Non-rhabdomyosarcoma Soft Tissue Sarcomas Research Articles

Autologous hematopoietic stem cell transplantation following high-dose chemotherapy for nonrhabdomyosarcoma soft tissue sarcomas.

Soft tissue sarcomas (STS) are a highly heterogeneous group of rare malignant solid tumors. Nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) comprise all STS except rhabdomyosarcoma. In people with advanced local or metastatic disease, autologous hematopoietic stem cell transplantation (HSCT) applied after high-dose chemotherapy (HDCT) is a planned rescue therapy for HDCT-related severe hematologic toxicity. The rationale for this update is to determine whether any randomized controlled trials (RCTs) have been conducted and to clarify whether HDCT followed by autologous HSCT has a survival advantage. To assess the efficacy and safety of high-dose chemotherapy (HDCT) followed by autologous hematopoietic stem cell transplantation (HSCT) for all stages of nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) in children and adults. For this update, we revised the search strategy to improve the precision and reduce the number of irrelevant hits. We searched the following electronic databases: Cochrane Central Register of Controlled Trials (CENTRAL; 2016, Issue 8), PubMed from 2012 to 6 September 2016, and Embase from 2012 to 26 September 2016. We searched online trial registries and congress proceedings from 2012 to 26 September 2016. Terms representing STS and autologous HSCT were required in the title or abstract. We restricted the study design to RCTs. We included studies if at least 80% of participants had a diagnosis listed in any version of the World Health Organization (WHO) classification and classified as malignant. The search included children and adults with no age limits. We used standard methodologic procedures expected by Cochrane. The primary outcomes were overall survival and treatment-related mortality. We identified 1549 records; 85 items from electronic databases, 45 from study registries, and 1419 from congress proceedings. The revised search strategy did not identify any additional RCTs. In the previous version of the review, we identified one RCT comparing HDCT followed by autologous HSCT versus standard-dose chemotherapy (SDCT). The trial randomized 87 participants who were considerably heterogeneous with respect to 19 different tumor entities. The data from 83 participants were available for analysis.In the single included trial, overall survival at three years was 32.7% in the HDCT arm versus 49.4% in the SDCT arm and there was no difference between the treatment groups (hazard ratio (HR) 1.26, 95% confidence interval (CI) 0.70 to 2.29, P = 0.44; 1 study, 83 participants; high quality evidence). In a subgroup of participants who had a complete response before HDCT, overall survival was higher in both treatment groups and overall survival at three years was 42.8% in the HDCT arm versus 83.9% in the SDCT arm and favored the SDCT group (HR 2.92, 95% CI 1.1 to 7.6, P = 0.028; 1 study, 39 participants).In the single included trial, the authors reported one treatment-related leukemia death two years after HDCT. They also evaluated severe adverse events WHO grade 3 to 4 in 22 participants in the HDCT arm and in 51 participants in the SDCT arm. The authors reported 11 events concerning digestive-, infection-, pain-, or asthenia-related toxicity in the HDCT arm and one event in the SDCT arm (moderate quality evidence). The development of secondary neoplasia was not addressed. We judged the study to have an overall unclear risk of bias as three of seven items had unclear and four items had low risk of bias. For GRADE, we judged three items as high quality and three items were not reported. The limited data of a single RCT with an unclear risk of bias and moderate to high quality evidence showed no survival advantage for HDCT. If this treatment is offered it should only be given after careful consideration on an individual person basis and possibly only as part of a well-designed RCT.

Pediatric nonrhabdomyosarcoma soft tissue sarcomas arising at visceral sites

Pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) may rarely occur in visceral tissues, and little is known about their clinical history. The present study retrospectively analyzed a group of patients prospectively registered in Italian pediatric protocols conducted between 1979 and 2004. Inclusion criteria for the study were as follows: a pathological diagnosis of "adult-type NRSTS," arising at visceral sites (lung-pleurae, liver, kidney, and mesentery-bowel); age under 18 years; no previous treatment except for primary surgery; available clinical data; and written consent. Thirty cases with visceral NRSTS were collected and analyzed. Sites of origin were as follows: mesentery-bowel in 12 cases, lung-pleurae in 11, liver in 5, and kidney in 2. According to the Intergroup Rhabdomyosarcoma Study (IRS) surgical grouping system, patients were classified as follows: nine IRS group I, three group II, 12 group III, and six group IV. Patients were treated with a multimodal approach including surgery, radiotherapy, and/or chemotherapy, according to their characteristics. For the series as a whole, the 5-year event-free and overall survival rates were 33.3% and 40.0%, respectively. The IRS group (reflecting the feasibility of initial complete resection) emerged as the main prognostic factor. Survival rates also correlated with tumor size and local invasiveness, histological subtype, and tumor sites (the worst outcome was seen for tumors arising in the lung and pleurae). This study confirmed that visceral NRSTS are aggressive tumors carrying a worse prognosis than pediatric NRSTS arising in soft tissues of the extremities. Local treatment remains the main challenge for these tumors.

