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    Desmoid tumor of the abdominal wall A case report and literature review.
    Ann Ital Chir 2017 Nov 20;6. Epub 2017 Nov 20.
    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. Read More

    Differential Diagnosis of Benign Spindle Cell Lesions.
    Surg Pathol Clin 2018 Mar 9;11(1):91-121. Epub 2017 Dec 9.
    Department of Medical and Surgical Sciences and Advanced Technologies, G.F. Ingrassia, Anatomic Pathology, University of Catania, Via S. Sofia 87, Catania 95123, Italy. Electronic address:
    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. Read More

    Complete resection of a rectus abdominis muscle invaded by desmoid tumors and subsequent management with an abdominal binder: a case report.
    J Med Case Rep 2018 Feb 7;12(1):29. Epub 2018 Feb 7.
    Intensive Care Unit, Kochi Health Sciences Center, Ike 2125-1, Kochi City, Kochi Prefecture, Japan.
    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. Read More

    Recurrent Enlarging Mesenteric Desmoid Tumor following Remote Surgical Resection.
    Case Rep Radiol 2017 18;2017:2312617. Epub 2017 Dec 18.
    Faculty of Medicine, Memorial University, St. John's, NL, Canada.
    Intra-abdominal desmoid tumors are commonly associated with genetic syndromes such as familial polyposis coli. Radiological cross imaging studies such as CT and MRI are used in the preoperative work-up of such tumors. Postoperatively, CT and MRI are useful in the assessment of recurrent desmoid tumors. Read More

    The evaluation of circulating endothelial progenitor cells and related angiogenic markers as prognostic factors in soft-tissue tumors.
    Eur J Surg Oncol 2018 Jan 31. Epub 2018 Jan 31.
    Department of Surgery, Mannheim University Medical Center, University of Heidelberg, Germany. Electronic address:
    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. Read More

    Pazopanib therapy for desmoid tumors in adolescent and young adult patients.
    Pediatr Blood Cancer 2018 Jan 31. Epub 2018 Jan 31.
    Division of Oncology, Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
    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. Read More

    Impact of Pathologist Involvement in Sarcoma and Rare Tumor Patient Support Groups on Facebook: A Survey of 542 Patients and Family Members.
    Arch Pathol Lab Med 2018 Jan 29. Epub 2018 Jan 29.
    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. Read More

    Chest wall - a structure underestimated in ultrasonography. Part III: Neoplastic lesions.
    J Ultrason 2017 Dec 29;17(71):281-288. Epub 2017 Dec 29.
    Self-education Sonography Group, Genetics Division, Pomeranian Medical University, Szczecin, Poland.
    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. Read More

    Screening-detected desmoid tumor of the breast: findings at conventional imaging and digital breast tomosynthesis.
    Acta Radiol Open 2018 Jan 19;7(1):2058460117752034. Epub 2018 Jan 19.
    Department of Radiology, Oslo University Hospital, University of Oslo, Oslo, Norway.
    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. Read More

    APC mosaicism in a young woman with desmoid type fibromatosis and familial adenomatous polyposis.
    Fam Cancer 2018 Jan 24. Epub 2018 Jan 24.
    Section of Inherited Cancer, Department of Medical Genetics, Oslo University Hospital, Oslo, Norway.
    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. Read More

    A Metabolomics Pilot Study on Desmoid Tumors and Novel Drug Candidates.
    Sci Rep 2018 Jan 12;8(1):584. Epub 2018 Jan 12.
    Developmental & Stem Cell Biology Program, Hospital for Sick Children, Toronto, ON, Canada.
    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). Read More

    [Excision of giant desmoid in the abdominal wall, method of abdominal wall reconstruction, and follow-up of long-termed effect].
    Zhonghua Wai Ke Za Zhi 2018 Jan;56(1):52-55
    Diagnostic and Therapeutic Center of Hernia and Abdominal Wall Diseases, Department of General Surgery, First Affiliated Hospital of People's Liberation Army General Hospital, Beijing 100048, China.
    To explore the ideal procedure of excision and repair for giant desmoid in the abdominal wall and long-termed follow-up results.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. Twenty-one female patients with the mean age of 34. Read More

