TumorsDesmoid tumorSpindle cell carcinomaBenignLocallyCarcinoma of the breastMyofibroblasticSuperficialRecurrentSynonymMetaplasticHereditaryCongenitalPathologyLesionsCommonlyTypeTreatmentConditionRare softFemaleExperienceAbdominalTumoursPlantar FibromatosisNodular fasciitisExcision of the tumorType fibromatosisLocally aggressiveDeep fibromatosesResectionMusculoaponeuroticBenign tumorTumourMalignantClassificationFibrousSarcomaModalityDIAGNOSISSoft tissuesHistopathologicalRareRecurrenceMesenchymalClinicalSurgicalPatientsConsistsOccurCorrelationGeneticDiseaseSpindle
Tumors12
- Desmoid tumors are a type of fibromatosis and related to sarcoma, though without the ability to spread throughout the body (metastasize). (wikipedia.org)
- However, aggressive fibromatosis is locally aggressive and can cause life-threatening problems or even death when the tumors compress vital organs such as intestines, kidneys, lungs, blood vessels, or nerves. (wikipedia.org)
- The World Health Organization reclassified desmoid tumors (termed desmoid-type fibromatosis) as a specific type of tumor in the category of intermediate (locally aggressive) fibroblastic and myofibroblastic tumors. (wikipedia.org)
- Desmoid tumors often appear as infiltrative, usually well-differentiated, firm overgrowths of fibrous tissue, and they are locally aggressive. (medscape.com)
- The DeFi trial randomized 142 patients with desmoid tumors (or aggressive fibromatosis) to receive oral treatment with either a placebo or nirogacestat, an investigational, selective gamma-secretase inhibitor that blocks Notch pathway signaling and cell growth. (mskcc.org)
- Gardner syndrome is also associated with familial adenomatous polyposis and may manifest as aggressive fibromatosis (desmoid tumors) of the retroperitoneum. (wikipedia.org)
- Desmoid tumors, also called aggressive fibromatosis or desmoid-type fibromatosis, are rare connective tissue tumors that typically arise in the upper and lower extremities, abdominal wall, head and neck area, mesenteric root, and chest wall, or other parts of the body. (itbusinessnet.com)
- To summarize the general characteristics, management principles and prognosis of benign and aggressive bone tumors (BABT) involving the acetabulum. (shsmu.edu.cn)
- Aggressive tumors and/or tumors with cortical bone damage have a higher recurrence rate following simple curettage, and the surgical margin needs to be strictly determined prior to surgery. (shsmu.edu.cn)
- Surgical approach and prognosis evaluation of primary benign and aggressive bone tumors involving the acetabulum[J]. JOURNAL OF SHANGHAI JIAOTONG UNIVERSITY (MEDICAL SCIENCE), 2021, 41(11): 1470-1477. (shsmu.edu.cn)
- Chen, HY, Nguyen, TMT & Chang, IW 2021, ' Low-Grade Fibromatosis-Like Spindle Cell Carcinoma of the Breast: a Mimicker of Benign Mesenchymal Tumors ', Indian Journal of Surgery , vol. 83, pp. 535-538. (tmu.edu.tw)
- These tumors, also called aggressive fibromatosis, are considered benign with no metastatic potential. (medscape.com)
Desmoid tumor4
- Aggressive fibromatosis or desmoid tumor is a rare condition. (wikipedia.org)
- Desmoid tumor classified as deep fibromatoses , a type of benign myofibroblastic neoplasm originating from the muscle aponeurosis(1). (iomcworld.com)
- 2) despite of their aggressive local infiltration desmoid tumor lack a metastatic potential (3). (iomcworld.com)
- Desmoid tumor, also known as aggressive fibromatosis, is well-characterized by abnormal Wnt/β-catenin signaling. (e-crt.org)
Spindle cell carcinoma2
- We described a case of low-grade fibromatosis-like spindle cell carcinoma (FLSCC) of the breast of a 49-year-old woman, which was diagnosed by excisional biopsy. (tmu.edu.tw)
- Low-grade fibromatosis-like spindle cell carcinoma is a rare subtype of metaplastic carcinomas of the breast and typically occurs as palpable breast mass in an older woman. (tmu.edu.tw)
Benign1
- The differential diagnosis is made with the benign forms of infantile fibromatoses rich in cells and other infantile sarcomas particularly the congenital rhabdomyosarcoma more aggressive [2, 3]. (jortho.org)
Locally4
- Histologically they resemble very low-grade fibrosarcomas, but they are very locally aggressive and tend to recur even after complete resection. (wikipedia.org)
- however, locally they show an aggressive and infiltrative behaviour. (minervamedica.it)
- Finally, desmoid type fibromatosis, a locally aggressive tumor that can present in the pleura, has been shown to frequently have CTNNB1 gene mutations and express β-catenin by immunohistochemistry. (nih.gov)
