http://www.hindawi.comThe latest articles from Hindawi Publishing Corporation© 2012, Hindawi Publishing Corporation. All rights reserved.http://www.hindawi.com/journals/srcm/2012/410973/Background. We report on 19 cases of giant cell tumor of bone (GCT) affecting the spine or sacrum and evaluate the outcome of different treatment modalities. Methods. Nineteen patients with GCT of the spine (n=6) or sacrum (n=13) have been included in this study. The mean followup was 51.6 months. Ten sacral GCT were treated by intralesional procedures of which 4 also received embolization, and 3 with irradiation only. All spinal GCT were surgically treated. Results. Two (15.4%) patients with sacral and 4 (66.7%) with spinal tumors had a local recurrence, two of the letter developed pulmonary metastases. One local recurrence of the spine was successfully treated by serial arterial embolization, a procedure previously described only for sacral tumors. At last followup, 9 patients had no evidence of disease, 8 had stable disease, 1 had progressive disease, 1 died due to disease. Six patients had neurological deficits. Conclusions. GCT of the axial skeleton have a high local recurrence rate. Neurological deficits are common. En-bloc spondylectomy combined with embolization is the treatment of choice. In case of inoperability, serial arterial embolization seems to be an alternative not only for sacral but also for spinal tumors.Maurice Balke, Marcel P. Henrichs, Georg Gosheger, Helmut Ahrens, Arne Streitbuerger, Michael Koehler, Viola Bullmann, and Jendrik HardesCopyright © 2012 Maurice Balke et al. All rights reserved.http://www.hindawi.com/journals/srcm/2012/987239/The Ewing sarcoma family of tumors (ESFT) represents an aggressive spectrum of malignant tumour types with common defining histological and cytogenetic features. To evaluate the functional activation of signal transducer and activator of transcription 3 (STAT3) in ESFT, we evaluated its activation in primary tissue sections and observed the functional consequences of its inhibition in ESFT cell lines. STAT3 was activated (tyrosine 705-phosphorylated) in 18 out of 31 primary tumours (58%), either diffusely (35%) or focally (23%). STAT3 was constitutively activated in 3 out of 3 ESFT cell lines tested, and its specific chemical inhibition resulted in complete loss of cell viability. STAT3 inhibition in ESFT cell lines was associated with several consistent changes in chemokine profile suggesting a role of STAT3 in ESFT in both cell survival and modification of the cellular immune environment. Together these data support the investigation of STAT3 inhibitors for the Ewing family of tumors.Sam Behjati, B. Piku Basu, Rebecca Wallace, Nelly Bier, Neil Sebire, Fyeza Hasan, and John AndersonCopyright © 2012 Sam Behjati et al. All rights reserved.http://www.hindawi.com/journals/srcm/2012/326210/Resistance of rhabdomyosarcoma to current therapies remains one of the key issues in pediatric oncology. Since the success of most cytotoxic therapies in the treatment of cancer, for example, chemotherapy, depends on intact signaling pathways that mediate programmed cell death (apoptosis), defects in apoptosis programs in cancer cells may result in resistance. Evasion of apoptosis in rhabdomyosarcoma may be caused by defects in the expression or function of critical mediators of apoptosis or in aberrant expression of antiapoptotic proteins. Therefore, the identification of the molecular mechanisms that confer primary or acquired resistance to apoptosis in rhabdomyosarcoma presents a critical step for the rational development of molecular targeted drugs. This approach will likely open novel perspectives for the treatment of rhabdomyosarcoma.Simone FuldaCopyright © 2012 Simone Fulda. All rights reserved.http://www.hindawi.com/journals/srcm/2011/325189/Introduction. The symptom burden and role of palliative care (PC) in patients with advanced soft tissue sarcoma (STS) are not well defined. Methods. This study retrospectively reviewed both symptoms and PC involvement in patients known to an STS referral centre who died in one calendar year. Results. 81 patients met inclusion criteria of which 27% had locally advanced disease and 73% metastases at initial referral. The median number of symptoms was slowly progressive ranging from 2 (range 0–5) before first-line chemotherapy (n=50) to 3 (range 1–6) at the time of best supportive care (BSC) decision (n=48). Pain and dyspnoea were the commonest symptoms. Median overall survival from BSC decision was 3.4 weeks. 