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Aggressive fibromatosis

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https://www.readbyqxmd.com/read/29330550/a-metabolomics-pilot-study-on-desmoid-tumors-and-novel-drug-candidates
#1
Kelly A Mercier, Mushriq Al-Jazrawe, Raymond Poon, Zachery Acuff, Benjamin Alman
Desmoid tumors (aggressive fibromatosis) are locally invasive soft tissue tumors that lack the ability to metastasize. There are no directed therapies or standard treatment plan, and chemotherapeutics, radiation, and surgery often have temporary effects. The majority of desmoid tumors are related to T41A and S45F mutations of the beta-catenin encoding gene (CTNNB1). Using broad spectrum metabolomics, differences were investigated between paired normal fibroblast and desmoid tumor cells from affected patients...
January 12, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29316163/systemic-therapy-of-aggressive-fibromatosis-in-children-and-adolescents-report-of-the-cooperative-weichteilsarkom-studiengruppe-cws
#2
Monika Sparber-Sauer, Guido Seitz, Thekla von Kalle, Christian Vokuhl, Ivo Leuschner, Monika Scheer, Marc Münter, Gustaf Ljungman, Stefan S Bielack, Felix Niggli, Ruth Ladenstein, Thomas Klingebiel, Joerg Fuchs, Ewa Koscielniak
BACKGROUND: Treatment algorithms for patients with aggressive fibromatosis (AF) are challenging. There are limited data available about the use of systemic therapy (ST) in pediatric patients with AF. METHODS: Patient-, tumor-, and treatment-related factors of 90 children and adolescents with AF treated on multiple prospective trials of the Cooperative Weichteilsarkom Studiengruppe (1981-2015) were analyzed with focus on response and outcome of ST. RESULTS: Median age was 9...
January 5, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29304783/desmoid-tumours-of-the-extremity-and-trunk-a-retrospective-study-of-44-patients
#3
Laura Wirth, Alexander Klein, Andrea Baur-Melnyk, Thomas Knösel, Lars H Lindner, Falk Roeder, Volkmar Jansson, Hans Roland Dürr
BACKGROUND: Desmoid-type fibromatosis (DF) is a aggressive (myo)fibroblastic neoplasm with an infiltrative growth and a tendency to local recurrence. Resection of the tumour and/or radiation were proposed as principal treatment. The aim of this retrospective study was to analyze the local control rates focusing on the effect of surgical margins and radiotherapy. METHODS: From 1981 to 2014, 44 patients had been treated. Fifty four therapies had been applied, in 50 cases surgery +/- radiation therapy, NSAIDs or chemotherapy...
January 5, 2018: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/29232006/reconstruction-of-a-large-full-thickness-abdominal-wall-defect-with-flow-through-based-alt-flaps-a-case-report
#4
Dong Yeon Kim, Junho Lee, Jeong Tae Kim, Hye Kyung Chang, Suk-Ho Moon
An extensive 35 × 20 cm sized full-thickness abdominal wall defect was created after resection of aggressive abdominal fibromatosis in a 19-year-old male patient. Immediate reconstruction was not possible due to prolonged operation time and resulting severe bowel edema. A silicone sheet with NPWT was applied over the exposed viscera. After 1 week, silicone sheet was substituted with a composite mesh. Then, abdominal wall reconstruction with bilateral free anterolateral thigh (ALT) flaps (30 × 12 cm and 25 × 12 cm sized) was performed...
December 12, 2017: Microsurgery
https://www.readbyqxmd.com/read/29220303/locally-aggressive-connective-tissue-tumors
#5
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/29170886/phase-ii-study-of-doxorubicin-and-thalidomide-in-patients-with-refractory-aggressive-fibromatosis
#6
Xin Liu, Huijie Wang, Xianghua Wu, Xiaonan Hong, Zhiguo Luo
Background To evaluate the efficacy and safety of doxorubicin (ADM) combined with thalidomide (THA) as a first-line treatment for patients with refractory aggressive fibromatosis (AF). Patients and Methods Eligible patients were treated with ADM 30 mg/m(2) on days 1-2 and THA 200 mg nightly on days 1-21 every 3 weeks for a maximum of six cycles. THA was then continued for a total of 1 year. The primary end point was response rate (RR). Results Fifteen patients were enrolled in the study. No patient had a complete response, but five patients had partial responses, resulting in a RR of 33%...
