ESFT. ESFT, which includes Ewing's sarcoma, extraosseous Ewing's sarcoma, Askin tumor, and primitive neuroectodermal tumor, is the second most common primary malignant bone tumor in children and adolescents. It accounts for 10% of primary malignant bone tumors in children and 3% of all childhood malignancies. The most common presenting symptoms of ESFT are pain or swelling. Treatment for ESFT consists of a multimodal approach, including chemotherapy, radiation therapy, and surgery. Children and young adults with Ewing's sarcoma face many physical challenges from their illness and the complications of their treatments. Nurses play an instrumental role in assessment techniques, which lead to prompt evaluation and intervention. Nurses are vital in the education and reinforcement of supportive care needs for this patient population.     

Immunotherapeutic effect of allogeneic tumor cells on the growth of autochthonous 3-methylcholanthrene-induced sarcomas in rats.

The tumor-inhibitory effect of injections of allogeneic tumor cells was observed. Established autochthonous sarcomas induced in the subcutaneous tissue of rats by 3-methylcholanthrene (MCA) were treated with immunization by using allogeneic Hirosaki sarcoma cells. When MCA-induced sarcomas grew to approximately 1 cm in mean diameter, Hirosaki sarcoma cells were inoculated into various tissues of primary tumor-bearing rats. Immunizing procedures consisted of intraperitoneal and subcutaneous injections in one experimental group, and of intradermal and intraperitoneal injections in another. Significantly inhibitory effect on the growth of autochthonous sarcomas was observed in the initial stage up to 2 cm in diameter as compared with that of control sarcomas. No significant inhibition was seen in the course of the growth of sarcomas larger than 2 cm in diameter. This results may indicate that immunotherapy by using allogeneic tumor cells should be considered to be valuable for the treatment of human cancer.     

颅底粒细胞肉瘤MRI诊断(附3例报道)

目的提高对颅底粒细胞肉瘤的认识及诊断水平。方法报道3例发生于颅底的粒细胞肉瘤,详细分析了其临床症状、影像学表现,并复习相关文献。结果 3例粒细胞肉瘤均发生于急性髓细胞性白血病的儿童。其中2例以动眼神经麻痹为首发症状来诊,1例确诊为急性粒细胞白血病M2a型后出现头痛来就诊。影像上所有3例颅底骨骨髓信号异常,T_1WI信号减低,并出现髓外软组织肿块。其中1例肿块位于右侧上颌窦外侧壁和鞍旁。1例肿块浸润眼眶、海绵窦和动眼神经。1例广泛浸润脑膜、颅神经根和鼻窦,表现为多结节、多处软组织肿块。MR上所有肿块、结节T_1WI均为呈等信号,T_2WI上为稍低信号,增强扫描显著性强化。结论儿童颅底粒细胞肉瘤可以周围神经病变为首发症状,MRI上如颅底广泛弥漫性骨髓信号异常并出现单发或多发软组织结节或肿块应注意排除粒细胞肉瘤。     

粒细胞肉瘤的诊断与鉴别诊断

目的 探讨粒细胞肉瘤(granulocytic sarcoma,GS)的诊断与鉴别诊断。方法 对12例GS的组织形态学及免疫表型特征进行研究，并结合外周血、骨髓涂片检查及骨髓活检对全部病例进行FAB分型诊断。结果 12例患者均以淋巴结肿大、结外骨及软组织肿块为首发症状。组织学上瘤细胞核呈圆形、卵圆形或不规则形，胞浆少，核分裂易见，呈弥漫分布。骨髓活检示11例有幼稚细胞弥漫单一性增生，形态与髓外浸润的瘤组织相似。1例没有白血病的形态学改变。免疫组化显示瘤细胞表达CD45 100％阳性、溶菌酶100％阳性、MPO 92％阳性、CD68 83％阳性、CD34 42％阳性、TdT 17％阳性。CD15和Mac387仅表达在分化较成熟的粒细胞。CD3、CD45RA，、CD20、CD45RA、CD79A及CD30阴性。结合 外周血及骨髓涂片检查结果确诊1例为孤立性粒细胞肉瘤(非白血病性粒细胞肉瘤)，11例为急性髓系白血病髓外浸润(白血病性粒细胞肉瘤)，11例中1例为AML-Mo，2例为AML-M1，8例为AML-M2。结论 GS在石蜡切片上因其形态学与非霍奇金淋巴瘤极其相似而容易造成误诊，免疫表型及全面的临床检查，特别是外周血、骨髓涂片检查及骨髓活检对明确诊断有很大帮助。     