- It behaves as a locally aggressive disease but with a low potential of regional lymph node or distant metastasis. (tmu.edu.tw)
Carcinoma of the breast1
- Matrix-producing carcinoma of the breast : An aggressive subtype of metaplastic carcinoma. (webpathology.com)
Myofibroblastic1
- It can be histologically confused with nodular fasciits, desmoids-type fibromatosis, and inflammatory myofibroblastic tumor. (tmu.edu.tw)
Superficial1
- They are classified into a less aggressive superficial variant and a deeper aggressive variant commonly called aggressive angiomyxomas (AA). (asjo.in)
Recurrent1
- Unrecognized neuromuscular choristoma with recurrent desmoid-type fibromatosis and Marjolin ulcer: expanding the spectrum of neuromuscular choristoma sequelae within the nerve territory? (elsevierpure.com)
Synonym1
- The synonym aggressive fibromatosis describes the marked cellularity and aggressive local behavior. (medscape.com)
Metaplastic1
- It is an aggressive subtype of metaplastic breast carcinoma in which there is a direct transition from carcinoma to the osseous, chondroid, or chondromyxoid elements . (webpathology.com)
Hereditary2
- BACKGROUND AND OBJECTIVE:Activated potassium channels were found to be strongly correlated with gingival overgrowth (GO) phenotype as we reviewed syndromic hereditary gingival fibromatosis (HGF). (shengsci.com)
- The aim of the present study was to explore the pathogenic role of aberrantly activated potassium channel in Hereditary Gingival Fibromatosis (HGF). (shengsci.com)
Congenital1
- Arthur Purdy Stout coined the term fibromatosis (in the name congenital generalized fibromatosis, describing myofibromatosis) in 1954. (wikipedia.org)
Pathology2
- Postoperative pathology demonstrated an aggressive histology in 18 cases, 14 cases of which were accompanied by bone cortical damage (77.8%), and a total of 5 cases had local recurrence. (shsmu.edu.cn)
- A pathology review of the previous biopsy showed findings suggestive of a sclerotic process and a diagnosis of desmoid fibromatosis was proposed, but definitive diagnosis was not possible on the limited tissue available. (fortuneonline.org)
Lesions1
- The fibromatoses are a group of lesions that can infiltrate widely, replacing muscle, fat and bone with fibrous tissue of varying cellularity. (minervamedica.it)
Commonly1
- Intra-abdominal desmoid fibromatosis (IADF) is a rare condition, comprising 8-15% of all presentations of desmoid fibromatosis (DF), most commonly arising from the small bowel mesentery. (fortuneonline.org)
Type2
- Comparison of Sporadic and FAP-associated Desmoid-Type Fibromatoses. (cancer.net)
- Histopathology demonstrated features of a desmoid-type abdominal fibromatosis (Figure 1a) with most areas showing near total hyalinisation (Figure 1b). (fortuneonline.org)
Treatment1
- It is important to establish the appropriate treatment to avoid unnecessary aggressive therapy. (bvsalud.org)
Condition1
- Also known as aggressive fibromatosis, it is a rare condition that does not usually impact lifespan but can cause debilitating symptoms in some patients. (keyopinionleaders.com)
Rare soft1
- Aggressive angiomyxoma (AA) is a very rare soft tissue mesenchymal tumor, typically found in the perineum of females in reproductive age group. (asjo.in)
Female1
- We report a rare case of Aggressive angiomyxoma in a 28-year-old female. (asjo.in)
Experience1
- Aggressive Fibromatosis in Children and Adolescents: The Italian Experience. (cancer.net)
Abdominal7
- We present here the case of a 47-year-old woman, without medical history, who was referred to our hospital for a painful swelling of the left iliac fossa, diagnosed with aggressive fibromatosis of the anterior abdominal wall based on radiological and histological findings. (thieme-connect.com)
- Aggressive fibromatosis of the abdominal wall, limbs and limb girdles. (thieme-connect.com)
- Abdominal wall reconstruction after desmoid type fibromatosis radical resection: case series from a single institution and review of the literature. (thieme-connect.com)
- Thanks to the mesenteric fibromatosis, abdominal surgery is forever off the table. (cancer.org)
- The adult form of abdominal fibromatosis is FIBROMATOSIS, ABDOMINAL . (nih.gov)
- Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution. (ijcto.org)
- The desmoid type of infantile fibromatosis represents the childhood counterpart of extra abdominal desmoids in adults. (waocp.com)
Tumours3
