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Desmoid tumor

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https://www.readbyqxmd.com/read/28099210/pazopanib-a-promising-option-for-the-treatment-of-aggressive-fibromatosis
#1
Zoltan Szucs, Christina Messiou, Han Hsi Wong, Helen Hatcher, Aisha Miah, Shane Zaidi, Winette T A van der Graaf, Ian Judson, Robin L Jones, Charlotte Benson
Desmoid tumour/aggressive fibromatosis (DT/AF) is a rare soft-tissue neoplasm that is locally aggressive but does not metastasize. There is no standard systemic treatment for symptomatic patients, although a number of agents are used. Tyrosine kinase inhibitors have recently been reported to show useful activity. We reviewed our bi-institutional (Royal Marsden Hospital, Cambridge University Hospitals) experience with the tyrosine kinase inhibitor pazopanib in the treatment of progressing DT/AF. Eight patients with DT/AF were treated with pazopanib at Royal Marsden Hospital and Cambridge University Hospitals between June 2012 and June 2016...
January 17, 2017: Anti-cancer Drugs
https://www.readbyqxmd.com/read/28044201/radiotherapy-in-desmoid-tumors-treatment-response-local-control-and-analysis-of-local-failures
#2
Kirsi Santti, Annette Beule, Laura Tuomikoski, Mikko Rönty, Anna-Stina Jääskeläinen, Kauko Saarilahti, Hanna Ihalainen, Maija Tarkkanen, Carl Blomqvist
BACKGROUND: Desmoid tumors (aggressive fibromatosis) are rare soft tissue tumors which frequently recur after surgery. Desmoid tumors arise from musculoaponeurotic tissue in the extremities, head and neck, abdominal wall, or intra-abdominally. Our aim was to examine the outcome of radiotherapy of desmoid tumors in a single institution series. PATIENTS AND METHODS: We evaluated 41 patients with desmoid tumors treated with 49 radiotherapies between 1987 and 2012...
January 2, 2017: Strahlentherapie und Onkologie: Organ der Deutschen Röntgengesellschaft ... [et Al]
https://www.readbyqxmd.com/read/28027119/comparison-of-%C3%AE-catenin-and-lef1-immunohistochemical-stains-in-desmoid-type-fibromatosis-and-its-selected-mimickers-with-unexpected-finding-of-lef1-positivity-in-scars
#3
Youran Zou, Yaxia Zhang, James Church, Xiuli Liu
β-catenin immunohistochemical stain can be useful in the diagnosis of many tumors including desmoid-type fibromatosis (DTF). Lymphoid enhancer-factor 1 (LEF1), a recently emerged marker, is part of the Wnt pathway with β-catenin but has not been studied in DTF. We performed LEF1 and β-catenin immunohistochemistry in DTF (n=26), superficial fibromatosis (n=19), sclerosing mesenteritis (n=12), gastrointestinal stromal tumor (n=17), and cutaneous scar (n=14) using tissue microarray and whole sections. The staining intensity was scored as strong (visible at ×2 objective, value of 3), moderate (visible at ×4, value of 2), weak (visible at ×10, value of 1), and negative (not visible at ×10, value of 0)...
December 23, 2016: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/27994217/desmoid-fibromatosis-presenting-as-deep-venous-thrombosis-a-case-report-and-discussion
#4
Lisa M Marks, Susan J Neuhaus
BACKGROUND Venous thromboembolism (VTE), comprising deep vein thrombosis (DVT) and pulmonary embolism (PE), is a common, serious cardiovascular event. Predisposing factors include genetic disorders, immobility, and underlying malignancy. Soft tissue tumors are by contrast rare, but should be included in the differential etiology of DVT, especially when the patient is young and has few thrombotic risk factors. CASE REPORT We present a 40-year-old patient whose initial diagnosis was spontaneous DVT of the lower leg, treated conventionally...
December 20, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27975036/breast-desmoid-tumor-after-ductal-carcinoma-treatment-salvaging-a-diep-flap-reconstruction
#5
Dmitry Zavlin, Eric S Ruff, Steven Benjamin Albright
Supplemental Digital Content is available in the text.
