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Soft tissu sarcomas

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https://www.readbyqxmd.com/read/28525423/congenital-infantile-fibrosarcoma-associated-with-a-lipofibromatosis-like-component-one-train-may-be-hiding-another
#1
Romain Swiadkiewicz, Louise Galmiche, Kahina Belhous, Olivia Boccara, Sylvie Fraitag, Florence Pedeutour, Bérangère Dadone, Jacques Buis, Arnaud Picard, Daniel Orbach, Natacha Kadlub
Congenital infantile fibrosarcoma (CIFS) is a soft tissue sarcoma of infants mainly involving lower extremities and usually developing during the first year of life. At another end of the spectrum of pediatric fibroblastic lesions, lipofibromatosis is a rare benign infiltrative soft tissue tumor that affects children. The authors report in this study a particular presentation with a CIFS surrounded by lipofibromatosis-like areas. The presence of a surrounding benign tumor confused and delayed CIFS diagnosis...
June 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28521631/immunohistochemical-profile-of-myc-protein-in-pediatric-small-round-blue-cell-tumors
#2
Karen M Chisholm, Chandra Krishnan, Amy Heerema-McKenney, Yasodha Natkunam
Deregulation of MYC oncoprotein in cancers can result from multiple oncogenic mechanisms. Although MYC translocations define Burkitt lymphoma and MYC protein expression is a poor prognostic factor in undifferentiated neuroblastomas, the distribution of MYC protein (c-MYC) across other pediatric small round blue cell tumors (SRBCT) has not been well characterized. We undertook this study to assess MYC protein expression in a large cohort of pediatric lymphomas, sarcomas, and other SRBCT. Tissue microarrays containing 302 SRBCT were successfully evaluated by immunohistochemistry using anti-MYC clone Y69, with nuclear positivity scored as 0%, 1%-25%, 26%-50%, 51%-75%, or 76%-100%...
June 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28515258/evolving-treatment-of-soft-tissue-sarcoma
#3
Suzanne George
Soft tissue sarcomas comprise multiple histologic subtypes, occur at a number of anatomic sites, and require individualized treatment. Over the past 5 years, 4 new drugs were approved for sarcoma, most of which are driven by histology or the anticipated response to treatment. Surgical resection remains the primary treatment for resectable tumors. For unresectable or metastatic disease, doxorubicin remains the backbone of chemotherapy, but other agents have improved on its single-agent efficacy. Chief among them is olaratumab, which, in combination with doxorubicin, is preferred over doxorubicin alone in the updated NCCN Clinical Practice Guidelines in Oncology for Soft Tissue Sarcoma...
May 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/28512869/patterns-of-head-and-neck-sarcoma-in-australia
#4
Roger H Woods, J Alexa Potter, Jessica L Reid, Jennie Louise, Taryn Bessen, Gelareh Farshid, Susan J Neuhaus
BACKGROUND: Sarcomas affecting the head and neck often require complex management due to the combination of anatomic, aesthetic and oncological considerations. The incidence and patterns of presentation are poorly understood and have not been reviewed in the Australian population. METHOD: This study sourced incidence and demographic data from the National Cancer Registry at the Australian Institute of Health and Welfare for the years 1982-2009 (corresponding to 97...
May 16, 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/28512389/evaluation-of-quality-of-life-at-progression-in-patients-with-soft-tissue-sarcoma
#5
Stacie Hudgens, Anna Forsythe, Ilias Kontoudis, David D'Adamo, Ashley Bird, Hans Gelderblom
Introduction. Soft Tissue Sarcoma (STS) is a rare malignancy of mesodermal tissue, with international incidence estimates between 1.8 and 5 per 100,000 per year. Understanding quality of life (QoL) and the detrimental impact of disease progression is critical for long-term care and survival. Objectives. The primary objective was to explore the relationship between disease progression and health-related quality of life (HRQoL) using data from Eisai's study (E7389-G000-309). Methods. This was a 1 : 1 randomized, open-label, multicenter, Phase 3 study comparing the efficacy and safety of eribulin versus dacarbazine in patients with advanced STS...
