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https://www.readbyqxmd.com/read/29220303/locally-aggressive-connective-tissue-tumors
#1
Mrinal M Gounder, David M Thomas, William D Tap
In this review, we highlight the complexities of the natural history, biology, and clinical management of three intermediate connective tissue tumors: desmoid tumor (DT) or aggressive fibromatosis, tenosynovial giant cell tumor (TGCT) or diffuse-type pigmented villonodular synovitis (dtPVNS), and giant cell tumor of bone (GCTB). Intermediate histologies include tumors of both soft tissue and bone origin and are locally aggressive and rarely metastatic. Some common aspects to these tumors are that they can be locally infiltrative and/or impinge on critical organs, which leads to disfigurement, pain, loss of function and mobility, neurovascular compromise, and occasionally life-threatening consequences, such as mesenteric, bowel, ureteral, and/or bladder obstruction...
December 8, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29207578/molecular-insights-into-desmoid-tumors
#2
EDITORIAL
Nam Bui, Shivaani Kummar
No abstract text is available yet for this article.
October 31, 2017: Oncotarget
https://www.readbyqxmd.com/read/29142465/efficacy-of-vinorelbine-combined-with-low-dose-methotrexate-for-treatment-of-inoperable-desmoid-tumor-and-prognostic-factor-analysis
#3
Shu Li, Zhengfu Fan, Zhiwei Fang, Jiayong Liu, Chujie Bai, Ruifeng Xue, Lu Zhang, Tian Gao
Objective: To assess the efficacy of conservative chemotherapy for inoperable desmoid tumor (DT) and analyze the prognostic factors. Methods: From November 2008 to April 2016, 71 patients of inoperable DT were treated with vinorelbine and low-dose methotrexate in the Department of Bone and Soft Tissue Tumors, Peking University Cancer Hospital & Institute, and enrolled in this retrospective study. The chemotherapy duration is one year. The efficacy of chemotherapy and the prognosis were observed...
October 2017: Chinese Journal of Cancer Research, Chung-kuo Yen Cheng Yen Chiu
https://www.readbyqxmd.com/read/29121373/pediatric-sinonasal-desmoid-tumor
#4
Jonathan Choi, Evan Walgama, John McClay, Pete S Batra, Ron B Mitchell
No abstract text is available yet for this article.
October 2017: Ear, Nose, & Throat Journal
https://www.readbyqxmd.com/read/29104207/-resection-of-a-chest-wall-desmoid-tumor-with-chest-wall-reconstruction-report-of-a-case
#5
Sakiko Nakamori, Masayuki Okui, Hirotoshi Horio, Takashi Yamamichi, Ayaka Asakawa, Masahiko Harada, Jumpei Kashima, Toru Motoi, Koji Sakaguchi
A primary desmoid tumor arising from the chest wall is extremely rare. We report the case of a 57-year-old man presenting with a desmoid tumor arising from his chest wall. Chest radiograph at a regular medical checkup indicated an abnormal shadow. By computed tomography-guided biopsy, he was diagnosed as having a desmoid tumor. He underwent right-sided chest wall resection and reconstruction. Desmoid tumor is histopathologically benign tumor, however, they tend to show high rates of local recurrence after surgery...