A phase I trial of hyperthermia-induced interleukin-12 gene therapy in spontaneously arising feline soft tissue sarcomas

Interleukin-12 (IL-12), a proinflammatory cytokine, shows anticancer properties. Systemically administered IL-12 causes dose-dependent toxicity. To achieve localized intratumoral gene expression, an adenoviral gene therapy vector with IL-12 controlled by a heat-inducible promoter (heat shock promoter 70B) was developed and tested in a phase I clinical trial in cats with spontaneously arising soft tissue sarcoma. A feasibility study was done in 16 cats with soft tissue sarcoma using murine IL-12 and/or enhanced green fluorescent protein adenoviral vectors under cytomegalovirus or heat shock promoter 70 control. Subsequently, we conducted a phase I clinical trial using an adenoviral feline IL-12 construct in 13 cats with soft tissue sarcoma. The soft tissue sarcomas were irradiated (48 Gy/16 fractions) followed by intratumoral injection of adenovirus. Twenty-four hours postinjection, tumors were heated (41 degrees C, 60 min). Tumor expression of feline IL-12 and IFN-gamma was determined. Cats were monitored for systemic toxicity. For the murine IL-12 construct, an association was noted between viral dose and murine IL-12 levels within tumor, whereas serum levels were minimal. Mild toxicity was noted at 10(11) plaque-forming units (pfu). With the feline IL-12 construct, high levels of feline IL-12 mRNA were detected in tumor biopsies with low or absent IFN-gamma mRNA following gene therapy. Hematologic and hepatic toxicities were noted at the highest viral doses and were associated with detection of IFN-gamma mRNA in tumor. It is possible to localize gene expression and limit systemic toxicity of IL-12 using the hyperthermia-induced gene therapy approach. The maximum tolerated dose of the feline IL-12 adenoviral vector was 10(10) pfu/tumor as dose-limiting toxicities were noted at the 4 x 10(10) pfu dose.     

Carbon ion radiotherapy for recurrent calf myxoid liposarcoma: a case report

Liposarcoma (LPS) is the most common soft tissue sarcoma. Myxoid LPS (MLPS) is the second most common subtype of LPS and accounts for 25% to 50% of all LPSs. Like most other soft tissue sarcomas, the mainstay of treatment for LPS is inevitably surgery. Multidisciplinary approaches, including surgery, chemotherapy, and radiotherapy, have been successful in the treatment of LPS during the last three decades. Even so, recurrence of LPS remains challenging. Carbon ion beams produce increased energy deposition at the end of their range to form a Bragg peak while minimizing irradiation damage to surrounding tissues, which facilitates more precise dosage and localization than that achieved with photon beams. Furthermore, carbon ion beams have high relative biologic effectiveness. We herein describe a patient who developed recurrent MLPS in the right calf after two surgeries and underwent carbon ion radiotherapy (CIRT), achieving complete disappearance of the tumor. The patient developed Grade 1 radiation dermatitis 30 days after CIRT, but no other acute toxicities were observed. The tumor had completely disappeared by 120 days after CIRT, and the patient remained disease-free for 27 months after CIRT. The CARE guidelines were followed in the reporting of this case.     

Peptide vaccination therapy for bone and soft tissue sarcoma

Bone and soft tissue sarcomas are relatively difficult to treat or cure. Despite a multidisciplinary approach involving surgery, chemotherapy, and radiotherapy, metastatic or relapsed diseases are difficult to cure. Therefore, novel therapeutic options need to be explored. We investigated the use of peptide vaccination therapy for synovial sarcoma and osteosarcoma. The present trial for synovial sarcoma showed the safety, immunogenic properties, and partial clinical efficacy of peptides. However, the power of the vaccine, at least in the present trial, is not strong enough to suppress tumor growth in the presence of gross residual tumors. Therefore, the use of the vaccination therapy as an adjuvant to chemotherapy for the treatment of refractory tumors may be a promising tool.     