Re-excision after unplanned resection of nonmetastatic nonrhabdomyosarcoma soft tissue sarcoma in children: Comparison with planned excision

BackgroundIt is not exceptional to come by children with initial unplanned surgical intervention for nonrhabdomyosarcoma soft tissue sarcomas (NRSTS). The aim of this study was to evaluate the presence of residual disease in these patients after re-excision and compare the treatment outcomes with patients who had planned upfront excision. MethodsThe data of patients with primary nonmetastatic NRSTS with initially unplanned excision who underwent re-excision between March 2006 and December 2014 were analyzed and the results compared with patients having planned upfront excision in the similar period. ResultsOf the 84 patients, 40 (48%) had an unplanned excision elsewhere; 35 of these patients had a re-excision. Twenty-one of the remaining 44 patients underwent upfront planned excision. A residual tumor was present in 16 (45.7%) patients. There was no significant difference in the local recurrence, distant metastases, or deaths in patients with re-excision or planned excision. The 5-year overall, disease-free survival and local control rates were, respectively, 93.5%, 90.2% and 96.6% for the re-excision group and 84.9%, 65.2% and 88.5% in the planned excision group (p=NS). ConclusionsThe probability of residual disease following unplanned excision of NRSTS is high. The outcomes following re-excision are similar to that with planned excision. Prognosis studyLevel II evidence.

Access to clinical trials for adolescents with soft tissue sarcomas: Enrollment in European pediatric Soft tissue sarcoma Study Group (EpSSG) protocols.

Adolescents with cancer are enrolled in clinical trials at far lower rates than children. This report compares the number of adolescents (15-19-year-olds) and children (0-14-year-olds) enrolled in the protocols of the European pediatric Soft tissue sarcoma Study Group (EpSSG) with the number of cases expected to occur. The observed-to-expected (O/E) ratio was detected in the EpSSG countries contributing most of the cases, that is, Italy, France, Spain, the Netherlands, United Kingdom, and Ireland. The observed cases included patients enrolled in any of the EpSSG protocols from October 2008 to October 2015, when all EpSSG protocols were open in these countries. The number of expected cases was calculated from the incidence rates estimated throughout the RARECAREnet database in the countries' population-based cancer registries. In the countries considered, 2,118 cases aged 0-19 years were enrolled in the EpSSG trials from 2008 to 2015: 82.8% were children and 17.2% were adolescents. The O/E ratio was 0.30 among patients 15-19 years old, as opposed to 0.64 for those 0-14 years old. The O/E ratio differed for the different subtypes: in adolescents, it was 0.64 and 0.18 for rhabdomyosarcoma (RMS) and non-rhabdomyosarcoma soft tissue sarcomas (NRSTS), respectively; in children, it was 0.77 and 0.50, respectively. The O/E ratios differed across the countries considered. Adolescents were less well represented than children on the EpSSG protocols, with better enrolment for RMS than for NRSTS for all age groups.

Low-grade Nonrhabdomyosarcoma Soft Tissue Sarcoma: What is Peculiar for Childhood.

