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https://www.readbyqxmd.com/read/28515694/fam20a-gene-mutation-amelogenesis-or-ectopic-mineralization
#1
Guilhem Lignon, Fleur Beres, Mickael Quentric, Stephan Rouzière, Raphael Weil, Muriel De La Dure-Molla, Adrien Naveau, Renata Kozyraki, Arnaud Dessombz, Ariane Berdal
Background and objective:FAM20A gene mutations result in enamel renal syndrome (ERS) associated with amelogenesis imperfecta (AI), nephrocalcinosis, gingival fibromatosis, and impaired tooth eruption. FAM20A would control the phosphorylation of enamel peptides and thus enamel mineralization. Here, we characterized the structure and chemical composition of unerupted tooth enamel from ERS patients and healthy subjects. Methods: Tooth sections were analyzed by Scanning Electron Microscopy (SEM), Energy Dispersive Spectroscopy (EDS), X-Ray Diffraction (XRD), and X-Ray Fluorescence (XRF)...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28509377/h3-1-k36m-mutation-in-a-congenital-onset-soft-tissue-neoplasm
#2
Kristin D Kernohan, David Grynspan, Raveena Ramphal, Eric Bareke, You Chang Wang, Elizabeth Nizalik, Jiannis Ragoussis, Nada Jabado, Kym M Boycott, Jacek Majewski, Sarah L Sawyer
We describe a patient who presented with a congenital soft tissue lesion initially diagnosed as infantile fibromatosis at 15 days of age. Unusually, the mass demonstrated malignant progression leading to death at 20 months of age. Biological progression to malignancy is not known to occur in fibromatosis, and fibrosarcoma is not known to progress from a benign lesion. Whole-exome sequencing of the tumor identified a driver mutation in histone H3.1 at lysine (K)36. Our findings support the link between oncohistones and infantile soft tissue tumors and provide additional evidence for the oncogenic effects of p...
May 16, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28502899/the-expression-of-p450-genes-mediating-fenpropathrin-resistance-is-regulated-by-cncc-and-maf-in-tetranychus-cinnabarinus-boisduval
#3
Li Shi, Mengyao Wang, Yichao Zhang, Guangmao Shen, Haishan Di, Yue Wang, Lin He
Although overexpression of genes encoding detoxification enzymes is a well known mechanism of pesticide resistance of mites, the regulators involved in this process are still illiterate. Previous studies in our laboratory demonstrated that the overexpression of six P450 genes contributes to fenpropathrin resistance in T. cinnabarinus. In this study, six transcription factor genes that likely regulate the expression of P450 genes were identified and characterized. Quantitative PCR (qPCR) analysis showed that three transcription factor genes were highly expressed in a fenpropathrin-resistant (FeR) strain of T...
May 11, 2017: Comparative Biochemistry and Physiology. Toxicology & Pharmacology: CBP
https://www.readbyqxmd.com/read/28469831/hereditary-gingival-fibromatosis-characteristics-and-treatment-approach
#4
Pedro J Almiñana-Pastor, Pedro J Buitrago-Vera, Francisco M Alpiste-Illueca, Montserrat Catalá-Pizarro
Hereditary gingival fibromatosis (HGF) is a rare disorder characterized by a benign, non-hemorrhagic, fibrous gingival overgrowth that can appear in isolation or as part of a syndrome. Clinically, a pink gingiva with marked stippling can be seen to cover almost all the tooth, in many cases preventing eruption. HGF usually begins during the transition from primary to permanent teeth, giving rise to a condition that can have negative psychological effects at that age. As it does not resolve spontaneously, the treatment of choice is gingivectomy, which can be performed with an internal or external bevel incision, depending on each case and bearing in mind the changes that will take place at the dentogingival junction (DGJ)...
April 2017: Journal of Clinical and Experimental Dentistry
https://www.readbyqxmd.com/read/28462604/desmoplastic-fibroma-versus-soft-tissue-desmoid-tumour-of-forearm-a-case-series-of-diagnosis-surgical-approach-and-outcome
#5
K Jamshidi, A Bagherifard, A Mirzaei
We report a case series of aggressive fibromatosis of the forearm and discuss the differentiation criteria of overlapped intra- or extra-osseous origin of aggressive fibromatosis, surgical approach, and outcome. Ten cases of aggressive fibromatosis of the forearm were evaluated. When there was no involvement of vital adjacent structures, such as nerves, vessels, and tendons, en-bloc excision was performed for the removal of the soft-tissue mass. Bone involvement was observed in nine cases. Intercalary allograft was performed whenever one of the forearm bones was involved (six cases), while extensive curettage was used if both forearm bones were affected (three cases)...
