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Fibromatosis

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https://www.readbyqxmd.com/read/28425619/analysis-of-mutations-in-the-sos-1-gene-in-two-polish-families-with-hereditary-gingival-fibromatosis
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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...
March 28, 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
#11
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
#12
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
https://www.readbyqxmd.com/read/28298625/periodontal-disease-and-fam20a-mutations
#13
Piranit Nik Kantaputra, Chotika Bongkochwilawan, Mark Lubinsky, Supansa Pata, Massupa Kaewgahya, Huei Jinn Tong, James R Ketudat Cairns, Yeliz Guven, Nipon Chaisrisookumporn
Enamel-renal-gingival syndrome (ERGS; OMIM #204690), a rare autosomal recessive disorder caused by mutations in FAM20A, is characterized by nephrocalcinosis, nephrolithiasis, amelogenesis imperfecta, hypoplastic type, gingival fibromatosis and other dental abnormalities, including hypodontia and unerupted teeth with large dental follicles. We report three patients and their families with findings suggestive of ERGS. Mutation analysis of FAM20A was performed in all patients and their family members. Patients with homozygous frameshift and compound heterozygous mutations in FAM20A had typical clinical findings along with periodontitis...
March 16, 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/28275596/an-unusual-location-of-fibrous-hamartoma-of-infancy-in-the-eyelid
#14
Ofira Zloto, Sarit Aviel-Ronen, Mordechai Rosner
AIM: To describe the clinical, imaging, and histopathological features of fibrous hamartoma of infancy (FHI) in the eyelid, a rare differential diagnosis for eyelid lesions. METHODS: We describe the case of a 7-month-old patient with a FHI in the eyelid that was diagnosed after surgical removal. The unique histopathological appearance of the triphasic histologic components provided the diagnosis. CONCLUSIONS: FHI is included in the long list of differential diagnosis for eyelid lesions in infancy...
January 2017: Ocular Oncology and Pathology
https://www.readbyqxmd.com/read/28248815/recurrent-srf-rela-fusions-define-a-novel-subset-of-cellular-myofibroma-myopericytoma-a-potential-diagnostic-pitfall-with-sarcomas-with-myogenic-differentiation
#15
Cristina R Antonescu, Yun-Shao Sung, Lei Zhang, Narasimhan P Agaram, Christopher D Fletcher
Cellular myofibroblastic tumors other than desmoid-type fibromatosis are often diagnostically challenging due to their relative rarity, lack of known genetic abnormalities, and expression of muscle markers which may be confused with sarcomas with myogenic differentiation. In this study we investigate the molecular alterations of a group of cellular myofibroblastic lesions with in the myofibroma and myopericytoma spectrum for better subclassification. Two index cases were studied by paired-end RNA sequencing for potential fusion gene discovery...
May 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28199014/meta-analysis-of-the-influence-of-surgical-margin-and-adjuvant-radiotherapy-on-local-recurrence-after-resection-of-sporadic-desmoid-type-fibromatosis
#16
REVIEW
M L Janssen, D L M van Broekhoven, J M M Cates, W M Bramer, J J Nuyttens, A Gronchi, S Salas, S Bonvalot, D J Grünhagen, C Verhoef
BACKGROUND: Extra-abdominal desmoid-type fibromatosis (DF) is a rare, locally aggressive neoplasm that is usually managed conservatively. When treatment is indicated, it typically involves surgical resection, possibly with adjuvant radiotherapy. The indications for postoperative radiotherapy and its effectiveness are unclear. The objective of this study was to estimate the effect of surgical resection margins and adjuvant radiotherapy on rates of recurrence of DF. METHODS: Literature published between 1999 and 2015 was extracted from MEDLINE, Embase, Cochrane Central Registry of Trials, Web of Science and Google Scholar...
March 2017: British Journal of Surgery
https://www.readbyqxmd.com/read/28182064/myofibromatosis-utility-of-fine-needle-aspiration-cytology-in-the-diagnosis-of-an-underreported-entity
#17
Sandhya V Poflee, Anjali N Bode, Sneha Chavarkar, Pradeep S Umap
Myofibromatosis (MFS) was recognized as a distinct form of childhood fibromatosis. Infantile myofibromatosis (IMF) is now identified as a solitary or multicentric tumor that predominantly occurs in neonates and infants. The adult counterpart of IMF, though of rare occurrence, is identified and is known as MFS. Morphological diagnosis of MFS is made by histopathological examination of the biopsy or surgically excised mass and confirmed on the basis of specific immunoprofile. We report a case of multicentric MFS occurring in an adolescent in whom diagnosis was suggested on the basis of fine needle aspiration cytology (FNAC) that avoided surgical excision of multiple nodules...
January 2017: Journal of Cytology
https://www.readbyqxmd.com/read/28173672/-fibromatosis-like-metaplastic-carcinoma-of-breast-a-clinicopathological-analysis-of-3-cases
#18
M Li, J B Lu, P Sun, R Z Luo, J H He
No abstract text is available yet for this article.
February 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28171221/the-significance-of-electron-microscopic-examination-of-gingiva-in-cases-of-hunter-syndrome-and-hereditary-gingival-fibromatosis
#19
Michal Straka, Lubos Danisovic, Vladimir Bzduch, Stefan Polak, Ivan Varga
INTRODUCTION: Electron microscopy has been for decades a basic morphological method still used in diagnostic protocols of some pathological conditions affecting the ultrastructure of cells and extracellular matrix. The aim of this study was an ultrastructural description of gingiva of patients with Hunter syndrome and hereditary gingival fibromatosis. PATIENTS AND METHODS: Gingival biopsies were obtained during surgical periodontal treatment from a 9-year-old boy with Hunter disease (with enzyme replacement therapy with recombinant human idursulphase) and a 15-year-old girl with hereditary gingival fibromatosis...
October 2016: Neuro Endocrinology Letters
https://www.readbyqxmd.com/read/28149737/endoscopic-subtotal-fasciectomy-of-the-foot
#20
Tun Hing Lui
Plantar fibromatosis is a rare benign but often locally aggressive tumor of the plantar aponeurosis. Nonsurgical treatment is the first line of treatment for symptomatic lesions. Because of the high recurrence rate associated with surgical treatment, operation is indicated only when the lesions are highly symptomatic and conservative measures fail or the diagnosis is in question. The purpose of this technical note is to report the details of endoscopic subtotal fasciectomy. This may reduce the risks of skin necrosis and dehiscence, infection, and formation of painful hypertrophic scars...
December 2016: Arthroscopy Techniques
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