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sarcoma soft tissue

Neimar de Paula Silva, Rejane de Souza Reis, Rafael Garcia Cunha, Júlio Fernando Pinto Oliveira, Marceli de Oliveira Santos, Maria S Pombo-de-Oliveira, Beatriz de Camargo
BACKGROUND: Several maternal and birth characteristics have been reported to be associated with an increased risk of many childhood cancers. Our goal was to evaluate the risk of childhood embryonal solid tumors in relation to pre- and perinatal characteristics. METHODS: A case-cohort study was performed using two population-based datasets, which were linked through R software. Tumors were classified as central nervous system (CNS) or non-CNS-embryonal (retinoblastoma, neuroblastoma, renal tumors, germ cell tumors, hepatoblastoma and soft tissue sarcoma)...
2016: PloS One
Francesca Megiorni, Simona Camero, Simona Ceccarelli, Heather P McDowell, Olga Mannarino, Francesco Marampon, Barry Pizer, Rajeev Shukla, Antonio Pizzuti, Cinzia Marchese, Anna Clerico, Carlo Dominici
Aberrant DNA methylation has been frequently observed in many human cancers, including rhabdomyosarcoma (RMS), the most common soft tissue sarcoma in children. To date, the expression and function of the de novo DNA methyltransferase (DNMT) 3B in RMS have not yet been investigated. Our study show for the first time a significant up-regulation of DNMT3B levels in 14 RMS tumour samples and 4 RMS cell lines in comparison to normal skeletal muscle. Transfection of RD and TE671 cells, two in vitro models of embryonal RMS (ERMS), with a synthetic DNMT3B siRNA decreased cell proliferation by arresting cell cycle at G1 phase, as demonstrated by the reduced expression of Cyclin B1, Cyclin D1 and Cyclin E2, and by the concomitant up-regulation of the checkpoint regulators p21 and p27...
October 15, 2016: Oncotarget
Petra Balogh, Rita Bánusz, Monika Csóka, Zsófia Váradi, Edit Varga, Zoltán Sápi
BACKGROUND: Rhabdomyosarcoma (RMS) is a malignant tumor of mesenchymal origin and comprises the largest category of soft-tissue sarcomas both in children and adolescents. From a pediatric oncology point of view, RMS has traditionally been classified into alveolar (ARMS) and embryonal (ERMS) subtypes. The anatomical localization of the tumor may vary, but commonly involve the head/neck regions, male and female urogenital tract or the trunk and extremities. CASE PRESENTATION: Here, we report two challenging cases involving 17- and 9-years-olds males where diffuse and multiplex bone lesions suggested either a hematological disease or a primary bone tumor (mesenchymal chondrosarcoma)...
October 18, 2016: Diagnostic Pathology
Cai-Hua Wang, Chuan-Cai Xu, Jun-Hong Jiang, Yan-Bin Chen, Sheng-Hua Zhan
Primary pulmonary synovial sarcoma (PPSS) is a rare disease. Diagnosis is made postoperatively following resection of the tumor. We describe the case of a 39-year-old non-smoking woman whose chest imaging revealed a heterogeneous mass (5.4 cm × 4.6 cm), with soft tissue density in the right upper lobe and pleural effusion in the right hemithorax. The tumor was enhanced on a computed tomography scan, in which enlargement of the mediastinal lymph nodes compressing the adjacent superior vena cava was observed...