Nearly half of soft tissue sarcomas are nonrhabdomyosarcomas (NRSTSs). The low-grade (LG) form comprises a heterogenous group of diseases that rarely metastasize but are known for local recurrence. The aim of the study was to retrospectively evaluate pediatric LG-NRSTS with regard to demography, survival, and factors affecting outcome in Egyptian patients. The study reviewed 66 NRSTS patients who presented to the Pediatric Oncology Department, National Cancer Institute, Cairo University, between January 2008 and December 2013. Out of the reviewed cases 32 patients had LG tumors and were eligible for analysis. The male to female ratio was 1:1 and the median age was 7.5 years (range, 1 mo to 18 y). Desmoid fibromatosis (N=18) showed frequent local recurrence and nearly half of this group was alive without disease. No recurrence of the disease occurred in the nonfibromatosis group (n=14) and all patients were alive and free of disease. The 5-year overall survival was 88% for the entire group of study patients versus 45% for event-free survival. Tumors >5 cm in diameter and fibromatosis histology subtype were associated with lower EFS. LG-NRSTS generally has good prognosis, with overall survival reaching 90%. However, aggressive fibromatosis usually runs a poorer course in the form of high incidence of local recurrence and lower survival rates. This needs to be further assessed in larger prospective studies including novel therapies in addition to the current conventional modalities.

Non-Rhabdomyosarcoma Soft Tissue Sarcoma in Pediatric and Young Adult Patients: Results From a Prospective Study Using Limited Margin Radiation Therapy

Non-Rhabdomyosarcoma Soft Tissue Sarcoma in Pediatric and Young Adult Patients: Results From a Prospective Study Using Limited Margin Radiation Therapy

Non-rhabdomyosarcoma

Pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) are a heterogeneous group encompassing more than 50 different histological diagnoses arising from primitive mesenchymal tissue. Together, they comprise about half the soft tissue sarcomas diagnosed in children and young adults. Despite each histologies relative rarity, their management schema is similar among the different NRSTS histologies. Surgical management is an important component of the multimodal treatment strategy of all these tumors. Resection with negative margins, while maintaining function, plays an important role as a primary treatment of these patients as well as diminishing the risks of local and distant recurrence.

Opportunities for Improvement in Pathology Reporting of Childhood Nonrhabdomyosarcoma Soft Tissue Sarcomas: A Report From Children's Oncology Group (COG) Study ARST0332.

Treatment of soft tissue tumors in young patients relies on the diagnostic information conveyed in the pathology report. We examined pathology reports from Children's Oncology Group ARST0332 for inclusion of data elements required in published guidelines. Pathology reports for 551 eligible patients were examined for required data elements defined by the College of American Pathologists, including tissue type, procedure, tumor site, tumor maximum diameter, macroscopic extent of tumor, histologic type, mitotic rate, extent of necrosis, tumor grade, margin status, use of ancillary studies, and pathologic stage. Only 65 (12%) of 551 reports included all required data elements. Of reports containing synoptic templates, 57% were complete. This study reveals significant opportunity to improve the quality of pathology reports in young patients with soft tissue tumors. Use of templates or checklists improves completeness of reports.

BERNIE: Open-label, randomized, phase II study of bevacizumab plus chemotherapy in pediatric metastatic rhabdomyosarcoma (RMS) and non-rhabdomyosarcoma soft tissue sarcoma (NRSTS).

11054Background: Thephase II BERNIE study evaluated the role of bevacizumab as part of the multi-modality treatment of children and adolescents with metastatic RMS/NRSTS. Methods: Eligible patients...

Outcome of extracranial malignant rhabdoid tumours in children registered in the European Paediatric Soft Tissue Sarcoma Study Group Non-Rhabdomyosarcoma Soft Tissue Sarcoma 2005 Study—EpSSG NRSTS 2005

