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    Embolization of a large progressive symptomatic desmoid tumor in the rectus muscle of a female patient with multiple sclerosis: a case report.
    Acta Chir Belg 2017 Jun 22:1-4. Epub 2017 Jun 22.
    a Department of General and Abdominal Surgery , ZNA Middelheim , Antwerp , Belgium.
    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. Read More

    Fibroblastic connective tissue nevus: Clinicopathological and immunohistochemical study of 14 cases.
    J Cutan Pathol 2017 Jun 20. Epub 2017 Jun 20.
    Dermatopathology, Bodensee, Siemensstrasse 6/1, 88048, Friedrichshafen, Germany.
    Background: We present herein a series of 14 lesions showing overlapping features with the newly defined benign cutaneous mesenchymal neoplasm labelled as fibroblastic connective tissue nevus (FCTN).

    Methods And Results: Eight patients were male and five 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). Read More

    Malignant tumours of the foot and ankle.
    EFORT Open Rev 2017 May 11;2(5):261-271. Epub 2017 May 11.
    Laboratoire d'anatomie et cytologie pathologiques, Institut Universitaire du Cancer de Toulouse- Oncopole, 1 avenue Irène Joliot-Curie. 31059 Toulouse Cedex 9, France.
    Most of tumours of the foot are tumour-like (synovial cyst, foreign body reactions and epidermal inclusion cyst) or benign conditions (tenosynovial giant cells tumours, planta fibromatosis). Malignant tumours of the soft-tissue and skeleton are very rare in the foot and their diagnosis is often delayed with referral to specialised teams after initial inappropriate procedures or unplanned excisions. The adverse effect of these misdiagnosed tumours is the increasing rate of amputation or local recurrences in the involved patients. Read More

    β-catenin in Desmoid-Type Fibromatosis: deep insights on the role of T41A and S45F mutations on protein structure and gene expression.
    Mol Oncol 2017 Jun 19. Epub 2017 Jun 19.
    Laboratory of Experimental Molecular Pathology, Department of Pathology, Fondazione IRCCS Istituto Nazionale Tumori, G. Venezian 1, 20133, Milan, Italy.
    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. Read More

    Multi-parametric (mp) MRI for the diagnosis of abdominal wall desmoid tumors.
    Eur J Radiol 2017 Jul 17;92:103-110. Epub 2017 Apr 17.
    Department of Clinical Imaging, The Ottawa Hospital, Ottawa, Ontario, Canada. Electronic address:
    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. Read More

    Thermal diffusivity and perfusion constants from in vivo MR-guided focused ultrasound treatments: a feasibility study.
    Int J Hyperthermia 2017 Jun 8:1-39. Epub 2017 Jun 8.
    a Department of Radiology and Imaging Sciences , University of Utah , 729 Arapeen Drive , Salt Lake City , UT 84108.
    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. Read More

    Sporadic giant intra-abdominal desmoid tumor: A radiological case report.
    Mol Clin Oncol 2017 Jun 8;6(6):896-898. Epub 2017 May 8.
    School of Medicine, University of Zagreb, 10000 Zagreb, Croatia.
    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). Read More

    Desmoid Tumor of Rectus Abdominis Presenting with Grey-Turner's and Cullen's Sign: A Report of a Rare Case.
    Indian J Dermatol 2017 May-Jun;62(3):318-320
    Department of Dermatology, SVS Medical College, Mahbubnagar, Telangana, India.
    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. Read More

    Biology and Treatment of Aggressive Fibromatosis or Desmoid Tumor.
    Mayo Clin Proc 2017 Jun;92(6):947-964
    Department of Medicine, University of Minnesota Medical School, Minneapolis. Electronic address:
    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. Read More

    Comparison of sporadic and FAP-associated desmoid-type fibromatoses.
    J Surg Oncol 2017 Jun 1. Epub 2017 Jun 1.
    Department of Gastrointestinal Surgery, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.
    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. Read More

    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.
    Int J Surg Case Rep 2017 21;36:110-115. Epub 2017 May 21.
    University of São Paulo, São Paulo, Brazil.
    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. Read More

    An Intra-Abdominal Desmoid Tumor, Embedded in the Pancreas, Preoperatively Diagnosed as an Extragastric Growing Gastrointestinal Stromal Tumor.
    Case Rep Oncol 2017 Jan-Apr;10(1):301-307. Epub 2017 Apr 4.
    aDepartment of Gastroenterology, Tokai University School of Medicine, Isehara, Japan.
    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. Read More

