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Aggressive fibromatosis

Massimo Valentino, Mauro Liberatore, Fabrizio Maghella, Valeria De Soccio, Carlo De Felice, Daniela Messineo, Francesco Maria Drudi
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. Treatment is necessary when the disease is symptomatic, especially in case of compression of critical structures. When possible, surgical resection is the treatment of choice; however, recurrence is common...
March 21, 2018: Journal of Ultrasound
David Martin, Mirza Muradbegovic, Snezana Andrejevic-Blant, David Petermann, Luca Di Mare
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. Omental fibromatosis, also called intra-abdominal desmoid, is a rare and benign tumour but can be locally aggressive. Majority of cases are asymptomatic, and difficult to diagnose based on clinical presentation and radiological investigation...
February 2018: Intractable & Rare Diseases Research
Xiaoxia Liu, Shan Zong, Yingli Cui, Ying Yue
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...
March 2018: Medicine (Baltimore)
Brian Chang, Jennifer F Ha, David Zopf
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. A 5-year old patient was found to have an aggressive fibromatosis causing oropharyngeal stenosis following tonsillectomy...
April 2018: International Journal of Pediatric Otorhinolaryngology
Maria Carmela Grimaldi, Chiara Trentin, Roberto Lo Gullo, Enrico Cassano
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. In this report, we discuss a case of breast fibromatosis, mimicking a breast carcinoma both clinically and radiologically, that presented clinically with dimpling of the skin of the left breast in a 31-year-old woman...
February 2018: Radiology Case Reports
James E Bates, Christopher G Morris, Nicole M Iovino, Michael Rutenberg, Robert A Zlotecki, C Parker Gibbs, Mark Scarborough, Daniel J Indelicato
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. METHODS AND MATERIALS: A total of 101 patients treated with RT for aggressive fibromatosis between 1975 and 2015 at a single institution were identified...
March 15, 2018: International Journal of Radiation Oncology, Biology, Physics
Sunil Munakomi
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. Since there was no aggravation in his trismus following surgery and because of his poor socioeconomic status, he was advised to attend regular follow-up visits without any adjuvant therapy...
February 19, 2018: Journal of Medical Case Reports
Gaetano Magro
Spindle cell lesions of the breast cover a wide spectrum of diseases ranging from reactive tumor-like lesions to high-grade malignant tumors. The recognition of the benign spindle cell tumor-like lesions (nodular fasciitis; reactive spindle cell nodule after biopsy, inflammatory pseudotumor/inflammatory myofibroblastic tumor; fascicular variant of pseudoangiomatous stromal hyperplasia) and tumors (myofibroblastoma, benign fibroblastic spindle cell tumor, leiomyoma, schwannoma, spindle cell lipoma, solitary fibrous tumor, myxoma) is crucial to avoid confusion with morphologically similar but more aggressive bland-appearing spindle cell tumors, such as desmoid-type fibromatosis, low-grade (fibromatosis-like) spindle cell carcinoma, low-grade fibrosarcoma/myofibroblastic sarcoma and dermatofibrosarcoma protuberans...
March 2018: Surgical Pathology Clinics
Tatsuhiko Ogawa
BACKGROUND: Desmoid-type fibromatosis is characterized by desmoid tumors, which are benign soft tissue tumors that can be locally aggressive but typically do not metastasize. Desmoid tumors can manifest anywhere in the body, and those in the abdominal cavity account for approximately 30 to 50% of all such tumors. Complete resection with free margins has been the standard treatment, but non-surgical therapies have been implemented recently. However, if tumors are strongly invasive and/or persistently recur, radical surgical resection with free margins remains the primary treatment...
February 7, 2018: Journal of Medical Case Reports
Laura Agresta, Hee Kim, Brian K Turpin, Rajaram Nagarajan, Alexandra Plemmons, Sara Szabo, Roshni Dasgupta, Joel I Sorger, Joseph G Pressey
BACKGROUND: Desmoid tumors/aggressive fibromatosis (DT/AF) lack a reliably effective medical therapy. Surgical resection may be morbid and does not preclude recurrence. Radiation may carry severe late effects, particularly detrimental in young patients. At our institution, we recently observed promising results with pazopanib therapy for DT/AF in adolescent and young adult (AYA) patients. PROCEDURE: Retrospective single-institution chart review. RESULTS: Six DT/AF patients of 3-21 years with previously treated DT/AF received pazopanib; 31 DT/AF patients received established therapies only...