BackgroundExtracranial malignant rhabdoid tumours (MRT) are rare lethal childhood cancers that often occur in infants and have a characteristic genetic mutation in the SMARCB1 gene. The European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) conducted a multinational prospective study of registered cases of extracranial MRT to test an intensive multimodal approach of treatment for children with newly diagnosed extracranial MRT. MethodsBetween December 2005 and June 2014, we prospectively registered 100 patients from 12 countries with a diagnosis of MRT tumour at an extracranial site on the EpSSG Non-Rhabdomyosarcoma Soft Tissue Sarcoma 2005 Study (NRSTS 2005). They were all treated on a standard multimodal protocol of surgery, radiotherapy, and chemotherapy over 30 weeks as follows: vincristine, cyclophosphamide, and doxorubicin (VDCy) at weeks 1, 10, 13, 22, and 28; vincristine was also given alone on weeks 2, 3, 11, 12, 14, 15, 23, 24, 29, and 30. Cyclophosphamide, carboplatin, and etoposide (Cy*CE) was given at weeks 4, 7, 16, 19, and 25. Radiotherapy was recommended for all primary tumour sites and all sites of metastatic disease. ResultsForty-three patients completed the protocol treatment. Median follow-up for alive patients of the complete cohort was 44.6 months (range 11.5–84.6). For the whole cohort, the 3-year event-free survival (EFS) was 32.3% (95% confidence interval [CI] 23.2–41.6%) with a 3-year overall survival (OS) of 38.4% (95% CI 28.8–47.9%). For localised disease, the 4-year EFS was 39.3% (95% CI 28.2–50.1%) with a 4-year OS of 40.1% (95% CI 28.4–51.5%). For metastatic disease, the 2-year EFS was 8.7% (95% CI 1.5–24.2%) with a 2-year OS of 13.0% (95% CI 3.3–29.7%). Multivariable analysis disclosed that all patients ≤1 year of age were associated with at higher risk of death (hazard ratio [HR]: 2.6; 95% CI 1.0–6.8; p-value = 0.0094). Risk of death was also related with gender in metastatic patients (HR for males: 2.9, 95% CI 1.0–8.0; p-value = 0.0077). ConclusionsThe EpSSG NRSTS 2005 protocol of intensive therapy can be delivered to extracranial MRT patients, with a possible improvement in outcome. The outcome, however, remains poor for patients who progress or with metastatic disease.

Pediatric non-rhabdomyosarcoma soft tissue sarcomas: multimodality approach results and validation of American Children's Oncology Group risk stratifications——a study of single-institutional series

Objective To investigate the clinical characteristics, survival, factors of prognosis and therapeutical strategy of pediatric non-rhabdomyosarcoma soft tissue sarcomas(NRSTS). Methods There were 34 cases of NRSTS treated in Department of Orthopaedic Oncology, Peking University Cancer Hospital during July 2007 and March 2015.The clinical and pathological data and outcome of multimodality approach were retrospectively studied, the survival analysis was performed. Results There were 21 male and 13 female cases, and the median age was 14 years, ranging from 7 to 18 years old.Mean and median size was 5.0 cm and 3.0 cm, ranged 0.5-22.0 cm.Synovial sarcoma was the most common type among the 9 sorts of histologies[29.4%(10/34 cases)], and limb was the most common site [67.6%(23/34 cases), accounting for 50.0% (17/34 cases)by lower limb]. There were 28 cases, 2 cases, 2 cases, 2 cases in Intergroup Rhabdomyosarcoma Study (IRG) groupⅠ, Ⅱ, Ⅲ, Ⅳ, respectively, and 23 cases, 7 cases, 4 cases in Children's Oncology Group (COG) low, moderate and high risk group respectively.The median follow-up was 35 months, ranging from 6 to 96 months.There were 2 cases of local recurrence, 1 case of distant recurrence, 2 cases of metastases of lymph node and distant sites, and 1 case of local and distant recurrence.The 2-year overall survival rate was 89.2%, 100.0%, 70.0% for total group, COG low risk group, COG moderate and high risk group, respectively.For 28 cases of tumors without any nodular or distant metastasis, the 2-year local recurrence-free survival and distant recurrence-free survival rate was 96.4% and 87.6% respectively.IRS group, COG risk group and size of tumor were associated with the prognosis.The groups of completely resected tumor(P=0.007), COG low risk (P=0.014)and tumors no larger than 5.0 cm in diameter(P=0.037) had more favorable overall survivals.There were 14 cases, 10 cases, 4 cases, 2 cases, 2 cases, 2 cases who had surgery alone, surgery plus chemotherapy, surgery plus chemoradiotherapy, surgery plus radiation, chemoradiotherapy alone and chemotherapy alone, respectively, as multimodality approaches.Mesna+ Adriamycin+ Ifosfamide regimen(Pirarubicin 40 mg/m2, Ifosfamide 10 g/m2) was the main(17/18 cases) choice as chemotherapy, with a total and mean cycles of chemotherapy for every case of 2-12 and 4.6 respectively. Conclusions This series of NRSTS showed favorable oncological outcomes after a multimodality approach composed of surgery, radiation and chemotherapy on MAI regimen.The COG risk stratifications were firstly adopted in domestic study of pediatric NRSTS, with prognostic efficacy being validated.Risk-based multimodality approach was also recommended. Key words: Non-rhabdomyosarcoma soft tissue sarcomas; Survival analysis; Risk stratifications; Multimodality approach

Nonrhabdomyosarcomatous abdominopelvic sarcomas: Analysis of prognostic factors.