    Soft-Tissue Sarcomas of the Abdomen and Pelvis: Radiologic-Pathologic Features, Part 2-Uncommon Sarcomas.
    Radiographics 2017 May-Jun;37(3):797-812
    From the Department of Radiology, Medstar Georgetown University Hospital, 3800 Reservoir Rd NW, Washington, DC 20007 (A.D.L., M.A.M.); the American Institute for Radiologic Pathology, Silver Spring, Md (M.A.M.); and the Center of Cancer Research, National Cancer Institute, Bethesda, Md (M.M.M.).
    Soft-tissue sarcomas occurring in the abdomen and pelvis are an uncommon but important group of malignancies. Recent changes to the World Health Organization classification of soft-tissue tumors include the movement of gastrointestinal stromal tumors (GISTs) into the soft-tissue tumor classification. GIST is the most common intraperitoneal sarcoma. Read More

    Adult desmoid tumors: biology, management and ongoing trials.
    Curr Opin Oncol 2017 Jul;29(4):268-274
    aDepartment of Medical Oncology, Centre Oscar Lambret, Lille bDepartment of Biopathology, Institut Bergonié, Comprehensive Cancer Centre, Institut National de la Santé et de la Recherche Medicale (INSERM) U916, Bordeaux cDivision of Adult Oncology, Department of Medicine, Aix-Marseille Université, INSERM U910 and Timone Hospital, Marseille, France.
    Purpose Of Review: To summarize the current knowledge about the biology and clinical management of adult desmoid tumors.

    Recent Findings: In the past decade, we have learned that desmoid tumors are driven by alterations of the Wnt/APC/β-catenin pathway, sporadic desmoid tumors are associated with somatic mutations of CTNNB1, and germline mutations of APC and somatic mutations of CTNNB1 are probably mutually exclusive. One-third of desmoid tumors are misdiagnosed; a second pathological opinion is therefore of major importance for desmoid tumor. Read More

    Desmoplastic fibroma versus soft-tissue desmoid tumour of forearm: a case series of diagnosis, surgical approach, and outcome.
    J Hand Surg Eur Vol 2017 Apr 1:1753193417705045. Epub 2017 Apr 1.
    Bone and Joint Reconstruction Research Center, Iran University of Medical Sciences, Tehran, Iran.
    We report a case series of aggressive fibromatosis of the forearm and discuss the differentiation criteria of overlapped intra- or extra-osseous origin of aggressive fibromatosis, surgical approach, and outcome. Ten cases of aggressive fibromatosis of the forearm were evaluated. When there was no involvement of vital adjacent structures, such as nerves, vessels, and tendons, en-bloc excision was performed for the removal of the soft-tissue mass. Read More

    Gradually shrinking intra-abdominal desmoid tumor derived from the stomach in a young boy: a case report.
    Surg Case Rep 2017 Dec 20;3(1):54. Epub 2017 Apr 20.
    Division of Surgical Oncology, Department of Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan.
    Background: Intra-abdominal desmoid tumors, particularly those derived from the stomach, are rare. Such tumors are associated with a history of familial adenomatous polyposis (FAP), trauma, or surgical procedures in general. In addition, spontaneous shrinking of an intra-abdominal desmoid tumor is rarer. Read More

    Human microRNA expression in sporadic and FAP-associated desmoid tumors and correlation with beta-catenin mutations.
    Oncotarget 2017 Mar 19. Epub 2017 Mar 19.
    General Surgery and Liver Transplantation Unit, Department of Emergency and Organ Transplantation (DETO), University of Bari "Aldo Moro", Bari, Italy.
    Desmoid tumors (DT) are rare, benign, fibroblastic neoplasm with challenging histological diagnosis. DTs can occur sporadically or associated with the familial adenomatous polyposis coli (FAP). Most sporadic DTs are associated with β-catenin gene (CTNNB1) mutations, while mutated APC gene causes FAP disease. Read More

    Desmoid-type fibromatosis in a boy with Down syndrome.
    Pediatr Int 2017 May 18;59(5):624-626. Epub 2017 Apr 18.
    Department of Pediatrics, Okayama University Hospital, Okayama, Japan.
    Patients with Down syndrome (DS) have a markedly higher incidence of childhood leukemia, but a lower incidence of most solid tumors, compared with age-matched euploid individuals. Trisomy 21 might be protective against tumorigenesis because of several tumor suppressive mechanisms. Desmoid-type fibromatosis (DF) is a rare monoclonal, fibroblastic proliferation characterized by a variable clinical course. Read More