88% had PC involvement (either hospital, community, or both) with median time from first PC referral to death of 16 (range 0–110) weeks. Conclusions. Patients with metastatic STS have a significant symptom burden which justifies early PC referral. Pain, including neuropathic pain, is a significant problem. Dyspnoea is common, progressive and appears to be undertreated. Time from BSC decision to death is short, and prospective studies are required to determine whether this is due to overtreatment or very rapid terminal disease progression.Nicholas J. Gough, Clare Smith, Joy R. Ross, Julia Riley, and Ian JudsonCopyright © 2011 Nicholas J. Gough et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/538085/Background. This study aimed to investigate prognostic factors for patients with myxoid/round-cell liposarcoma (MRCLS), in particular the significance of the round cell component, and to identify metastatic patterns as well as possibly suggest a suitable strategy for followup. Methods. Clinical, morphologic, and follow-up data from 160 patients with MRCLS was reviewed and statistically analysed. Results. Of 130 tumours with the round cell component evaluated, 61 had no round cell component, 27 had <5% round cell component, and 42 had >5%. All patients underwent surgical excision, 15 requiring amputation. 107 patients received adjuvant radiotherapy. Local recurrence occurred in 19 patients (12%), predominantly in patients with marginal or intralesional margins and a round cell component. Overall disease specific survival was 75% at 5 years and 56% at 10 years and was related to the proportion of round cell component. Of 52 patients who developed metastases, 38 (73%) had purely extrapulmonary metastases. We could not identify any factors predicting the site of metastases developing. Conclusions. The occurrence of any round cell component is the most important adverse prognostic factor for patients with MRCLS; patients with >5% round cell component are at higher risk of local recurrence, metastasis and tumour-related death and should be considered for adjuvant radiotherapy and possibly chemotherapy. The best method of monitoring extrapulmonary metastases remains to be established.J. Haniball, V. P. Sumathi, L.-G. Kindblom, A. Abudu, S. R. Carter, R. M. Tillman, L. Jeys, D. Spooner, D. Peake, and R. J. GrimerCopyright © 2011 J. Haniball et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/395180/Introduction. Ewing's sarcomas (EWSs) of bone and soft tissue are neuroectodermal tumors that affect both axial and appendicular locations. We hypothesized that axial location predicted poor outcome in EWS patients. Materials and Methods. Sixty-seven patients (57 with bone EWS and 10 with soft tissue EWS) were identified from our database. Thirty-four (51%) had axial EWS and 33 (49%) had appendicular EWS. Statistical analyses identified predictors of poor outcome. Results and Discussion. Axial location, large size, metastases at presentation, lack of definitive treatment, and positive surgical margins all correlated with poor outcome in univariate analysis. In multivariate analysis, axial location still predicted poor outcome when adjusted for pretreatment variables. Axial location was not statistically predictive of poor outcome when adjusted for treatment variables. Conclusions. Anatomic location has a negative effect on outcome in EWS that cannot be completely explained by pretreatment or treatment factors. Additional studies are required to determine if there is a biologic difference between axial and appendicular EWS.Kurt R. Weiss, David J. Biau, Rej Bhumbra, Anthony M. Griffin, Martin E. Blackstein, Peter Chung, Charles Catton, Brian O'Sullivan, Peter C. Ferguson, and Jay S. WunderCopyright © 2011 Kurt R. Weiss et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/289673/Background and Purpose. Lymphoedema is a serious complication following limb salvage for extremity soft tissue sarcomas (STSs) for which little is known. We aimed to evaluate its incidence, its, severity and its associated risk factors. Material and Method. Patient and tumor characteristics, treatment modalities and complications and functional outcomes (MSTS 1987, TESS), and lymphoedema severity (Stern) were all collected from prospective databases. Charts were retrospectively abstracted for BMI and comorbidities. Results. There were 289 patients (158 males). Mean age was 53 (16–88). Followup ranged between 12 and 60 months with an average of 35 and a median of 36 months. Mean BMI was 27.4 (15.8–52.1). 72% had lower extremity tumors and 38% upper extremity. Mean tumor size was 8.1 cm (1.0–35.6 cm). 