Background:Data concerning treatment outcome and prognostic factors in sarcomas of abdomen and pelvis are sparse in literature. Methods and Results:Of 696 patients with nonrhabdomyosarcomatous soft tissue sarcoma registered at our center between June 2003 and December 2012, 112 (16%) patients of sarcomas arising from abdomen and pelvis were identified, of which 88 patients were analyzed for treatment outcome and prognostic factors. The median age was 40 years (range: 1–78 years) with a male: female ratio of 0.7:1. Twenty-one (24%) patients were metastatic at baseline. The most common tumor sites were retroperitoneum in 70% patients and abdominal wall in 18% patients. Leiomyosarcoma was the most common histological subtype in 36% patients followed by liposarcoma in 17% patients. Thirty-five (40%) patients had Grade III tumors. Forty-six (52%) patients underwent surgical resection. At a median follow-up of 43 months (range: 2–94 months), the 5-year event-free survival (EFS) and overall survival (OS) were 35% and 42%, with a median of 22 months and 43 months, respectively. Multivariate analysis identified male gender (P - 0.03, hazard ratio [HR] - 0.46, 95% confidence interval [CI] - 0.23–0.92), baseline metastatic disease (P - 0.01, HR - 2.98, 95% CI - 1.27–6.98) and Grade III tumors (P - 0.02, HR - 1.84, 95% CI - 1.08–3.13) as factors associated with poor EFS, whereas baseline metastatic disease (P < 0.001, HR - 5.45, 95% CI - 2.31–12.87) and unresectability (P - 0.01, HR - 2.72, 95% CI - 1.27–5.83) were associated with poor OS.Conclusion:This is a single-institutional study of patients with abdominopelvic sarcomas where gender was identified as a new factor affecting survival apart from baseline presentation, histologic grade, and surgical resection.

Monophasic synovial sarcoma: a case report and literature review

Synovial sarcoma is an aggressive soft tissue tumor which accounts for 7% - 8% of all human malignant sarcomas. Although this tumor generally affects adults, about 30% of reported cases occur in children and adolescents. Synovial sarcoma is the most common malignant non-rhabdomyosarcomatous soft tissue sarcoma in children and adolescents. We report a case of synovial sarcoma presented with pain and swelling in the left knee. MRI of left knee shows 25×54×56 mm soft tissue lesion in infrapatellar bursa. FNAC left knee swelling reported malignant spindle cell tumor. The mass was completely resected in a surgical procedure. In pathological examination of the mass, monomorphic fibroblastic synovial sarcoma was reported. Patient was treated with Ifosfamide and Adriamycin.

Trends in survival for teenagers and young adults with cancer in the UK 1992–2006

BackgroundAlthough relatively rare, cancer in teenagers and young adults (TYA) is the most common disease-related cause of death and makes a major contribution to years of life lost in this age group. There is a growing awareness of the distinctive needs of this age group and drive for greater understanding of how outcomes can be improved. We present here the latest TYA survival trends data for the United Kingdom (UK). MethodsUsing national cancer registry data, we calculated five-year relative survival for all 15–24year olds diagnosed with cancer or a borderline/benign CNS tumour in the UK during the periods 1992–1996, 1997–2001 and 2002–2006. We analysed trends in survival for all cancers combined and for eighteen specified groups that together represent the majority of TYA cancers. We compared our data with published data for Europe, North America and Australia. ResultsFive-year survival for all cancers combined increased from 75.5% in 1992–1996 to 82.2% in 2002–2006 (P<0.001). Statistically significant improvements were seen for all disease groups except osteosarcoma, rhabdomyosarcoma, non-gonadal and ovarian germ cell tumours and ovarian and thyroid carcinomas. During the earliest time period, females had significantly better survival than males for five of the twelve non-gender-specific disease groups. By the latest period, only melanomas and non-rhabdomyosarcoma soft tissue sarcomas had differential survival by gender. Survival in the UK for the most recent period was generally similar to other comparable countries. ConclusionFive-year survival has improved considerably in the UK for most cancer types. For some disease groups, there has been little progress, either because survival already approaches 100% (e.g. thyroid carcinomas) or, more worryingly for some cancers with poor outcomes, because they remain resistant to existing therapy (e.g. rhabdomyosarcoma). In addition, for a number of specific cancer types and for cancer as a whole males continue to have worse outcomes than females.