November 23, 2017: Investigational New Drugs
https://www.readbyqxmd.com/read/29113152/effect-of-surgical-margins-on-prognosis-in-aggressive-fibromatosis-a-single-institutional-analysis-of-90-patients
#7
Kamran Harati, Anais Jaenisch, Björn Behr, Ole Goertz, Ali Harati, Tobias Hirsch, Ingo Stricker, Marcus Lehnhardt, Adrien Daigeler
The treatment of aggressive fibromatosis poses a therapeutic challenge in an interdisciplinary setting. The extent of surgical resection is still discussed controversially. The present retrospective analysis aimed to determine prognostic factors leading to recurrence. Between 2000 and 2014, 114 patients with aggressive fibromatosis were treated surgically at BG-University Hospital Bergmannsheil (Bochum, Germany). Univariate and multivariate analyses were restricted to 90 participants with information available on surgical margins at the initial procedure...
November 2017: Oncology Letters
https://www.readbyqxmd.com/read/29096658/safety-and-efficacy-of-intralesional-steroid-injection-for-aggressive-fibromatosis
#8
Dumnoensun Pruksakorn, Sratwadee Lorsomradee, Areerak Phanphaisarn, Pimpisa Teeyakasem, Jeerawan Klangjorhor, Parunya Chaiyawat, Natapong Kosachunhanun, Jongkolnee Settakorn, Olarn Arpornchayanon
BACKGROUND: Treatment of recurrent aggressive fibromatosis (AF) following surgical resection is a clinical challenge. Non-steroidal anti-inflammatory drugs (NSAIDs) have been reported to be an effective option for controlling the disease. However, long-term NSAID use can result in unfavorable complications. This study was a trial of the use of intralesional steroid injection (ILSI) including investigation of safety margins and clinical outcomes of high-dose steroids for local use treatment of AF...
November 2, 2017: World Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29056131/late-presentation-of-aggressive-fibromatosis-involving-head-neck-and-chest-wall
#9
Waseem M Hajjar, Amjad F AlShehri, Mohammed A Alessa, Sami A Al-Nassar
Fibromatosis is a rare, benign, slow-growing and locally infiltrative tumour, caused by uncontrolled proliferation of fibrous tissue arising from muscles, connective tissue, fasciae and aponeurosis. It is also called desmoid tumour, which is very rare pathology representing only 0.03% of all neoplasms in human and around 3% of all soft tissue tumours. It is locally aggressive and usually invades the surrounding structures and has a high recurrence rate, even after surgical complete resection, which should be the first line of treatment...
October 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28961825/an-update-on-the-management-of-sporadic-desmoid-type-fibromatosis-a-european-consensus-initiative-between-sarcoma-patients-euronet-spaen-and-european-organization-for-research-and-treatment-of-cancer-eortc-soft-tissue-and-bone-sarcoma-group-stbsg
#10
B Kasper, C Baumgarten, J Garcia, S Bonvalot, R Haas, F Haller, P Hohenberger, N Penel, C Messiou, W T van der Graaf, A Gronchi
Desmoid-type fibromatosis is a rare and locally aggressive monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Currently, there is no established or evidence-based treatment approach available for this disease. Therefore, in 2015 the European Desmoid Working Group published a position paper giving recommendations on the treatment of this intriguing disease. Here, we present an update of this consensus approach based on professionals' AND patients' expertise following a round table meeting bringing together sarcoma experts from the European Organization for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group with patients and patient advocates from Sarcoma PAtients EuroNet...