    [Benign mesenchymal tumors of the abdomen].
    Radiologe 2018 Jan;58(1):25-35
    FE MRT, Radiologische Universitätsklinik Bonn, Sigmund-Freud-Straße 25, 53127, Bonn, Deutschland.
    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. Read More

    Systemic therapy of aggressive fibromatosis in children and adolescents: Report of the Cooperative Weichteilsarkom Studiengruppe (CWS).
    Pediatr Blood Cancer 2018 Jan 5. Epub 2018 Jan 5.
    Stuttgart Cancer Center, Zentrum für Kinder-, Jugend- und Frauenmedizin, Pediatrics 5 (Oncology, Hematology, and Immunology), Klinikum Stuttgart-Olgahospital, Stuttgart, Germany.
    Background: Treatment algorithms for patients with aggressive fibromatosis (AF) are challenging. There are limited data available about the use of systemic therapy (ST) in pediatric patients with AF.

    Methods: Patient-, tumor-, and treatment-related factors of 90 children and adolescents with AF treated on multiple prospective trials of the Cooperative Weichteilsarkom Studiengruppe (1981-2015) were analyzed with focus on response and outcome of ST. Read More

    Plantar Fibromatosis: Pathophysiology, Surgical and Nonsurgical Therapies: An Evidence-Based Review.
    Foot Ankle Spec 2018 Jan 1:1938640017751184. Epub 2018 Jan 1.
    Division of Podiatric Surgery, Center for Wound Healing, MedStar Georgetown University Hospital, Washington, DC (PC).
    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. Read More

    Desmoid Tumours of the extremity and trunk. A retrospective study of 44 patients.
    BMC Musculoskelet Disord 2018 01 5;19(1). Epub 2018 Jan 5.
    Musculoskeletal Oncology, Department of Orthopaedic Surgery, Physical Medicine and Rehabilitation, Campus Grosshadern, University Hospital, LMU Munich, Marchioninistr. 15, 81377, Munich, Germany.
    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. Read More

    Diagnosis and Differential Diagnosis of Desmoplastic Fibroblastoma by Clinical, Radiological, and Histopathological Analyses.
    Chin Med J (Engl) 2018 Jan;131(1):32-36
    Department of Pathology, Beijing Jishuitan Hospital, The Fourth Medical College of Peking University, Beijing 100035, China.
    Background: Desmoplastic fibroblastoma (collagenous fibroma) is an uncommon benign soft-tissue tumor, rarely involving bone. It shares some overlapping features with other infiltrate tumors, such as desmoid-type fibromatosis, neurofibroma, and low-grade fibromyxoid sarcoma. The misdiagnosis may cause unnecessary surgical overtreatment, especially for those involving bone. Read More

    Reconstruction of a large full-thickness abdominal wall defect with flow-through-based alt flaps: A case report.
    Microsurgery 2017 Dec 12. Epub 2017 Dec 12.
    Department of Plastic and Reconstructive Surgery, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
    An extensive 35 × 20 cm sized full-thickness abdominal wall defect was created after resection of aggressive abdominal fibromatosis in a 19-year-old male patient. Immediate reconstruction was not possible due to prolonged operation time and resulting severe bowel edema. A silicone sheet with NPWT was applied over the exposed viscera. Read More

    Solitary Fibrous Tumors in Pediatric Patients: A Rare and Potentially Overdiagnosed Neoplasm, Confirmed by STAT6 Immunohistochemistry.
    Pediatr Dev Pathol 2017 Jan 1:1093526617745431. Epub 2017 Jan 1.
    1 Department of Pathology, Stanford University Medical Center, Stanford, California.
    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. Read More