2017: Sarcoma
https://www.readbyqxmd.com/read/28511645/therapeutic-potential-of-tas-115-via-c-met-and-pdgfr%C3%AE-signal-inhibition-for-synovial-sarcoma
#6
Shutaro Yamada, Yoshinori Imura, Takaaki Nakai, Sho Nakai, Naohiro Yasuda, Keiko Kaneko, Hidetatsu Outani, Satoshi Takenaka, Kenichiro Hamada, Akira Myoui, Nobuhito Araki, Takafumi Ueda, Kazuyuki Itoh, Hideki Yoshikawa, Norifumi Naka
BACKGROUND: The prognosis of synovial sarcoma (SS), an aggressive soft tissue sarcoma, remains poor. We previously reported that c-MET or platelet-derived growth factor receptor α (PDGFRα) signalling pathway is related to SS progression based upon the findings of phospho-receptor tyrosine kinase (RTK) arrays. TAS-115 is a novel c-MET/ vascular endothelial growth factor receptor-targeting tyrosine kinase inhibitor that has been shown to inhibit multiple RTKs. Here we aimed to investigate the therapeutic potential of TAS-115 against SS...
May 16, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28510278/tfg-met-fusion-in-an-infantile-spindle-cell-sarcoma-with-neural-features
#7
Uta Flucke, Max M van Noesel, Marc Wijnen, Lei Zhang, Chun-Liang Chen, Yun-Shao Sung, Cristina R Antonescu
An increasing number of congenital and infantile sarcomas displaying a primitive, monomorphic spindle cell phenotype have been characterized to harbor recurrent gene fusions, including infantile fibrosarcoma and congenital spindle cell rhabdomyosarcoma. Here we report an unusual spindle cell sarcoma presenting as a large and infiltrative pelvic soft tissue mass in a 4-month-old girl, which revealed a novel TFG-MET gene fusion by whole transcriptome RNA sequencing. The tumor resembled the morphology of an infantile fibrosarcoma with both fascicular and patternless growth, however, it expressed strong S100 protein immunoreactivity, while lacking SOX10 staining and retaining H3K27me3 expression...
May 16, 2017: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/28506772/treatment-of-epithelioid-angiosarcoma-with-topical-ala-pdt-in-the-course-of-surgery
#8
Yang Gao, Wen-Sheng Wang, Hai-Lin Wang, Jia Liu, Yuan-Gang Lu
BACKGROUND: Epithelioid angiosarcoma is a kind of high grade malignancy sarcoma of soft tissue, which is hard to diagnose and completely exsect without causing functional and cosmetic problems. Moreover, the high rate of recurrence is hard to handle. Photodynamic therapy is a novel treatment protocol which can selectively destroy tumor cell with good functional and cosmetic outcomes. METHODS: This is a case about a 81 years old patient with Epithelioid angiosarcoma in the right medial angle of eye, which received surgery and photodynamic therapy...
May 12, 2017: Photodiagnosis and Photodynamic Therapy
https://www.readbyqxmd.com/read/28505003/the-adequacy-of-core-biopsy-in-the-assessment-of-smooth-muscle-neoplasms-of-soft-tissues-implications-for-treatment-and-prognosis
#9
Nina Schneider, Dirk C Strauss, Myles J Smith, Aisha B Miah, Shane Zaidi, Charlotte Benson, Winan J van Houdt, Robin L Jones, Andrew J Hayes, Cyril Fisher, Khin Thway
The grading of soft tissue sarcomas is one of the most important prognostic factors and determines patient management. Although grading of most adult-type soft tissue sarcomas on biopsies correlates highly with the final grading on the excision specimen, it appears less reliable for tumors of smooth muscle. We assessed the pathologic findings for smooth muscle neoplasms diagnosed by core biopsy at our tertiary sarcoma center, and compared them with those in the subsequent excision specimens. A total of 100 patients with leiomyosarcoma first diagnosed on core biopsy and with a subsequent excision were identified and the accuracy of the biopsy grade determined by comparison with the excision grade...
May 12, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28502707/surgical-innovation-in-sarcoma-surgery
#10
L Jeys, G Morris, S Evans, J Stevenson, M Parry, J Gregory
The field of orthopaedic oncology relies on innovative techniques to resect and reconstruct a bone or soft tissue tumour. This article reviews some of the most recent and important innovations in the field, including biological and implant reconstructions, together with computer-assisted surgery. It also looks at innovations in other fields of oncology to assess the impact and change that has been required by surgeons; topics including surgical margins, preoperative radiotherapy and future advances are discussed...