27% had no adjuvant radiation, 62% had 50 Gy, and 11% received 66 Gy. The incidence of lymphoedema was 28.8% (206 none, 58 mild, 22 moderate, 3 severe, and 0 very severe). Mean MSTS score was 32 (11–35) and TESS was 89.4 (32.4–100). Radiation dose was significantly correlated with tumor size>5 cm (P=0.0001) and TESS score (P=0.001), but not MSTS score (P=0.090). Only tumor size>5 cm and depth were found to be independent predictors of significant lymphoedema. Conclusion. Nine percent of STS patients in our cohort developed significant (grade≥2) lymphoedema. Tumor size>5 cm and deep tumors were associated with an increased occurrence of lymphoedema but not radiation dosage.Daniel Friedmann, Jay S. Wunder, Peter Ferguson, Brian O'Sullivan, David Roberge, Charles Catton, Carolyn Freeman, Neil Saran, and Robert E. TurcotteCopyright © 2011 Daniel Friedmann et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/352580/Ewing's sarcoma family tumors (ESFT) are characterized by specific chromosomal translocations, which give rise to EWS-ETS chimeric proteins. These aberrant transcription factors are the main pathogenic drivers of ESFT. Elucidation of the factors influencing EWS-ETS expression and/or activity will guide the development of novel therapeutic agents against this fatal disease.David Herrero-Martin, Argyro Fourtouna, Stephan Niedan, Lucia T. Riedmann, Raphaela Schwentner, and Dave N. T. AryeeCopyright © 2011 David Herrero-Martin et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/847409/One important mechanism by which cancer cells parasitize their host is by escaping apoptosis. Thus, selectively facilitating apoptosis is a therapeutic mechanism by which oncotherapy may prove highly advantageous. One major apoptotic pathway is mediated by Fas ligand (FasL). The death-inducing signaling Ccmplex (DISC) and subsequent death-domain aggregations are created when FasL is bound by its receptor thereby enabling programmed cell death. Conceptually, if a better understanding of the Fas pathway can be garnered, an oncoselective prodeath therapeutic approach can be tailored. Herein, we propose that EGF and CTGF play essential roles in the regulation of the Fas apoptotic pathway in sarcomas. Tumor and in vitro data suggest viable cells counter the prodeath signal induced by FasL by activating EGF, which in turn induces prosurvival CTGF. The prosurvival attributes of CTGF ultimately predominate over the death-inducing FasL. Cells destined for elimination inhibit this prosurvival response via a presently undefined pathway. This scenario represents a novel role for EGF and CTGF as regulators of the Fas pathway in sarcomas.David E. Joyner, Kevin B. Jones, Stephen L. Lessnick, Joshua D. Schiffman, and R. Lor RandallCopyright © 2011 David E. Joyner et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/898257/Patients with human immunodeficiency virus infection are known to have increased risk of various neoplasms, including Kaposi sarcoma, which classically involves the skin and mucosal locations. The anaplastic variant of Kaposi sarcoma is rare and poorly documented in the literature. It is characterised clinically by a more aggressive behaviour and increased metastatic potential, and histologically by increased cellularity, mitotic rate, and rarely by epithelioid angiosarcoma-like morphology. We report herein a 64-year-old man with a long-standing history of human immunodeficiency virus infection who developed a right adrenal tumor with a high-grade anaplastic angiosarcoma-like morphology. Immunohistochemistry for human herpes virus-8 was strongly positive in the tumor cells. To the best of our knowledge, this is the first report of an anaplastic Kaposi sarcoma in the adrenal gland.Hassan Huwait, Adam Meneghetti, and Torsten O. NielsenCopyright © 2011 Hassan Huwait et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/978617/A 23-year-old female with Ewing's Sarcoma underwent tibial resection and skeletal reconstruction using proximal tibial allograft prosthetic reconstruction with distal femur endoprosthetic reconstruction and rotating hinge. Human acellular dermal matrix, (Alloderm, LifeCell, Branchburg, NJ, USA), was used to wrap the skeletal reconstruction. Soft tissue reconstruction was completed with a rotational gastrocnemius muscle flap and skin graft. Despite prolonged immobilization, the patient quickly regained full range of motion of her skeletal reconstruction. Synthetic mesh, tapes and tubes are used to perform capsule reconstruction of megaprosthesis. This paper describes the role of human acellular dermal matrix in capsule reconstruction around a megaprosthesis.Robert M. Whitfield, Jeremy Rinard, and David KingCopyright © 2011 Robert M. Whitfield et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/732690/A limited number of reports have investigated the role of microRNAs in