November 2016: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/27957320/pazopanib-a-novel-treatment-option-for-aggressive-fibromatosis
#6
Gulcan Bulut, Anil Ozluk, Atike Pınar Erdogan, Ruchan Uslu, Nevra Elmas, Burcak Karaca
BACKGROUND: Aggressive fibromatosis (AF), also known as desmoid tumor, is an uncommon soft tissue neoplasm. AF does not metastasize, but it is locally invasive and its propensity for recurrence after conservative resection is well documented. No effective cytotoxic treatment has been reported, hence there is a need for novel treatment strategies. CASE PRESENTATION: We present the case of an AF successfully treated with an oral tyrosine kinase inhibitor, pazopanib, with mild side effects...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27920876/bilateral-perirenal-space-fibromatosis-with-renal-infiltration-case-report-and-review-of-literature
#7
Avinash Sabale, Anil Pralhadan, Kalirajan Kalidos, Krishnankutty Ramachandran
Fibromatosis and/or desmoid tumors which constitute less than 1% of all neoplasms and 3.0% of all soft-tissue tumors are pathologically benign proliferations of the fibroblasts but are locally aggressive with infiltrative type of growth and tendency toward recurrence. Bilateral symmetrical perirenal involvement has been described in many conditions which can be renal, subcapsular, or perirenal in origin. However, bilateral perirenal fibromatosis as an isolated presentation was very uncommon. We report an exceptionally rare case of bilateral perirenal fibromatosis with renal infiltration...
December 2016: Radiology case reports
https://www.readbyqxmd.com/read/27913912/genetic-counselor-practices-involving-pediatric-patients-with-fap-an-investigation-of-their-self-reported-strategies-for-genetic-testing-and-hepatoblastoma-screening
#8
Caitlin E Lawson, Thomas M Attard, Hongying Dai, Seth Septer
Familial adenomatous polyposis (FAP) is a cancer predisposition syndrome that causes early-onset polyposis and is associated with an increased risk for hepatoblastoma. There is currently a lack of consensus on when to order APC (adenomatous polyposis coli) gene testing or implement surveillance for hepatoblastoma. An online questionnaire was completed by 62 genetic counselors to capture their current practices regarding these questions. Extracolonic findings associated with FAP that were most likely to prompt APC testing in an otherwise asymptomatic 10 year-old child with a negative family history were multiple desmoid tumors, congenital hypertrophy of the retinal pigment epithelium (CHRPE), jaw osteomas, and hepatoblastoma...
December 3, 2016: Journal of Genetic Counseling
https://www.readbyqxmd.com/read/27912995/rehabilitation-strategies-and-outcomes-of-the-sarcoma-patient
#9
REVIEW
Sean Robinson Smith
Sarcomas are a relatively rare cancer that, depending on the location, can cause significant neuromusculoskeletal dysfunction and require rehabilitation interventions to reduce pain, restore function, and improve quality of life. This review focuses on sarcoma subtypes that frequently cause these complications: bony and soft tissue sarcomas leading to limb salvage or amputation, desmoid tumors, and malignant peripheral nerve sheath tumors. Rehabilitation approaches and outcomes are discussed, as well as considerations for childhood sarcoma survivors transitioning to adulthood...
February 2017: Physical Medicine and Rehabilitation Clinics of North America
https://www.readbyqxmd.com/read/27905688/liposomal-doxorubicin-effective-treatment-for-pediatric-desmoid-fibromatosis
#10
Prasanna Ananth, Annette Werger, Stephan Voss, Carlos Rodriguez-Galindo, Katherine A Janeway
Efficacy of liposomal doxorubicin (LD) in treating desmoid fibromatosis (DF) in children has not been well evaluated. This retrospective case series examines five children with progressive DF, treated with LD. We report progression-free intervals (PFIs) and radiographic as well as clinical responses for each medication received. LD was well tolerated, with an average 4.5% reduction in tumor size and median PFI of 29 months. Treatment with LD conferred the longest PFI of all medical therapies pursued. Thus, LD is an important treatment option for DF in pediatrics...
