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https://www.readbyqxmd.com/read/29220303/locally-aggressive-connective-tissue-tumors
#1
Mrinal M Gounder, David M Thomas, William D Tap
In this review, we highlight the complexities of the natural history, biology, and clinical management of three intermediate connective tissue tumors: desmoid tumor (DT) or aggressive fibromatosis, tenosynovial giant cell tumor (TGCT) or diffuse-type pigmented villonodular synovitis (dtPVNS), and giant cell tumor of bone (GCTB). Intermediate histologies include tumors of both soft tissue and bone origin and are locally aggressive and rarely metastatic. Some common aspects to these tumors are that they can be locally infiltrative and/or impinge on critical organs, which leads to disfigurement, pain, loss of function and mobility, neurovascular compromise, and occasionally life-threatening consequences, such as mesenteric, bowel, ureteral, and/or bladder obstruction...
December 8, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29220289/osteosarcoma-chondrosarcoma-and-chordoma
#2
Jeremy S Whelan, Lara E Davis
Osteosarcoma (OS), chondrosarcoma, and chordoma are characterized by multiple challenges to the investigator, clinician, and patient. One consequence of their rarity among sarcomas, as well as their biologic and clinical heterogeneity, is that management guidelines are inadequate to inform the range of individual patient-treatment decisions from diagnosis, approaches to surgery, chemotherapy, radiotherapy, treatment of recurrence, palliative care, and quality of survivorship. Of high-grade sarcomas, OSs are among the most curable, with more than two-thirds of patients with localized disease likely to achieve long-term survival...
December 8, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29218692/euro-b-o-s-s-a-european-study-on-chemotherapy-in-bone-sarcoma-patients-aged-over-40-outcome-in-primary-high-grade-osteosarcoma
#3
Stefano Ferrari, Stefan S Bielack, Sigbjørn Smeland, Alessandra Longhi, Gerlinde Egerer, Kirsten Sundby Hall, Davide Donati, Matthias Kevric, Otte Brosjö, Alessandro Comandone, Mathias Werner, Odd Monge, Emanuela Palmerini, Wolfgang E Berdel, Bodil Bjerkehagen, Anna Paioli, Sylvie Lorenzen, Mikael Eriksson, Marco Gambarotti, Per-Ulf Tunn, Nina L Jebsen, Marilena Cesari, Thekla von Kalle, Virginia Ferraresi, Rudolf Schwarz, Rossella Bertulli, Anne-Katrin Kasparek, Giovanni Grignani, Fatime Krasniqi, Benjamin Sorg, Stefanie Hecker-Nolting, Piero Picci, Peter Reichardt
INTRODUCTION: The EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.S.S.) was the first prospective international study for patients 41-65 years old with high-grade bone sarcoma treated with an intensive chemotherapy regimen derived from protocols for younger patients with high-grade skeletal osteosarcoma. METHODS: Chemotherapy based on doxorubicin, cisplatin, ifosfamide, and methotrexate was suggested, but patients treated with other regimens at the investigators' choice were also eligible for the study...
December 5, 2017: Tumori
https://www.readbyqxmd.com/read/29216884/intensity-modulated-radiation-therapy-and-surgery-for-management-of-retroperitoneal-sarcomas-a-single-institution-experience
#4
Pippa F Cosper, Jeffrey Olsen, Todd DeWees, Brian A Van Tine, William Hawkins, Jeff Michalski, Imran Zoberi
BACKGROUND: Peri-operative radiation of retroperitoneal sarcomas (RPS) is an important component of multidisciplinary treatment. All retrospective series thus far included patients treated with older radiation therapy (RT) techniques including 2D and 3DRT. Intensity modulated radiation therapy (IMRT) allows for selective dose escalation while sparing adjacent organs. We therefore report the first series of patients with RPS treated solely with IMRT, surgery and chemotherapy. We hypothesized that IMRT would permit safe dose escalation and superior rates of local control (LC) in this high-risk patient population...
