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Desmoid tumor

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https://www.readbyqxmd.com/read/29901254/novel-intra-genic-large-deletions-of-ctnnb1-gene-identified-in-wt-desmoid-type-fibromatosis
#1
Chiara Colombo, Milena Urbini, Annalisa Astolfi, Paola Collini, Valentina Indio, Antonino Belfiore, Nicholas Paielli, Federica Perrone, Giuseppe Tarantino, Elena Palassini, Marco Fiore, Andrea Pession, Silvia Stacchiotti, Maria Abbondanza Pantaleo, Alessandro Gronchi
A wait and see approach for desmoid tumors (DT) has become part of the routine treatment strategy. However, predictive factors to select the risk of progressive disease are still lacking. A translational project was run in order to identify genomic signatures in patients enrolled within an Italian prospective observational study. Among 12 DT patients (ten CTNNB1-mutated and two WT) enrolled from our Institution only two patients (17%) showed a progressive disease. Tumor biopsies were collected for whole exome sequencing...
June 14, 2018: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/29879959/sporadic-abdominal-wall-desmoid-type-fibromatosis-treatment-paradigm-after-thirty-two-years
#2
S D Couto Netto, F Teixeira, C A M Menegozzo, H M Leão-Filho, A Albertini, F O Ferreira, E H Akaishi, E M Utiyama
BACKGROUND: Desmoid-type fibromatosis is a benign mesenchymal neoplastic process. It exhibits an uncertain growth pattern and high recurrence rate. Previously radical surgical resection was the mainstay of treatment, but recently more surgeons are opting for conservative management with observation ("wait and see" policy). The authors intend to evaluate different therapeutic modalities and oncological outcomes for abdominal wall desmoid tumors. METHODS: We performed a retrospective study of patients who underwent surgical, hormonal or chemotherapy treatment for abdominal wall desmoid tumors between 1982 to 2014 at two institutions affiliated with the University of São Paulo, Brazil...
June 7, 2018: BMC Surgery
https://www.readbyqxmd.com/read/29878366/high-cyclin-a-expression-but-not-ki67-is-associated-with-early-recurrence-in-desmoid-tumors
#3
Kirsi Santti, Hanna Ihalainen, Mikko Rönty, Tom Böhling, Christina Karlsson, Caj Haglund, Maija Tarkkanen, Carl Blomqvist
BACKGROUND AND OBJECTIVES: Desmoid tumors are soft-tissue tumors originating from myofibroblasts with a tendency to recur after surgery. High expression of proliferation markers is associated with shortened progression-free and/or overall survival in many neoplasms, including soft-tissue sarcomas. We investigated the prognostic role of cyclin A and Ki67 in desmoid tumors by immunohistochemistry. METHODS: The study included 76 patients with desmoid tumor operated at Helsinki University Hospital between 1987 and 2011...
June 7, 2018: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29863809/evaluation-of-pan-trk-immunohistochemistry-in-infantile-fibrosarcoma-lipofibromatosis-like-neural-tumor-and-histologic-mimics
#4
Yin P Hung, Christopher D M Fletcher, Jason L Hornick
AIMS: Infantile fibrosarcoma is characterized by intersecting fascicles of spindle cells and ETV6-NTRK3 gene fusion in most cases. Given histologic overlap with other spindle-cell tumors, the diagnosis can be challenging and often requires molecular confirmation. A recently developed pan-TRK antibody shows promise for identifying tumors with NTRK fusions. The purpose of this study was to evaluate the potential diagnostic utility of pan-TRK immunohistochemistry for infantile fibrosarcoma...
June 4, 2018: Histopathology
https://www.readbyqxmd.com/read/29804951/giant-desmoid-tumor-of-the-chest-wall-with-intrathoracic-growth
#5
Florencio Quero Valenzuela, Inmaculada Piedra Fernández, Francisco Hernández Escobar
No abstract text is available yet for this article.
May 24, 2018: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/29795437/nuclear-%C3%AE-catenin-localization-and-mutation-of-the-ctnnb1-gene-a-context-dependent-association
#6
Grace Kim, Katherine C Kurnit, Bojana Djordjevic, Charanjeet Singh, Mark F Munsell, Wei-Lien Wang, Alexander J Lazar, Wei Zhang, Russell Broaddus
Although the majority of low-grade, early-stage endometrial cancer patients have good survival with surgery alone, patients who recur tend to do poorly. Identification of patients at high risk of recurrence who would benefit from adjuvant treatment or more extensive surgical staging would help optimize individualized care of endometrial cancer patients. CTNNB1 (encodes β-catenin) mutations identify a subset of low-grade, early-stage endometrial cancer patients at high risk of recurrence. Mutation of CTNNB1 exon 3 is classically associated with translocation of the β-catenin protein from the membrane to the nucleus and activation of Wnt/β-catenin signaling...
May 24, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29794626/caring-for-a-patient-with-a-desmoid-tumor
#7
(no author information available yet)
No abstract text is available yet for this article.
