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    2635 results match your criteria Desmoid Tumor

    1 OF 53

    Systematic Review of Clinical Outcomes Following Various Treatment Options for Patients with Extraabdominal Desmoid Tumors.
    Ann Surg Oncol 2018 Apr 11. Epub 2018 Apr 11.
    Peter MacCallum Cancer Centre, Melbourne, VIC, Australia.
    Background: Desmoid tumors (DT) are rare clonal proliferations that arise from mesenchymal cells. These tumors do not metastasize but are locally aggressive, and their growth may lead to significant morbidity. Their clinical course is both variable and unpredictable; tumors may rapidly progress but in other instances remain stable or regress without intervention. Read More

    Desmoid Fibromatosis of the Lower Abdominal Wall in Irrua Nigeria.
    Niger J Surg 2018 Jan-Jun;24(1):52-55
    Department of Radiology, Irrua Specialist Teaching Hospital, Ekpoma, Edo State, Nigeria.
    Desmoid fibromatosis (desmoid tumors) is rare tumors. It can occur as intra-abdominal, extraabdominal, or abdominal wall tumor depending on the site. The abdominal wall type is usually sporadic, but few have been associated with familial adenomatous polyposis. Read More

    Fatal Outcome of Recurrent Infantile Pelvic Desmoid Tumor Treated with Tamoxifene.
    Gulf J Oncolog 2018 Jan;1(26):75-78
    Department of Medical Oncology, Hassan II University Hospital FEZ, Morocco.
    Desmoid tumors are rare benign neoplasms with an aggressive local growth. In children, intra-abdominal localization is less frequent and few reports exist in the literature about the management of DTs in those special patients. In our report, we describe a case of a 13-year old patient with a bifocal intra-abdominal DT, treated unsuccessfully with tamoxifene, and we discuss briefly the existing literature data. Read More

    Ovarian dysgerminoma in pregnancy: A case report and literature review.
    Cancer Biol Ther 2018 Mar 26:1-10. Epub 2018 Mar 26.
    a Obstetrics and Gynecology Department , Tianjin Medical University General Hospital , No.154, Anshan Road, Heping District , Tianjin , China.
    Background: Dysgerminoma is an uncommon malignant tumor arising from the germ cells of the ovary. Its association with pregnancy is extremely rare; the incidence is about 0.2-1 per 100,000 pregnancies. Read More

    Diagnostic imaging and CEUS findings in a rare case of Desmoid-type fibromatosis. A case report.
    J Ultrasound 2018 Mar 21. Epub 2018 Mar 21.
    Department of Radiological Sciences and Nuclear Medicine, University of Rome "La Sapienza", viale del Policlinico 155, Rome, Italy.
    Desmoid-type fibromatosis (DF), also known as aggressive fibromatosis, is a locally aggressive benign fibroblastic neoplasm that can infiltrate or recur but cannot metastasize. It is rare, with an estimated annual incidence of two to four new cases per million people. Most DFs occur sporadically, but it may also be associated with the hereditary syndrome familial adenomatous polyposis. Read More

    Sporadic Retroperitoneal Fibrosis: a Gentle Giant.
    Indian J Surg Oncol 2018 Mar 25;9(1):71-73. Epub 2017 Sep 25.
    1Department of Surgical Oncology, Tata Memorial Center, Mumbai, Maharashtra 400012 India.
    Fibromatosis covers a broad spectrum of benign fibrous tissue proliferation and is characterized by slow growing, locally infiltrative growth pattern with a high propensity for local recurrence. We report on a case of multicentric fibromatosis originating from the retroperitoneal space and submandibular triangle, in an 18-year-old lady. Computed tomography revealed a retroperitoneal abdominopelvic tumor extending from the left sub-diaphragmatic space to the pelvic inlet which had enveloped the solid viscera in the left upper quadrant with a displaced celiac axis. Read More

    Omental fibromatosis treated by laparoscopic wide surgical resection.
    Intractable Rare Dis Res 2018 Feb;7(1):51-53
    Department of Visceral Surgery, University Hospital CHUV, Lausanne, Switzerland.
    The current report presents a case of an omental fibromatosis discovered incidentally in a 46-year-old woman with no particular medical history and few symptoms. A surgical biopsy was performed initially, and microscopic examination revealed myofibroblastic proliferation. After additional immunohistochemical and molecular analyses, omental fibromatosis was diagnosed. Read More

    New intra-abdominal mass after operation for colorectal cancer: desmoid tumor versus peritoneal seeding.
    Abdom Radiol (NY) 2018 Mar 17. Epub 2018 Mar 17.
    Department of Nuclear Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-Ro, Gangnam-Gu, Seoul, 06351, Republic of Korea.
    Purpose: To identify differential clinical and imaging findings between intra-abdominal desmoid tumors and peritoneal seeding that developed after surgery for colorectal cancer.