    Desmoid Fibromatosis of the Abdominal Wall: Surgical Resection and Reconstruction with Biological Matrix Egis(®).
    Case Rep Oncol 2017 Jan-Apr;10(1):205-211. Epub 2017 Feb 17.
    Surgical Oncology Unit, Veneto Insitute of Oncology, IOV-IRCCS, Padua, Italy.
    Desmoid tumor is a rare monoclonal fibroblast proliferation that is regarded as benign. The clinical management of desmoid tumors is very complex and requires a multidisciplinary approach because of the unpredictable disease course. For those cases localized in the anterior abdominal wall, symptomatic and unresponsive to medical treatment, radical resection and reconstruction with a prosthetic device are indicated. Read More

    Long-term Efficacy of Methotrexate Plus Vinblastine/Vinorelbine in a Large Series of Patients Affected by Desmoid-Type Fibromatosis.
    Cancer J 2017 Mar/Apr;23(2):86-91
    From the Departments of *Medical Oncology, †Statistics, and ‡Surgery, IRCCS Fondazione Istituto Nazionale Tumori; §Department of Medical Oncology, Humanitas Cancer Center, IRCCS; and Departments of ∥Radiology and ¶Pathology, IRCCS Fondazione Istituto Nazionale Tumori, Milan, Italy.
    Purpose: Today, surgery and radiation therapy have a limited role in desmoid-type fibromatosis. Different systemic treatments were shown to be effective. Herein, we report on our institutional experience with low-dose methotrexate (MTX) + vinca alkaloids in this disease over the last 25 years. Read More

    Clinical Activity of the γ-Secretase Inhibitor PF-03084014 in Adults With Desmoid Tumors (Aggressive Fibromatosis).
    J Clin Oncol 2017 May 28;35(14):1561-1569. Epub 2017 Mar 28.
    Shivaani Kummar, Geraldine O'Sullivan Coyne, Khanh T. Do, Baris Turkbey, Paul S. Meltzer, Eric Polley, Peter L. Choyke, Robert Meehan, Yvonne Horneffer, Ann Lih, Amul Choudhary, Sandra A. Mitchell, Lee J. Helman, James H. Doroshow, and Alice P. Chen, National Cancer Institute, National Institutes of Health, Bethesda; and Rasa Vilimas and Lamin Juwara, Leidos Biomedical Research, Frederick National Laboratory for Cancer Research, Frederick, MD.
    Purpose Desmoid tumors (aggressive fibromatosis) arise from connective tissue cells or fibroblasts. In general, they are slow growing and do not metastasize; however, locally aggressive desmoid tumors can cause severe morbidity and loss of function. Disease recurrence after surgery and/or radiation and diagnosis of multifocal desmoid tumors highlight the need to develop effective systemic treatments for this disease. Read More

    Partial fasciectomy is a useful treatment option for symptomatic plantar fibromatosis.
    Foot (Edinb) 2017 Jun 21;31:31-34. Epub 2017 Feb 21.
    Royal Liverpool and Broadgreen University Hospitals NHS Trust, Prescot Street, Liverpool, L7 8XP, United Kingdom.
    Plantar fibromatosis is a rare, benign fibroproliferative disorder of the plantar fascia. It is considered as a low-grade tumour and it can be locally aggressive. It can present as painful swelling with impairment of local function. Read More

    Solid-Cystic Pancreatic Tail Desmoid Tumor with Beta-Catenin Positivity.
    ACG Case Rep J 2017 15;4:e40. Epub 2017 Mar 15.
    Department of Gastroenterology, Hepatology and Nutrition, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX; Ertan Digestive Disease Center, Memorial Hermann Hospital, Texas Medical Center, Houston, TX.