September 10, 2016: Thoracic Cancer
Olivier Mir, Thomas Brodowicz, Antoine Italiano, Jennifer Wallet, Jean-Yves Blay, François Bertucci, Christine Chevreau, Sophie Piperno-Neumann, Emmanuelle Bompas, Sébastien Salas, Christophe Perrin, Corinne Delcambre, Bernadette Liegl-Atzwanger, Maud Toulmonde, Sarah Dumont, Isabelle Ray-Coquard, Stéphanie Clisant, Sophie Taieb, Cécile Guillemet, Maria Rios, Olivier Collard, Laurence Bozec, Didier Cupissol, Esma Saada-Bouzid, Christine Lemaignan, Wolfgang Eisterer, Nicolas Isambert, Loïc Chaigneau, Axel Le Cesne, Nicolas Penel
BACKGROUND: Regorafenib is a multikinase inhibitor with proven activity in refractory gastrointestinal stromal tumours and chemotherapy-refractory advanced colorectal cancers. We assessed this agent's efficacy and safety in patients with metastatic soft tissue sarcomas previously treated with anthracycline. METHODS: In this randomised, double-blind, phase 2 trial undertaken in France and Austria, we enrolled patients aged 18 years and older with advanced soft tissue sarcomas who had received previous doxorubicin or other anthracycline treatment...
October 14, 2016: Lancet Oncology
Maureen O'Sullivan
Since its foundation by remarkably talented and insightful individuals, prominently including Pepper Dehner, pediatric soft tissue tumor pathology has developed at an immense rate. The morphologic classification of tumoral entities has extensively been corroborated, but has also evolved with refinement or realignment of these classifications, through accruing molecular data, with many derivative ancillary diagnostic assays now already well-established. Tumors of unclear histogenesis, classically morphologically undifferentiated, are prominent amongst pediatric sarcomas, however, the classes of undifferentiated round- or spindle-cell-tumors-not-otherwise-specified are being dismantled gradually with the identification of their molecular underpinnings...
September 5, 2016: Seminars in Diagnostic Pathology
Maria W Gunnes, Rolv T Lie, Tone Bjørge, Sara Ghaderi, Astri Syse, Ellen Ruud, Finn Wesenberg, Dag Moster
Suicide risk in adult cancer patients is found to be elevated, but limited information exists regarding risks of suicide and non-suicidal violent deaths when diagnosed with cancer in young age. We investigate suicide and violent deaths in a national cohort including individuals diagnosed with cancer before age 25. Through the linkage of different national registries (Cancer Registry of Norway, Norwegian Causes of Death Registry, and the National Registry) a cohort of all live births in Norway during 1965-1985 was defined and followed up through 2008...
October 17, 2016: International Journal of Cancer. Journal International du Cancer
Imen Ben Salha, Shane Zaidi, Jonathan Noujaim, Aisha B Miah, Cyril Fisher, Robin L Jones, Khin Thway
Dedifferentiated liposarcoma (DDL) is a histologically pleomorphic sarcoma, traditionally defined as well-differentiated liposarcoma with abrupt transition to high grade, non-lipogenic sarcoma. It can occur as part of recurrent well-differentiated liposarcoma, or may arise de novo. DDL most frequently occurs within the retroperitoneum, and while it is prone to local recurrence, it usually has a lower rate of metastasis than other pleomorphic sarcomas. We describe a case of retroperitoneal dedifferentiated liposarcoma in a 63-year-old male, who showed MDM2 amplification with fluorescence in situ hybridization, which displayed unusually aggressive behavior, with brain, lung and subcutaneous soft tissue metastases...
September 5, 2016: Rare Tumors
Min Jung Kim, Min Seok Hur, Byung Gon Choi, Soo Young Kim, Yang Won Lee, Yong Beom Choe, Kyu Joong Ahn
Dermatofibrosarcoma protuberans (DFSP) is a rare disease of dermal fibroblastic origin that accounts for less than 5% of all soft tissue sarcomas in adults. DFSP grows slowly and is an asymptomatic lesion at the initial diagnosis. Herein, we report a case of multiple pedunculated nodules as a variant of DFSP. A 47-year-old man presented with a 7-month history of multiple well-circumscribed, firm, pedunculated nodules on the inguinal area. Histopathologic examination results showed densely packed uniform spindle cells with a storiform and cartwheel pattern, and positivity for CD34...