18F-FDG PET/CT监测骨与软组织肉瘤术后复发及转移的价值

目的：评价18 F-FDG PET/CT显像监测骨与软组织肉瘤术后复发和/或转移的临床价值及对临床治疗决策的影响。方法回顾性分析32例骨与软组织肉瘤术后行18 F-FDG PET/CT检查患者的随访及临床资料,将PET/CT检查结果与临床随访（再次手术后病理和长期随访观察）的结果进行对比分析,并分析PET/CT对临床决策的影响。结果32例患者,18 F-FDG PET/CT提示局部复发13例,正确诊断12例,假阳性及假阴性各1例,PET/CT诊断骨与软组织肉瘤局部复发的灵敏度、特异性、准确率、阳性预测值及阴性预测值分别为92.3%（12/13）、94.7%（18/19）、93.7%（30/32）、92.3%和94.7%（18/19）;PET/CT提示远处转移11例,正确诊断10例,假阳性1例,假阴性2例,PET/CT诊断远处转移的灵敏度、特异性、准确率、阳性预测值及阴性预测值分别为83.3%（10/12）、95%（19/20）、90.1%（29/32）、90.9%（10/11）和90.5%（19/21）。基于PET/CT结果,37.5%（12/32）患者的治疗方案发生改变。结论18 F-FDG PET/CT在骨与软组织术后复发和/或转移的诊断中具有重要的临床价值,是一种较理想的监测方法。     

FURTHER STUDIES ON SPECIFIC TRANSPLANTATION ANTIGENS IN ROUS SARCOMA OF MICE

Mice allografted with different sarcomas, induced by the Schmidt-Ruppin variant of Rous sarcoma virus (RSV-SR), showed a resistance against subsequent isografting of 9 different Rous sarcomas. Transplantation resistance could also be induced by Rous mouse tumor cells x-irradiated with 8000 r or with cell-free tumor extracts, containing no demonstrable virus. No transplantation resistance could be demonstrated after allograft pretreatment with various polyoma tumors or non-viral tumors. Allograft pretreatment with Rous tumors induced no demonstrable resistance against isografting of polyoma tumors. Inoculation of RSV-SR or Rous chicken sarcoma suspension into adult mice gave no clear cut resistance against isografting of mouse sarcomas. Neither after allografting of Rous tumors nor after virus or chicken sarcoma inoculation into adult mice could virus-neutralizing activity be demonstrated in the sera. The results demonstrate the presence of common, specific transplantation antigen(s) in different Rous sarcomas in mice and speak against an identity between the transplantation antigen(s) and viral antigen(s).     

The role of MR imaging in soft tissue tumor evaluation: perspective of the orthopedic oncologist and musculoskeletal pathologist

Soft tissue masses are common in both children and adults. Clinicians must evaluate patients carefully to avoid management errors. The most effective management decisions are made when a working group composed of clinicians, radiologists, and pathologists participates in the interpretation of the imaging studies. Plain-film radiographs and MR imaging scans are the two main imaging modalities used in patients with soft tissue masses. The working group assimilates the clinical and radiographic data to determine if they can identify the nature of the soft tissue mass. When the group can assign a definitive diagnosis, the lesion is designated as a determinate lesion. Determinate lesions include lipomas, ganglions, hemangiomas, neurofibromas, diabetic myonecrosis, muscle tears, myositis ossificans (heterotopic ossification), and pigmented villonodular synovitis. When the process cannot be identified, the lesion is classified as indeterminate. All soft tissue sarcomas are indeterminate lesions. Many benign lesions are also indeterminate. Common examples include schwannomas, myxomas, and giant cell tumor of tendon sheath. Based on the clinical and radiologic features, these diagnoses may be suspected, but because of the inability to distinguish them from sarcomas based on the MR imaging features, they are usually classified as indeterminate. When lesions are judged to be determinate, observation or excisional biopsy are the two major treatment choices. When lesions cannot be identified on the imaging studies, incisional or needle biopsy is performed to establish a diagnosis. Once a diagnosis is made, the proper management choice can be selected. Inappropriate excisional biopsy is the major treatment error in the management of soft tissue tumors. When a high-grade soft tissue sarcoma is resected with multiple positive margins, the risk of local failure after definitive resection is much higher than if the patient had been treated initially with only a needle or incisional biopsy. Also, if a major complication, such as an infection, a major wound-healing problem, or contamination of the major neurovascular structures, occurs at the time of incisional biopsy, amputation of the limb may be necessary. Inappropriate excisional biopsy can occur when a surgeon is not familiar with the features of sarcomas or when a radiologist mistakenly interprets the signal features as a benign lesion.     