April 1, 2017: Journal of Hand Surgery, European Volume
https://www.readbyqxmd.com/read/28439797/desmoid-type-fibromatosis-who-when-and-how-to-treat
#6
REVIEW
Javier Martínez Trufero, Isabel Pajares Bernad, Irene Torres Ramón, Jorge Hernando Cubero, Roberto Pazo Cid
Desmoid-type fibromatosis is a sarcoma subtype that gathers some singular characteristics, making it a difficult challenge to face in clinical practice. Despite its excellent survival prognosis, these tumors may be unpredictable, ranging from an asymptomatic indolent course to persistent, local, and extended recurrences that significantly impair quality of life. Although surgery was initially considered the first elective treatment, collected published data during the past few years are now pointing to the "wait and see" approach as a reasonable initial strategy because many patients can live a long life with the disease without having symptoms...
May 2017: Current Treatment Options in Oncology
https://www.readbyqxmd.com/read/28439607/late-metastatic-endometrial-carcinoma-at-the-repair-site-of-an-abdominal-wall-incisional-hernia
#7
Abdul-Wahed N Meshikhes, Suha H Al-Badr, Ehsan A Sulais, Hibba M Al-Qudaihi
The abdominal wall is a very rare site for endometrial cancer metastases. Its appearance generally indicates advanced cancer with poor prognosis. We report a case of a 55-year-old female who presented with an incisional hernia 4 years after abdominal panhysterectomy for endometrioid adenocarcinoma in 2009. Open hernia mesh repair was performed but on follow-up, she complained of pain and a swelling at the repair site. This was radiologically diagnosed as fibromatosis, but tru-cut biopsy confirmed presence of fibromatosis as well as a metastatic endometrial carcinoma...
May 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/28433619/lack-of-pathogenic-mutations-in-sos1-gene-in-phenytoin-induced-gingival-overgrowth-patients
#8
Katia Margiotti, Giulia Pascolini, Federica Consoli, Valentina Guida, Carlo Di Bonaventura, Anna Teresa Giallonardo, Antonio Pizzuti, Alessandro De Luca
OBJECTIVE: Gingival overgrowth is a side effect associated with some distinct classes of drugs, such as anticonvulsants, immunosuppressants, and calcium channel blockers. One of the main drugs associated with gingival overgrowth is the antiepileptic phenytoin, which affects gingival tissues by altering extracellular matrix metabolism. It has been shown that mutation of human SOS1 gene is responsible for a rare hereditary gingival fibromatosis type 1, a benign gingival overgrowth. The aim of the present study is to evaluate the possible contribution of SOS1 mutation to gingival overgrowth-related phenotype...
April 5, 2017: Archives of Oral Biology
https://www.readbyqxmd.com/read/28425619/analysis-of-mutations-in-the-sos-1-gene-in-two-polish-families-with-hereditary-gingival-fibromatosis
#9
Katarzyna Gawron, Grzegorz Bereta, Zuzanna Nowakowska, Katarzyna Łazarz-Bartyzel, Jan Potempa, Maria Chomyszyn-Gajewska, Renata Górska, Paweł Plakwicz
OBJECTIVES: To establish whether two families from Malopolska and Mazovia Provinces in Poland are affected by hereditary gingival fibromatosis type 1, caused by a single-cytosine insertion in exon 21 of the Son-of-Sevenless-1 gene. MATERIAL AND METHODS: Six subjects with hereditary gingival fibromatosis and five healthy subjects were enrolled in the study. Gingival biopsies were collected during gingivectomy or tooth extraction and used for histopathological evaluation...