osteosarcoma. In this study, we performed miRNA expression profiling of osteosarcoma cell lines, tumor samples, and normal human osteoblasts. Twenty-two differentially expressed microRNAs were identified using high throughput real-time PCR analysis, and 4 (miR-135b, miR-150, miR-542-5p, and miR-652) were confirmed and validated in a different group of tumors. Both miR-135b and miR-150 have been previously shown to be important in cancer. We hypothesize that dysregulation of differentially expressed microRNAs may contribute to tumorigenesis. They might also represent molecular biomarkers or targets for drug development in osteosarcoma.Rishi R. Lulla, Fabricio F. Costa, Jared M. Bischof, Pauline M. Chou, Maria de F. Bonaldo, Elio F. Vanin, and Marcelo B. SoaresCopyright © 2011 Rishi R. Lulla et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/984340/Aims. This phase II study explored activity/safety of front-line dose-dense chemotherapy in high-grade STS (soft tissue sarcoma) patients and tested ezrin as prognostic factor. Patients and Methods. The protocol consisted of three cycles of doxorubicin (DOXO) 30 mg/m2 on days 1–3 every 2 weeks, followed by three cycles of ifosfamide (IFO) 2.5 g/m2 two hours a day on days 1–5 every 3 weeks, with GCSF support. Ezrin was assessed immunohistochemically. Results. Twenty patients, 13 metastatic and 7 locally advanced, were enrolled. Median age was 39 years (25–60). Median dose intensities were 42 mg/m2/week and 3.6 g/m2/week for DOXO and IFO, respectively. Grade 3/4 toxicities occurred in 18 patients. Response rate was 15% (3 of 20) by RECIST. Patients younger than 45 years with locally advanced disease and synovial histology presented longer survival. A trend towards longer survival was observed among ezrin-positive patients. Conclusions. This dose-dense schedule should not be routinely used due to its high frequency of toxic events; however, a sequential strategy with DOXO and IFO may benefit selected patients and should be further explored with lower doses. The role of ezrin as a prognostic marker should be confirmed in a larger group of patients.G. F. G. Almeida, G. Castro Jr., I. M. L. Snitcovsky, S. A. Siqueira, E. H. Akaishi, O. P. Camargo, C. R. G. C. M. Oliveira, and M. H. H. FedericoCopyright © 2011 G. F. G. Almeida et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/312802/Primary meningeal rhabdomyosarcoma is a rare primary brain malignancy, with scant case reports. While most reports of primary intracranial rhabdomyosarcoma occur in pediatric patients, a handful of cases in adult patients have been reported in the medical literature. We report the case of a 44-year-old male who developed primary meningeal rhabdomyosarcoma. After developing episodes of right lower extremity weakness, word finding difficulty, and headaches, a brain magnetic resonance imaging (MRI) demonstrated a vertex lesion with radiographic appearance of a meningeal-derived tumor. Subtotal surgical resection was performed due to sagittal sinus invasion and initial pathology was interpreted as an anaplastic meningioma. Re-review of pathology demonstrated rhabdomyosarcoma negative for alveolar translocation t(2;13). Staging studies revealed no evidence of disseminated disease. He was treated with stereotactic radiotherapy with concurrent temozolamide to be followed by vincristine, actinomycin-D, and cyclophosphamide (VAC) systemic therapy.Manisha Palta, Richard F. Riedel, James J. Vredenburgh, Thomas J. Cummings, Scott Green, Zheng Chang, and John P. KirkpatrickCopyright © 2011 Manisha Palta et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/450743/Background. Teratoma with malignant transformation (TMT) is rare and most commonly encountered in adult patient with germ cell tumor (GCT). Method. We report a rare case of testicular teratoma with metastatic TMT/embryonal rhabdomyosarcoma (ERMS). A 44-year-old man underwent right orchiectomy which revealed a malignant teratoma, he subsequently had right pneumonectomy with two pulmonary masses containing a high-grade embryonal rhabdomyosarcoma. The patient developed liver metastasis three months after initial diagnosis. He was treated with a chemotherapy regimen with vincristine, dactinomycin, and cyclophosphamide (VAC) alternating with vincristine and irinotecan (VI) with complete resolution of his liver lesion. The tumors were examined with a battery of cytogenetic, immunohistochemical, and molecular assays. Results. The malignant cells were immunohistochemically positive for desmin, myogenin, and MyoD1. Molecular cytogenetics of embryonal rhabdomyosarcoma tissue revealed the presence of i(12p). The tumor expressed high level of