October 2016: Annals of Dermatology
Randall Winnette, Lisa M Hess, Steven J Nicol, Datchen Fritz Tai, Catherine Copley-Merriman
BACKGROUND: Soft tissue sarcomas (STS) are a heterogenous group of rare tumors that involve the connective tissue in the body (e.g. muscle, tendons). As with many rare tumors, little is known about the impact of STS on patient well-being. OBJECTIVE: The aim of this review was to better understand current knowledge related to patient experience and quality of life (QOL) following diagnosis of STS. METHODS: A systematic review of English-language articles published from 2005 to 2015 was conducted in the PubMed/MEDLINE, Embase, PsychINFO, and Evidence-Based Medicine databases...
October 15, 2016: Patient
Shimpei Miyamoto, Masahide Fujiki, Fumihiko Nakatani, Eisuke Kobayashi, Masanobu Sakisaka, Minoru Sakuraba
BACKGROUND: Complex groin defects after sarcoma resection require reconstruction of multiple vital structures that can include the major vessels, the overlying skin, the abdominal wall, and the hip joint. We investigated the feasibility of limb preservation after complex groin reconstruction. METHODS: The subjects were 12 consecutive patients who underwent complex groin reconstruction after sarcoma resection. In all patients, the defect included a major artery (external iliac or femoral) and overlying skin...
October 6, 2016: Annals of Plastic Surgery
Zeeshan Khan, Adam M Gerrish, Robert J Grimer
INTRODUCTION: The scapula is not an uncommon site for bone and soft tissue tumours and can be difficult to delineate on examination. Furthermore, these lesions can be potentially challenging to biopsy due to its close anatomical relationship with important structures. We present an epidemiological survey of all the scapular and periscapular lesions presenting to our institution. METHODOLOGY: This was a retrospective study with data obtained from a prospectively held electronic database over a 30-year period...
2016: SICOT-J
O Kalita, K Cwiertka, D Vrána, M Vaverka, L Tučková, M Megová
BACKGROUND: Malignant peripheral nerve sheath tumor schwannoma (MPNST), also known as malignant schwannoma, is a very rare tumor accounting for only 2% of all sarcomas. The prognosis is relatively poor, with a 5-year survival rate of 46-69%. The treatment of MPNST has not been standardized yet. Mainstay treatment is radical resection. Oncological adjuvant or neoadjuvant treatment has equivocal indications with unclear effects. CASE: The case report presents a 55-year-old patient who showed resistance in the medial-ventral area of the left lower limb...
2016: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
Michiro Susa, Kazutaka Kikuta, Robert Nakayama, Kazumasa Nishimoto, Keisuke Horiuchi, Sota Oguro, Masanori Inoue, Hideki Yashiro, Seishi Nakatsuka, Masaya Nakamura, Morio Matsumoto, Kazuhiro Chiba, Hideo Morioka
BACKGROUND: Historically, local control of recurrent sarcomas has been limited to radiotherapy when surgical re-resection is not feasible. For metastatic carcinomas to the bone or soft tissue, radiotherapy and some interventional radiology treatment along with other systemic therapies have been widely advocated due to the possibility of disseminated disease. These techniques are effective in alleviating pain and achieving local control for some tumor types, but it has not been effective for prolonged local control of most tumors...
October 13, 2016: BMC Cancer
Masaoki Sasaki, Hiroaki Izumi, Takaaki Yokoyama, Motohiro Kojima, Ako Hosono
Follicular dendritic cell sarcoma (FDCS) is a very rare malignant tumor derived from follicular dendritic cells. Radical resection is the standard therapy for patients with local disease, but an optimal chemotherapy regimen has not been determined for unresectable disease. We report our experience of an FDCS patient with multiorgan involvement. In the present case, disease was only located in the pancreas initially and radical resection was performed. Multiple metastasis developed after the treatment and several factors that indicated a poor prognosis were observed...