    Abdominal wall reconstruction after desmoid type fibromatosis radical resection: Case series from a single institution and review of the literature.
    Int J Surg Case Rep 2017 1;33:167-172. Epub 2017 Mar 1.
    Departament of Surgery, Division of Surgical Clinic III, Hospital das Clínicas, University of Sao Paulo School of Medicine, Sao Paulo, Brazil.
    Background: Abdominal wall desmoid type fibromatosis management has been changing over recent years, from an aggressive approach towards a more conservative one. When radical resection is indicated, the surgical team faces the challenge of abdominal wall reconstruction, for which optimal technique is still debated. The present study reports the experience from a single center with abdominal closures after desmoid type fibromatosis resection. Read More

    A Patient With Desmoid Tumors and Familial FAP Having Frame Shift Mutation of the APC Gene.
    Acta Med Iran 2017 Feb;55(2):134-138
    Department of Medical Genetics, Cancer Research Center, Cancer Institute of Iran, Tehran University of Medical Sciences, Tehran, Iran.
    Desmoids tumors, characterized by monoclonal proliferation of myofibroblasts, could occur in 5-10% of patients with familial adenomatous polyposis (FAP) as an extra-colonic manifestation of the disease. FAP can develop when there is a germ-line mutation in the adenomatous polyposis coli gene. Although mild or attenuated FAP may follow mutations in 5΄ extreme of the gene, it is more likely that 3΄ extreme mutations haveamore severe manifestation of thedisease. Read More

    Imatinib induces sustained progression arrest in RECIST progressive desmoid tumours: Final results of a phase II study of the German Interdisciplinary Sarcoma Group (GISG).
    Eur J Cancer 2017 May 8;76:60-67. Epub 2017 Mar 8.
    University of Heidelberg, Mannheim University Medical Center, Interdisciplinary Tumor Center Mannheim, Sarcoma Unit, Theodor-Kutzer-Ufer 1-3, D-68167 Mannheim, Germany; University of Heidelberg, Mannheim University Medical Center, Division of Surgical Oncology, Theodor-Kutzer-Ufer 1-3, D-68167 Mannheim, Germany.
    Background: Desmoid tumours describe a rare monoclonal, fibroblastic proliferation characterised by an often unpredictable clinical course. Surgery is one therapeutic option for progressing patients, except if mutilating and associated with considerable function loss. Different systemic treatment approaches have been investigated and promising results could be demonstrated using imatinib. Read More

    Functional and Aesthetic Thorax Reconstruction after Desmoid Tumor Resection.
    Plast Reconstr Surg Glob Open 2017 Feb 22;5(2):e1248. Epub 2017 Feb 22.
    Plastic and Reconstructive Surgery Service, Surgical Oncology Service, and Pathology Service, Hospital Angeles Puebla, Puebla 72190, México.
    This study describes a case report of a 31-year-old patient who presented with a left thoracic tumor on costal cartilages 5 and 6 that was diagnosed as a desmoid tumor 3 years after receiving retropectoral breast implants for cosmetic reasons. The integral reconstruction of the thoracic wall, functional and aesthetic, was planned for a single surgical period. The defect secondary to the tumor resection, which left the pericardium and lung exposed, was closed using the pectoral muscle as a "pre-expanded" flap by the breast implant, and the breast aesthetic was treated bilaterally with new implants in the retromammary position. Read More

    An Unusual Location of Fibrous Hamartoma of Infancy in the Eyelid.
    Ocul Oncol Pathol 2017 Jan 6;3(1):8-10. Epub 2016 Sep 6.
    Goldschleger Eye Institute, Tel Hashomer, Israel; Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
    Aim: To describe the clinical, imaging, and histopathological features of fibrous hamartoma of infancy (FHI) in the eyelid, a rare differential diagnosis for eyelid lesions.

    Methods: We describe the case of a 7-month-old patient with a FHI in the eyelid that was diagnosed after surgical removal. The unique histopathological appearance of the triphasic histologic components provided the diagnosis. Read More

    The treatment of desmoid tumors associated with familial adenomatous polyposis: the results of a Japanese multicenter observational study.
    Surg Today 2017 Mar 1. Epub 2017 Mar 1.
    Department of Surgical Oncology, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan.
    Purpose: Familial adenomatous polyposis (FAP)-associated desmoid tumor (DT) is sometimes life threatening. However, the optimal treatment for DTs has not been established. The aim of this study was to analyze the outcomes of surgical and pharmacological treatments for DT in Japanese FAP patients. Read More

    A Mesenteric Desmoid Tumor with Rapid Progression.
    Intern Med 2017 1;56(5):505-508. Epub 2017 Mar 1.
    Division of Gastroenterology and Hepatology, Niigata University Graduate School of Medical and Dental Sciences, Japan.
    We herein report the case of a rapidly progressive sporadic mesenteric desmoid tumor (DT). A 62-year-old woman presented with a 4-cm-diameter palpable mass in the left supraumbilical area. The mass showed an ill-defined margin with heterogeneous delayed enhancement on computed tomography and heterogeneous high intensity on T2-weighted magnetic resonance imaging. Read More