TOPO2A, TOPO1, MRP1, MGMT, BCRP, ERCC1, RRM1, and TS. Conclusion. The activity of topoisomerase inhibitors and the potential usefulness of topoisomerase expression as biomarkers should be further tested in aprospective study.Jue Wang and Syed A. Jaffar KazmiCopyright © 2011 Jue Wang and Syed A. Jaffar Kazmi. All rights reserved.http://www.hindawi.com/journals/srcm/2011/978319/Objective. Ewing’s sarcoma of the kidney is rare and is usually found in young adults. We present here a single case study of Ewing's sarcoma found in an elderly man. Material and methods. A 73-year-old man underwent routine surgery for hydrocoele of the testis. He developed urinary obstruction symptoms, and radiological examinations revealed a tumour in the right kidney. Results. Microscopical, immunohistochemical, and molecular pathological analysis of the tumour was consistent with Ewing's sarcoma. FISH showed rearrangement of chromosomes 22q12 (EWSR1). The patient subsequently underwent nephrectomy followed by 6 adjuvant chemotherapy cycles. Follow-up after 7 months shows no recurrence. Conclusion. This case report presents not only the rare finding of Ewing's sarcoma in the kidney, but also the occurrence of this tumour entity in an elderly patient. Treatment options for the different types of renal tumours are vastly different and the need for a correct diagnosis is, therefore, vital.T. B. Wedde, I. V. K. Lobmaier, B. Brennhovd, F. Lohne, and K. S. HallCopyright © 2011 T. B. Wedde et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/813483/The prevalence of pain in patients with sarcoma is not well documented. We investigated this in outpatients at a tertiary cancer referral centre, assessing the adequacy of pain control and for risk factors leading to higher prevalence and severity of pain. 149 patients were surveyed. Patients with pain within the previous 7 days completed pain assessment tools (BPI, S-LANSS, PMI). 53% of patients had pain within the previous 7 days, and 25% had significant pain. Of those with pain, 63% was inadequately controlled and neuropathic pain was identified in 36%. Age, gender, tumour type, and the type of cancer treatment were not significant predictors of the prevalence or severity of the pain. Based on our results, patients with sarcoma should be actively screened for pain and have regular reviews of their analgesic requirements.P. Y. Kuo, J. T. C. Yen, G. M. Parker, S. Chapman, S. Kandikattu, I. Sohanpal, Y. Barbachano, and J. E. WilliamsCopyright © 2011 P. Y. Kuo et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/160369/Angiosarcoma (AS) is a rare and aggressive vascular neoplasm with very poor prognosis. Patients with extensive cutaneous AS who are not surgical candidates have very limited options since there is no standard treatment. Treatment options include radiation, chemotherapy, and angiogenesis inhibitor with varying success rates. Here, we report a case an 88 year old patient with extensive scalp angiosarcoma having biopsy proven remission with bevacizumab and radiotherapy without undergoing surgery.Jocelyn T. De Yao, Danyu Sun, Angela T. Powell, and Esther H. RehmusCopyright © 2011 Jocelyn T. De Yao et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/130484/Rhabdomyosarcoma is an aggressive childhood malignancy, accounting for more than 50% of all soft-tissue sarcomas in children. Even with extensive therapy, the survival rate among alveolar rhabdomyosarcoma patients with advanced disease is only 20%. The receptor tyrosine kinase Epidermal Growth Factor Receptor (EGFR) has been found to be expressed and activated in human rhabdomyosarcomas. In this study we have used a genetically engineered mouse model for alveolar rhabdomyosarcoma (ARMS) which faithfully recapitulates the human disease by activating the pathognomic Pax3:Fkhr fusion gene and inactivating p53 in the maturing myoblasts. We have demonstrated that tumors from our mouse model of alveolar rhabdomyosarcoma express EGFR at both the mRNA and protein levels. We then tested the EGFR inhibitor, Erlotinib, for its efficacy in this mouse model of alveolar rhabdomyosarcoma. Surprisingly, Erlotinib had no effect on tumor progression, yet mice treated with Erlotinib showed 10–20% loss of body weight. These results suggest that EGFR might not be an a priori monotherapy target in alveolar rhabdomyosarcoma.Jinu Abraham, Laura D. Nelon, Courtney B. Kubicek, Aoife Kilcoyne, Sheila T. Hampton, Lee Ann Zarzabal, Francis J. Giles, Joel E. Michalek, Brian P. Rubin, and Charles KellerCopyright © 2011 Jinu Abraham et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/815190/Radiotherapy used in the treatment of pediatric musculoskeletal sarcomas