November 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/29095310/sonography-used-in-the-infantile-desmoid-fibromatosis-of-postcricoid-area-a-case-report
#6
Chunxia Xia, Qiang Zhu, Changli Yue, Minxia Hu, Pingdong Li, Zheng Li
RATIONALE: Infantile desmoid fibromatosis of the postcricoid area is a rare disease and is characterized by a proliferation of fibrous tissue with non-metastasis, local infiltration, and a high rate of recurrence after surgical resection. Currently, ultrasound is scarcely used in the hypopharynx and larynx area. PATIENT CONCERNS: A 4-year-old boy presented with hoarseness, deep voice and snoring for 2∼4 years without any surgical history. On sonography, the lesion was found in the postcricoid area, and the left larynx showed impaired mobility in real time observation...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29056131/late-presentation-of-aggressive-fibromatosis-involving-head-neck-and-chest-wall
#7
Waseem M Hajjar, Amjad F AlShehri, Mohammed A Alessa, Sami A Al-Nassar
Fibromatosis is a rare, benign, slow-growing and locally infiltrative tumour, caused by uncontrolled proliferation of fibrous tissue arising from muscles, connective tissue, fasciae and aponeurosis. It is also called desmoid tumour, which is very rare pathology representing only 0.03% of all neoplasms in human and around 3% of all soft tissue tumours. It is locally aggressive and usually invades the surrounding structures and has a high recurrence rate, even after surgical complete resection, which should be the first line of treatment...
October 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/29033773/laparoscopic-resection-of-a-jejunal-mesenteric-pseudocyst
#8
Takumi Yamabuki, Masato Suzuoki, Tsuzuku Murakami, Satoshi Hirano
An unusual case of a jejunal mesenteric pseudocyst treated by laparoscopic resection is reported. A 44-year-old woman was admitted to our hospital with intermittent upper abdominal pain and diarrhea. Physical examination revealed slight periumbilical tenderness, and no masses were palpable. Contrast-enhanced computed tomography showed a 4-cm-sized nonenhancing high-density mass with a heterogeneous pattern on a proximal small bowel loop. Based on these findings, a gastrointestinal stromal tumor accompanied by hemorrhagic and cystic change, a mesenteric hematoma, or a desmoid tumor was diagnosed...
September 2017: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/28982225/not-all-abdominal-wall-masses-in-women-are-endometriomas-or-desmoids-endometriosis-associated-abdominal-wall-cancer
#9
Leigh Archer, Daniel Wong, Robert Nairn, Rupert Hodder
No abstract text is available yet for this article.
October 5, 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/28979721/desmoid-type-fibromatosis-a-case-report-with-an-unusual-etiology
#10
Syed Faisal Jafri, Obada Obaisi, Gerardo G Vergara, Joe Cates, Jaswinder Singh, Jennifer Feeback, Harathi Yandrapu
Desmoid type fibromatosis (DTF) is a rare, locally invasive, non-metastasizing soft tissue tumor. We report an interesting case of DTF involving the pancreatic head of a 54-year-old woman. She presented with intermittent dysphagia and significant weight loss within a 3-mo period. Laboratory findings showed mild elevation of transaminases, significant elevation of alkaline phosphatase and direct hyperbilirubinemia, indicating obstructive jaundice. Computerized tomography of the abdomen revealed a mass in the head of the pancreas, dilated common bile duct, and dilated pancreatic duct...
September 15, 2017: World Journal of Gastrointestinal Oncology
https://www.readbyqxmd.com/read/28974244/multiple-rapidly-growing-desmoid-tumors-that-were-difficult-to-distinguish-from-recurrence-of-rectal-cancer
#11
Koki Nakanishi, Dai Shida, Shunsuke Tsukamoto, Hiroki Ochiai, Junichi Mazaki, Hirokazu Taniguchi, Yukihide Kanemitsu
BACKGROUND: Intra-abdominal desmoid tumors are usually slow growing and solitary, but multifocal desmoid tumors develop on rare occasions. Diagnosing desmoid tumors before histological examination of a surgical biopsy is often difficult. In particular, if a patient has a prior history of malignancy, it may be difficult to differentiate between these lesions and disease recurrence or metastasis. CASE PRESENTATION: We present a rare case of multiple rapidly growing intra-abdominal desmoid tumors after surgical trauma, without familial adenomatous polyposis...