October 1, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/28916434/effects-of-icos-t-cell-depletion-via-afucosylated-monoclonal-antibody-medi-570-on-pregnant-cynomolgus-monkeys-and-the-developing-offspring
#11
Simone M Nicholson, Gianluca Carlesso, Lily I Cheng, Halie Cook, Karma DaCosta, Joel Leininger, Kathleen McKeever, Stephen Weasel Scott, Devon Taylor, Katie Streicher, Steve Eck, Molly Reed, Raffaella Faggioni, Ronald Herbst, Rakesh Dixit, Patricia C Ryan
MEDI-570 is a fully human afucosylated monoclonal antibody (MAb) against Inducible T-cell costimulator (ICOS), highly expressed on CD4+ T follicular helper (TFH) cells. Effects of MEDI-570 were evaluated in an enhanced pre-postnatal development toxicity (ePPND) study in cynomolgus monkeys. Administration to pregnant monkeys did not cause any abortifacient effects. Changes in hematology and peripheral blood T lymphocyte subsets in maternal animals and infants and the attenuated infant IgG immune response to keyhole limpet hemocyanin (KLH) were attributed to MEDI-570 pharmacology...
September 13, 2017: Reproductive Toxicology
https://www.readbyqxmd.com/read/28883708/rarity-among-benign-gastric-tumors-plexiform-fibromyxoma-report-of-two-cases
#12
Kinga Szurian, Holger Till, Eva Amerstorfer, Nicole Hinteregger, Hans-Jörg Mischinger, Bernadette Liegl-Atzwanger, Iva Brcic
Plexiform fibromyxoma is a very rare mesenchymal tumor of the stomach, found almost exclusively in the antrum/pylorus region. The most common presenting symptoms are anemia, hematemesis, nausea and unintentional weight loss, without sex or age predilection. We describe here two cases of plexiform fibromyxoma, involving a 16-year-old female and a 34-year-old male. Both patients underwent complete resection (R0) by distal gastrectomy and retrocolic gastrojejunostomy (according to Billroth 2); for both, the postoperative course was uneventful...
August 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28881160/effective-treatment-of-aggressive-fibromatosis-with-celecoxib-guided-by-genetic-testing
#13
Shanshan Yang, Xufu Wang, Haiping Jiang, Yongjie Wang, Zhuokun Li, Haijun Lu
Aggressive fibromatosis (AF) or desmoid tumors is an aggressive fibroblastic proliferation which is locally invasive but can not metastasize. The treatment of AF is challenging. Surgery was the main treatment modality for AF in the past, other strategies including radiotherapy, systemic therapies and wait-and-see policy. The use of non-steroidal anti-inflammatory drugs (NSAIDs) and targeted therapies has demonstrated good results. In the case report, a 39-year-old man presented with progressive chest wall pain...
October 3, 2017: Cancer Biology & Therapy
https://www.readbyqxmd.com/read/28851389/immunohistochemical-staining-with-non-phospho-%C3%AE-catenin-as-a-diagnostic-and-prognostic-tool-of-cox-2-inhibitor-therapy-for-patients-with-extra-peritoneal-desmoid-type-fibromatosis
#14
Tomohisa Sakai, Yoshihiro Nishida, Shunsuke Hamada, Hiroshi Koike, Kunihiro Ikuta, Takehiro Ota, Naoki Ishiguro
BACKGROUND: Immunohistochemical staining with conventional anti-β-catenin antibody has been applied as a diagnostic tool for desmoid-type fibromatosis (DF). This study aimed to evaluate the diagnostic and prognostic value of immunohistochemical staining with anti-non-phospho β-catenin antibody, which might more accurately reflect the aggressiveness of DF, in comparison to the conventional anti-β-catenin antibody. METHODS: Between 2003 and 2015, 40 patients with extra-peritoneal sporadic DF were prospectively treated with meloxicam or celecoxib, a COX-2 inhibitor, therapy...
August 29, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28828097/radiologic-images-of-an-aggressive-implant-associated-fibromatosis-of-the-breast-and-chest-wall-case-report-and-review-of-the-literature
#15
Lourdes Alanis, Robyn Roth, Nati Lerman, Julieta E Barroeta, Pauline Germaine
Fibromatosis of the breast is a rare benign disease compromising <0.2% of all primary breast tumors. Although the chest wall is a common location, occurrences of implant-associated fibromatosis of the breast are extremely rare; only 33 cases have been reported. We present a case of a 42-year-old female who underwent breast augmentation with silicone breast implants, and 2 years later developed an aggressive implant-associated fibromatosis of the breast and chest wall. On imaging studies, the tumor mimicked breast carcinoma, and despite chemotherapy, the fibromatosis rapidly enlarged and was locally invasive requiring wide surgical excision...