December 8, 2017: Radiation Oncology
https://www.readbyqxmd.com/read/29212287/the-benefit-of-everolimus-in-recurrent-epithelioid-angiosarcoma-patients-case-reports-and-literature-review
#5
Shi-Long Zhang, Li Liang, Yuan Ji, Zhi-Ming Wang, Yu-Hong Zhou
Epithelioid angiosarcoma (EA) is a kind of rare malignant soft tissue sarcoma, with high recurrence/metastatic rate and poor prognosis. To date, no effective standardized treatment regimen has been available for patients with recurrence/metastatic EA. Everolimus is an oral rapamycin derivative that highly inhibits the mechanistic target of rapamycin(mTOR) signal pathway. Previous studies have suggested that everolimus is effective and safe in some soft tissue sarcoma. We reported two cases with recurrence/metastatic EA, who received everolimus after failure of surgery, radiotherapy, chemotherapy or interventional therapy...
November 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/29202127/obesity-does-not-exacerbate-the-protumorigenic-systemic-environment-in-sarcoma-subjects
#6
Claire M Buchta, Shannon K Boi, Benjamin J Miller, Mohammed M Milhem, Lyse A Norian
Sarcomas are a rare but fatal tumor type that accounts for <1% of adult solid malignancies and ~15% of childhood malignancies. Although the use of immunotherapy is being actively investigated for other solid tumors, advances in immunotherapy for sarcoma patients are lacking. To better understand the systemic immune environment in sarcoma patients, we performed a detailed multiplex analysis of serum cytokines, chemokines, and protumorigenic factors from treatment-naive subjects with localized, high-grade sarcoma...
June 1, 2017: ImmunoHorizons
https://www.readbyqxmd.com/read/29200103/bcor-internal-tandem-duplication-in-high-grade-uterine-sarcomas
#7
Adrián Mariño-Enriquez, Alexandra Lauria, Joanna Przybyl, Tony L Ng, Magdalena Kowalewska, Maria Debiec-Rychter, Raji Ganesan, Vaiyapuri Sumathi, Suzanne George, W Glenn McCluggage, Marisa R Nucci, Cheng-Han Lee, Jonathan A Fletcher
Endometrial stromal sarcomas (ESSs) are mesenchymal uterine tumors characterized by recurrent genetic events, most commonly chromosomal rearrangements, that create oncogenic gene fusions. High-grade endometrial stromal sarcomas (HG-ESSs), as defined in the 2014 World Health Organization Classification, typically contain oncogenic YWHAE-NUTM2 fusions; however, although not well characterized, there are tumors morphologically overlapping with HG-ESS that do not contain the YWHAE-NUTM2 fusions. These fusions are also found in certain pediatric primitive sarcomas, including clear cell sarcoma of the kidney and soft tissue undifferentiated round cell sarcoma of infancy...
December 1, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29198676/case-report-primary-de-novo-sarcoma-in-transplant-pancreas-allograft
#8
S Nagaraju, S J Grethlein, S Vaishnav, A A Sharfuddin, J A Powelson, J A Fridell
BACKGROUND: The majority of malignancies after transplantation appear to be virally mediated and of recipient origin. Donor-derived neoplasms occur early, whereas recipient-origin tumors typically occur many years after transplantation. Sarcomas are a relatively rare form of cancer. The etiology of sarcomas remains largely unknown, although some are linked to viruses, familial cancer syndromes, or therapeutic radiation exposure. Primary sarcomas are extremely rare, accounting for <0...
December 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/29196822/osteogenic-melanoma-report-of-a-case-mimicking-osteosarcoma-and-review-of-the-literature
#9
Deepika Savant, Shachar Kenan, Samuel Kenan, Leonard Kahn
A 32-year-old male presented with a right thumb lesion of 11-month duration. A clinical diagnosis of pyogenic granuloma was entertained but the lesion failed to respond to conservative therapy and eventually necessitated amputation of his thumb. MRI of the right thumb showed an ill-defined, heterogeneously enhancing, infiltrating mass within the dorsal soft tissues abutting the distal phalanx and measuring 4.2 × 2.4 × 0.7 cm. Histologically, the tumor was composed of a high-grade osteosarcoma with a chondrosarcomatous component localized within the underlying bone and a more superficial spindle cell component in the overlying soft tissue...
