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Desmoid tumor

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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
#1
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
#2
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
#3
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
#4
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
#5
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...
July 21, 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
#6
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
https://www.readbyqxmd.com/read/28668823/desmoid-tumors-in-familial-adenomatous-polyposis
#7
REVIEW
Maria Laura DE Marchis, Francesco Tonelli, Davide Quaresmini, Domenica Lovero, David Della-Morte, Franco Silvestris, Fiorella Guadagni, Raffaele Palmirotta
Familial adenomatous polyposis (FAP) is a cancer syndrome caused by a germline mutation in the adenomatous polyposis coli (APC) gene. It is characterized by the presence of hundreds of colonic polyps, which have a high tendency to undergo malignant transformation. Among associated lesions in FAP, desmoid tumors represent a common possible life-threatening condition that requires special attention. They are rare tumors occurring with a particularly high incidence in FAP, especially after surgery. In agreement with Knudson's 'two-hit' theory, the inactivation of the residual APC gene in FAP is a critical step in the development of both colorectal cancer and desmoids...
July 2017: Anticancer Research
https://www.readbyqxmd.com/read/28666150/mesenteric-desmoid-tumour-presenting-with-recurrent-abdominal-abscess-and-duodenal-fistula-a-case-report-and-review-of-literature
#8
Kai Huang, Heather Stuart, Kirill Lyapichev, Andrew E Rosenberg, Alan S Livingstone
INTRODUCTION: Desmoid tumors are locally destructive but histologically benign. Their management involves close observation and surgical, medical, or hormonal treatment. PRESENTATION OF THE CASE: A 36-year-old male was admitted for abdominal pain and fever. A CT scan showed fluid collections and air within a mesenteric mass. Diagnostic laparotomy was performed with drainage of the abscess and biopsy of the mass. The pathology suggested a desmoid tumor. His fever and abdominal pain persisted...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28657872/desmoid-tumors-and-celecoxib-with-sorafenib
#9
LETTER
Nicolas Benech, Thomas Walter, Jean-Christophe Saurin
New England Journal of Medicine, Volume 376, Issue 26, Page 2595-2597, June 2017.
June 29, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28641503/embolization-of-a-large-progressive-symptomatic-desmoid-tumor-in-the-rectus-muscle-of-a-female-patient-with-multiple-sclerosis-a-case-report
#10
I Diebels, M Blockhuys, P Willemsen, Y Pirenne
INTRODUCTION: Desmoid tumors are benign tumors, yet can lead to significant morbidity due to aggressive local expansions. Treatment starts with a wait-and-see policy, however, more aggressive treatments like broad margin resection surgery might be necessary in case of tumor progression. PATIENTS AND METHODS: We report the case of a 26-year-old female with a symptomatic desmoid tumor in the left rectus muscle. The initial wait-and-see policy led to an increase in tumor size and progression of symptoms...
June 22, 2017: Acta Chirurgica Belgica
https://www.readbyqxmd.com/read/28632950/fibroblastic-connective-tissue-nevus-clinicopathological-and-immunohistochemical-study-of-14-cases
#11
Ilaria Pennacchia, Heinz Kutzner, Dmitry V Kazakov, Thomas Mentzel
BACKGROUND: We present herein a series of 14 lesions showing overlapping features with the newly defined benign cutaneous mesenchymal neoplasm labeled as fibroblastic connective tissue nevus (FCTN). METHODS AND RESULTS: Total of 8 patients were male and 5 were female, ranging in age from 1 to 56 years. Lesions appeared as isolated nodules or plaques on the trunk (7 cases), the limbs (4 cases) and the neck (2 cases). Histologically, all cases were composed of bundles of bland spindle cells of fibroblastic/myofibroblastic lineage irregularly branching within the reticular dermis and along fibrous septa in the subcutis...
June 20, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28627792/%C3%AE-catenin-in-desmoid-type-fibromatosis-deep-insights-on-the-role-of-t41a-and-s45f-mutations-on-protein-structure-and-gene-expression
#12
C Colombo, A Belfiore, N Paielli, L De Cecco, S Canevari, E Laurini, M Fermeglia, S Pricl, P Verderio, S Bottelli, M Fiore, S Stacchiotti, E Palassini, A Gronchi, S Pilotti, F Perrone
Desmoid- type fibromatosis (DF) is a rare mesenchymal lesion with high risk of local recurrence. Specific β-catenin mutations (S45F) appeared to be related to this higher risk compared to T41A mutated or wild type (WT). We explored the influence of both mutations and WT on structure stability and affinity of β-catenin for α-catenin and the pattern of gene expression that may influence DF behavior. Using 33 surgically resected primary DFs harboring T41A (n=14), S45F (n=10) or WT (n=9), we performed a comparative molecular analysis by protein/protein interaction modeling, gene expression by DASL microarrays, human inflammation gene panel and assessment of immune system-based biomarkers by immunohistochemistry...
June 19, 2017: Molecular Oncology
https://www.readbyqxmd.com/read/28624006/multi-parametric-mp-mri-for-the-diagnosis-of-abdominal-wall-desmoid-tumors
#13
Maneesh Khanna, Subramaniyan Ramanathan, Aalaa Salaheldin Kambal, Mohammed Al-Berawi, Santosh Yadav, Devendra Kumar, Nicola Schieda
INTRODUCTION: Desmoid tumors are benign myofibroblastic neoplasms, originating from the muscle aponeurosis and classified as deep fibromatoses. The aim of this study was to evaluate the utility of multi-parametric (mp)-MRI for the diagnosis of abdominal wall desmoid tumor (awdt). MATERIAL AND METHODS: This Institutional review board approved retrospective study compared 10 patients (mean age±SD; 38.2±13years; 9 females and 1 male) with awdt to 14 subjects (mean age±SD; 45...