    TAMOXIFEN RETINOPATHY DURING TREATMENT OF AN INOPERABLE DESMOID TUMOR.
    Retin Cases Brief Rep 2017 Dec 8. Epub 2017 Dec 8.
    *School of Medicine, University of Texas Health at San Antonio, San Antonio, Texas;†TOC Eye and Face, Austin, Texas;‡Austin Retina Associates, Austin, Texas;§Department of Surgery and Perioperative Services, Dell Medical School, University of Texas at Austin, Austin, Texas; and¶Department of Sarcoma Medical Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas.
    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. Read More

    Locally Aggressive Connective Tissue Tumors.
    J Clin Oncol 2018 Jan 8;36(2):202-209. Epub 2017 Dec 8.
    Mrinal M. Gounder and William D. Tap, Memorial Sloan Kettering Cancer Center and Weil Cornell Medical School, New York, NY; and David M. Thomas, Garvan Institute of Medical Research, Darlinghurst, Australia.
    In this review, we highlight the complexities of the natural history, biology, and clinical management of three intermediate connective tissue tumors: desmoid tumor (DT) or aggressive fibromatosis, tenosynovial giant cell tumor (TGCT) or diffuse-type pigmented villonodular synovitis (dtPVNS), and giant cell tumor of bone (GCTB). Intermediate histologies include tumors of both soft tissue and bone origin and are locally aggressive and rarely metastatic. Some common aspects to these tumors are that they can be locally infiltrative and/or impinge on critical organs, which leads to disfigurement, pain, loss of function and mobility, neurovascular compromise, and occasionally life-threatening consequences, such as mesenteric, bowel, ureteral, and/or bladder obstruction. Read More

    Phase II study of doxorubicin and thalidomide in patients with refractory aggressive fibromatosis.
    Invest New Drugs 2018 Feb 23;36(1):114-120. Epub 2017 Nov 23.
    Department of Medical Oncology, Fudan University Shanghai Cancer Center, 270 Dong-An Road, Shanghai, 200032, China.
    Background To evaluate the efficacy and safety of doxorubicin (ADM) combined with thalidomide (THA) as a first-line treatment for patients with refractory aggressive fibromatosis (AF). Patients and Methods Eligible patients were treated with ADM 30 mg/mon days 1-2 and THA 200 mg nightly on days 1-21 every 3 weeks for a maximum of six cycles. THA was then continued for a total of 1 year. Read More

    Efficacy of vinorelbine combined with low-dose methotrexate for treatment of inoperable desmoid tumor and prognostic factor analysis.
    Chin J Cancer Res 2017 Oct;29(5):455-462
    Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Bone and Soft Tissue Tumors, Peking University Cancer Hospital & Institute, Beijing 100142, China.
    Objective: To assess the efficacy of conservative chemotherapy for inoperable desmoid tumor (DT) and analyze the prognostic factors.

    Methods: From November 2008 to April 2016, 71 patients of inoperable DT were treated with vinorelbine and low-dose methotrexate in the Department of Bone and Soft Tissue Tumors, Peking University Cancer Hospital & Institute, and enrolled in this retrospective study. The chemotherapy duration is one year. Read More

    Effect of surgical margins on prognosis in aggressive fibromatosis: A single-institutional analysis of 90 patients.
    Oncol Lett 2017 Nov 1;14(5):5129-5134. Epub 2017 Sep 1.
    Department of Plastic Surgery, BG-University Hospital Bergmannsheil, D-44789 Bochum, Germany.
    The treatment of aggressive fibromatosis poses a therapeutic challenge in an interdisciplinary setting. The extent of surgical resection is still discussed controversially. The present retrospective analysis aimed to determine prognostic factors leading to recurrence. Read More

    [Resection of a Chest Wall Desmoid Tumor with Chest Wall Reconstruction;Report of a Case].
    Kyobu Geka 2017 Nov;70(12):1041-1043
    Department of Thoracic Surgery, Tokyo Metropolitan Cancer and Infectious Diseases Center, Komagome Hospital, Tokyo, Japan.
    A primary desmoid tumor arising from the chest wall is extremely rare. We report the case of a 57-year-old man presenting with a desmoid tumor arising from his chest wall. Chest radiograph at a regular medical checkup indicated an abnormal shadow. Read More