- Whilst most fibromatoses are benign tumours and do not metastasise (spread to other parts of the body), the desmoid tumours although they do not metastasise like malignant cancers can be locally aggressive. (dermnetnz.org)
- In some types of fibromatosis such as desmoid tumours, it is thought that the condition may be related to trauma , hormonal factors, or have a genetic association. (dermnetnz.org)
- The primary differential medical diagnosis of GIST contains simple muscles tumours (leiomyoma and leiomyosarcoma), nerve sheath tumours (schwannoma and neurofibroma), inflammatory fibroid polyp and desmoid fibromatosis. (moonphase2018.com)
Plantar Fibromatosis1
- The key symptoms of Plantar Fibromatosis (Ledderhose Disease) are the growing nodules and eventually cords that form within and in between the tissue layers of the plantar fascia and the skin surface on the soles of the feet. (dupuytren-online.info)
Nodular fasciitis1
- Aggressive fibromatosis, nodular fasciitis, myositis ossificans , and inflammatory pseudotumor are among the most important differential diagnoses. (medscape.com)
Excision of the tumor1
- Treatment of infantile fibromatosis includes surgical excision of the tumor through wide or radical resections, both of which are limb-sparing surgeries. (orthopediconcologist.com)
Type fibromatosis10
- Sporadic desmoid-type fibromatosis (DTF) is a rare, chronic, non-metastasising, disease of the soft tissues. (nih.gov)
- Desmoid-type fibromatosis is a rare, potentially locally aggressive disease. (biomedcentral.com)
- Aggressive fibromatosis (AF), also named desmoid-type fibromatosis, is characterised by monoclonal myofibroblastic proliferation in soft tissues. (biomedcentral.com)
- For many centres, first line systemic treatment of non-resectable or symptomatic desmoid-type fibromatosis consists of hormonal manipulation, with or without a NSAID. (biomedcentral.com)
- 6. Approach to screening for Familial Adenomatous Polyposis (FAP) in a cohort of 226 patients with Desmoid-type Fibromatosis (DF): experience of a specialist center in the UK. (nih.gov)
- 8. Differential diagnosis and mutation stratification of desmoid-type fibromatosis on MRI using radiomics. (nih.gov)
- 14. Yield of Colonoscopy in Identification of Newly Diagnosed Desmoid-Type Fibromatosis with Underlying Familial Adenomatous Polyposis. (nih.gov)
- 18. Radiation-induced sarcomas Occurring in Desmoid-type Fibromatosis Are Not Always Derived From the Primary Tumor. (nih.gov)
- Desmoid-type fibromatosis rarely occurs in the oral tongue. (johnshopkins.edu)
- Background: Treatment modality of desmoid-type fibromatosis (DF) has changed from surgery with a wide surgical margin to conservative treatment. (elsevierpure.com)
Locally aggressive2
- Histologically they resemble very low-grade fibrosarcomas, but they are very locally aggressive and tend to recur even after complete resection. (wikipedia.org)
- This tumor is locally aggressive, but rarely metastatic, and occurs in the extremity in 70% of patients. (medscape.com)
Deep fibromatoses3
- juvenile vs adult fibromatoses) and localisation (ie, superficial vs deep fibromatoses). (dermnetnz.org)
- The following table lists the distinguishing features between superficial and deep fibromatoses. (dermnetnz.org)
- Desmoid tumor classified as deep fibromatoses , a type of benign myofibroblastic neoplasm originating from the muscle aponeurosis(1). (iomcworld.com)
Resection1
- 17. Fibromatosis of the Sigmoid Colon With ctnnb 1 (β-Catenin) Gene Mutation, Arising at the Site of Ileocolic Anastomosis for Resection of Gastrointestinal Stromal Tumor. (nih.gov)
Musculoaponeurotic4
- Introduction Aggressive fibromatosis is a benign tumor, thought to arise from deep musculoaponeurotic structures, rarely found in the head or neck. (medscape.com)
- Aggressive fibromatosis (AF) is a benign tumor, thought to arise from deep musculoaponeurotic structures, [ 1 ] characterized by local invasion and recurrence that can occur anywhere in the body. (medscape.com)
- Aggressive fibromatosis describes rare benign lesions, which arise from musculoaponeurotic structures. (biomedcentral.com)
- This is a rare benign tumour that develops from the tissue of the musculoaponeurotic system and is characterized by local aggressive behaviour. (wingsandwishes.org)
Benign tumor1
- Aggressive fibromatosis is a rare and benign tumor of soft tissues, locally invasive but never metastasizes. (thieme-connect.com)
Tumour1
- It really is generally decided that the main prognostic elements in GIST are size from the tumour and mitotic count number, which defines the chance of aggressive behavior. (moonphase2018.com)