    Methods: 8 patients (9 desmoid tumors) and 11 patients (13 peritoneal seeding masses) were enrolled in our retrospective study. Patients with three or more tumors were excluded. Read More

    Sorafenib inhibits proliferation and invasion in desmoid-derived cells by targeting Ras/MEK/ERK and PI3K/Akt/mTOR pathways.
    Carcinogenesis 2018 Mar 10. Epub 2018 Mar 10.
    Department of Surgery, Brigham and Women's Hospital, Boston, MA.
    Desmoid tumors (DTs) are unusual neoplasms of mesenchymal origin that exhibit locally invasive behavior. Surgical resection is the initial treatment of choice for DTs. For patients with recurrent or unresectable disease, however, medical options are limited. Read More

    [Lipofibromatosis: a clinicopathological analysis of eight cases].
    Zhonghua Bing Li Xue Za Zhi 2018 Mar;47(3):186-191
    Department of Pathology, Fudan University Shanghai Cancer Center; Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China.
    To investigate the clinicopathological characteristics and differential diagnosis of lipofibromatosis. The clinicopathological features and immunohistochemical profiles in 8 cases of lipofibromatosis diagnosed at Fudan University Shanghai Cancer Center from January 2008 to June 2017 were studied. Molecular analysis of β-catenin mutation by Sanger sequencing, NTKR1 and ETV6 rearrangements by FISH were performed. Read More

    Use of Aromatase Inhibitors in IVF for Fertility Preservation of Non-Breast Cancer Patients: A Case Series.
    Isr Med Assoc J 2018 Mar;20(3):145-146
    Department of Obstetrics and Gynecology, Davidoff Center, Rabin Medical Center (Beilinson Campus), Petah Tikva, associated with Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
    Background: Controlled ovarian hyperstimulation (COH) followed by oocyte retrieval is a leading option for fertility preservation before chemotherapy, yet this procedure causes excessive serum levels of estradiol (E2), which are often detrimental for cancer patients. Aromatase inhibitors are often used in breast cancer patients during COH to prevent elevated levels of E2.

    Objectives: To describe our experience with COH for oocyte cryopreservation in non-breast cancer patients using aromatase inhibitors. Read More

    Misdiagnosis of aggressive fibromatosis of the abdominal wall: A case report and literature review.
    Medicine (Baltimore) 2018 Mar;97(10):e9925
    Gynecology Oncology Department, Norman Bethune First Hospital, Jilin University, Changchun, China.
    Rationale: Aggressive fibromatosis (AF) of abdominal wall is also called desmoid tumor, ligament tumor, fibrous tissue tumor hyperplasia, tendon membrane fibroma or soft tissue ligament fibroma, etc. Aggressive fibromatosis of abdominal wall was first described by MacFarlane in 1832, and it was named for the first time by Muller according to its general appearance and texture in 1838. This disease has been mistaken for a benign lesions for a long time because when the cells were examined by pathology often show normal mitosis, and distant metastases are not found clinically, but actually the disease is locally invasive and shows a local invasive growth. Read More

    Aggressive laryngeal fibromatosis: A case report and brief review.
    J Cancer Res Ther 2018 Jan-Mar;14(2):465-467
    Department of Pathology, Apollo Gleneagles Hospital, Kolkata, West Bengal, India.
    We describe a rare case of aggressive fibromatosis of the larynx occurring in a 72-year-old man. The lesion manifested with complaints of dyspnea and stridor. A computerized tomographic scan of the neck revealed irregular polypoidal growth invading right vocal cord, crossing midline. Read More