may result in crippling defects of skeletal growth. Several radioprotective strategies have shown potential for preserving function of the irradiated epiphysis but have not been evaluated in a tumor-bearing animal model. We developed two bioluminescent human rhabdomyosarcoma cell lines that were used to establish xenograft tumors in skeletally immature mice. Bioluminescence imaging and radiography allowed serial evaluation of tumor growth and tibial elongation following localized radiotherapy. High-dose (10 Gy) radiotherapy significantly reduced tumor growth velocity and prolonged the median survival of tumor-bearing mice but also resulted in a significant 3.3% shortening of the irradiated limb. Exposure to a lower, 2 Gy dose resulted in 4.1% decrease in limb length but did not extend survival. This new model provides a clinically relevant means to test the efficacy and safety of novel radioprotectant and radiorecovery strategies for use in this context.Jason A. Horton, Judith A. Strauss, Matthew J. Allen, and Timothy A. DamronCopyright © 2011 Jason A. Horton et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/231789/Background. There remains controversy on the routine use of chemotherapy in localized SS. Methods. The records of 87 adult (AP) and 15 pediatric (PP) patients with localized SS diagnosed between 1986 and 2007 at 2 centres in Toronto were reviewed. Results. Median age for AP and PP was 37.6 (range 15–76) and 14 (range 0.4–18) years, respectively. 65 (64%) patients had large tumours (>5 cm). All patients underwent en bloc surgical resection resulting in 94 (92.2%) negative and 8 (7.8%) microscopically positive surgical margins. 72 (82.8%) AP and 8 (53%) PP received radiotherapy. Chemotherapy was administered to 12 (13.8%) AP and 13 (87%) PP. 10 AP and 5 PP were evaluable for response to neoadjuvant chemotherapy, with response rate of 10% and 40%, respectively. 5-year EFS and OS was 69.3±4.8% and 80.3±4.3%, respectively, and was similar for AP and PP, In patients with tumors >5 cm, in whom chemotherapy might be considered most appropriate, relapse occurred in 9/19 (47%) with chemotherapy, compared to 17/46 (37%) In those without. Conclusions. Patients with localized SS have a good chance of cure with surgery and RT. Evidence for a well-defined role of chemotherapy to improve survival In localized SS remains elusive.H. Al-Hussaini, D. Hogg, M. E. Blackstein, B. O'Sullivan, C. N. Catton, P. W. Chung, A. M. Griffin, D. Hodgson, S. Hopyan, R. Kandel, P. C. Ferguson, J. S. Wunder, and A. A. GuptaCopyright © 2011 H. Al-Hussaini et al. All rights reserved.http://www.hindawi.com/journals/srcm/2011/545104/Reconstruction of the extensor mechanism is essential for good extremity function after endoprosthetic knee replacement following tumor resection. Only a few biological methods have been able to reliably restore a functional extensor mechanism, but they are often associated with significant complication rates. Reattachment of the patellar tendon to the prosthesis using an alloplastic patellar ligament (Trevira cord) can be an appropriate alternative. In vivo and in vitro studies have already shown that complete fibrous ingrowth in polyethylene chords can be seen after a period of six months. However, until now, no biomechanical study has shown the efficacy of an alloplastic cord and its fixation device in providing sufficient stability and endurance in daily life-activity until newly formed scar tissue can take over this function. In a special test bench developed for this study, different loading regimes were applied to simulate loads during everyday life. Failure loads and failure modes were evaluated. The properties of the cord were compared before and after physiological conditioning. It was shown that rubbing was the mode of failure under dynamic loading. Tensile forces up to 2558 N did not result in material failure. Thus, using an artificial cord together with this fixation device, temporary sufficient stable fixation can be expected.Boris Michael Holzapfel, Hans Rechl, Stefan Lehner, Hakan Pilge, Hans Gollwitzer, and Erwin SteinhauserCopyright © 2011 Boris Michael Holzapfel et al. All rights reserved.http://www.hindawi.com/journals/srcm/2010/751304/Background. Optimal treatment of nongastrointestinal stromal tumor soft-tissue sarcomas (non-GIST STSs) is resection with wide margins. This study investigates the prognostic impact of the angiogenesis-associated platelet-derived growth factors (PDGFs) and their receptors (PDGFRs) in non-GIST STS patients with wide and nonwide resection margins. Method. Tumor samples and clinical data from 249 patients with non-GIST STS were obtained, and tissue