January 31, 2018: Pediatric Blood & Cancer
Jasmine Haller, Marjorie Parker David, Nathan E Lee, Sara C Shalin, Jerad M Gardner
CONTEXT: - Patients with rare tumors have difficulty finding reliable information about their disease. Facebook patient support groups allow patients to educate one another. OBJECTIVE: - To investigate how these patients perceive the value of pathologists, both in Facebook groups and real-world patient care. DESIGN: - Survey links were posted in 12 Facebook patient groups: 6 with an active pathologist member (angiosarcoma, epithelioid hemangioendothelioma, epithelioid sarcoma, dermatofibrosarcoma protuberans [×2], and desmoid fibromatosis), and 6 without "active" pathologist involvement (aggressive angiomyxoma, chondrosarcoma, Ewing sarcoma, leiomyosarcoma, liposarcoma, and osteosarcoma)...
January 29, 2018: Archives of Pathology & Laboratory Medicine
Andrzej Smereczyński, Katarzyna Kołaczyk, Elżbieta Bernatowicz
Chest wall neoplasms mainly include malignancies, metastatic in particular. Differential diagnosis should include clinical data; tumor location, extent, delineation; the degree of homogeneity; the presence of calcifications; the nature of bone destruction and the degree of vascularization. The aim of the paper is to present both the benefits and limitations of ultrasound for the diagnosis of chest wall neoplasms. The neoplastic process may be limited to the chest wall; it may spread from the chest wall into the intrathoracic structures or spread from the inside of the chest towards the chest wall...
December 2017: Journal of Ultrasonography
Kelly A Mercier, Mushriq Al-Jazrawe, Raymond Poon, Zachery Acuff, Benjamin Alman
Desmoid tumors (aggressive fibromatosis) are locally invasive soft tissue tumors that lack the ability to metastasize. There are no directed therapies or standard treatment plan, and chemotherapeutics, radiation, and surgery often have temporary effects. The majority of desmoid tumors are related to T41A and S45F mutations of the beta-catenin encoding gene (CTNNB1). Using broad spectrum metabolomics, differences were investigated between paired normal fibroblast and desmoid tumor cells from affected patients...
January 12, 2018: Scientific Reports
Monika Sparber-Sauer, Guido Seitz, Thekla von Kalle, Christian Vokuhl, Ivo Leuschner, Monika Scheer, Marc Münter, Gustaf Ljungman, Stefan S Bielack, Felix Niggli, Ruth Ladenstein, Thomas Klingebiel, Joerg Fuchs, Ewa Koscielniak
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. RESULTS: Median age was 9...
January 5, 2018: Pediatric Blood & Cancer
Laura Wirth, Alexander Klein, Andrea Baur-Melnyk, Thomas Knösel, Lars H Lindner, Falk Roeder, Volkmar Jansson, Hans Roland Dürr
BACKGROUND: Desmoid-type fibromatosis (DF) is a aggressive (myo)fibroblastic neoplasm with an infiltrative growth and a tendency to local recurrence. Resection of the tumour and/or radiation were proposed as principal treatment. The aim of this retrospective study was to analyze the local control rates focusing on the effect of surgical margins and radiotherapy. METHODS: From 1981 to 2014, 44 patients had been treated. Fifty four therapies had been applied, in 50 cases surgery +/- radiation therapy, NSAIDs or chemotherapy...
January 5, 2018: BMC Musculoskeletal Disorders
Dong Yeon Kim, Junho Lee, Jeong Tae Kim, Hye Kyung Chang, Suk-Ho Moon
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. After 1 week, silicone sheet was substituted with a composite mesh. Then, abdominal wall reconstruction with bilateral free anterolateral thigh (ALT) flaps (30 × 12 cm and 25 × 12 cm sized) was performed...
December 12, 2017: Microsurgery
Mrinal M Gounder, David M Thomas, William D Tap
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...
January 10, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
Xin Liu, Huijie Wang, Xianghua Wu, Xiaonan Hong, Zhiguo Luo
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/m2 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. The primary end point was response rate (RR). Results Fifteen patients were enrolled in the study. No patient had a complete response, but five patients had partial responses, resulting in a RR of 33%...
February 2018: Investigational New Drugs
Kamran Harati, Anais Jaenisch, Björn Behr, Ole Goertz, Ali Harati, Tobias Hirsch, Ingo Stricker, Marcus Lehnhardt, Adrien Daigeler
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. Between 2000 and 2014, 114 patients with aggressive fibromatosis were treated surgically at BG-University Hospital Bergmannsheil (Bochum, Germany). Univariate and multivariate analyses were restricted to 90 participants with information available on surgical margins at the initial procedure...
November 2017: Oncology Letters
Dumnoensun Pruksakorn, Sratwadee Lorsomradee, Areerak Phanphaisarn, Pimpisa Teeyakasem, Jeerawan Klangjorhor, Parunya Chaiyawat, Natapong Kosachunhanun, Jongkolnee Settakorn, Olarn Arpornchayanon
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. This study was a trial of the use of intralesional steroid injection (ILSI) including investigation of safety margins and clinical outcomes of high-dose steroids for local use treatment of AF...
November 2, 2017: World Journal of Surgical Oncology
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