Malignant2
- The tumor is itself not malignant but it is very aggressive, especially in females. (cancer.org)
- Some conditions may be serious, and self-diagnosis can put a person at risk of improper treatment, e.g. in case of a benign or malignant soft tissue tumor such as the diagnosis of an aggressive fibromatosis (desmoid tumor). (dupuytren-online.info)
Classification2
- What is the classification of fibromatosis? (dermnetnz.org)
- According to the World Health Organization Classification, infantile fibromatosis is classified in diffuse (Mesenchymal and desmoid types) [2]. (waocp.com)
Fibrous1
- Infantile fibromatosis is a proliferative fibrous childhood condition that originates from skeletal muscles, fasciae or periosteum and may invade adjacent structures [1, 2] with unknown pathogenesis. (waocp.com)
Sarcoma2
- Efficacy of imatinib in aggressive fibromatosis: Results of a phase II multicenter Sarcoma Alliance for Research through Collaboration (SARC) trial. (medscape.com)
- Forty-two patients presenting to the combined sarcoma clinic at Johannesburg Hospital with aggressive fibromatosis from 1990 to 2003 were analysed retrospectively. (bvsalud.org)
Modality2
- Due to its aggressive behavior and tendency to invade local structures and recur, a multi-modality management strategy is usually employed. (medscape.com)
- On the basis of this case, we suggest that aggressive surgery is a viable management option and may be successfully used as a single modality treatment. (medscape.com)
DIAGNOSIS1
- Radiographic imaging is used to help form a diagnosis of infantile fibromatosis. (orthopediconcologist.com)
Soft tissues1
- Infantile fibromatosis can affect the skin, muscles, soft-tissues, bones and rarely visceral organs. (orthopediconcologist.com)
Histopathological2
- The mass was excised and histopathological analysis revealed a case of aggressive fibromatosis. (medscape.com)
- however, the histopathological confirmation was aggressive infantile fibromatosis and the management was appropriate. (waocp.com)
Rare2
- Aggressive fibromatosis or desmoid tumor is a rare condition. (wikipedia.org)
- The need for aggressive surgical management, rare site and operability in this region is very rarer of rarest. (waocp.com)
Recurrence1
- The lesion has a strong tendency for local recurrence and aggressive infiltrating growth. (waocp.com)
Mesenchymal1
- Aggressive fibromatosis (desmoid tumor) is derived from mesenchymal progenitor cells. (ijcto.org)
Clinical2
- Clinical and molecular studies of the effect of imatinib on advanced aggressive fibromatosis (desmoid tumor). (medscape.com)
- The Case Study describes the clinical radiographic Surgical Procedure with reconstruction and histopathology Infantile fibromatosis of infratemporal fossa extending to oral cavity and Oropharynx in a two-year-old male child. (waocp.com)
Surgical1
- Preceding traumatic events to the involved area, previous pregnancy as well as surgical scars have been identified as factors contributing to the development of aggressive fibromatosis. (biomedcentral.com)
Patients2
- A retrospective database was used to identify consecutive patients diagnosed with aggressive fibromatosis (AF) and treated with tamoxifen plus/minus non-steroidal anti-inflammatory drugs at our tertiary referral centre between 2007 and 2014. (biomedcentral.com)
- To determine the impact of prognostic variables on local control in patients with aggressive fibromatosis treated with or without radiation . (bvsalud.org)
Consists1
- The work-up for Infantile Fibromatosis often consists of a physical examination, X-ray, MRI, CT, and bone scans. (orthopediconcologist.com)
Occur1
- However, when it does occur in the head and neck region, it tends to be more aggressive and associated with significant morbidity, which may be attributed to the vital vascular, neurological or anatomical structures in close proximity. (medscape.com)
Correlation1
- 10 Lee JC, Thomas JM, Phillips S, Fisher C, Moskovic E. Aggressive fibromatosis: MRI features with pathologic correlation. (thieme-connect.com)
Genetic1
- Aside from the CTNNB1 and adenomatous polyposis coli (APC) mutations, the genetic profile of pediatric aggressive fibromatosis (AF) has remained poorly characterized. (nih.gov)
Disease1
- Management of fibromatosis depends on individual disease. (dermnetnz.org)
Spindle1
- Desmoid fibromatosis expresses fl-catenin in the nuclei from the spindle cells generally. (moonphase2018.com)