    Recurrent SRF-RELA Fusions Define a Novel Subset of Cellular Myofibroma/Myopericytoma: A Potential Diagnostic Pitfall With Sarcomas With Myogenic Differentiation.
    Am J Surg Pathol 2017 May;41(5):677-684
    *Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY †Department of Pathology, Brigham and Women Hospital, Boston, MA.
    Cellular myofibroblastic tumors other than desmoid-type fibromatosis are often diagnostically challenging due to their relative rarity, lack of known genetic abnormalities, and expression of muscle markers which may be confused with sarcomas with myogenic differentiation. In this study we investigate the molecular alterations of a group of cellular myofibroblastic lesions with in the myofibroma and myopericytoma spectrum for better subclassification. Two index cases were studied by paired-end RNA sequencing for potential fusion gene discovery. Read More

    Risk factors for local recurrence of fibromatosis.
    Rev Esp Cir Ortop Traumatol 2017 Mar - Apr;61(2):82-87. Epub 2017 Feb 21.
    Departamento de Cirugía Ortopédica y Traumatología, Clínica Universidad de Navarra, Pamplona, Navarra, España.
    Objective: To evaluate the clinical, radiological and histological factors that can predict local recurrence of fibromatosis.

    Methods: A retrospective study was conducted on 51 patients diagnosed with fibromatosis in this hospital from 1983 to 2014. The mean follow-up was 83 months. Read More

    Desmoid tumor following abdominally-based free flap breast reconstruction.
    Gland Surg 2017 Feb;6(1):89-92
    Division of Plastic Surgery, Department of Surgery, Mayo Clinic, Rochester, MN, USA.
    Desmoid tumors are fibroblastic connective tissue tumors that most commonly develop within the anterior abdominal wall. The etiology of desmoid tumors has not been well defined; however, hereditary, hormonal, traumatic, and surgery-related causes have been implicated. Desmoid tumors are believed to arise from musculoaponeurotic structures. Read More

    Sporadic desmoid tumors in the pediatric population: A single center experience and review of the literature.
    J Pediatr Surg 2017 Feb 4. Epub 2017 Feb 4.
    Department of Pediatric Hematology-Oncology, Schneider Children's Medical Center of Israel, Petach Tikva 4941492, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel. Electronic address:
    Background/purpose: We present our long experience with desmoid tumors in children.

    Methods: Data were retrospectively collected from 17 children/adolescents treated for sporadic desmoid tumors at a tertiary pediatric hospital in 1988-2016. There were 10 girls and 7 boys aged 1-17years. Read More

    Meta-analysis of the influence of surgical margin and adjuvant radiotherapy on local recurrence after resection of sporadic desmoid-type fibromatosis.
    Br J Surg 2017 Mar;104(4):347-357
    Departments of Surgical Oncology, Erasmus MC Cancer Institute, Rotterdam, The Netherlands.
    Background: Extra-abdominal desmoid-type fibromatosis (DF) is a rare, locally aggressive neoplasm that is usually managed conservatively. When treatment is indicated, it typically involves surgical resection, possibly with adjuvant radiotherapy. The indications for postoperative radiotherapy and its effectiveness are unclear. Read More

    MRgFUS for desmoid tumors within the thigh: early clinical experiences.
    J Ther Ultrasound 2017 3;5. Epub 2017 Feb 3.
    Department of Radiology and Biomedical Imaging, University of California San Francisco, 185 Berry Street, Suite 350, San Francisco, CA 94107-5705 USA.
    Background: Desmoid tumors are benign but locally aggressive non-malignant tumors derived from fibroblasts. Surgery, chemotherapy, and radiation therapy have been the mainstay of treatment, but recurrence is common and side effects can result in significant morbidity. In this case series, we highlight our experiences performing treatments in the thigh, including strategies for optimizing ablation size and safety. Read More

    Endoscopic Subtotal Fasciectomy of the Foot.
    Arthrosc Tech 2016 Dec 5;5(6):e1387-e1393. Epub 2016 Dec 5.
    Department of Orthopaedics and Traumatology, North District Hospital, Hong Kong SAR, China.
    Plantar fibromatosis is a rare benign but often locally aggressive tumor of the plantar aponeurosis. Nonsurgical treatment is the first line of treatment for symptomatic lesions. Because of the high recurrence rate associated with surgical treatment, operation is indicated only when the lesions are highly symptomatic and conservative measures fail or the diagnosis is in question. Read More