    Treatment of extensive post tonsillectomy oropharyngeal stenosis secondary to fibromatosis.
    Int J Pediatr Otorhinolaryngol 2018 Apr 2;107:107-109. Epub 2018 Feb 2.
    Department of Pediatrics Otolaryngology Head & Neck Surgery, Michigan Medicine, C.S. Mott Children's Hospital, 1540 East Hospital Drive, Ann Arbor, MI 48109, USA. Electronic address:
    Aggressive fibromatosis is an uncommon, benign tumor of fibroblastic origin with high potential for local invasion. Less than a quarter of these lesions are located in the head and neck, and although extremely rare, associations have been demonstrated with physical trauma. We describe a unique case of oropharyngeal fibromatosis with traumaticetiology, managed successfully with surgical excision of the lesion with negative surgical margins. Read More

    Fibromatosis of the breast mimicking cancer: A case report.
    Radiol Case Rep 2018 Feb 6;13(1):1-5. Epub 2017 Nov 6.
    Breast Imaging Division, European Institute of Oncology, via G. Ripamonti 435, 20141 Milan, Italy.
    Breast fibromatosis, also referred to as desmoid tumor or aggressive fibromatosis, is a very rare, locally aggressive disease that does not metastasize. Bilateral lesions are extremely rare and are found in only 4% of patients with breast fibromatosis. Tumor recurrence following surgery occurs in 18%-29% of patients, most often within the first 2 years after surgery. Read More

    Radiation Therapy for Aggressive Fibromatosis: The Association Between Local Control and Age.
    Int J Radiat Oncol Biol Phys 2018 Mar 21;100(4):997-1003. Epub 2017 Dec 21.
    Department of Radiation Oncology, University of Florida College of Medicine, Jacksonville, Florida. Electronic address:
    Purpose: Radiation therapy (RT) is often used in the treatment of unresectable or recurrent aggressive fibromatosis (also known as desmoid tumor) typically with excellent local control. Prior reports have suggested that local control in pediatric patients with aggressive fibromatosis is poor. We aimed to report a long-term single-institution experience with the radiotherapeutic treatment of these tumors with a focus on age-dependent outcomes. Read More

    Plantar Fibromatosis and Other Fibroblastic and Fibrohistiocytic Soft Tissue Tumors of the Foot.
    J Surg Orthop Adv 2017 WINTER;26(4):266-270
    ProScan Imaging Education Foundation, Cincinnati, Ohio; Department of Radiology, University of Louisville, Louisville, Kentucky; Department of Radiology, University of Missouri-Kansas City School of Medicine, Kansas City, Missouri; Department of Orthopedic Surgery, University of Cincinnati, Cincinnati, Ohio; Saint George Children's Hospital, Puerto Rico; and World Care Clinical Research, Boston, Massachusetts.
    Plantar fibromatosis, also known as Ledderhose's disease, is a benign fibroblastic disorder of plantar aponeurosis, more specifically the medial side of the foot arch. Magnetic resonance imaging (MRI) has an important role in the diagnosis, staging, and determining the deep extension found in advanced, aggressive forms of plantar fibromatosis, thereby guiding appropriate clinical and surgical management. This case report aims to provide radiologists and clinicians with simple guidelines for the differential diagnosis of the fibroblastic and fibrohistiocytic soft tissue tumors of the foot with the emphasis on the MRI findings. Read More

    Aggressive fibromatosis in the infratemporal fossa presenting as trismus: a case report.
    J Med Case Rep 2018 Feb 19;12(1):41. Epub 2018 Feb 19.
    Nobel Teaching Hospital, Kachanbari, Biratnagar, Nepal.
    Background: Here we report a very rare entity of an infratemporal region aggressive fibromatosis in a 23-year-old Tharu man who had presented with the symptoms of painless but progressive trismus.

    Case Presentation: We describe a case of aggressive fibromatosis in a 23-year-old Tharu man. Radiological imaging as well as an immunohistochemistry panel from a biopsy indicated a diagnosis of an aggressive fibromatosis. Read More

    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 Apr 1;44(4):496-501. Epub 2018 Feb 1.
    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 Jun 31;65(6):e26968. 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 May 5;65(5):e26943. 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 Apr 9;11(2):168-176. Epub 2018 Jan 9.
    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/m on 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

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