    Safety and efficacy of intralesional steroid injection for aggressive fibromatosis.
    World J Surg Oncol 2017 Nov 2;15(1):195. Epub 2017 Nov 2.
    Bangkok Hospital Chiang Mai, Chiang Mai, Thailand.
    Background: Treatment of recurrent aggressive fibromatosis (AF) following surgical resection is a clinical challenge. Non-steroidal anti-inflammatory drugs (NSAIDs) have been reported to be an effective option for controlling the disease. However, long-term NSAID use can result in unfavorable complications. Read More

    Sonography used in the infantile desmoid fibromatosis of postcricoid area: A case report.
    Medicine (Baltimore) 2017 Nov;96(44):e8500
    aDepartment of Diagnostic Ultrasound, Beijing Tongren Hospital, Capital Medical University bDepartment of Pathology, Beijing Tongren Hospital, Capital Medical University cDepartment of Otorhinolaryngology Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University dDepartment of Diagnostic Ultrasound, Beijing Aerospace General Hospital, Beijing, China.
    Rationale: Infantile desmoid fibromatosis of the postcricoid area is a rare disease and is characterized by a proliferation of fibrous tissue with non-metastasis, local infiltration, and a high rate of recurrence after surgical resection. Currently, ultrasound is scarcely used in the hypopharynx and larynx area.

    Patient Concerns: A 4-year-old boy presented with hoarseness, deep voice and snoring for 2∼4 years without any surgical history. Read More

    Late Presentation of Aggressive Fibromatosis Involving Head, Neck and Chest Wall.
    J Coll Physicians Surg Pak 2017 Oct;27(10):654-656
    Department of Surgery, College of Medicine, King Saud University, Riyadh, Kingdom of Saudi Arabia.
    Fibromatosis is a rare, benign, slow-growing and locally infiltrative tumour, caused by uncontrolled proliferation of fibrous tissue arising from muscles, connective tissue, fasciae and aponeurosis. It is also called desmoid tumour, which is very rare pathology representing only 0.03% of all neoplasms in human and around 3% of all soft tissue tumours. Read More

    Laparoscopic Resection of a Jejunal Mesenteric Pseudocyst.
    Case Rep Gastroenterol 2017 Sep-Dec;11(3):526-530. Epub 2017 Sep 13.
    Department of Gastroenterological Surgery II, Hokkaido University Hospital, Sapporo, Japan.
    An unusual case of a jejunal mesenteric pseudocyst treated by laparoscopic resection is reported. A 44-year-old woman was admitted to our hospital with intermittent upper abdominal pain and diarrhea. Physical examination revealed slight periumbilical tenderness, and no masses were palpable. Read More

    Desmoid type fibromatosis: A case report with an unusual etiology.
    World J Gastrointest Oncol 2017 Sep;9(9):385-389
    Internal Medicine Department of Research Medical Center and Research Department of Kansas City Veterans Affairs Medical Center, Kansas City, MO 64132, United States.
    Desmoid type fibromatosis (DTF) is a rare, locally invasive, non-metastasizing soft tissue tumor. We report an interesting case of DTF involving the pancreatic head of a 54-year-old woman. She presented with intermittent dysphagia and significant weight loss within a 3-mo period. Read More

    An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG).
    Ann Oncol 2017 Oct;28(10):2399-2408
    Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
    Desmoid-type fibromatosis is a rare and locally aggressive monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Currently, there is no established or evidence-based treatment approach available for this disease. Therefore, in 2015 the European Desmoid Working Group published a position paper giving recommendations on the treatment of this intriguing disease. Read More

    A First-in-Human Phase I Study of the Anticancer Stem Cell Agent Ipafricept (OMP-54F28), a Decoy Receptor for Wnt Ligands, in Patients with Advanced Solid Tumors.
    Clin Cancer Res 2017 Dec 27;23(24):7490-7497. Epub 2017 Sep 27.
    University of Michigan, Ann Arbor, Michigan.
    Wnt signaling is implicated in tumor cell dedifferentiation and cancer stem cell function. Ipafricept (OMP-54F28) is a first-in-class recombinant fusion protein with the extracellular part of human frizzled 8 receptor fused to a human IgG1 Fc fragment that binds Wnt ligands. This trial evaluated ipafricept in patients with solid tumors. Read More