July 2017: European Journal of Radiology
https://www.readbyqxmd.com/read/28595499/thermal-diffusivity-and-perfusion-constants-from-in-vivo-mr-guided-focused-ultrasound-treatments-a-feasibility-study
#14
Christopher R Dillon, Viola Rieke, Pejman Ghanouni, Allison Payne
PURPOSE: This study investigates the feasibility of non-invasively determining thermal diffusivity (α) and the Pennes perfusion parameter (w) from preclinical and clinical magnetic resonance-guided focused ultrasound (MRgFUS) temperature data. MATERIALS AND METHODS: Pre-clinical MRgFUS experiments were performed in rabbit muscle (N = 3, 28 sonications) using three-dimensional MR thermometry. 8 sonications were made in a clinical QA phantom with two-dimensional thermometry...
June 8, 2017: International Journal of Hyperthermia
https://www.readbyqxmd.com/read/28588785/sporadic-giant-intra-abdominal-desmoid-tumor-a-radiological-case-report
#15
Karla Kovačević, Dragica Obad-Kovačević, Jelena Popić-Ramač
Desmoid tumor (DT) is a locally invasive form of fibromatosis, comprising only 0.03% of all tumors. DTs occur more frequently in patients with familial adenomatous polyposis and Gardner's syndrome, as intra-abdominal or anterior abdominal wall tumors, whereas sporadic DTs are more likely to be extra-abdominal (only 5% of sporadic DTs are intra-abdominal). There is also an association of DTs with prior trauma, surgery, estrogen exposure and childbirth. Imaging studies, such as computed tomography (CT) and magnetic resonance imaging (MRI) are used for preoperative diagnosis and for the planning of the surgery...
June 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28584378/desmoid-tumor-of-rectus-abdominis-presenting-with-grey-turner-s-and-cullen-s-sign-a-report-of-a-rare-case
#16
Angoori Gnaneshwar Rao, T Swathi, Saba Syeda Farheen, Amit Kolli, Sharanya Hari, Uday Deshmukh Reddy, Kondapi Deepak, Kranthi Jagadevapuram
Desmoid tumor of rectus abdominis presenting with Grey-Turner's and Cullen's sign is rare. Herein, we report desmoid tumor of rectus abdominis in a 64-year-old multiparous female who presented with ecchymosis involving left flank and around the umbilicus. Histopathological examination of biopsy from the tumor confirmed the diagnosis of the desmoid tumor. She was referred to a surgeon for radical resection.
May 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28578783/biology-and-treatment-of-aggressive-fibromatosis-or-desmoid-tumor
#17
REVIEW
Keith M Skubitz
Aggressive fibromatosis, also known as desmoid-type fibromatosis (DTF) or desmoid tumor, is an uncommon locally invasive tumor. Because of its low incidence and variable behavior, DTF is often first seen by physicians who are not familiar with it, and recent advances in understanding this disease have led to changes in treatment approaches. The Wnt (β-catenin) pathway appears to play a key role in DTF pathogenesis, and recent studies of DTF biology suggest a possible model of DTF pathogenesis. Histologically, DTF shows a poorly circumscribed proliferation of myofibroblast-like cells with variable collagen deposition, similar to the proliferative phase of wound healing, and DTF has been associated with trauma and pregnancy...
June 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28570749/comparison-of-sporadic-and-fap-associated-desmoid-type-fibromatoses
#18
Laura Koskenvuo, Ari Ristimäki, Anna Lepistö
BACKGROUND AND OBJECTIVES: Desmoid-type fibromatosis is a rare disease of which 7.5-16% have been reported to be related to familial adenomatous polyposis (FAP). We sought to compare the characteristics and treatment of sporadic and FAP-related desmoid-type fibromatoses. METHODS: Altogether 220 patients were included in the study after receiving a diagnosis of desmoid-type fibromatosis by the Pathology Department of Helsinki University Hospital, with adequate follow-up...
June 1, 2017: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28554106/chest-wall-reconstruction-following-axillary-breast-augmentation-and-desmoid-tumor-resection-using-capsular-flaps-and-a-form-stable-silicone-implant-a-case-report-diagnosis-and-surgical-technique
#19
Alexandre Mendonça Munhoz, Ary de Azevedo Marques, José Ribas Milanez, Rolf Gemperli
INTRODUCTION: Chest desmoid tumors (CDT) are rare lesions characterized by fibroblastic proliferation from the connective tissue. Although CDT have been studied previously, no cases following subfascial transaxillary breast augmentation (TBA) have been described. PRESENTATION OF CASE: The authors describe a case of CDT in a 28-year-old woman one year after TBA, which presented as a painful and progressive mass in the lower-inner right breast quadrant. MRI showed a soft-tissue tumor (6×3×4cm) that affected the region of the right anterior costal margin, without signs of structural costal invasion...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28512414/an-intra-abdominal-desmoid-tumor-embedded-in-the-pancreas-preoperatively-diagnosed-as-an-extragastric-growing-gastrointestinal-stromal-tumor
#20
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
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