December 1, 2017: Skeletal Radiology
https://www.readbyqxmd.com/read/29192652/zc3h7b-bcor-high-grade-endometrial-stromal-sarcomas-a-report-of-17-cases-of-a-newly-defined-entity
#10
Natasha Lewis, Robert A Soslow, Deborah F Delair, Kay J Park, Rajmohan Murali, Travis J Hollmann, Ben Davidson, Francesca Micci, Ioannis Panagopoulos, Lien N Hoang, Javier A Arias-Stella, Esther Oliva, Robert H Young, Martee L Hensley, Mario M Leitao, Meera Hameed, Ryma Benayed, Marc Ladanyi, Denise Frosina, Achim A Jungbluth, Cristina R Antonescu, Sarah Chiang
High-grade endometrial stromal sarcoma likely encompasses underrecognized tumors harboring genetic abnormalities besides YWHAE-NUTM2 fusion. Triggered by three initial endometrial stromal sarcomas with ZC3H7B-BCOR fusion characterized by high-grade morphology and aggressive clinical behavior, we herein investigate the clinicopathologic features of this genetic subset by expanding the analysis to 17 such tumors. All of them occurred in adult women with a median age of 54 (range, 28-71) years. They were predominantly based in the endomyometrium and demonstrated tongue-like and/or pushing myometrial invasion...
December 1, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29192019/pre-and-postoperative-chemotherapy-in-localized-extremity-soft-tissue-sarcoma-a-european-organization-for-research-and-treatment-of-cancer-expert-survey
#11
REVIEW
Christian Rothermundt, Galina F Fischer, Sebastian Bauer, Jean-Yves Blay, Viktor Grünwald, Antoine Italiano, Bernd Kasper, Attila Kollár, Lars H Lindner, Aisha Miah, Stefan Sleijfer, Silvia Stacchiotti, Paul Martin Putora
BACKGROUND: The management of localized extremity soft tissue sarcomas (STS) is challenging and the role of pre- and postoperative chemotherapy is unclear and debated among experts. MATERIALS AND METHODS: Medical oncology experts of the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group were asked to participate in this survey on the use of pre- and postoperative chemotherapy in STS. Experts from 12 centers in Belgium, France, Germany, Great Britain, Italy, Switzerland, and The Netherlands agreed to participate and provided their treatment algorithm...
November 30, 2017: Oncologist
https://www.readbyqxmd.com/read/29190507/results-of-methotrexate-etoposide-ifosfamide-based-regimen-m-ei-in-osteosarcoma-patients-included-in-the-french-os2006-sarcome-09-study
#12
Nathalie Gaspar, Bob-Valéry Occean, Hélène Pacquement, Emmanuelle Bompas, Corine Bouvier, Hervé J Brisse, Marie-Pierre Castex, Nadir Cheurfa, Nadège Corradini, Jessy Delaye, Natacha Entz-Werlé, Jean-Claude Gentet, Antoine Italiano, Cyril Lervat, Perrine Marec-Berard, Eric Mascard, Françoise Redini, Laure Saumet, Claudine Schmitt, Marie-Dominique Tabone, Cécile Verite-Goulard, Marie-Cécile Le Deley, Sophie Piperno-Neumann, Laurence Brugieres
BACKGROUND: In most countries, reference chemotherapy for osteosarcoma is MAP regimen (M = high-dose methotrexate, AP = doxorubicin-cisplatinum). In France, the standard preoperative chemotherapy for children/adolescents combines M and etoposide-ifosfamide (EI), based on the OS94-trial. We report the safety and efficacy results of patients ≤25 years treated with preoperative M-EI regimen enroled in the French OS2006-study, between 2007 and 2014. METHODS: Treatment comprised preoperative chemotherapy with the 7 M-courses and 2 EI-courses, then surgery and postoperative chemotherapy assigned by risk's groups: standard-risk (good histological response without metastases) received 12 M-courses, 3 EI-courses; high-risk (poor histologic response, initial metastases or unresectable primary) received 5 M-courses alternated with 5 AP-courses...