microarrays were constructed for each specimen. Immunohistochemistry was used to evaluate the expression of PDGF-A, -B, -C, and -D and PDGFR-α and -β. Results. In the multivariate analysis of patients with wide resection margins, high expression of PDGF-B (P=.013, HR = 2.954, and 95% CI = 1.255–6.956) and the coexpression of PDGF-B and PDGFR-α (overall; P=.016, high-low/low-high; P=.051, HR = 2.678, 95% CI = 0.996–7.200, high/high; P=.004, HR = 3.930, 95% CI = 1.542–10.015) were independent negative prognostic markers for disease-specific survival. Conclusion. PDGF-B and the coexpression of PDGF-B and PDGFR-α are strong and independent prognostic factors in non-GIST STSs with wide resection margins.Thomas Karsten Kilvaer, Andrej Valkov, Sveinung W. Sorbye, Tom Donnem, Eivind Smeland, Roy Martin Bremnes, and Lill-Tove BusundCopyright © 2010 Thomas Karsten Kilvaer et al. All rights reserved.http://www.hindawi.com/journals/srcm/2010/630129/Immortal tumor cell lines are an important model system for cancer research, however, misidentification and cross-contamination of cell lines are a common problem. Seven chordoma cell lines are reported in the literature, but none has been characterized in detail. We analyzed gene expression patterns and genomic copy number variations in five putative chordoma cell lines (U-CH1, CCL3, CCL4, GB60, and CM319). We also created a new chordoma cell line, U-CH2, and provided genotypes for cell lines for identity confirmation. Our analyses revealed that CCL3, CCL4, and GB60 are not chordoma cell lines, and that CM319 is a cancer cell line possibly derived from chordoma, but lacking expression of key chordoma biomarkers. U-CH1 and U-CH2 both have gene expression profiles, copy number aberrations, and morphology consistent with chordoma tumors. These cell lines also harbor genetic changes, such as loss of p16, MTAP, or PTEN, that make them potentially useful models for studying mechanisms of chordoma pathogenesis and for evaluating targeted therapies.Silke Brüderlein, Joshua B. Sommer, Paul S. Meltzer, Sufeng Li, Takuya Osada, David Ng, Peter Möller, David A. Alcorta, and Michael J. KelleyCopyright © 2010 Silke Brüderlein et al. All rights reserved.http://www.hindawi.com/journals/srcm/2010/317462/These guidelines have been developed in order to provide an overview and a set of broad-based key recommendations for the management of patients with bone sarcomas in the UK. They have taken into consideration the most up-to-date scientific literature along with the recent recommendations by the European Society of Medical Oncology. The principles of the NICE guidance on both “improving outcomes for patients with sarcomas” and “improving outcomes with children and young people with cancer” have been incorporated. As care evolves, it is acknowledged that these guidelines will need updating. The key recommendations are that bone pain or a palpable mass should always lead to further investigation and patients with clinicoradiological findings suggestive of a primary bone tumour should be sent to a reference centre. Patients should then have their care managed at such a specialist centre by a fully accredited multidisciplinary team.Robert Grimer, Nick Athanasou, Craig Gerrand, Ian Judson, Ian Lewis, Bruce Morland, David Peake, Beatrice Seddon, and Jeremy WhelanCopyright © 2010 Robert Grimer et al. All rights reserved.http://www.hindawi.com/journals/srcm/2010/125162/Pelvic sarcoma is associated with a relatively poor prognosis, due to the difficulty in obtaining an adequate surgical margin given the complex pelvic anatomy. Magnetic resonance imaging and computerized tomography allow valuable surgical resection planning, but intraoperative localization remains hazardous. Surgical navigation systems could be of great benefit in surgical oncology, especially in difficult tumor location; however, no commercial surgical oncology software is currently available. A customized navigation software was developed and used to perform a synovial sarcoma resection and allograft reconstruction. The software permitted preoperative planning with defined target planes and intraoperative navigation with a free-hand saw blade. The allograft was cut according to the same planes. Histological examination revealed tumor-free resection margins. Allograft fitting to the pelvis of the patient was excellent and allowed stable osteosynthesis. We believe this to be the first case of combined computer-assisted tumor resection and reconstruction with an allograft.Pierre-Louis Docquier, Laurent Paul, Olivier Cartiaux, Christian Delloye, and Xavier BanseCopyright © 2010 Pierre-Louis Docquier et al. All rights