April 20, 2017: Oral Diseases
https://www.readbyqxmd.com/read/28420393/medical-treatment-of-mammary-desmoid-type-fibromatosis-which-benefit
#10
Louise Scheer, Massimo Lodi, Sébastien Molière, Jean-Emmanuel Kurtz, Carole Mathelin
BACKGROUND: Breast fibromatosis is a rare disease characterized by monoclonal fibroblast proliferation. It has no ability to metastasize but has a high local recurrence rate and often infiltrates surrounding tissues. Surgical treatment is the reference, but recently, new targeted therapies have emerged. We report an original case of a patient with breast fibromatosis who received exclusive medical treatment. Our aim was to analyze these treatments based on the clinical and radiological outcome, iatrogenic effects, and pharmacological action...
April 18, 2017: World Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28417531/desmoid-type-fibromatosis-in-a-boy-with-down-syndrome
#11
Hisashi Ishida, Kousuke Chayama, Kiichiro Kanamitsu, Kana Washio, Takehiro Tanaka, Akira Shimada
Patients with Down syndrome (DS) have a markedly higher incidence of childhood leukemia, but a lower incidence of most solid tumors, compared with age-matched euploid individuals. Trisomy 21 might be protective against tumorigenesis because of several tumor suppressive mechanisms. Desmoid-type fibromatosis (DF) is a rare monoclonal, fibroblastic proliferation characterized by a variable clinical course. In recent reports, almost all cases of DF involved genomic alterations associated with activation of the Wnt/β-catenin pathway...
April 18, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28413398/desmoid-fibromatosis-of-the-abdominal-wall-surgical-resection-and-reconstruction-with-biological-matrix-egis-%C3%A2
#12
Saveria Tropea, Simone Mocellin, Roberto Stramare, Maria Giuseppina Bonavina, Carlo Riccardo Rossi, Marco Rastrelli
Desmoid tumor is a rare monoclonal fibroblast proliferation that is regarded as benign. The clinical management of desmoid tumors is very complex and requires a multidisciplinary approach because of the unpredictable disease course. For those cases localized in the anterior abdominal wall, symptomatic and unresponsive to medical treatment, radical resection and reconstruction with a prosthetic device are indicated. We present here a case of desmoid fibromatosis of the left anterolateral abdominal wall with a marked increase of the mass that required a large excision followed by reconstruction with biological matrix...
January 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28410294/methotrexate-and-vinblastine-or-vinorelbine-remain-useful-treatments-for-desmoid-type-fibromatosis-30-years-later
#13
Scott M Schuetze
No abstract text is available yet for this article.
March 2017: Cancer Journal
https://www.readbyqxmd.com/read/28410293/long-term-efficacy-of-methotrexate-plus-vinblastine-vinorelbine-in-a-large-series-of-patients-affected-by-desmoid-type-fibromatosis
#14
Elena Palassini, Anna Maria Frezza, Luigi Mariani, Luca Lalli, Chiara Colombo, Marco Fiore, Antonella Messina, Alessandra Casale, Carlo Morosi, Paola Collini, Silvia Stacchiotti, Paolo Giovanni Casali, Alessandro Gronchi
PURPOSE: Today, surgery and radiation therapy have a limited role in desmoid-type fibromatosis. Different systemic treatments were shown to be effective. Herein, we report on our institutional experience with low-dose methotrexate (MTX) + vinca alkaloids in this disease over the last 25 years. METHODS: We retrospectively reviewed data from all adult patients with sporadic desmoid-type fibromatosis treated with MTX and vinca alkaloids at our institution between 1989 and 2014...
March 2017: Cancer Journal
https://www.readbyqxmd.com/read/28400645/juvenile-hyaline-fibromatosis-a-10-year-follow-up
#15
Esra Baltacioglu, Esra Guzeldemir, Erkan Sukuroglu, Kadriye Yildiz, Pinar Yuva, Güven Aydin, Naci Karacal
Juvenile hyaline fibromatosis (JHF) is a rare hereditary disease with an autosomal recessive transmission. JHF is characterized by papulonodular skin lesions, osteolytic bone lesions, flexural joint contractures, and gingival hyperplasia and usually diagnosed in infancy or early childhood. JHF is thought to be a disorder of collagen metabolism and characterized by homogenous amorphous eosinophilic material and fibrous tissue. We report the case of a 14-year-old male child with multiple papulonodular skin lesions, progressive flexion contractures of joints, and severe gingival hyperplasia, with a 10-year follow-up...