Establishment and characterization of a clonal human extraskeletal Ewing's sarcoma cell line, EES1

Ewing's sarcoma, a small round cell sarcoma arising in soft tissue as well as the bone, is one of the most malignant tumors in children and young adults. Few established cell lines of extraskeletal Ewing's sarcoma (EES) have been reported, which made it difficult to examine the biological features of EES. Therefore, we have established a new clonal cell line of EES. We report its morphological characters, results of chromosomal and immunohistochemical analysis. A piece of tumor obtained from the 18-year-old female patient with EES was xenografted in a nude mouse. In vitro subcultured cells were then obtained from this xenograft. A clonal cell line was subsequently established by limiting dilution and designated EES1. EES1 cells had a doubling time of 24 hours. In the xenografted tumor, the cells expressed vimentin, CD99 (MIC2), neuron specific enolase (NSE) and cytokeratin. The original tumor cells also expressed vimentin, CD 99, and NSE, but was negative for cytokeratin. The morphological and immunohistochemical features of this cell line established, except for cytokeratin expression, were consistent with those of the primary tumor. Cytogenetic analysis of EES1 revealed chromosomal translocation of t(11; 12)(q24;ql2). The chimeric fusion of the Ewing's sarcoma gene in band 22q12 with the Friend leukemia virus integration-1 gene in band 11q24 was also demonstrated. Fluorescence in situ hybridization further confirmed the presence of translocation involving the Ewing's sarcoma gene in both the primary tumor and EES1 cells. In conclusion, we have established a human EES cell line EES1, which will provide a useful model for studying various aspects of human EES.     

躯干和肢体软组织肉瘤的围手术期化疗与放疗

虽然手术是肢体和躯干原发软组织肉瘤的根治性治疗手段,但对于高危患者单纯手术常难以获得疾病的长期控制。治疗失败的主要原因为远处转移,其次为局部复发。一些局部进展期的患者可能面临无法手术切除,或仅边缘可切除,因此预后很差。围手术期的系统治疗和放疗可提高手术疗效。如何确立围手术期系统治疗的价值颇具挑战。系统治疗可以缩小肿瘤。但要证明其对局部控制的价值从而促进实施更为保守的外科手术的证据仍不充分。对于那些原发肿瘤被认定为难以或不可能切除的患者,新辅助化疗可能为疾病的局部控制提供一种选择。围手术期的系统治疗也可以避免远处转移的发生。近期的研究表明,以蒽环类药物/异环磷酰胺为基础的辅助治疗能够使患者获益,虽然证据来自于对一项大型研究的再次分析。一项新辅助化疗的随机研究提示,对某些经过选择的肉瘤组织学亚型,以蒽环类药物/异环磷酰胺为基础的系统化疗可能改善患者无病生存和总生存期。其疗效的获得主要是通过改善远处无转移生存而不是局控率的提高。这两项研究所采用的方法均存在一定的局限性,因此需要进一步探索。尽管如此,我们相信这些结果支持在局部进展期、肢体和躯干原发的软组织肉瘤患者中进行新辅助化疗。放疗与手术结合在软组织肉瘤的治疗中有确切的疗效。虽然预后并不因放疗和手术的先后顺序而异,但不良反应却存在差异。辅助放疗较新辅助放疗有更低的围手术期伤口并发症,但相对于新辅助放疗而言,辅助放疗需要更高的放射剂量,对长期功能的预后差于新辅助放疗。因此,我们相信围手术期放疗与化疗一样,在可能的情况下,应尽可能在术前进行。     