December 1, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27895871/desmoid-tumor-of-the-chest-wall-mimicking-recurrent-breast-cancer-multimodality-imaging-findings
#11
Kyeong A Choi, Yeong Yi An
Desmoid tumor of breast is a rare benign, locally aggressive tumor with a high recurrence rate. It has been associated with scar from previous breast surgery or trauma. Especially in breast cancer patients with previous operation history, it may simulate recurrent breast cancer clinically and radiologically. We presented multimodality imaging findings (ultrasound, computed tomography, magnetic resonance imaging and positron emission tomography/computed tomography) of chest wall desmoid tumor mimicking recurrent breast cancer in a 38-year-old patient with a history of left modified mastectomy...
October 2016: Iranian Journal of Radiology: a Quarterly Journal Published By the Iranian Radiological Society
https://www.readbyqxmd.com/read/27895766/primary-mesenchymal-tumors-of-the-pancreas-in-a-single-center-over-15-years
#12
Hongkai Zhang, Shuangni Yu, Wenze Wang, Yin Cheng, Yu Xiao, Zhaohui Lu, Jie Chen
In total, 95% of primary tumors in the pancreas are epithelial tumors; primary mesenchymal tumors at this site are extremely rare. At present, only one comprehensive study about these rare tumors has been performed. Another retrospective analysis of these rare tumors is performed in the present study, which, to the best of our knowledge, is the first to be performed in China. In the present study, 10 patients that underwent resection for primary mesenchymal tumors of the pancreas were identified in a 15-year period at the Chinese Academy of Medical Sciences and Peking Union Medical College, which accounted for 0...
November 2016: Oncology Letters
https://www.readbyqxmd.com/read/27882239/desmoplastic-fibroma-of-the-distal-tibia-a-case-report-of-a-minimally-invasive-histological-diagnosis
#13
Gabriele Levrini, Pierpaolo Pattacini
Desmoplastic fibroma (DF) is a benign, rare fibroblastic intraosseous neoplasm histologically resembling a desmoid soft tissue tumor. Although classified as benign, DF frequently exhibits an aggressive behavior, has a moderate-to-high recurrence rate, and often causes pathological fractures and extensive bone destruction. This case report presents an incidentally detected DF of the tibia, which was diagnosed using a minimally invasive approach. A 36-year-old African female patient was referred to the Department of Diagnostic Imaging of Arcispedale Santa Maria Nuova-IRCCS (Reggio Emilia, Italy), to be examined by a computed tomography scan on an outpatient basis, after an x-ray examination of the tibia, which was performed after an injury to exclude the presence of a fracture, revealed a hyperlucency of unknown origin...
November 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/27875709/iodine-125-interstitial-brachytherapy-for-pediatric-desmoid-type-fibromatosis-of-the-head-and-neck-a-case-report
#14
Wen-Jie Wu, Hua-Qiu Guo, Guang-Yan Yu, Jian-Guo Zhang
Desmoid-type fibromatosis (DF) is a locally aggressive benign soft tissue tumor. It is rarely observed in the head and neck region and is particularly uncommon in the parotid gland. This report describes the case of a 32-month-old girl with DF of the head and neck. The tumor was resected with gross residual tumors. Recurrence occurred 3 months later and then the patient was treated with iodine-125 interstitial brachytherapy. The tumor was completely absent 6 months after brachytherapy. No recurrence was found 60 months after brachytherapy during follow-up...
October 29, 2016: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/27870683/increasing-role-of-image-guided-ablation-in-the-treatment-of-musculoskeletal-tumors
#15
Anil Nicholas Kurup, Matthew R Callstrom
In the last decade, percutaneous treatment of musculoskeletal (MSK) tumors has become more established in routine clinical care while also undergoing a number of advancements. Ablative techniques to palliate painful skeletal metastases have gained wide acceptance, while goals for ablation have evolved to include local control of oligometastases and desmoid tumors. Bone consolidation or augmentation is now frequently used in conjunction with or instead of ablation of skeletal tumors to stabilize pathologic fractures or prevent further morbidity that could result from fractures caused by tumor progression...