October 13, 2016: Hematological Oncology
Sha Lou, Arjen H G Cleven, Benjamin Balluff, Marieke de Graaff, Marie Kostine, Inge Briaire-de Bruijn, Liam A McDonnell, Judith V M G Bovée
BACKGROUND: Previous studies on high grade sarcomas using mass spectrometry imaging showed proteasome activator complex subunit 1 (PSME1) to be associated with poor survival in soft tissue sarcoma patients. PSME1 is involved in immunoproteasome assembly for generating tumor antigens presented by MHC class I molecules. In this study, we aimed to validate PSME1 as a prognostic biomarker in an independent and larger series of soft tissue sarcomas by immunohistochemistry. METHODS: Tissue microarrays containing leiomyosarcomas (n = 34), myxofibrosarcomas (n = 14), undifferentiated pleomorphic sarcomas (n = 15), undifferentiated spindle cell sarcomas (n = 4), pleomorphic liposarcomas (n = 4), pleomorphic rhabdomyosarcomas (n = 2), and uterine leiomyomas (n = 7) were analyzed for protein expression of PSME1 using immunohistochemistry...
2016: Clinical Sarcoma Research
A Gronchi, S Stacchiotti, P Verderio, S Ferrari, J Martin Broto, A Lopez-Pousa, A Llombart-Bosch, A P Dei Tos, P Collini, J Cruz Jurado, A De Paoli, D M Donati, A Poveda, V Quagliuolo, A Comandone, G Grignani, C Morosi, A Messina, R De Sanctis, S Bottelli, E Palassini, P G Casali, Piero Picci
BACKGROUND: To report on long-term results of a phase 3 trial comparing three versus five cycles of adjuvant chemotherapy (CT) with full-dose epirubicin+ifosfamide in high-risk soft tissue sarcomas (STS). METHODS: Patients (pts) were randomized to receive three preoperative cycles of epirubicin 120 mg/m(2) and ifosfamide 9 g/m(2) (Arm A) or to receive the same three preoperative cycles plus two postoperative cycles (Arm B). Radiotherapy could be either delivered in the preoperative or in the postoperative setting...
October 11, 2016: Annals of Oncology: Official Journal of the European Society for Medical Oncology
Fien Hoefkens, Charlotte Dehandschutter, Johan Somville, Paul Meijnders, Dirk Van Gestel
Soft tissue sarcomas are uncommon tumours of mesenchymal origin, most commonly arising in the extremities. Treatment includes surgical resection in combination with radiotherapy. Resection margins are of paramount importance in surgical treatment of soft tissue sarcomas but unambiguous guidelines for ideal margins of resection are still missing as is an uniform guideline on the use of radiotherapy.The present paper reviews the literature on soft tissue sarcomas of the extremities regarding the required resection margins, the impact of new radiotherapy techniques and the timing of radiotherapy, more particularly if it should be administered before or after surgical resection...
October 12, 2016: Radiation Oncology
Joel C Thompson, Gary M Woods, Michael A Arnold, Charles Elmaraghy, Samir B Kahwash, Timothy P Cripe, Bhuvana A Setty
Pediatric soft tissue sarcomas of the oral/maxillofacial region are rare neoplasms that present significant difficulty with respect to treatment and local control measures. We report four cases of pediatric oral/maxillofacial soft tissue sarcomas from our tertiary care pediatric hospital and emphasize the rarity of these malignancies and the challenges encountered in treating these lesions, and suggest areas for further research. We conclude that multimodal therapy and interdisciplinary cooperation are paramount to successful management of these lesions...
May 2016: Case Reports in Oncology
Catherine H Davis, Halim Yammine, Puja G Khaitan, Edward Y Chan, Min P Kim
INTRODUCTION: Soft tissue sarcomas of the chest wall are exceptionally rare entities that present as painless slow growing masses. Resection is often precarious due to involvement of vital structures, and patients are left with large chest wall defects postoperatively requiring extensive reconstruction. PRESENTATION OF CASE: We present a case report of a 29 year-old man who presented with a giant soft tissue sarcoma of the chest that had been growing slowly for one year prior to presentation...
October 4, 2016: International Journal of Surgery Case Reports
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