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Desmoid tumor

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https://www.readbyqxmd.com/read/28429313/gradually-shrinking-intra-abdominal-desmoid-tumor-derived-from-the-stomach-in-a-young-boy-a-case-report
#1
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
#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/28418912/human-microrna-expression-in-sporadic-and-fap-associated-desmoid-tumors-and-correlation-with-beta-catenin-mutations
#3
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
#4
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/28413499/identification-a-nonsense-mutation-of-apc-gene-in-chinese-patients-with-familial-adenomatous-polyposis
#5
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
#6
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
#7
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
#8
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/28331879/solid-cystic-pancreatic-tail-desmoid-tumor-with-beta-catenin-positivity
#9
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
#10
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
#11
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...
March 13, 2017: Neuroradiology
https://www.readbyqxmd.com/read/28282712/a-patient-with-desmoid-tumors-and-familial-fap-having-frame-shift-mutation-of-the-apc-gene
#12
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
#13
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
https://www.readbyqxmd.com/read/28251376/the-treatment-of-desmoid-tumors-associated-with-familial-adenomatous-polyposis-the-results-of-a-japanese-multicenter-observational-study
#14
Yasuhiro Inoue, Hideyuki Ishida, Hideki Ueno, Hirotoshi Kobayashi, Tatsuro Yamaguchi, Tsuyoshi Konishi, Naohiro Tomita, Nagahide Matsubara, Fumio Ishida, Takao Hinoi, Yukihide Kanemitsu, Toshiaki Watanabe, Kenichi Sugihara
PURPOSE: Familial adenomatous polyposis (FAP)-associated desmoid tumor (DT) is sometimes life threatening. However, the optimal treatment for DTs has not been established. The aim of this study was to analyze the outcomes of surgical and pharmacological treatments for DT in Japanese FAP patients. METHODS: We retrospectively reviewed the data of 303 patients who underwent colectomy for FAP between 2000 and 2012. We analyzed 41 patients with DTs in which the location was apparent...
March 1, 2017: Surgery Today
https://www.readbyqxmd.com/read/28250295/a-mesenteric-desmoid-tumor-with-rapid-progression
#15
Kazunao Hayashi, Masaaki Takamura, Hisashi Yokoyama, Yuichi Sato, Satoshi Yamagiwa, Hitoshi Nogami, Toshifumi Wakai, Go Hasegawa, Shuji Terai
We herein report the case of a rapidly progressive sporadic mesenteric desmoid tumor (DT). A 62-year-old woman presented with a 4-cm-diameter palpable mass in the left supraumbilical area. The mass showed an ill-defined margin with heterogeneous delayed enhancement on computed tomography and heterogeneous high intensity on T2-weighted magnetic resonance imaging. Sixteen months after the initial observation, the mass had grown in size, reaching 13 cm in diameter. The resected mass was histologically confirmed as a DT of the mesentery...
2017: Internal Medicine
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
#16
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/28219574/intrathoracic-desmoid-tumor-arising-at-thoracotomy-site-mimicking-lung-cancer-pleural-recurrence
#17
Luis Gorospe, Gemma María Muñoz-Molina, Percy Carvajal-Serrano, Raquel García-Latorre
No abstract text is available yet for this article.
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28210557/desmoid-tumor-following-abdominally-based-free-flap-breast-reconstruction
#18
Christine Oh, Ziyad S Hammoudeh, Brian T Carlsen
Desmoid tumors are fibroblastic connective tissue tumors that most commonly develop within the anterior abdominal wall. The etiology of desmoid tumors has not been well defined; however, hereditary, hormonal, traumatic, and surgery-related causes have been implicated. Desmoid tumors are believed to arise from musculoaponeurotic structures. Development in the breast is very rare. Several reports of desmoid tumors arising in the vicinity of the fibrous capsule of a breast implant have been described, but to date, the authors are not aware of any published cases following autologous breast reconstruction...
February 2017: Gland Surgery
https://www.readbyqxmd.com/read/28209418/sporadic-desmoid-tumors-in-the-pediatric-population-a-single-center-experience-and-review-of-the-literature
#19
Vered Shkalim Zemer, Helen Toledano, Liora Kornreich, Enrique Freud, Eli Atar, Smadar Avigad, Galina Feinberg-Gorenshtein, Suzana Fichman, Josephine Issakov, Tal Dujovny, Isaac Yaniv, Shifra Ash
BACKGROUND/PURPOSE: We present our long experience with desmoid tumors in children. METHODS: Data were retrospectively collected from 17 children/adolescents treated for sporadic desmoid tumors at a tertiary pediatric hospital in 1988-2016. There were 10 girls and 7 boys aged 1-17years. Tumor sites included head and neck, trunk, extremity, and groin. Eight patients underwent radical resection, with complete remission in 7 and local relapse in one which was treated with chemotherapy...
February 4, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28174660/mrgfus-for-desmoid-tumors-within-the-thigh-early-clinical-experiences
#20
Matthew D Bucknor, Viola Rieke
BACKGROUND: Desmoid tumors are benign but locally aggressive non-malignant tumors derived from fibroblasts. Surgery, chemotherapy, and radiation therapy have been the mainstay of treatment, but recurrence is common and side effects can result in significant morbidity. In this case series, we highlight our experiences performing treatments in the thigh, including strategies for optimizing ablation size and safety. CASE PRESENTATION: Since December 2014, 14 magnetic resonance-guided focused ultrasound (MRgFUS) treatments for desmoid tumors were performed at our institution in seven patients...
2017: Journal of Therapeutic Ultrasound
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