November 2016: Cancer Journal
https://www.readbyqxmd.com/read/27870147/management-strategies-in-lynch-syndrome-and-familial-adenomatous-polyposis-a-national-healthcare-survey-in-japan
#16
Tomoki Yamano, Michiko Hamanaka, Akihito Babaya, Kei Kimura, Masayoshi Kobayashi, Miki Fukumoto, Kiyoshi Tsukamoto, Masafumi Noda, Nagahide Matsubara, Naohiro Tomita, Kenichi Sugihara
Lynch syndrome (LS) and familial adenomatous polyposis (FAP) are major sources of hereditary colorectal cancer (CRC) and associated with other malignancies. There is some heterogeneity in management strategies in Japan. We performed a survey of management of hereditary CRC in hospitals that are members of the Japan Society of Colorectal Cancer Research (JSCCR). One hundred and ninety departments responded, of which 127 were from designated cancer care hospitals (DCCHs) according to the Japanese government. There were 25 488 operations for CRC in these departments in 2015...
November 21, 2016: Cancer Science
https://www.readbyqxmd.com/read/27844202/does-routine-colonoscopy-help-diagnose-familial-adenomatous-polyposis-in-patients-presenting-with-desmoid-tumors-but-no-gastrointestinal-symptoms
#17
Rumi Shin, Hwan-Seong Cho, Duck-Woo Kim, So Yeon Ahn, Myong Hoon Ihn, Hyo Jin Park, Heung-Kwon Oh, Sung-Bum Kang
PURPOSE: Desmoid tumor (DT) is a rare myofibroblastic proliferative neoplasm, but can occur in up to 26% of patients with familial adenomatous polyposis (FAP). Therefore, DT may be an extraintestinal manifestation of FAP that precedes the development of advanced malignancies. The aim of this study was to identify the incidence of polyps in DT patients and investigate value of colonoscopy for diagnosis of FAP in DT patients without gastrointestinal symptoms. MATERIALS AND METHODS: The records of patients diagnosed with DT were retrospectively reviewed using the clinical data warehouse (CDW) system...
January 2017: International Journal of Colorectal Disease
https://www.readbyqxmd.com/read/27817254/cytohistological-and-immunohistochemical-characteristics-of-spindle-shaped-mesenchymal-neoplasms-occurring-in-the-gastrointestinal-tract
#18
Antonio Ieni, Valeria Barresi, Luca Reggiani Bonetti, Giovanni Branca, Rosario Alberto Caruso, Giovanni Tuccari
The purpose of the present review is to analyze the cytohistological and immunohistochemical characteristics of spindle-shaped mesenchymal gastrointestinal neoplams (MGNs), a group of unusual neoplastic conditions with different biological behavior. These tumors exhibit clinical pictures strictly related to the site of origin and dimensions, even if they appear generally with an intramural localization. This latter point may suggest an useful application of endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA), mainly followed by the cell-block procedure (CBP) in the differential diagnostic approach...
March 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/27810608/rapid-progression-of-a-pregnancy-associated-intra-abdominal-desmoid-tumor-in-the-post-partum-period-a-case-report
#19
David Hanna, Michail Magarakis, William S Twaddell, H Richard Alexander, Susan B Kesmodel
INTRODUCTION: The clinical behavior of desmoid tumors can be unpredictable, particularly when they arise in the ante-partum or post-partum period. We present a case of an intra-abdominal desmoid tumor that was identified in the ante-partum period, progressed rapidly in the post-partum period, and was subsequently resected. PRESENTATION OF CASE: The patient is a 19 year-old female who was found to have a 12cm intra-abdominal mass on a fetal assessment ultrasound...
2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27762441/free-fillet-flap-application-to-cover-forequarter-or-traumatic-amputation-of-an-upper-extremity-a-case-report
#20
Mario F Scaglioni, Nicole Lindenblatt, André A Barth, Bruno Fuchs, Walter Weder, Pietro Giovanoli
Reusing tissue of amputated or unsalvageable limbs to reconstruct soft tissue defects is one aspect of the "spare parts concept." Using a free fillet flap in such situations enables the successful formation of a proximal stump with the length needed to cover a large defect from forequarter amputation without risking additional donor-site morbidity. The use of free fillet flaps for reconstruction after forequarter and traumatic upper extremity amputations is illustrated here in a case report. A 41-year old patient required a forequarter amputation to resect a desmoid tumor, resulting in an extensive soft-tissue defect of the upper extremity...
November 2016: Microsurgery
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