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Desmoid

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https://www.readbyqxmd.com/read/29424372/desmoid-tumor-of-the-abdominal-wall-a-case-report-and-literature-review
#1
Angelo Guttadauro, Matteo Maternini, Silvia Frassani, Elena Guanziroli, Giulia Lo Bianco, Francesco Gabrielli
BACKGROUND: Desmoid tumor is a rare soft tissues neoplasia characterized by local invasiveness and by a tendency towards local recurrence although not towards metastasization. DISCUSSION: Etiology is not clear. Desmoid tumors originate from the monoclonal proliferation of one mesenchymal cell and develop in the context of fascial, muscular and aponeurotic tissue. They are free of capsule and do not usually metastatize, although they do present a high risk of local recurrence CONCLUSION: In the absence of a systematic data collection and of a clear distinction, in the existing databases, between primitive and recurrent lesions and between lesions of different sites, there are no standard guidelines for a correct management of desmoids...
November 20, 2017: Annali Italiani di Chirurgia
https://www.readbyqxmd.com/read/29413661/differential-diagnosis-of-benign-spindle-cell-lesions
#2
REVIEW
Gaetano Magro
Spindle cell lesions of the breast cover a wide spectrum of diseases ranging from reactive tumor-like lesions to high-grade malignant tumors. The recognition of the benign spindle cell tumor-like lesions (nodular fasciitis; reactive spindle cell nodule after biopsy, inflammatory pseudotumor/inflammatory myofibroblastic tumor; fascicular variant of pseudoangiomatous stromal hyperplasia) and tumors (myofibroblastoma, benign fibroblastic spindle cell tumor, leiomyoma, schwannoma, spindle cell lipoma, solitary fibrous tumor, myxoma) is crucial to avoid confusion with morphologically similar but more aggressive bland-appearing spindle cell tumors, such as desmoid-type fibromatosis, low-grade (fibromatosis-like) spindle cell carcinoma, low-grade fibrosarcoma/myofibroblastic sarcoma and dermatofibrosarcoma protuberans...
March 2018: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/29409537/complete-resection-of-a-rectus-abdominis-muscle-invaded-by-desmoid-tumors-and-subsequent-management-with-an-abdominal-binder-a-case-report
#3
Tatsuhiko Ogawa
BACKGROUND: Desmoid-type fibromatosis is characterized by desmoid tumors, which are benign soft tissue tumors that can be locally aggressive but typically do not metastasize. Desmoid tumors can manifest anywhere in the body, and those in the abdominal cavity account for approximately 30 to 50% of all such tumors. Complete resection with free margins has been the standard treatment, but non-surgical therapies have been implemented recently. However, if tumors are strongly invasive and/or persistently recur, radical surgical resection with free margins remains the primary treatment...
February 7, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29397265/the-evaluation-of-circulating-endothelial-progenitor-cells-and-related-angiogenic-markers-as-prognostic-factors-in-soft-tissue-tumors
#4
Ioannis Karampinis, Elena Joas, Anna Dreyer, Ulrich Ronellenfitsch, Jens Jakob, Peter Hohenberger, Kai Nowak
INTRODUCTION: Neovascularisation is a critical step in the progression of malignant tumors. Circulating endothelial progenitor cells (cEPC) have been proposed as surrogate markers of vasculogenesis in malignancies. In this project, we studied the impact of tumor-specific therapy on cEPC and associated angiogenic factors in patients with soft tissue tumors. MATERIALS AND METHODS: Fifty-three patients with soft tissue tumors (25 soft tissue sarcomas, 19 GIST, 9 desmoids) and 15 healthy controls were included...