June 2018: Nursing
https://www.readbyqxmd.com/read/29791426/-familiar-adenomatous-polyposis-report-of-2-cases
#8
Luis Vásquez Elera, Patricia Guzman Rojas, Manuel Sánchez Herrera, Víctor Prado, Carlos García Encinas, Eduar Albán Bravo Paredes, Alejandro Bussalleu
Familial Adenomatous polyposis (FAP) it is based on an autosomal dominant mutation which results in loss of function of theAPC tumor suppressor gene. On the other hand, Gardner syndrome is a type of FAP and is characterized for multiple colonic adenomatous polyps and extracolonic abnormalities as desmoid tumors, osteomas, lipomas, dental abnormalities, dermoid cysts and duodenal adenomas. This report aims to present two patients with FAP: The first one is a patient who presented with osteomas and hematochezia, being diagnosed with Gardner Syndrome after the colonoscopy...
January 2018: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
https://www.readbyqxmd.com/read/29768363/ex-vivo-resection-and-intestinal-autotransplantation-for-a-large-mesenteric-desmoid-tumor-secondary-to-familial-adenomatous-polyposis-a-case-report-and-literature-review
#9
Chao Cheng, Shuang Guo, Dakinah Eastman G B Kollie, Wanli Zhang, Jun Xiao, Jun Liu, Xiaoming Lu, Yong Xiao
RATIONALE: The mesenteric desmoid tumor requires special attention and the most demanding treatment. PATIENT CONCERNS: Here we present a rare case of a large mesenteric desmoid tumor secondary to familial adenomatous polyposis (FAP) in a 34-year-old man accepted the ex vivo resection, and intestinal autotransplantation. DIAGNOSES: A 34-year-old man was referred to our department with a 6-year history of intermittent hematochezia without any other discomfort after undergoing partial colectomy in February 2013, and 5 endoscopic mucosal resections of colon polyps between May 2012 and July 2015 due to pathological diagnosis of FAP...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29766555/hand-foot-skin-reaction-related-to-use-of-the-multikinase-inhibitor-sorafenib-and-hard-orthotics
#10
Ayan Kusari, Jenna Borok, Allison M Han, Alix Jessika Valderrama, Sheila Fallon Friedlander
Hand-foot-skin reaction is a distinct clinical condition arising in association with the use of multikinase inhibitors, including sorafenib. Because multikinase inhibitors are increasingly being used in children with cancer, recognition of this previously unfamiliar condition is of importance to pediatric dermatologists. We describe the diagnosis and successful treatment of a case of hand-foot-skin reaction in a child taking sorafenib for an unresectable desmoid tumor.
May 15, 2018: Pediatric Dermatology
https://www.readbyqxmd.com/read/29757868/caring-for-a-patient-with-a-desmoid-tumor
#11
Janette OʼSullivan, Margaret Vanderbeek-Warren
No abstract text is available yet for this article.
June 2018: Nursing
https://www.readbyqxmd.com/read/29752328/soft-tissue-sarcoma-version-2-2018-nccn-clinical-practice-guidelines-in-oncology
#12
Margaret von Mehren, R Lor Randall, Robert S Benjamin, Sarah Boles, Marilyn M Bui, Kristen N Ganjoo, Suzanne George, Ricardo J Gonzalez, Martin J Heslin, John M Kane, Vicki Keedy, Edward Kim, Henry Koon, Joel Mayerson, Martin McCarter, Sean V McGarry, Christian Meyer, Zachary S Morris, Richard J O'Donnell, Alberto S Pappo, I Benjamin Paz, Ivy A Petersen, John D Pfeifer, Richard F Riedel, Bernice Ruo, Scott Schuetze, William D Tap, Jeffrey D Wayne, Mary Anne Bergman, Jillian L Scavone
Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for STS provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumors, desmoid tumors, and rhabdomyosarcoma...
May 2018: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/29751773/an-unusual-finding-in-a-desmoid-type-fibromatosis-of-the-pancreas-a-case-report-and-review-of-the-literature
#13
Joseph Clarence Torres, Chen Xin
BACKGROUND: Desmoid-type fibromatoses are rare benign and fibrous tumors that account for approximately 0.03% of total neoplasms. Within this category of neoplasms, pancreatic desmoid-type fibromatosis is an extremely rare subgroup, accounting for approximately 5% of desmoid-type fibromatoses. Although the etiology is unknown, some risk factors include trauma, surgery, family history of desmoid tumor, pregnancy, use of contraceptives, genetic mutation, and familial adenomatous polyposis or Gardner syndrome...
May 12, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29735028/adult-intestinal-transplantation
#14
REVIEW
Cal S Matsumoto, Sukanya Subramanian, Thomas M Fishbein
Adult intestinal transplantation differs significantly from pediatric intestinal transplantation. While indications have remained largely consistent since 2000, indications for adults have expanded over the last two decades to include motility disorders and desmoid tumors. Graft type in adult recipients depends on the distinct anatomic characteristics of the adult recipient. Colonic inclusion, while initially speculated to portend unfavorable outcomes due to complex host-bacterial interactions has increased over the past two decades with superior graft survival and improved patient quality of life...