reserved.http://www.hindawi.com/journals/srcm/2010/702573/Retroperitoneal sarcomas are rare and treatment should optimally be centralized. Despite successful centralization with 90% of the patients referred prior to surgery, delays occur, which led us to assess lead times in a population-based series. Method. Patients diagnosed with retroperitoneal sarcoma in the southern Sweden health care region 2003–2009 were eligible for the study. Data on referrals and diagnostic investigations were collected from clinical files from primary health care, local hospitals, and from the sarcoma centre. Lead times were divided into patient delays and health care delays caused by primary health care, local hospitals, or procedures at the sarcoma centre. Results. Complete data were available from 33 patients and demonstrated a median patient delay of 23 days (0–17 months) and median health care delay of 94 days (1–40 months) with delays of median 15 days at the general practitioner, 36 days at local hospitals, and 55 days at the sarcoma centre. Conclusion. Centralization per se is not sufficient for optimized and efficient management. Our findings suggest that delays can be minimized by direct referral of patients from primary health care to sarcoma centers and indicate that development of coordinated diagnostic packages could shorten delays at the sarcoma centre.Jojanneke Seinen, Martin Almquist, Emelie Styring, Anders Rydholm, and Mef NilbertCopyright © 2010 Jojanneke Seinen et al. All rights reserved.http://www.hindawi.com/journals/srcm/2010/829498/Soft-tissue sarcoma (STS) is a histopathologically diverse group of tumors accounting for approximately 10,000 new malignancies in the US each year. The proximal lower extremity is the most common site for STS, accounting for approximately one-third of all cases. Coordinated multimodality management in the form of surgery and radiation is often critical to local control, limb preservation, and functional outcome. Based on a review of currently available Medline literature and professional experience, this paper provides an overview of the treatment of STS of the lower extremity with a particular focus on the modern role of radiotherapy.Brendan Prendergast, John B. Fiveash, C. Parker Gibbs, Mark T. Scarborough, and Daniel J. IndelicatoCopyright © 2010 Brendan Prendergast et al. All rights reserved.http://www.hindawi.com/journals/srcm/2010/174528/Tenosynovial giant cell tumor is a neoplastic disease of joints that can cause severe morbidity. Recurrences are common following local therapy, and no effective medical therapy currently exists. Recent work has demonstrated that all cases overexpress macrophage colony-stimulating factor (CSF1), usually as a consequence of an activating gene translocation, resulting in an influx of macrophages that form the bulk of the tumor. New anti-CSF1 drugs have been developed; however there are no preclinical models suitable for evaluation of drug benefits in this disease. In this paper, we describe a novel renal subcapsular xenograft model of tenosynovial giant cell tumor. Using this model, we demonstrate that an anti-CSF1 monoclonal antibody significantly inhibits host macrophage infiltration into this tumor. The results from this model support clinical trials of equivalent humanized agents and anti-CSF1R small molecule drugs in cases of tenosynovial giant cell tumor refractory to conventional local therapy.Hongwei Cheng, Paul W. Clarkson, Dongxia Gao, Marina Pacheco, Yuzhuo Wang, and Torsten O. NielsenCopyright © 2010 Hongwei Cheng et al. All rights reserved.http://www.hindawi.com/journals/srcm/2010/312648/Introduction. The ‘‘two-week wait’’ was established as a potential means of diagnosing malignant tumours earlier. This paper investigated whether these clinics are leading to earlier diagnosis of malignant soft-tissue lumps. Method. We identified all referrals to our centre from a database over a 4-year period. Results. 2225 patients were referred to our unit for investigation of a soft-tissue mass. 576 (26%) were referred under the ‘‘two-week wait’’ criteria. 153 (27%) of which were found to have a malignant or borderline malignant diagnosis. 1649 patients were referred nonurgently. 855 (52%) of which were diagnosed with a malignant or borderline lesion. The average size at diagnosis was 9.4 cm with no difference in size between the different referral routes. Conclusion. There is little evidence that the two-week wait clinic is leading to earlier diagnosis of soft-tissue sarcomas with the majority still being referred nonurgently.W. St. J. Taylor, R. J. Grimer, S. R. Carter, R. M. Tillman, A. Abudu, and L. JeysCopyright © 2010 W. St. J. Taylor et al. All rights reserved.