    Effects of ICOS+ T cell depletion via afucosylated monoclonal antibody MEDI-570 on pregnant cynomolgus monkeys and the developing offspring.
    Reprod Toxicol 2017 Dec 13;74:116-133. Epub 2017 Sep 13.
    Toxicology, Research & Development, MedImmune Inc., One MedImmune Way, Gaithersburg, MD 20854, United States.
    MEDI-570 is a fully human afucosylated monoclonal antibody (MAb) against Inducible T-cell costimulator (ICOS), highly expressed on CD4+ T follicular helper (T) cells. Effects of MEDI-570 were evaluated in an enhanced pre-postnatal development toxicity (ePPND) study in cynomolgus monkeys. Administration to pregnant monkeys did not cause any abortifacient effects. Read More

    [Latissimus dorsi flap in reconstruction following treatment of giant tumor of the abdominal wall: about a rare case].
    Pan Afr Med J 2017 6;27:181. Epub 2017 Jul 6.
    Service de Chirurgie Plastique, Hôpital Avicenne, Rabat, Maroc.
    We report the case of a 16-year old patient presenting with giant, multinodular, mesenchymal tumor of the abdominal wall occupying the left abdominal region and measuring 25 cm on the vertical axis, 20 cm on the transverse axis, mobile when compared with the deep structures and gradually increasing in volume over childhood and neglected. After small biopsy, which showed desmoid tumor, the patient underwent complete surgical resection of the tumor with immediate reconstruction by free muscolo skin flap of the latissimus dorsi attached to the large blood vessels of the inguinal fold (left iliac artery and left external iliac vein), connected by termino lateral anastomosis. Flap survival was correctly performed and reconstruction was successful. Read More

    Role of Histone H3K27 Trimethylation Loss as a Marker for Malignant Peripheral Nerve Sheath Tumor in Fine-Needle Aspiration and Small Biopsy Specimens.
    Am J Clin Pathol 2017 Aug;148(2):179-189
    Department of Pathology, Brigham and Women's Hospital, Boston, MA.
    Objectives: Accurate diagnosis of malignant peripheral nerve sheath tumor (MPNST) is often challenging on fine-needle aspiration (FNA) or core needle biopsy. Recurrent mutations in EED and SUZ12, which encode subunits of polycomb repressive complex 2 (PRC2), have been identified in 70% to 92% of MPNSTs; PRC2 inactivation leads to loss of trimethylation of lysine 27 of histone H3 (H3K27me3). We evaluated the utility of H3K27me3 immunohistochemistry for distinguishing MPNST from its cytomorphologic mimics. Read More

    Outcomes and complications of radiation therapy in patients with familial adenomatous polyposis.
    J Gastrointest Oncol 2017 Aug;8(4):643-649
    Department of Radiation Oncology, Huntsman Cancer Hospital, University of Utah; Salt Lake City, UT, USA.
    Background: The outcomes, complications, and rates of secondary malignancies from radiation therapy (RT) are not known for patients with familial adenomatous polyposis (FAP).

    Methods: We queried the Hereditary Gastrointestinal Cancer Registry (HGCR) for patients with FAP who received RT. Outcomes assessed included acute and late treatment toxicity and secondary malignancies. Read More

    Long-Term Follow-Up of Desmoid Fibromatosis Treated with PF-03084014, an Oral Gamma Secretase Inhibitor.
    Ann Surg Oncol 2018 Mar 8;25(3):768-775. Epub 2017 Sep 8.
    Division of Medical Oncology, University of Colorado Denver School of Medicine - Anschutz Medical Campus, Aurora, CO, USA.
    Background: Desmoid fibromatosis is a fibroblastic neoplasm driven by aberrations within the WNT pathway, exhibiting mutations in β-catenin or APC. We review the long-term follow-up of patients in a phase I study treated with an oral gamma secretase inhibitor, PF-03084014.

