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Desmoid tumor

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https://www.readbyqxmd.com/read/28512414/an-intra-abdominal-desmoid-tumor-embedded-in-the-pancreas-preoperatively-diagnosed-as-an-extragastric-growing-gastrointestinal-stromal-tumor
#1
Mari Mizuno, Yoshiaki Kawaguchi, Aya Kawanishi, Yohei Kawashima, Atsuko Maruno, Masami Ogawa, Mifuji Tomioku, Daisuke Furukawa, Kazuhito Nabeshima, Kenji Nakamura, Kenichi Hirabayashi, Tetsuya Mine
A 45-year-old woman was found to have a pancreatic tumor by abdominal ultrasound performed for a medical check-up. Abdominal contrast-enhanced computed tomography showed a hypovascular tumor measuring 30 mm in diameter in the pancreatic tail. Endoscopic ultrasound-guided fine needle aspiration was performed. An extragastric growing gastrointestinal stromal tumor was thereby diagnosed preoperatively, and surgical resection was planned. Laparoscopic surgery was attempted but conversion to open surgery was necessitated by extensive adhesions, and distal pancreatectomy, splenectomy, and partial gastrectomy were performed...
January 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28493803/soft-tissue-sarcomas-of-the-abdomen-and-pelvis-radiologic-pathologic-features-part-2-uncommon-sarcomas
#2
Angela D Levy, Maria A Manning, Markku M Miettinen
Soft-tissue sarcomas occurring in the abdomen and pelvis are an uncommon but important group of malignancies. Recent changes to the World Health Organization classification of soft-tissue tumors include the movement of gastrointestinal stromal tumors (GISTs) into the soft-tissue tumor classification. GIST is the most common intraperitoneal sarcoma. Liposarcoma is the most common retroperitoneal sarcoma, and leiomyosarcoma is the second most common. GIST, liposarcoma, and leiomyosarcoma account for the majority of sarcomas encountered in the abdomen and pelvis and are discussed in part 1 of this article...
May 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28489620/adult-desmoid-tumors-biology-management-and-ongoing-trials
#3
Nicolas Penel, Frédéric Chibon, Sébastien Salas
PURPOSE OF REVIEW: To summarize the current knowledge about the biology and clinical management of adult desmoid tumors. RECENT FINDINGS: In the past decade, we have learned that desmoid tumors are driven by alterations of the Wnt/APC/β-catenin pathway, sporadic desmoid tumors are associated with somatic mutations of CTNNB1, and germline mutations of APC and somatic mutations of CTNNB1 are probably mutually exclusive. One-third of desmoid tumors are misdiagnosed; a second pathological opinion is therefore of major importance for desmoid tumor...
May 9, 2017: Current Opinion in Oncology
https://www.readbyqxmd.com/read/28468632/erratum-to-carbon-ion-radiotherapy-for-desmoid-tumor-of-the-abdominal-wall-a-case-report
#4
Takuya Nagata, Yusuke Demizu, Tomoyuki Okumura, Shinichi Sekine, Naoki Hashimoto, Nobukazu Fuwa, Tomoaki Okimoto, Yutaka Shimada
No abstract text is available yet for this article.
May 3, 2017: World Journal of Surgical Oncology
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/28447874/management-of-abdominal-wall-desmoid-tumors
#6
Olivier Detry
No abstract text is available yet for this article.
April 27, 2017: Acta Chirurgica Belgica
https://www.readbyqxmd.com/read/28439797/desmoid-type-fibromatosis-who-when-and-how-to-treat
#7
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/28429313/gradually-shrinking-intra-abdominal-desmoid-tumor-derived-from-the-stomach-in-a-young-boy-a-case-report
#8
Kazushi Miyata, Masahide Fukaya, Masato Nagino
BACKGROUND: Intra-abdominal desmoid tumors, particularly those derived from the stomach, are rare. Such tumors are associated with a history of familial adenomatous polyposis (FAP), trauma, or surgical procedures in general. In addition, spontaneous shrinking of an intra-abdominal desmoid tumor is rarer. And desmoid tumors most commonly arise during the fourth decade of life. CASE PRESENTATION: A 17-year-old boy with lower abdominal pain was diagnosed with a gastrointestinal stromal tumor (GIST) or a hematoma at a local hospital...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28420393/medical-treatment-of-mammary-desmoid-type-fibromatosis-which-benefit
#9
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/28418912/human-microrna-expression-in-sporadic-and-fap-associated-desmoid-tumors-and-correlation-with-beta-catenin-mutations
#10
Aldo Cavallini, Maria Teresa Rotelli, Catia Lippolis, Domenico Piscitelli, Rosa Digennaro, Claudia Covelli, Nicola Carella, Matteo Accetturo, Donato Francesco Altomare
Desmoid tumors (DT) are rare, benign, fibroblastic neoplasm with challenging histological diagnosis. DTs can occur sporadically or associated with the familial adenomatous polyposis coli (FAP). Most sporadic DTs are associated with β-catenin gene (CTNNB1) mutations, while mutated APC gene causes FAP disease. microRNAs (miRNAs) are involved in many human carcinogenesis.The miRNA profile was analyzed by microarray in formalin-fixed, paraffin-embedded (FFPE) specimens of 12 patients (8 sporadic, 4 FAP-associated) and 4 healthy controls...