NCCN软组织肉瘤临床实践指南腹膜后软组织肉瘤部分解读

软组织肉瘤是一类来源于结缔组织的少见恶性肿瘤。美国国立综合癌症网络(NCCN)持续更新并发布了关于软组织肉瘤的临床实践指南。本文对其中腹膜后软组织肉瘤(retroperitoneal sarcoma,RPS)部分进行解读,主要涉及RPS治疗流程中多学科诊疗模式(MDT)的应用及其分级分期、活检原则、手术原则、放疗治疗原则等内容,为其临床规范诊治提供参考。     

18FDG-PET术前评估骨与软组织肿瘤治疗效果的Meta分析

背景:18FDG-PET可为骨和软组织肿瘤的良恶性鉴别,病变定位,恶性程度评价,活检部位的确定,治疗效果评估和判断预后提供重要信息。目的:采用Meta分析方法评价18FDG-PET在术前骨与软组织肿瘤治疗疗效评估方面的应用价值。方法:检索PubMed、EMBASE、Cochrance、CNKI、万方数据库及维普中文科技期刊数据库获得PET用于骨与软组织肿瘤诊治方面的临床研究。结果与结论:对符合纳入标准的9项临床非随机对照研究进行Meta分析,文献质量评价循证医学推荐级别3项研究为B级,6项为C级,检验提示无发表偏倚。将治疗前后最大标准摄取值SUV2/SUV1≤0.5作为预测术后肿瘤坏死率≥90%的敏感性和特异性分别为0.82(95%CI,0.72～0.89)和0.61(95%CI,0.49～0.71)。SROC为6.17(95%CI,2.84～13.39)。说明将化疗前后最大标准摄取值比值作为评价术前疗效的敏感性较好,但缺乏特异性;应综合18FDG-PET检查结果与其他辅助检查结果对术前疗效进行综合评估。     

Descriptive epidemiology of soft tissue and bone sarcomas in Lebanon

ObjectivesMost epidemiologic studies on soft tissue sarcomas (STS) and bone sarcomas (BS) are performed in western countries, with few in the Middle East and North Africa region. We describe the epidemiology of sarcomas in Lebanon using the medical records database at the American University of Beirut Medical Center (AUBMC).MethodsThis single-center retrospective cohort study included patients with sarcomas registered in the database between 2015 and 2019. Their charts were reviewed for baseline characteristics, tumor biology and location, treatment modalities, recurrence, metastasis, and death.ResultsThe cohort included 234 patients with STS and 99 patients with BS. Most tumors were <10 cm in size. The most common subtypes were liposarcoma for STS and osteosarcoma for BS. The most common location of STS was the thigh. The most frequent sites of STS metastasis were the lungs. Histological subtype, smoking status, and tumor size and grade were significant for progression-free survival (PFS) in patients with STS. By multivariable analysis, smoking was significantly associated with poorer PFS in STS. For BS, only tumor grade was significant for PFS.ConclusionThe epidemiology of sarcomas at AUBMC is similar to that previously reported. Smoking history was associated with poorer survival in patients with STS.     

颌面部骨源性肿瘤与类肿瘤病变的CT表现

目的回顾性分析颌面部骨源性肿瘤与类肿瘤病变的CT表现,旨在提高对此类病变CT表现的认识.方法回顾颌面部骨源性肿瘤与类肿瘤病变20例,与病理对照,分析各类病变的影像学特点.结果恶性肿瘤8例,CT表现为不规则软组织肿物,其中骨肉瘤3例,明显溶骨性骨质破坏3例,肿物内均有瘤骨形成;软骨肉瘤3例,局部有骨质破坏,肿物内有钙化灶者2例,有瘤骨及瘤软骨形成者1例;尤文氏肉瘤1例,内见钙化;良性肿瘤及类肿瘤病变7例,为边缘规则肿物,未见钙化、瘤骨及骨膜反应.成釉细胞瘤5例,为边缘规则的分房状肿物、骨质膨胀性破坏4例,广泛侵犯1例.骨纤维异常增殖症4例,均为多骨膨胀性改变,其内骨结构紊乱,正常骨小梁消失.结论各类颌面部骨源性肿瘤与类肿瘤病变有不同的影像学特点.CT扫描对颌面部骨源性的肿瘤与类肿瘤病变定性诊断及侵犯范围的准确评估有重要意义.