January 31, 2018: European Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29394664/-the-characteristics-and-treatment-outcomes-for-desmoid-tumors-associated-with-familial-adenomatous-polyposis
#5
Kunihiko Amano, Noriyasu Chika, Tetsuya Ito, Azusa Yamamoto, Satoshi Hatano, Toru Ishiguro, Minoru Fukuchi, Youichi Kumagai, Keiichiro Ishibashi, Erito Mochiki, Takeo Iwama, Hidetaka Eguchi, Koji Okazaki, Shigehisa Inokuma, Hideyuki Ishida
INTRODUCTION: The characteristics of desmoid tumors(DTs)associated with familial adenomatous polyposis(FAP)and relationships between the development of DTs and the sites of APC germline mutation have not closely been examined Japan. PATIENTS AND METHODS: This retrospective study was performed to address these issues by examining patients with FAP who underwent proctocolectomy between 1981 and 2015. RESULTS: The cumulative 2-year incidence of DT development was 50%...
November 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/29384266/pazopanib-therapy-for-desmoid-tumors-in-adolescent-and-young-adult-patients
#6
Laura Agresta, Hee Kim, Brian K Turpin, Rajaram Nagarajan, Alexandra Plemmons, Sara Szabo, Roshni Dasgupta, Joel I Sorger, Joseph G Pressey
BACKGROUND: Desmoid tumors/aggressive fibromatosis (DT/AF) lack a reliably effective medical therapy. Surgical resection may be morbid and does not preclude recurrence. Radiation may carry severe late effects, particularly detrimental in young patients. At our institution, we recently observed promising results with pazopanib therapy for DT/AF in adolescent and young adult (AYA) patients. PROCEDURE: Retrospective single-institution chart review. RESULTS: Six DT/AF patients of 3-21 years with previously treated DT/AF received pazopanib; 31 DT/AF patients received established therapies only...
January 31, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29377717/impact-of-pathologist-involvement-in-sarcoma-and-rare-tumor-patient-support-groups-on-facebook-a-survey-of-542-patients-and-family-members
#7
Jasmine Haller, Marjorie Parker David, Nathan E Lee, Sara C Shalin, Jerad M Gardner
CONTEXT: - Patients with rare tumors have difficulty finding reliable information about their disease. Facebook patient support groups allow patients to educate one another. OBJECTIVE: - To investigate how these patients perceive the value of pathologists, both in Facebook groups and real-world patient care. DESIGN: - Survey links were posted in 12 Facebook patient groups: 6 with an active pathologist member (angiosarcoma, epithelioid hemangioendothelioma, epithelioid sarcoma, dermatofibrosarcoma protuberans [×2], and desmoid fibromatosis), and 6 without "active" pathologist involvement (aggressive angiomyxoma, chondrosarcoma, Ewing sarcoma, leiomyosarcoma, liposarcoma, and osteosarcoma)...
January 29, 2018: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/29375904/chest-wall-a-structure-underestimated-in-ultrasonography-part-iii-neoplastic-lesions
#8
REVIEW
Andrzej Smereczyński, Katarzyna Kołaczyk, Elżbieta Bernatowicz
Chest wall neoplasms mainly include malignancies, metastatic in particular. Differential diagnosis should include clinical data; tumor location, extent, delineation; the degree of homogeneity; the presence of calcifications; the nature of bone destruction and the degree of vascularization. The aim of the paper is to present both the benefits and limitations of ultrasound for the diagnosis of chest wall neoplasms. The neoplastic process may be limited to the chest wall; it may spread from the chest wall into the intrathoracic structures or spread from the inside of the chest towards the chest wall...
December 2017: Journal of Ultrasonography
https://www.readbyqxmd.com/read/29375894/screening-detected-desmoid-tumor-of-the-breast-findings-at-conventional-imaging-and-digital-breast-tomosynthesis
#9
Tatjana Samardzic, Jon Lømo, Per Skaane
Desmoid tumor of the breast is a rare benign entity that usually is mistaken for carcinoma clinically and radiologically. We report two cases of desmoid tumor of the breast detected by mammography screening using digital breast tomosynthesis (DBT). The larger tumor was detected at both full-field digital mammography (FFDM) and DBT. The smaller desmoid tumor, however, was identified only at tomosynthesis. Mammographic and ultrasonographic findings at diagnostic work-up were consistent with carcinoma of the breast...