March 19, 2017: Oncotarget
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...
May 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28413499/identification-a-nonsense-mutation-of-apc-gene-in-chinese-patients-with-familial-adenomatous-polyposis
#12
Haishan Li, Lingling Zhang, Quan Jiang, Zhenwang Shi, Hanxing Tong
Familial adenomatous polyposis (FAP; Mendelian of Inherintance in Man ID, 175100) is a rare autosomal dominant disorder characterized by the development of numerous adenomatous polyps throughout the colon and rectum associated with an increased risk of colorectal cancer. FAP is at time accompanied with certain extraintestinal manifestations such as congenital hypertrophy of the retinal pigment epithelium, dental disorders and desmoid tumors. It is caused by mutations in the adenomatous polyposis coli (APC) gene...
April 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28413398/desmoid-fibromatosis-of-the-abdominal-wall-surgical-resection-and-reconstruction-with-biological-matrix-egis-%C3%A2
#13
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/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/28350521/clinical-activity-of-the-%C3%AE-secretase-inhibitor-pf-03084014-in-adults-with-desmoid-tumors-aggressive-fibromatosis
#15
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/28331879/solid-cystic-pancreatic-tail-desmoid-tumor-with-beta-catenin-positivity
#16
Harsh D Patel, Neil R Desai, Aniruddh Som, Shinil K Shah, Nirav C Thosani
No abstract text is available yet for this article.
2017: ACG Case Reports Journal
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
#17
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/28289810/non-odontogenic-tumors-of-the-facial-bones-in-children-and-adolescents-role-of-multiparametric-imaging
#18
REVIEW
Minerva Becker, Salvatore Stefanelli, Anne-Laure Rougemont, Pierre Alexandre Poletti, Laura Merlini
Tumors of the pediatric facial skeleton represent a major challenge in clinical practice because they can lead to functional impairment, facial deformation, and long-term disfigurement. Their treatment often requires a multidisciplinary approach, and radiologists play a pivotal role in the diagnosis and management of these lesions. Although rare, pediatric tumors arising in the facial bones comprise a wide spectrum of benign and malignant lesions of osteogenic, fibrogenic, hematopoietic, neurogenic, or epithelial origin...
April 2017: Neuroradiology
https://www.readbyqxmd.com/read/28282712/a-patient-with-desmoid-tumors-and-familial-fap-having-frame-shift-mutation-of-the-apc-gene
#19
Sanambar Sadighi, Mahsa Ghaffari-Moghaddam, Mojtaba Saffari, Mohammad Ali Mohagheghi, Reza Shirkoohi
Desmoids tumors, characterized by monoclonal proliferation of myofibroblasts, could occur in 5-10% of patients with familial adenomatous polyposis (FAP) as an extra-colonic manifestation of the disease. FAP can develop when there is a germ-line mutation in the adenomatous polyposis coli gene. Although mild or attenuated FAP may follow mutations in 5΄ extreme of the gene, it is more likely that 3΄ extreme mutations haveamore severe manifestation of thedisease. A 28-year-old woman was admitted to the Cancer Institute of Iran with an abdominal painful mass...
February 2017: Acta Medica Iranica
https://www.readbyqxmd.com/read/28280682/functional-and-aesthetic-thorax-reconstruction-after-desmoid-tumor-resection
#20
Carlos Olvera-Caballero, Eric Acosta-Ponce de Leon, Sergio Sanchez-Sosa
This study describes a case report of a 31-year-old patient who presented with a left thoracic tumor on costal cartilages 5 and 6 that was diagnosed as a desmoid tumor 3 years after receiving retropectoral breast implants for cosmetic reasons. The integral reconstruction of the thoracic wall, functional and aesthetic, was planned for a single surgical period. The defect secondary to the tumor resection, which left the pericardium and lung exposed, was closed using the pectoral muscle as a "pre-expanded" flap by the breast implant, and the breast aesthetic was treated bilaterally with new implants in the retromammary position...
February 2017: Plastic and Reconstructive Surgery. Global Open
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