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Desmoid tumor

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https://www.readbyqxmd.com/read/29033773/laparoscopic-resection-of-a-jejunal-mesenteric-pseudocyst
#1
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/28979721/desmoid-type-fibromatosis-a-case-report-with-an-unusual-etiology
#2
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
#3
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/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
#4
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
#5
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...
September 16, 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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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...
September 7, 2017: Cancer Biology & Therapy
https://www.readbyqxmd.com/read/28857656/diaphragm-reconstruction-combined-with-thoraco-abdominal-wall-reconstruction-after-tumor-resection
#12
Hiroaki Kuwahara, Juho Salo, Erkki Tukiainen
BACKGROUND: Thoraco-abdominal wall resection including diaphragm resection results in a challenging surgical defect. Various methods have been used for diaphragm reconstruction. The aim of this study was to describe our methods of diaphragm and thoraco-abdominal wall reconstruction after combined resection of these anatomical structures. METHODS: Twenty-one patients underwent diaphragm resection at our institution between 1997 and 2015. We used a mesh or direct closure for diaphragm defect and a mesh for chest wall stabilization...
August 31, 2017: Journal of Plastic Surgery and Hand Surgery
https://www.readbyqxmd.com/read/28851389/immunohistochemical-staining-with-non-phospho-%C3%AE-catenin-as-a-diagnostic-and-prognostic-tool-of-cox-2-inhibitor-therapy-for-patients-with-extra-peritoneal-desmoid-type-fibromatosis
#13
Tomohisa Sakai, Yoshihiro Nishida, Shunsuke Hamada, Hiroshi Koike, Kunihiro Ikuta, Takehiro Ota, Naoki Ishiguro
BACKGROUND: Immunohistochemical staining with conventional anti-β-catenin antibody has been applied as a diagnostic tool for desmoid-type fibromatosis (DF). This study aimed to evaluate the diagnostic and prognostic value of immunohistochemical staining with anti-non-phospho β-catenin antibody, which might more accurately reflect the aggressiveness of DF, in comparison to the conventional anti-β-catenin antibody. METHODS: Between 2003 and 2015, 40 patients with extra-peritoneal sporadic DF were prospectively treated with meloxicam or celecoxib, a COX-2 inhibitor, therapy...
August 29, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28842766/desmoid-tumor-and-silicone-breast-implant-surgery-is-there-really-a-connection-a-literature-review
#14
REVIEW
R Tzur, E Silberstein, Y Krieger, Y Shoham, Y Rafaeli, A Bogdanov-Berezovsky
BACKGROUND: Desmoid tumors are borderline tumors of the connective tissue, arising in the musculo-aponeurotic stromal elements. A desmoid tumor (DT) has an infiltrative and locally aggressive growth pattern and usually does not metastasize; however, it has a high recurrence and complication rate. DT located in the breast (BDT) represents a rare extra-abdominal form. Recently, the presence of breast silicone implants was suggested by several researchers as a risk factor for developing BDT...
August 25, 2017: Aesthetic Plastic Surgery
https://www.readbyqxmd.com/read/28815038/management-of-desmoid-tumours-a-large-national-database-of-familial-adenomatous-patients-shows-a-link-to-colectomy-modalities-and-low-efficacy-of-medical-treatments
#15
T Walter, C Zhenzhen Wang, O Guillaud, E Cotte, A Pasquer, O Vinet, G Poncet, T Ponchon, J-C Saurin
BACKGROUND: Desmoid tumours represent a major complication of familial adenomatous polyposis. Our aims were to study the factors associated with the development of desmoid tumours in familial adenomatous polyposis patients, and to describe presentation and management of desmoid tumours. METHODS AND PATIENTS: We reviewed all patients with familial adenomatous polyposis followed at our institution between 1965-2013, with either identified adenomatous polyposis coli gene mutation, or a personal and family history suggesting adenomatous polyposis coli-related polyposis...