May 11, 2017: Clinical Oncology: a Journal of the Royal College of Radiologists
https://www.readbyqxmd.com/read/28499583/histotype-tailored-neoadjuvant-chemotherapy-versus-standard-chemotherapy-in-patients-with-high-risk-soft-tissue-sarcomas-isg-sts-1001-an-international-open-label-randomised-controlled-phase-3-multicentre-trial
#11
Alessandro Gronchi, Stefano Ferrari, Vittorio Quagliuolo, Javier Martin Broto, Antonio Lopez Pousa, Giovanni Grignani, Umberto Basso, Jean-Yves Blay, Oscar Tendero, Robert Diaz Beveridge, Virginia Ferraresi, Iwona Lugowska, Domenico Franco Merlo, Valeria Fontana, Emanuela Marchesi, Davide Maria Donati, Elena Palassini, Emanuela Palmerini, Rita De Sanctis, Carlo Morosi, Silvia Stacchiotti, Silvia Bagué, Jean Michelle Coindre, Angelo Paolo Dei Tos, Piero Picci, Paolo Bruzzi, Paolo Giovanni Casali
BACKGROUND: Previous trials from our group suggested an overall survival benefit with five cycles of adjuvant full-dose epirubicin plus ifosfamide in localised high-risk soft-tissue sarcoma of the extremities or trunk wall, and no difference in overall survival benefit between three cycles versus five cycles of the same neoadjuvant regimen. We aimed to show the superiority of the neoadjuvant administration of histotype-tailored regimen to standard chemotherapy. METHODS: For this international, open-label, randomised, controlled, phase 3, multicentre trial, patients were enrolled from 32 hospitals in Italy, Spain, France, and Poland...
May 9, 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28498445/evaluation-of-expression-of-cancer-stem-cell-markers-and-fusion-gene-in-synovial-sarcoma-insights-into-histogenesis-and-pathogenesis
#12
Yang Zhou, Dongdong Chen, Yan Qi, Ruixue Liu, Shugang Li, Hong Zou, Jiaojiao Lan, Xinxin Ju, Jinfang Jiang, Weihua Liang, Yaoyuan Shen, Lijuan Pang, Feng Li
Synovial sarcoma (SS) is an aggressive soft tissue tumor, with uncertain histological and cellular origin. SYT-SSX is considered to be responsible for sarcoma initiation and progression. The histogenesis and pathogenesis of this tumor are poorly understood, and prognosis of patients of SS is unsatisfactory. Recent studies have shown an association of cancer stem cells with the initiation and development of tumors. We explored immunohistochemical expression level of stem cell associated markers to determine the possible histogenesis and pathogenesis of SS...
May 2, 2017: Oncology Reports
https://www.readbyqxmd.com/read/28494808/observational-studies-goldmines-of-information-on-rare-diseases
#13
EDITORIAL
Robert S Benjamin
The article by Savina et al. from the large METASARC database of the French Sarcoma Group (BMC Med 15:78, 2017) provides a wealth of information about the natural history and therapy of patients with metastatic soft tissue sarcomas. The information complements - and in some cases surpasses - that obtained from randomized clinical trials, and should not be overlooked because of its retrospective nature. For rare diseases, retrospective data are often more important than data from randomized trials because of the inherent restrictions on sample size...
May 12, 2017: BMC Medicine
https://www.readbyqxmd.com/read/28494701/spindle-cell-lipoma-in-dogs
#14
Giancarlo Avallone, Valeria Pellegrino, Luisa V Muscatello, Giuseppe Sarli, Paola Roccabianca
Spindle cell lipoma (SCL) is a benign neoplasm of the adipose tissue that may resemble an undifferentiated soft tissue sarcoma (STS). This report describes the histopathological features of 6 SCLs in dogs. All SCLs were located in the subcutis and were composed of bland, occasionally vacuolated spindle cells intermixed with ropey collagen and myxoid matrix. Sudan IV stain performed in 1 case demonstrated the lipid content of vacuoles. Mature adipocytes represented less than 10% of the neoplasm in 3 cases and were absent in the remaining 3...
January 1, 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/28494349/trabectedin-and-campthotecin-synergistically-eliminate-cancer-stem-cells-in-cell-of-origin-sarcoma-models
#15
Lucia Martinez-Cruzado, Juan Tornin, Aida Rodriguez, Laura Santos, Eva Allonca, Maria Teresa Fernandez-Garcia, Aurora Astudillo, Juana Maria Garcia-Pedrero, Rene Rodriguez
Trabectedin has been approved for second-line treatment of soft tissue sarcomas. However, its efficacy to target sarcoma initiating cells has not been addressed yet. Here, we used pioneer models of myxoid/round cell liposarcoma (MRCLS) and undifferentiated pleomorphic sarcoma (UPS) developed from transformed human mesenchymal stromal/stem cells (MSCs) to evaluate the effect of trabectedin in the cell type responsible for initiating sarcomagenesis and their derived cancer stem cells (CSC) subpopulations. We found that low nanomolar concentrations of trabectedin efficiently inhibited the growth of sarcoma-initiating cells, induced cell cycle arrest, DNA damage and apoptosis...