October 3, 2017: World Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28961825/an-update-on-the-management-of-sporadic-desmoid-type-fibromatosis-a-european-consensus-initiative-between-sarcoma-patients-euronet-spaen-and-european-organization-for-research-and-treatment-of-cancer-eortc-soft-tissue-and-bone-sarcoma-group-stbsg
#12
B Kasper, C Baumgarten, J Garcia, S Bonvalot, R Haas, F Haller, P Hohenberger, N Penel, C Messiou, W T van der Graaf, A Gronchi
Desmoid-type fibromatosis is a rare and locally aggressive monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Currently, there is no established or evidence-based treatment approach available for this disease. Therefore, in 2015 the European Desmoid Working Group published a position paper giving recommendations on the treatment of this intriguing disease. Here, we present an update of this consensus approach based on professionals' AND patients' expertise following a round table meeting bringing together sarcoma experts from the European Organization for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group with patients and patient advocates from Sarcoma PAtients EuroNet...
October 1, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/28954784/a-first-in-human-phase-1-study-of-the-anti-cancer-stem-cell-agent-ipafricept-omp-54f28-a-decoy-receptor-for-wnt-ligands-in-patients-with-advanced-solid-tumors
#13
Antonio Jimeno, Michael S Gordon, Rashmi Chugh, Wells Messersmith, David S Mendelson, Jakob Dupont, Robert J Stagg, Ann M Kapoun, Lu Xu, Shailaja Uttamsingh, Rainer Brachmann, David C Smith
PURPOSE: Wnt signaling is implicated in tumor cell de-differentiation and cancer stem cell function. Ipafricept (OMP-54F28) is a first-in-class recombinant fusion protein with the extracellular part of human Frizzled 8 receptor fused to a human IgG1 Fc fragment that binds Wnt ligands. This trial evaluated ipafricept in patients with solid tumors. EXPERIMENTAL DESIGN: A 3+3 design was used; ipafricept was given intravenously every 3 weeks. Objectives were determination of dose-limiting toxicities (DLTs), recommended phase 2 dose (RP2D), safety, pharmacokinetics (PK), immunogenicity, pharmacodynamics (PD), and preliminary efficacy...
September 27, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28918445/pre-emptive-intestinal-transplant-the-surgeon-s-point-of-view
#14
REVIEW
Augusto Lauro, Ignazio R Marino, Kishore R Iyer
Pre-emptive transplantation is a well-established practice for certain types of end-organ failure such as in the use of kidney transplantation. For irreversible intestinal failure, total parenteral nutrition (TPN) remains the gold standard, due to the suboptimal long-term results of intestinal transplantation. As such, the only role for pre-emptive transplantation, if at all, will be for patients identified to be at high risk of complications and mortality while on definitive long-term TPN. In these patients, the timing of early listing and transplantation could become life-saving, taking into account that mortality on the waiting list is still the highest for intestinal candidates...
November 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28904708/-latissimus-dorsi-flap-in-reconstruction-following-treatment-of-giant-tumor-of-the-abdominal-wall-about-a-rare-case
#15
Karim Bourra, Samir El Mazouz
We report the case of a 16-year old patient presenting with giant, multinodular, mesenchymal tumor of the abdominal wall occupying the left abdominal region and measuring 25 cm on the vertical axis, 20 cm on the transverse axis, mobile when compared with the deep structures and gradually increasing in volume over childhood and neglected. After small biopsy, which showed desmoid tumor, the patient underwent complete surgical resection of the tumor with immediate reconstruction by free muscolo skin flap of the latissimus dorsi attached to the large blood vessels of the inguinal fold (left iliac artery and left external iliac vein), connected by termino lateral anastomosis...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28898989/role-of-histone-h3k27-trimethylation-loss-as-a-marker-for-malignant-peripheral-nerve-sheath-tumor-in-fine-needle-aspiration-and-small-biopsy-specimens
#16
Jeffrey K Mito, Xiaohua Qian, Leona A Doyle, Jason L Hornick, Vickie Y Jo
Objectives: Accurate diagnosis of malignant peripheral nerve sheath tumor (MPNST) is often challenging on fine-needle aspiration (FNA) or core needle biopsy. Recurrent mutations in EED and SUZ12, which encode subunits of polycomb repressive complex 2 (PRC2), have been identified in 70% to 92% of MPNSTs; PRC2 inactivation leads to loss of trimethylation of lysine 27 of histone H3 (H3K27me3). We evaluated the utility of H3K27me3 immunohistochemistry for distinguishing MPNST from its cytomorphologic mimics...