Non-Rhabdomyosarcoma Soft Tissue Sarcomas in Children: A Surveillance, Epidemiology, and End Results Analysis Validating COG Risk Stratifications

Non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) are a heterogeneous group of sarcomas that encompass over 35 histologies. With an incidence of ∼500 cases per year in the United States in those <20 years of age, NRSTS are rare and therefore difficult to study in pediatric populations. We used the large Surveillance, Epidemiology, and End Results (SEER) database to validate the prognostic ability of the Children's Oncology Group (COG) risk classification system and to define patient, tumor, and treatment characteristics. From SEER data from 1988 to 2007, we identified patients ≤18 years of age with NRSTS. Data for age, sex, year of diagnosis, race, registry, histology, grade, primary size, primary site, stage, radiation therapy, and survival outcomes were analyzed. Patients with nonmetastatic grossly resected low-grade tumors of any size or high-grade tumors ≤5 cm were considered low risk. Cases of nonmetastatic tumors that were high grade, >5 cm, or unresectable were considered intermediate risk. Patients with nodal or distant metastases were considered high risk. A total of 941 patients met the review criteria. On univariate analysis, black race, malignant peripheral nerve sheath (MPNST) histology, tumors >5 cm, nonextremity primary, lymph node involvement, radiation therapy, and higher risk group were associated with significantly worse overall survival (OS) and cancer-specific survival (CSS). On multivariate analysis, MPNST histology, chemotherapy-resistant histology, and higher risk group were significantly poor prognostic factors for OS and CSS. Compared to low-risk patients, intermediate patients showed poorer OS (hazard ratio [HR]: 6.08, 95% confidence interval [CI]: 3.53-10.47, P<.001) and CSS (HR: 6.27; 95% CI: 3.44-11.43, P<.001), and high-risk patients had the worst OS (HR: 13.35, 95% CI: 8.18-21.76, P<.001) and CSS (HR: 14.65, 95% CI: 8.49-25.28, P<.001). The current COG risk group stratification for children with NRSTS has been validated with a large number of children in the SEER database.

Soft tissue sarcoma and radiation therapy advances, impact on toxicity.

Since adjuvant radiotherapy was introduced in the 1970s for soft tissue sarcoma (STS), sequential clinical trials characterized the toxicities induced by radiotherapy when given post-operatively and pre-operatively. Gradual technological advancements led to more precise radiotherapy delivery through intensity-modulated radiation therapy (IMRT) and more accurate targeting through image-guided radiotherapy (IGRT) to minimize normal tissues from high-dose irradiation. These improvements ultimately reduced the long-term toxicities from radiotherapy. Due to the rarity and complexity of the disease, patients with STS should be treated at institutes where multidisciplinary discussion and care can be provided. Patients with STS should ideally be offered the choice of participating in clinical trials. International phase III trials are ongoing through COG-NRG Oncology (Pazopanib Neoadjuvant Trial in Non-Rhabdomyosarcoma Soft Tissue Sarcomas (PAZNTIS)) to define the role of radiotherapy in combination with pazopanib in the clinical care of extremity STS and through EORTC (STRASS) to define the role of pre-operative radiotherapy in the treatment of retroperitoneal STS. Outside of clinical trials, extremity STS should be treated at centers of expertise where high-quality IMRT-IGRT is administered to lessen acute and long-term toxicities. In patients with extremity STS, pre-operative IMRT-IGRT is preferred as better target delineation and image guidance can be achieved. While acute wound complication remains a concern, patients treated using pre-operative IMRT-IGRT are largely spared of severe chronic irreversible radiation-related side effects such as bone fracture, fibrosis, edema, and joint stiffness that alter limb functions. For STS originating from the retroperitoneum, if radiotherapy is recommended following multidisciplinary case discussion, pre-operative radiotherapy is preferred over post-operative radiotherapy. Post-operatively, normal radiosensitive organs fill the surgical cavity, which is the targeted volume of radiotherapy; hence, post-operative radiotherapy for retroperitoneal STS is associated with severe to fatal toxicities. Pre-operative radiotherapy has a more favorable toxicity profile as the retroperitoneal STS displaces, and thus spares, normal structures and organs from the high-dose irradiation volume.

