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    1 OF 52

    Aggressive fibromatosis of the oral cavity in a 5 year old boy: a rare case report.
    Pan Afr Med J 2017 18;27:47. Epub 2017 May 18.
    Department of Oral Medicine and Radiology Bapuji Dental College and Hospital MCC B Block, Davangere, Karnataka, India.
    Fibrous tissue proliferations express a wide spectrum of histologic and morphologic variation in both infants and adults. This ranges from hypertrophic scar formation at one end to malignant fibrosarcoma at the other end of the spectrum. Aggressive fibromatosis is an intermediate tumor which is in proximity to fibrosarcomas. Read More

    Management of desmoid tumours: A large national database of familial adenomatous patients shows a link to colectomy modalities and low efficacy of medical treatments.
    United European Gastroenterol J 2017 Aug 28;5(5):735-741. Epub 2016 Oct 28.
    Gastroenterology Department, Edouard Herriot Hospital, Lyon, France.
    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. Read More

    Giant pancreatic solid cystic desmoid tumor with two ectopic adrenal tissues.
    Turk J Gastroenterol 2017 Aug 9. Epub 2017 Aug 9.
    Department of Pathology, İzmir Katip Çelebi University Atatürk Training and Research Hospital, İzmir, Turkey.
    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. Read More

    Desmoid-type fibromatosis of the breast: A report of 2 cases.
    Oncol Lett 2017 Aug 7;14(2):1433-1438. Epub 2017 Jun 7.
    Second Department of Gynecology and Obstetrics, Comenius University of Bratislava, Faculty of Medicine, 82606 Bratislava, Slovakia.
    Mammary fibromatosis is a rare and locally aggressive benign tumor of the breast; it originates from fibroblasts and myofibroblasts within the breast parenchyma and does not metastasize. The condition is locally aggressive and has a high rate of recurrence. The etiology of mammary fibromatosis is unknown. Read More

    Concurrent Imatinib and Radiation Therapy for Unresectable and Symptomatic Desmoid Tumors.
    Sarcoma 2017 5;2017:2316839. Epub 2017 Jul 5.
    Department of Medicine, Division of Oncology, Stanford University School of Medicine, Stanford, CA, USA.
    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. Read More

    Myofibroblastic, fibroblastic and myoid lesions of the breast.
    Semin Diagn Pathol 2017 May 28. Epub 2017 May 28.
    Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY, USA.
    Myofibroblastic, fibroblastic and/or myoid lesions are rare in the breast but comprise the majority of mammary mesenchymal spindle cell lesions. Whereas most have similar features to their counterparts at extramammary sites, pseudoangiomatous stromal hyperplasia is considered a breast-specific myofibroblastic proliferation on the same spectrum as myofibroblastoma. Other lesions with myofibroblastic/fibroblastic differentiation include fibromatosis and nodular fasciitis, as well as more aggressive tumors such as the rarely reported myofibrosarcoma, inflammatory myofibroblastic tumor and fibrosarcoma. Read More

    Cesarean section after abdominal mesh repair for pregnancy-related desmoid tumor: a case report.
    Int J Womens Health 2017 12;9:497-499. Epub 2017 Jul 12.
    Obstetrics and Gynaecology Department, Liverpool Hospital, Liverpool, NSW, Australia.
    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. Read More

    Gastroblastoma harbors a recurrent somatic MALAT1-GLI1 fusion gene.
    Mod Pathol 2017 Jul 21. Epub 2017 Jul 21.
    Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
    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. Read More

    Late toxicity and outcomes following radiation therapy for chest wall sarcomas in pediatric patients.
    Pract Radiat Oncol 2017 Apr 26. Epub 2017 Apr 26.
    Department of Radiation Oncology, St. Jude Children's Research Hospital Memphis, Tennessee.
    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). Cobb angles were used to quantify the extent of scoliosis. Read More

    Mesenteric desmoid tumour presenting with recurrent abdominal abscess and duodenal fistula: A case report and review of literature.
    Int J Surg Case Rep 2017 16;37:119-123. Epub 2017 Jun 16.
    Department of Surgery, Jackson Memorial Hospital/University of Miami Miller School of Medicine, Miami, USA.
    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. Read More

    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.
    Dermopathologie Bodensee, Friedrichshafen, Germany.
    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). 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

    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 Jun;8(26):41866-41875
    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

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