January 2018: Acta Radiologica Open
https://www.readbyqxmd.com/read/29373657/cardiac-desmoid-tumour
#10
Kanhua Yin, Yi Lin, Changfa Guo, Chunsheng Wang
No abstract text is available yet for this article.
January 23, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29368261/apc-mosaicism-in-a-young-woman-with-desmoid-type-fibromatosis-and-familial-adenomatous-polyposis
#11
Astrid Tenden Stormorken, Thomas Berg, Ole-Jacob Norum, Toto Hølmebakk, Kristin Aaberg, Sonja E Steigen, Eli Marie Grindedal
Familial adenomatous polyposis (FAP) is usually caused by germline mutations in the adenomatous polyposis coli (APC) gene. The classic form is characterized by hundreds to thousands of adenomas in the colorectum and early onset colorectal cancer (CRC) if left untreated. FAP is also associated with multiple extra-colonic manifestations such as gastroduodenal polyps, osteomas, epidermoid cysts, fibromas and desmoids. Most desmoid tumours in FAP patients occur intra-abdominally. Approximately 15-20% of the APC mutations are de novo mutations...
January 24, 2018: Familial Cancer
https://www.readbyqxmd.com/read/29330550/a-metabolomics-pilot-study-on-desmoid-tumors-and-novel-drug-candidates
#12
Kelly A Mercier, Mushriq Al-Jazrawe, Raymond Poon, Zachery Acuff, Benjamin Alman
Desmoid tumors (aggressive fibromatosis) are locally invasive soft tissue tumors that lack the ability to metastasize. There are no directed therapies or standard treatment plan, and chemotherapeutics, radiation, and surgery often have temporary effects. The majority of desmoid tumors are related to T41A and S45F mutations of the beta-catenin encoding gene (CTNNB1). Using broad spectrum metabolomics, differences were investigated between paired normal fibroblast and desmoid tumor cells from affected patients...
January 12, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29325354/-excision-of-giant-desmoid-in-the-abdominal-wall-method-of-abdominal-wall-reconstruction-and-follow-up-of-long-termed-effect
#13
Y Fei, J Y Li
Objective: To explore the ideal procedure of excision and repair for giant desmoid in the abdominal wall and long-termed follow-up results. Methods: Clinical and follow-up data of 24 patients with giant desmoid in the abdominal wall underwent radical removal and immediate abdominal wall reconstruction in Diagnostic and Therapeutic Center of Hernia and Abdominal Wall Diseases, First Affiliated Hospital of People's Liberation Army General Hospital from October 2006 to October 2016 were analyzed retrospectively...
January 1, 2018: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/29319019/port-site-desmoid-tumour-following-laparoscopic-cholecystectomy-a-case-report
#14
Gautham Krishnamurthy, Vijay Chetan Jha, Ganga Ram Verma
Desmoid tumours are locally aggressive tumours occurring either spontaneously or in familial conditions. History of trauma is invariably present with surgical trauma being a common cause. Port site desmoid tumours are extremely rare conditions. Inadequate treatment results in high recurrence rate and substantial morbidity. Reconstruction, if required, by the appropriate technique is vital to avoid an incisional hernia. Adjuvant therapy may be useful in large locally advanced or recurrent tumours. We describe a young female with large port site desmoid tumour following laparoscopic cholecystectomy managed with wide local excision and mesh placement...
January 10, 2018: Journal of Minimal Access Surgery
https://www.readbyqxmd.com/read/29318350/-benign-mesenchymal-tumors-of-the-abdomen
#15
REVIEW
P A Kupczyk, G M Kukuk
BACKGROUND: Benign mesenchymal tumors of the abdomen are-except for hemangiomas-rare neoplasms that are associated with potentially life-threatening complications. Thus, awareness of the most important entities and knowledge of their imaging characteristics is fundamental for the radiologist. OBJECTIVES: An overview of the most frequent benign abdominal soft tissue tumors and their common imaging features is given. Furthermore, other facts concerning the individual entities that are relevant to radiologic practice are outlined...