    [A Case of Familial Adenomatous Polyposis with a Desmoid Tumor Probably Communicating to the Intestinal Lumen That Was Successfully Treated with Non-Surgical Therapy].
    Gan To Kagaku Ryoho 2016 Nov;43(12):2316-2319
    Dept. of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University.
    A 44-year-old man with familial adenomatous polyposis underwent laparoscopic-assistedtotal proctocolectomy with ilealpouch anal anastomosis(IPAA). Computed tomography conducted 21 months after IPAA demonstrated bilateral hydronephrosis andan intra-abdominal mass with a maximal diameter of 22 cm, leading to a diagnosis of stage IV desmoid disease, according to the classification by Church and associates. Six courses of combination chemotherapy with doxorubicin plus dacarbazine were administered. Read More

    Pazopanib, a promising option for the treatment of aggressive fibromatosis.
    Anticancer Drugs 2017 Apr;28(4):421-426
    aSarcoma Unit, The Royal Marsden Hospital NHS Foundation Trust, London bDepartment of Oncology, Cambridge University Hospitals NHS Foundation Trust, Cambridge Biomedical Campus, Cambridge cThe Institute of Cancer Research, Sutton, UK.
    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. Read More

    TALENs and CRISPR/Cas9 fuel genetically engineered clinically relevant Xenopus tropicalis tumor models.
    Genesis 2017 Jan;55(1-2)
    Developmental Biology Unit, Department of Biomedical Molecular Biology, Ghent University, Belgium.
    The targeted nuclease revolution (TALENs, CRISPR/Cas9) now allows Xenopus researchers to rapidly generate custom on-demand genetic knockout models. These novel methods to perform reverse genetics are unprecedented and are fueling a wide array of human disease models within the aquatic diploid model organism Xenopus tropicalis (X. tropicalis). Read More

    Radiotherapy in desmoid tumors : Treatment response, local control, and analysis of local failures.
    Strahlenther Onkol 2017 Apr 2;193(4):269-275. Epub 2017 Jan 2.
    Comprehensive Cancer Center, Helsinki University Hospital and University of Helsinki, PO Box 180, 00029 HUS, Helsinki, Finland.
    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. Read More

    Comparison of β-Catenin and LEF1 Immunohistochemical Stains in Desmoid-type Fibromatosis and its Selected Mimickers, With Unexpected Finding of LEF1 Positivity in Scars.
    Appl Immunohistochem Mol Morphol 2016 Dec 23. Epub 2016 Dec 23.
    Departments of *Department of Pathology †Department of Colorectal Surgery, Cleveland Clinic, Cleveland, OH.
    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. Read More

    Fibromatosis Associated With Neuromuscular Choristoma: Evaluation by FDG PET/CT.
    Clin Nucl Med 2017 Mar;42(3):e168-e170
    From the Departments of *Radiology, †Neurosurgery, and ‡Orthopedic Surgery, Mayo Clinic, Rochester, MN.
    Neuromuscular choristoma (NMC) is a rare benign peripheral nerve lesion consisting of mature skeletal muscle fibers admixed with nerve fascicles. Aggressive fibromatosis frequently develops in association with NMC, often after surgery or biopsy, but the exact pathogenesis is not known. We present a case of NMC complicated by aggressive fibromatosis evaluated by F-FDG PET/CT and examine the relationship of metabolic activity and MRI signal characteristics. Read More

    Desmoid Fibromatosis Presenting as Deep Venous Thrombosis: A Case Report and Discussion.
    Am J Case Rep 2016 Dec 20;17:967-972. Epub 2016 Dec 20.
    Department of Surgery, University of Adelaide, Adelaide, SA, Australia.
    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. Read More

    Breast Desmoid Tumor after Ductal Carcinoma Treatment: Salvaging a DIEP Flap Reconstruction.
    Plast Reconstr Surg Glob Open 2016 Nov 23;4(11):e1142. Epub 2016 Nov 23.
    Institute for Reconstructive Surgery, Houston Methodist Hospital, Weill Cornell Medicine, Houston, Tex.; and College of Medicine, Texas A&M University College of Medicine, Houston, Tex.
    Supplemental Digital Content is available in the text. Read More

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