August 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28890814/outcomes-and-complications-of-radiation-therapy-in-patients-with-familial-adenomatous-polyposis
#17
Meng Gan, Dustin Boothe, Deborah W Neklason, N Jewel Samadder, Jonathan Frandsen, Megan B Keener, Shane Lloyd
BACKGROUND: The outcomes, complications, and rates of secondary malignancies from radiation therapy (RT) are not known for patients with familial adenomatous polyposis (FAP). METHODS: We queried the Hereditary Gastrointestinal Cancer Registry (HGCR) for patients with FAP who received RT. Outcomes assessed included acute and late treatment toxicity and secondary malignancies. RESULTS: We identified 15 patients undergoing 18 treatment courses...
August 2017: Journal of Gastrointestinal Oncology
https://www.readbyqxmd.com/read/28887726/long-term-follow-up-of-desmoid-fibromatosis-treated-with-pf-03084014-an-oral-gamma-secretase-inhibitor
#18
Victor Manuel Villalobos, Francis Hall, Antonio Jimeno, Lia Gore, Kenneth Kern, Rossano Cesari, Bo Huang, Jeffrey T Schowinsky, Patrick Judson Blatchford, Brianna Hoffner, Anthony Elias, Wells Messersmith
BACKGROUND: Desmoid fibromatosis is a fibroblastic neoplasm driven by aberrations within the WNT pathway, exhibiting mutations in β-catenin or APC. We review the long-term follow-up of patients in a phase I study treated with an oral gamma secretase inhibitor, PF-03084014. METHODS: PF-03084014 was administered orally at doses ranging from 20 to 330 mg twice daily. Tumor assessments were performed using computed tomography/magnetic resonance imaging (CT/MRI) within 4 weeks of study entry, and every other cycle through cycle 9...
September 8, 2017: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/28883708/rarity-among-benign-gastric-tumors-plexiform-fibromyxoma-report-of-two-cases
#19
Kinga Szurian, Holger Till, Eva Amerstorfer, Nicole Hinteregger, Hans-Jörg Mischinger, Bernadette Liegl-Atzwanger, Iva Brcic
Plexiform fibromyxoma is a very rare mesenchymal tumor of the stomach, found almost exclusively in the antrum/pylorus region. The most common presenting symptoms are anemia, hematemesis, nausea and unintentional weight loss, without sex or age predilection. We describe here two cases of plexiform fibromyxoma, involving a 16-year-old female and a 34-year-old male. Both patients underwent complete resection (R0) by distal gastrectomy and retrocolic gastrojejunostomy (according to Billroth 2); for both, the postoperative course was uneventful...
August 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28881160/effective-treatment-of-aggressive-fibromatosis-with-celecoxib-guided-by-genetic-testing
#20
Shanshan Yang, Xufu Wang, Haiping Jiang, Yongjie Wang, Zhuokun Li, Haijun Lu
Aggressive fibromatosis (AF) or desmoid tumors is an aggressive fibroblastic proliferation which is locally invasive but can not metastasize. The treatment of AF is challenging. Surgery was the main treatment modality for AF in the past, other strategies including radiotherapy, systemic therapies and wait-and-see policy. The use of non-steroidal anti-inflammatory drugs (NSAIDs) and targeted therapies has demonstrated good results. In the case report, a 39-year-old man presented with progressive chest wall pain...
October 3, 2017: Cancer Biology & Therapy
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