September 2017: Radiology Case Reports
https://www.readbyqxmd.com/read/28819469/aggressive-fibromatosis-of-the-oral-cavity-in-a-5-year-old-boy-a-rare-case-report
#16
Keerthi Krishnankutty Nair, Kanad Chaudhuri, Ashok Lingappa, Ranjani Shetty, Pramod Gujjar Vittobarao
Fibrous tissue proliferations express a wide spectrum of histologic and morphologic variation in both infants and adults. This ranges from hypertrophic scar formation at one end to malignant fibrosarcoma at the other end of the spectrum. Aggressive fibromatosis is an intermediate tumor which is in proximity to fibrosarcomas. These are locally invasive and often recur after excision, but do not metastasize. Histologically, they are characterized by proliferating fibroblasts with little mitotic activity. Aggressive fibromatosis in the head and neck region is not common, and very sporadically occurs in the oral cavity or jaw bones...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28812409/tumor-reduction-and-symptom-relief-after-electrochemotherapy-in-a-patient-with-aggressive-fibromatosis-a-case-report
#17
Joanna Vitfell-Rasmussen, Rikke Mulvad Sandvik, Karin Dahlstrøm, Gina Al-Farra, Anders Krarup-Hansen, Julie Gehl
No abstract text is available yet for this article.
August 16, 2017: Acta Oncologica
https://www.readbyqxmd.com/read/28751104/myofibroblastic-fibroblastic-and-myoid-lesions-of-the-breast
#18
REVIEW
Gregor Krings, Patrick McIntire, Sandra J Shin
Myofibroblastic, fibroblastic and/or myoid lesions are rare in the breast but comprise the majority of mammary mesenchymal spindle cell lesions. Whereas most have similar features to their counterparts at extramammary sites, pseudoangiomatous stromal hyperplasia is considered a breast-specific myofibroblastic proliferation on the same spectrum as myofibroblastoma. Other lesions with myofibroblastic/fibroblastic differentiation include fibromatosis and nodular fasciitis, as well as more aggressive tumors such as the rarely reported myofibrosarcoma, inflammatory myofibroblastic tumor and fibrosarcoma...
September 2017: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28630763/malignant-tumours-of-the-foot-and-ankle
#19
E Mascard, N Gaspar, L Brugières, C Glorion, S Pannier, A Gomez-Brouchet
Most of tumours of the foot are tumour-like (synovial cyst, foreign body reactions and epidermal inclusion cyst) or benign conditions (tenosynovial giant cells tumours, planta fibromatosis). Malignant tumours of the soft-tissue and skeleton are very rare in the foot and their diagnosis is often delayed with referral to specialised teams after initial inappropriate procedures or unplanned excisions. The adverse effect of these misdiagnosed tumours is the increasing rate of amputation or local recurrences in the involved patients...
May 2017: EFORT Open Reviews
https://www.readbyqxmd.com/read/28578783/biology-and-treatment-of-aggressive-fibromatosis-or-desmoid-tumor
#20
REVIEW
Keith M Skubitz
Aggressive fibromatosis, also known as desmoid-type fibromatosis (DTF) or desmoid tumor, is an uncommon locally invasive tumor. Because of its low incidence and variable behavior, DTF is often first seen by physicians who are not familiar with it, and recent advances in understanding this disease have led to changes in treatment approaches. The Wnt (β-catenin) pathway appears to play a key role in DTF pathogenesis, and recent studies of DTF biology suggest a possible model of DTF pathogenesis. Histologically, DTF shows a poorly circumscribed proliferation of myofibroblast-like cells with variable collagen deposition, similar to the proliferative phase of wound healing, and DTF has been associated with trauma and pregnancy...
June 2017: Mayo Clinic Proceedings
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