November 27, 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/29187496/mucinous-cystic-neoplasms-lined-by-abundant-mucinous-epithelium-frequently-involve-kras-mutations-and-malignant-progression
#13
Hideki Shibata, Nobuyuki Ohike, Tomoko Norose, Tomohide Isobe, Reika Suzuki, Hideyuki Imai, Akira Shiokawa, Takeshi Aoki, Masahiko Murakami, Hiroki Mizukami, Jun-Ichi Tanaka, Masafumi Takimoto
BACKGROUND: Pancreatic and hepatic mucinous cyst neoplasms (MCNs) have a malignant potential, but indolent MCNs are not uncommon. MATERIALS AND METHODS: The pathological and genetic characteristics of resected MCNs (n=15) categorized by the amount of mucin of the lining epithelium were investigated. RESULTS: MCNs were divided into two groups: (i) a rich (r)-MCN group (n=6), in which more than half of the epithelium was lined by abundant mucinous epithelium; and (ii) a poor (p)-MCN group (n=9), which consisted of the remaining cases...
December 2017: Anticancer Research
https://www.readbyqxmd.com/read/29187480/clinical-efficacy-of-carbon-ion-radiotherapy-for-unresectable-chondrosarcomas
#14
Reiko Imai, Tadashi Kamada, Nobuhito Araki
BACKGROUND: Carbon ion radiotherapy has precise dose distribution and high biological effectiveness and is likely a promising therapy for patients with sarcomas. We evaluated the outcomes of carbon ion radiotherapy in patients with unresectable chondrosarcomas. PATIENTS AND METHODS: A retrospective analysis of 75 tumors in 73 patients treated with carbon ion radiotherapy was performed. There were 26 spinal, 38 pelvic, and 11 other types of tumors. Seventy conventional and five dedifferentiated chondrosarcomas were treated in 69 and four patients, respectively...
December 2017: Anticancer Research
https://www.readbyqxmd.com/read/29152519/primary-carcinosarcoma-of-the-pancreas-with-cd10-positive-sarcoma-component
#15
Christine J Salibay, Julia Rewerska, Shweta Gupta, Nicholas Ree
Carcinosarcomas of the pancreas are rare entities with a dismal prognosis. We report a rare case of pancreatic carcinosarcoma in a 49-year-old African American female who underwent a total abdominal hysterectomy with right salpingo-oophorectomy and exploration of the pancreatic mass. The surgery revealed a sclerotic mass in the body and tail of the pancreas that was surgically unresectable, and a pancreatic biopsy confirmed the pathology of pancreatic carcinosarcoma. Histologically, the lesion showed a high-grade spindle cell sarcoma and adjacent moderately differentiated adenocarcinoma...
October 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29152444/excellent-pathologic-response-and-atypical-clinical-course-of-high-grade-extremity-sarcoma-to-neoadjuvant-pencil-beam-scanning-proton-therapy
#16
Jill Remick, William Regine, Robert Malyapa, Vincent Ng, Melissa Vyfhuis, Tejan Diwanji, Susan Shyu, James W Snider
Neoadjuvant radiation therapy, followed by definitive surgical resection, remains the standard of care for resectable high-grade and unresectable soft tissue sarcomas. Proton therapy offers the promise of highly conformal dose distributions with improved sparing of neighboring normal tissues as compared with conformal and intensity modulated photon techniques. It is unclear whether proton therapy may offer an improved tumoral response, especially with dose escalation, in this relatively radio-insensitive tumor type...