Non-rhabdomyosarcoma soft-tissue sarcomas in children: Contemporary appraisal and experience from a single centre.

Nonrhabdmyosarcoma soft tissue sarcomas (NRSTS) include a cluster of different types of soft tissue sarcomas clubbed together due to the rarity of individual subtypes. The diagnostic accuracy is lately reinforced due to the availability of immunohistochemical and molecular markers. Surgery is the central modality of treatment since many of them are insensitive to chemotherapy. With the availability of rational risk stratification system, efforts are in progress to evaluate the role of neoadjuvant chemotherapy and radiotherapy to improve outcomes especially for the locally advanced disease. The survival remains dismal for metastatic disease. This review highlights the current status of NRSTS and also describes the experience from a single centre in treatment of NRSTS.

Comparative Pathology of Canine Soft Tissue Sarcomas: Possible Models of Human Non-rhabdomyosarcoma Soft Tissue Sarcomas

Comparative analyses of canine and human soft tissue sarcomas (STSs) are lacking. This study compared the histological and immunohistochemical (labelling for desmin, smooth muscle actin [SMA], CD31, pancytokeratin, S100 and CD34) appearance of 32 archived, formalin-fixed, paraffin wax-embedded canine STS tumour specimens by board-certified veterinary and medical pathologists, both blinded to the other's interpretations. Comparison between the veterinary and human diagnoses revealed a generally consistent pattern of interpretation with few notable variations. Most tumours (13/32) were judged to display similar histomorphological appearance to human low-grade spindle cell sarcomas, appearing non-distinctive and morphologically of a fibroblastic/myofibroblastic type. Five canine cases resembled human liposarcoma, but with atypical desmin-positive epithelioid cells present. Five canine cases resembled human spindle cell sarcoma with myxoid features and two additional cases resembled human myxofibrosarcoma. Seven canine cases were noted to resemble human undifferentiated sarcoma. Findings in the present study demonstrate that canine STSs display histological and immunohistochemical features similar to their human equivalents. Because of these cross-species similarities, a particular opportunity exists to understand the biology and treatment of human STS by potentially including dogs as clinical models.

PO-0164 Paediatric Soft Tissue Sarcoma: A Ten Year Review

Background and aims Paediatric soft tissue sarcomas are rare tumours that account for about 7% of all childhood cancers. 50–60% of these are rhabdomyosarcoma (RMS), while the remainder are non rhabdomyosarcoma soft tissue sarcomas (NRSTS). To evaluate the clinical characteristics, treatment modalities and outcome of paediatric soft tissue sarcomas. Methods A retrospective analysis of data from 51 patients diagnosed and treated from 2003 to 2013. Results 51 patients, 30 male and 21 female, median age of 10 years old at diagnosis. 92% showed a mass at diagnosis. The most common site of disease was the extremities (20 cases). Histology: 51% RMS (embryonal: 12 patients; alveolar: 12 patients; other: 2 patients) and 49% NRSTS (PNET 5 patients; malignant peripheral nerve sheath tumour: 3 patients; other: 17 patients). IRS group: 29% group I, 14% group II, 28% group III and 29% group IV. Most patients received multimodality therapy (radiotherapy, chemotherapy and surgery). 31% (16 patients) died due to disease progression: 7 alveolar RMS, 11 ≥ 10 years old and 13 group III-IV. Time from diagnosis to death was between 7 months and 5 years. Of the 34 living patients, 74% were in first complete remission. The median follow-up time was 38 months. Conclusions Alveolar RMS is more common in the extremities and the embryonal in the genitourinary tract. NRSTS are more common in children ≥10 years. The absence of metastases and the tumour size ≤5 cm were associated with a better prognosis (p

Autologous haematopoietic stem cell transplantation following high-dose chemotherapy for non-rhabdomyosarcoma soft tissue sarcomas: a Cochrane systematic review*

ObjectivesWe conducted a systematic review to compare the efficacy and adverse events of autologous haematopoietic stem cell transplantation (HSCT) following high-dose chemotherapy (HDCT) versus standard-dose chemotherapy (SDCT) in patients with...