August 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/28797989/giant-pancreatic-solid-cystic-desmoid-tumor-with-two-ectopic-adrenal-tissues
#16
Neşe Ekinci, Fikret Dirilenoğlu, Arzu Avcı, Oğuzhan Özsay
A 19-year-old woman presented with painless swelling of the abdomen. During surgery, a giant mass measuring 37 cm×26 cm×12 cm within the distal pancreas invading the spleen was noted. The clinical diagnosis of a solid cystic pseudopapillary tumor of the pancreas was suspected. Distal pancreatectomy, splenectomy, and debulking surgery were performed. Histological examination showed that the tumor infiltrated the spleen and pancreatic parenchyma, and sections of the solid areas revealed a proliferation of spindle-shaped or stellate cells growing in fascicular and storiform patterns within a myxoid intercellular matrix...
August 9, 2017: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/28761389/concurrent-imatinib-and-radiation-therapy-for-unresectable-and-symptomatic-desmoid-tumors
#17
Everett J Moding, Lynn Million, Raffi Avedian, Pejman Ghanouni, Christian Kunder, Kristen N Ganjoo
Desmoid tumors are locally aggressive fibroproliferative neoplasms that can lead to pain and dysfunction due to compression of nerves and surrounding structures. Desmoid tumors often progress through medical therapy, and there is frequently a delay of multiple months before radiation can provide symptomatic relief. To achieve more rapid symptomatic relief and tumor regression for unresectable desmoid tumors causing significant morbidity such as brachial plexus impingement with loss of extremity function, we have selectively utilized a combination of imatinib and radiation therapy...
2017: Sarcoma
https://www.readbyqxmd.com/read/28744163/cesarean-section-after-abdominal-mesh-repair-for-pregnancy-related-desmoid-tumor-a-case-report
#18
Sara Ooi, Harry Ngo
We report the case of a 32-year-old gravida 2 para 1 woman with a background of partially resected desmoid tumor (DT) arising from the previous cesarean section (CS) scar. This case details the management of her DT by surgical resection and mesh repair and second pregnancy following this. Pregnancy-related DTs are a relatively rare entity, and there is a paucity of literature regarding their management during pregnancy. There are only five reported cases of DTs arising from CS scars. To our knowledge, this is the only report to illustrate that subsequent CS is possible after desmoid resection and abdominal mesh repair...
2017: International Journal of Women's Health
https://www.readbyqxmd.com/read/28731043/gastroblastoma-harbors-a-recurrent-somatic-malat1-gli1-fusion-gene
#19
Rondell P Graham, Asha A Nair, Jaime I Davila, Long Jin, Jin Jen, William R Sukov, Tsung-Teh Wu, Henry D Appelman, Jorge Torres-Mora, Kyle D Perry, Lizhi Zhang, Sara M Kloft-Nelson, Ryan A Knudson, Patricia T Greipp, Andrew L Folpe
Gastroblastoma is a rare distinctive biphasic tumor of the stomach. The molecular biology of gastroblastoma has not been studied, and no affirmative diagnostic markers have been developed. We retrieved two gastroblastomas from the consultation practices of the authors and performed transcriptome sequencing on formalin-fixed paraffin-embedded tissue. Recurrent predicted fusion genes were validated at genomic and RNA levels. The presence of the fusion gene was confirmed on two additional paraffin-embedded cases of gastroblastoma...
October 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28676226/late-toxicity-and-outcomes-following-radiation-therapy-for-chest-wall-sarcomas-in-pediatric-patients
#20
John T Lucas, Israel Fernandez-Pineda, Christopher L Tinkle, Michael W Bishop, Sue C Kaste, Rajiv Heda, Andrew M Davidoff, Matthew J Krasin
PURPOSE: To investigate the contribution of radiation therapy to acute and late toxicity in pediatric chest wall sarcoma patients and evaluate dosimetric correlates of higher incidence toxicities such as scoliosis and pneumonitis. METHODS AND MATERIALS: The data from 23 consecutively treated pediatric patients with chest wall sarcomas of various histologies (desmoid, Ewing, rhabdomyosarcoma, nonrhabdomyosarcoma-soft tissue sarcomas) were reviewed to evaluate the relationship between end-organ radiation dose, clinical factors, and the risk of subsequent late effects (scoliosis, pneumonitis)...
April 26, 2017: Practical Radiation Oncology
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