May 8, 2017: Neoplasia: An International Journal for Oncology Research
https://www.readbyqxmd.com/read/28493834/establishing-a-patient-derived-xenograft-model-of-human-myxoid-and-round-cell-liposarcoma
#16
Yiming Qi, Yu Hu, Hua Yang, Rongyuan Zhuang, Yingyong Hou, Hanxing Tong, Yi Feng, Yuan Huang, Quan Jiang, Qunsheng Ji, Qingyang Gu, Zhixiang Zhang, Xuzhen Tang, Weiqi Lu, Yuhong Zhou
Myxoid and round cell liposarcoma (MRCL) is a common type of soft tissue sarcoma. The lack of patient-derived tumor xenograft models that are highly consistent with human tumors has limited the drug experiments for this disease. Hence, we aimed to develop and validate a patient-derived tumor xenograft model of MRCL. A tumor sample from a patient with MRCL was implanted subcutaneously in an immunodeficient mouse shortly after resection to establish a patient-derived tumor xenograft model. After the tumor grew, it was resected and divided into several pieces for re-implantation and tumor passage...
April 21, 2017: Oncotarget
https://www.readbyqxmd.com/read/28493803/soft-tissue-sarcomas-of-the-abdomen-and-pelvis-radiologic-pathologic-features-part-2-uncommon-sarcomas
#17
Angela D Levy, Maria A Manning, Markku M Miettinen
Soft-tissue sarcomas occurring in the abdomen and pelvis are an uncommon but important group of malignancies. Recent changes to the World Health Organization classification of soft-tissue tumors include the movement of gastrointestinal stromal tumors (GISTs) into the soft-tissue tumor classification. GIST is the most common intraperitoneal sarcoma. Liposarcoma is the most common retroperitoneal sarcoma, and leiomyosarcoma is the second most common. GIST, liposarcoma, and leiomyosarcoma account for the majority of sarcomas encountered in the abdomen and pelvis and are discussed in part 1 of this article...
May 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28493287/soft-tissue-sarcoma-nomograms-and-their-incorporation-into-practice
#18
REVIEW
Dario Callegaro, Rosalba Miceli, Luigi Mariani, Chandrajit P Raut, Alessandro Gronchi
The accurate prediction of prognosis in patients with soft tissue sarcoma (STS) is a challenging issue. Extreme variability in the clinical and pathological characteristics of this family of tumors hinders the simple stratification of patients into meaningful prognostic cohorts. Precision medicine tools for the prediction of prognosis, such as nomograms, enable personalized computation of outcome based on clinical and pathological characteristics of both patient and tumor. The eighth edition of the American Joint Committee on Cancer staging manual moved from a "population-based" to a "personalized" approach endorsing high-quality nomograms to improve clinician prediction ability in definite patient subgroups...
May 10, 2017: Cancer
https://www.readbyqxmd.com/read/28492292/olaratumab-for-the-treatment-of-soft-tissue-sarcoma
#19
H A Deshpande, M Cecchini, S Ni Choileain, R Jones
Soft tissue sarcoma represents about 1% of all solid malignancies. The standard chemotherapy regimens have included doxorubicin alone or in combination with other agents. Despite recent advances in treatment beyond first line - with the FDA approval of pazopanib, eribulin and trabectidin - overall survival for patients with metastatic disease remains in the region of 12-19 months. Olaratumab is a monoclonal antibody directed against platelet-derived growth factor receptor alpha (PDGFRalpha). It was studied in a phase Ib and randomized phase II study in combination with doxorubicin in patients with soft tissue sarcoma who previously had not received doxorubicin for metastatic disease...
April 2017: Drugs of Today
https://www.readbyqxmd.com/read/28491193/indium-111-labeled-leukocyte-accumulation-in-extremity-soft-tissue-sarcoma
#20
Victor Fong, Lester Johnson
Nuclear medicine labeled leukocyte whole-body scintigraphy is commonly used to identify a source of infection in a patient with fever of unknown origin. White blood cells can also localize to other sites of inflammation, including sometimes tumors. A patient with a large myxofibrosarcoma in his left forearm was scanned due to chronic low-grade fever and persistent leukocytosis. This case demonstrates focal white blood cell activity in an extremity soft tissue sarcoma.
June 2017: Radiology case reports
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