    Methods: PF-03084014 was administered orally at doses ranging from 20 to 330 mg twice daily. Read More

    Rarity among benign gastric tumors: Plexiform fibromyxoma - Report of two cases.
    World J Gastroenterol 2017 Aug;23(31):5817-5822
    Institute of Pathology, Medical University of Graz, 8036 Graz, Austria.
    Plexiform fibromyxoma is a very rare mesenchymal tumor of the stomach, found almost exclusively in the antrum/pylorus region. The most common presenting symptoms are anemia, hematemesis, nausea and unintentional weight loss, without sex or age predilection. We describe here two cases of plexiform fibromyxoma, involving a 16-year-old female and a 34-year-old male. Read More

    Effective treatment of aggressive fibromatosis with celecoxib guided by genetic testing.
    Cancer Biol Ther 2017 Oct 7;18(10):757-760. Epub 2017 Sep 7.
    a Department of Oncology , The Affiliated Hospital of Qingdao University , Qingdao , Shandong , China.
    Aggressive fibromatosis (AF) or desmoid tumors is an aggressive fibroblastic proliferation which is locally invasive but can not metastasize. The treatment of AF is challenging. Surgery was the main treatment modality for AF in the past, other strategies including radiotherapy, systemic therapies and wait-and-see policy. Read More

    Diaphragm reconstruction combined with thoraco-abdominal wall reconstruction after tumor resection.
    J Plast Surg Hand Surg 2017 Aug 31:1-6. Epub 2017 Aug 31.
    a Department of Plastic Surgery , Helsinki University Hospital , Helsinki , Finland.
    Background: Thoraco-abdominal wall resection including diaphragm resection results in a challenging surgical defect. Various methods have been used for diaphragm reconstruction. The aim of this study was to describe our methods of diaphragm and thoraco-abdominal wall reconstruction after combined resection of these anatomical structures. Read More

    Expanding the borders: Image-guided procedures for the treatment of musculoskeletal tumors.
    Diagn Interv Imaging 2017 Sep 23;98(9):635-644. Epub 2017 Aug 23.
    Interventional Imaging, Nouvel hopital civil, 1, place de l'Hôpital, 67098 Strasbourg cedex, France. Electronic address:
    Interventional radiology has revolutionized the local management of bone tumours, offering new minimal invasive alternatives to surgery and radiotherapy. Its role has continuously increased over the past years for the treatment of benign tumors and also of some malignant ones, especially in palliative situations. The development of protective techniques and new ablative technologies, such as cryoablation, contributes to expand the role of the interventional radiologist to new fields of applications in musculoskeletal oncology. Read More

    Desmoid Tumor and Silicone Breast Implant Surgery: Is There Really a Connection? A Literature Review.
    Aesthetic Plast Surg 2018 Feb 25;42(1):59-63. Epub 2017 Aug 25.
    Plastic and Reconstructive Surgery Department, Soroka University Medical Center, Ben Gurion University of Negev, Beersheba, Israel.
    Background: Desmoid tumors are borderline tumors of the connective tissue, arising in the musculo-aponeurotic stromal elements. A desmoid tumor (DT) has an infiltrative and locally aggressive growth pattern and usually does not metastasize; however, it has a high recurrence and complication rate. DT located in the breast (BDT) represents a rare extra-abdominal form. Read More

    Radiologic images of an aggressive implant-associated fibromatosis of the breast and chest wall: case report and review of the literature.
    Radiol Case Rep 2017 Sep 28;12(3):431-438. Epub 2017 Jun 28.
    Department of Radiology, Cooper University Hospital, 1 Cooper Plaza, Suite B23, Camden, NJ 08103, USA.
    Fibromatosis of the breast is a rare benign disease compromising <0.2% of all primary breast tumors. Although the chest wall is a common location, occurrences of implant-associated fibromatosis of the breast are extremely rare; only 33 cases have been reported. Read More

    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

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