June 2018: Gastroenterology Clinics of North America
https://www.readbyqxmd.com/read/29719606/proof-of-concept-prognostic-value-of-the-plasmatic-concentration-of-circulating-cell-free-dna-in-desmoid-tumors-using-ddpcr
#15
Nicolas Macagno, Frédéric Fina, Nicolas Penel, Corinne Bouvier, Isabelle Nanni, Florence Duffaud, Raquel Rouah, Bruno Lacarelle, L'houcine Ouafik, Sylvie Bonvalot, Sébastien Salas
Since desmoid tumors (DT) exhibit an unpredictable clinical course, with stabilization and/or spontaneous regression, an initial "wait-and-see" policy is the new standard of care-thus, the actual challenge is to identify early factors of progression. We present a method of detection of CTNNB1 mutations using a targeted digital droplet PCR (ddPCR) on cell-free DNA (cfDNA) extracted from blood samples of 31 DT patients. Furthermore, we analyzed the correlation between DT evolution and plasmatic concentration of total and mutated cfDNA at the time of diagnosis...
April 6, 2018: Oncotarget
https://www.readbyqxmd.com/read/29709714/molecular-analyses-in-the-diagnosis-and-prediction-of-prognosis-in-non-gist-soft-tissue-sarcomas-a-systematic-review-and-meta-analysis
#16
REVIEW
Rita A Kandel, Xiaomei Yao, Brendan C Dickson, Michelle Ghert, Snezana Popovic, Bibianna M Purgina, Shailendra Verma, Joel Werier
BACKGROUND: The molecular pathogenesis of many forms of soft tissue sarcomas (STS) have been rigorously characterized in the medical literature, which may be particularly important for the diagnosis and prediction of prognosis in STS. METHODS: Electronic databases (2005 to October 2016) were searched. Gastrointestinal stromal tumor and pediatric sarcomas were excluded. The eligible individual study's risk of bias and the quality of aggregate evidence were assessed...
May 2018: Cancer Treatment Reviews
https://www.readbyqxmd.com/read/29705714/multifocal-occurrence-of-extra-abdominal-desmoid-type-fibromatosis-a-rare-manifestation-a-clinicopathological-study-of-6-sporadic-cases-and-1-hereditary-case
#17
Elise M Bekers, Danique L M van Broekhoven, Thijs van Dalen, Johan J Bonenkamp, Ingrid C M van der Geest, Jacky W J de Rooy, Joost M van Gorp, David H Creytens, Wendy W J de Leng, Blanca Scheijen, Astrid Eijkelenboom, Uta Flucke
Desmoid-type fibromatosis, also called desmoid tumor, is a locally aggressive myofibroblastic neoplasm that usually arises in deep soft tissue with significant potential for local recurrence. It displays an unpredictable clinical course. β-Catenin, the genetic key player of desmoid tumors shows nuclear accumulation due to mutations that prevent its degradation leading to activation of Wnt signaling and myofibroblastic cell proliferation. The corresponding hot spot mutations are located in exon 3 of the CTNNB1 gene or alternatively, in the APC tumor suppressor gene, most often as a germline mutation...
April 21, 2018: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/29644533/systematic-review-of-clinical-outcomes-following-various-treatment-options-for-patients-with-extraabdominal-desmoid-tumors
#18
Kortnye Smith, Jayesh Desai, Smaro Lazarakis, David Gyorki
BACKGROUND: Desmoid tumors (DT) are rare clonal proliferations that arise from mesenchymal cells. These tumors do not metastasize but are locally aggressive, and their growth may lead to significant morbidity. Their clinical course is both variable and unpredictable; tumors may rapidly progress but in other instances remain stable or regress without intervention. AIMS: To examine current treatment of DT and assist with decision-making at time of presentation. METHODS: A literature search was conducted of MEDLINE and Cochrane databases for published studies (1995-July 2015) using the search terms fibromatosis aggressive, desmoid with drug therapy, radiation therapy, prevention and control, radiotherapy, surgery, and therapy...
June 2018: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/29643736/desmoid-fibromatosis-of-the-lower-abdominal-wall-in-irrua-nigeria
#19
Oluwafemi Olasupo Awe, Sylvester Eluehike
Desmoid fibromatosis (desmoid tumors) is rare tumors. It can occur as intra-abdominal, extraabdominal, or abdominal wall tumor depending on the site. The abdominal wall type is usually sporadic, but few have been associated with familial adenomatous polyposis. They are commonly seen in young females who are pregnant with a history of the previous cesarean section scar or within the 1st year of the last childbirth. There is an association between this tumor, presence of estrogen receptors, and abdominal trauma...
January 2018: Nigerian Journal of Surgery: Official Publication of the Nigerian Surgical Research Society
https://www.readbyqxmd.com/read/29622108/desmoid-tumor
#20
Meryl C Nath, Michael S Torbenson, Lori A Erickson
No abstract text is available yet for this article.
April 2018: Mayo Clinic Proceedings
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