September 15, 2017: Curēus
https://www.readbyqxmd.com/read/29149644/assessing-the-risk-of-laparoscopic-morcellation-of-occult-uterine-sarcomas-during-hysterectomy-and-myomectomy-literature-review-and-the-isge-recommendations
#17
REVIEW
Ornella Sizzi, Lucia Manganaro, Alfonso Rossetti, Matteo Saldari, Giuseppe Florio, Alessandro Loddo, Robert Zurawin, Bruno van Herendael, Dusan Djokovic
OBJECTIVE: This project of the International Society for Gynecologic Endoscopy (ISGE) had the objective to review the literature and provide recommendations on the occult sarcoma risk assessment in patients who are candidates for minimally invasive gynecological surgery involving intra-abdominal electromechanical tissue morcellation. STUDY DESIGN: The ISGE Task Force for Estimation of the Risk in Endoscopic Morcellation initially defined key topics and clinical questions which may guide a comprehensive preoperative patient assessment...
November 7, 2017: European Journal of Obstetrics, Gynecology, and Reproductive Biology
https://www.readbyqxmd.com/read/29143983/growth-of-doxorubicin-resistant-undifferentiated-spindle-cell-sarcoma-pdox-is-arrested-by-metabolic-targeting-with-recombinant-methioninase
#18
Kentaro Igarashi, Shukuan Li, Qinghong Han, Yuying Tan, Kei Kawaguchi, Takashi Murakami, Tasuku Kiyuna, Kentaro Miyake, Yunfeng Li, Scott D Nelson, Sarah M Dry, Arun S Singh, Irmina A Elliott, Tara A Russell, Mark A Eckardt, Norio Yamamoto, Katsuhiro Hayashi, Hiroaki Kimura, Shinji Miwa, Hiroyuki Tsuchiya, Fritz C Eilber, Robert M Hoffman
Undifferentiated spindle-cell sarcoma (USCS) is a recalcitrant -cancer in need of individualized therapy. A high-grade USCS from a striated muscle of a patient was grown orthotopically in the right biceps femoris muscle of nude mice to establish a patient-derived orthotopic xenograft (PDOX) model. In a previous study, we evaluated the efficacy of standard first-line chemotherapy of doxorubicin (DOX), gemcitabine (GEM) combined with docetaxel (DOC), compared to pazopanib (PAZ), a multi-targeting tyrosine-kinase inhibitor, in an USCS PDOX model...
November 16, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/29140881/pdgfb-rearrangements-in-dermatofibrosarcoma-protuberans-of-the-vulva-a-study-of-11-cases-including-myxoid-and-fibrosarcomatous-variants
#19
Khadijeh Jahanseir, Deyin Xing, Patricia T Greipp, William R Sukov, Gary L Keeney, Brooke E Howitt, J Kenneth Schoolmeester
Dermatofibrosarcoma protuberans (DFSP) is a low-grade fibroblastic sarcoma that tends to arise in young to middle age adults and involve the trunk and proximal extremities. Rare examples of vulvar DFSP have been reported, including myxoid, myoid, and fibrosarcomatous variants, but detection of the characteristic t(17;22)(q22;q13) that produces COL1A1-PDGFB gene fusion has not been evaluated in a large series of primary vulvar tumors. The clinical, morphologic, immunohistochemical, and molecular cytogenetic features of 11 cases were examined...
November 14, 2017: International Journal of Gynecological Pathology
https://www.readbyqxmd.com/read/29137065/primary-leiomyosarcoma-of-the-bone-a-case-report-and-a-review-of-the-literature
#20
Federica Recine, Alberto Bongiovanni, Roberto Casadei, Federica Pieri, Nada Riva, Alessandro De Vita, Laura Mercatali, Chiara Liverani, Chiara Spadazzi, Giacomo Miserocchi, Valentina Fausti, Dino Amadori, Toni Ibrahim
RATIONALE: Leiomyosarcoma (LMS) is a malignant sarcoma that can occur in different anatomic sites, including the bone, showing similar histological characteristics but heterogeneous clinical behavior and prognosis. Primary bone LMS was first described in 1965. It is a very rare sarcoma, accounting for <0.7% of all primary malignant bone tumors. PATIENT CONCERNS: We report the case of a 52-year-old male with primary bone LMS who presented with a solitary osteolytic lesion with focal cortical destruction in the left clavicle, seen on an x-ray and subsequent computed tomography (CT) scan...
November 2017: Medicine (Baltimore)
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