January 9, 2018: Der Radiologe
https://www.readbyqxmd.com/read/29310463/plantar-fibromatosis-pathophysiology-surgical-and-nonsurgical-therapies-an-evidence-based-review
#16
Paul Carroll, Robert M Henshaw, Caitlin Garwood, Katherine Raspovic, Dhruv Kumar
Plantar fibromatosis (morbus Ledderhose), an extra-abdominal desmoid tumor of the plantar foot, is a rare benign hyperproliferative disorder of the plantar fascia with an unknown etiology. The main clinical characteristics include slow growing nodules on the medial and central bands of the plantar fascia, which may become painful and negatively affect ambulation. Most established conservative therapies today target symptomatic relief. As symptoms progress, therapies such as injections, shockwave ablation, radiation, and/or surgery may be required...
January 1, 2018: Foot & Ankle Specialist
https://www.readbyqxmd.com/read/29304783/desmoid-tumours-of-the-extremity-and-trunk-a-retrospective-study-of-44-patients
#17
Laura Wirth, Alexander Klein, Andrea Baur-Melnyk, Thomas Knösel, Lars H Lindner, Falk Roeder, Volkmar Jansson, Hans Roland Dürr
BACKGROUND: Desmoid-type fibromatosis (DF) is a aggressive (myo)fibroblastic neoplasm with an infiltrative growth and a tendency to local recurrence. Resection of the tumour and/or radiation were proposed as principal treatment. The aim of this retrospective study was to analyze the local control rates focusing on the effect of surgical margins and radiotherapy. METHODS: From 1981 to 2014, 44 patients had been treated. Fifty four therapies had been applied, in 50 cases surgery +/- radiation therapy, NSAIDs or chemotherapy...
January 5, 2018: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/29246931/periosteal-desmoid-tumour-a-rare-finding-in-the-oral-cavity
#18
Singh Shruti, Radhika M Bavle, Paremala Konda, Reshma Venugopal
A 55-year-old female patient reported with an intraoral well-localised asymptomatic swelling on the right side of the mandible in relation to the right mandibular first molar along with a history of trauma 6 months back. Panoramic radiograph revealed normal trabecular bone pattern in relation to the lesion. The lesion was excised along with the associated buccal cortical plate and tooth. The microscopic examination revealed a well-circumscribed lesion consisting of spindle cells arranged in storiform pattern associated with the buccal cortical plate...
December 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29228868/solitary-fibrous-tumors-in-pediatric-patients-a-rare-and-potentially-overdiagnosed-neoplasm-confirmed-by-stat6-immunohistochemistry
#19
Serena Y Tan, Linda J Szymanski, Carlos Galliani, David Parham, Eduardo Zambrano
Pathological diagnosis of solitary fibrous tumor (SFT) in the pediatric population is challenging, as it occurs uncommonly in this age-group and resembles other spindle cell neoplasms. SFT contains a NAB2-STAT6 fusion gene, which can be reliably detected using STAT6 immunohistochemistry. Positive staining is highly sensitive and specific. We sought to investigate the utility of STAT6 immunohistochemistry, to show how commonly SFT was historically recognized at 3 academic pediatric institutions, to reclassify them when appropriate, and to demonstrate features of major mimics of SFT...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29227350/tamoxifen-retinopathy-during-treatment-of-an-inoperable-desmoid-tumor
#20
Meredith Furst, Marie B Somogyi, Robert W Wong, Dejka Araujo, Clio A Harper
PURPOSE: To evaluate the clinical significance and rarity of tamoxifen retinopathy after a long-term tamoxifen treatment for an inoperable desmoid tumor. METHODS: Case report. RESULTS: Tamoxifen retinopathy is a condition rarely observed in clinical practice. Although tamoxifen is typically a treatment for breast cancer patients, we present a 68-year-old woman taking tamoxifen for an inoperable desmoid tumor, an equally rare condition. She presented with bilaterally deteriorating vision over the course of a year...
December 8, 2017: Retinal Cases & Brief Reports
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