March 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28396890/cowden-syndrome-%C3%A2-oral-presentations-of-a-paraneoplastic-syndrome-%C3%A2-case-report-and-review-of-the-literature
#16
Snober Tariq, Joseph Katz
Cowden syndrome is an autosomal dominant disorder with a predisposition to multiple benign and malignant tumors with unique oral manifestations. We present a case of Cowden syndrome in a patient diagnosed with breast cancer, traumatic fibromas, bronchial asthma, and multiple papillomatous fibromatosis of the oral cavity. Close association between oral papillomatosis associated with Cowden syndrome and PTEN gene mutation may increase the risk for malignant transformation. Therefore, patients of Cowden syndrome should be monitored carefully for all kinds of cancers...
2017: Quintessence International
https://www.readbyqxmd.com/read/28386395/outcome-of-surgery-for-primary-and-recurrent-desmoid-type-fibromatosis-a-retrospective-case-series-of-174-patients
#17
Panagiotis Tsagozis, Jonathan Daniel Stevenson, Robert Grimer, Simon Carter
BACKGROUND: The best management of relapsing desmoid-type fibromatosis, a benign but locally infiltrative soft-tissue tumour, is largely undecided. Our aim was to investigate the incidence and the factors influencing local relapse after surgery for primary and recurrent disease of the trunk and extremities. PATIENTS AND METHODS: Retrospective analysis of 174 patients who had surgical treatment for desmoid-type fibromatosis. The quality of the surgical margins and use of adjuvant radiotherapy or chemotherapy were analysed regarding local recurrences in primary and recurrent disease...
May 2017: Annals of Medicine and Surgery
https://www.readbyqxmd.com/read/28350521/clinical-activity-of-the-%C3%AE-secretase-inhibitor-pf-03084014-in-adults-with-desmoid-tumors-aggressive-fibromatosis
#18
Shivaani Kummar, Geraldine O'Sullivan Coyne, Khanh T Do, Baris Turkbey, Paul S Meltzer, Eric Polley, Peter L Choyke, Robert Meehan, Rasa Vilimas, Yvonne Horneffer, Lamin Juwara, Ann Lih, Amul Choudhary, Sandra A Mitchell, Lee J Helman, James H Doroshow, Alice P Chen
Purpose Desmoid tumors (aggressive fibromatosis) arise from connective tissue cells or fibroblasts. In general, they are slow growing and do not metastasize; however, locally aggressive desmoid tumors can cause severe morbidity and loss of function. Disease recurrence after surgery and/or radiation and diagnosis of multifocal desmoid tumors highlight the need to develop effective systemic treatments for this disease. In this study, we evaluate objective response rate after therapy with the γ-secretase inhibitor PF-03084014 in patients with recurrent, refractory, progressive desmoid tumors...
May 10, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28334642/partial-fasciectomy-is-a-useful-treatment-option-for-symptomatic-plantar-fibromatosis
#19
Haji Khairul Abd Kadir, Coonoor R Chandrasekar
Plantar fibromatosis is a rare, benign fibroproliferative disorder of the plantar fascia. It is considered as a low-grade tumour and it can be locally aggressive. It can present as painful swelling with impairment of local function. Upon failure of non-operative management, surgical treatment options include total fasciectomy or partial fasciectomy. Although surgical excision is the mainstay of treatment, recurrence rate can be up to 60%. The aim of this study was to determine the recurrence and complication rates of surgically treated plantar fibromatosis...
February 21, 2017: Foot
https://www.readbyqxmd.com/read/28327422/abdominal-wall-reconstruction-after-desmoid-type-fibromatosis-radical-resection-case-series-from-a-single-institution-and-review-of-the-literature
#20
S D Couto Netto, F Teixeira, C A M Menegozzo, A Albertini, E H Akaishi, E M Utiyama
BACKGROUND: Abdominal wall desmoid type fibromatosis management has been changing over recent years, from an aggressive approach towards a more conservative one. When radical resection is indicated, the surgical team faces the challenge of abdominal wall reconstruction, for which optimal technique is still debated. The present study reports the experience from a single center with abdominal closures after desmoid type fibromatosis resection. MATERIAL AND METHODS: Retrospective analysis of patients who underwent abdominal wall closure after sporadic abdominal desmoid type fibromatosis radical resection from 1982 to 2013...
March 1, 2017: International Journal of Surgery Case Reports
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