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1. Targeting Hedgehog Signalling as a Drug Therapy in Aggressive Fibromatosis
by Ghanbari Azarnier, Ronak
11/2012
Aggressive fibromatosis is a benign fibroproliferative tumour that can occur as a sporadic lesion or a manifestation in patients with familial syndromes, such... 
0992 | hedgehog signalling | 0571 | aggressive fibromatosis
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2. Full Text Systemic therapy of aggressive fibromatosis in children and adolescents: Report of the Cooperative Weichteilsarkom Studiengruppe (CWS)
by Sparber‐Sauer, Monika and Seitz, Guido and Kalle, Thekla and Vokuhl, Christian and Leuschner, Ivo and Scheer, Monika and Münter, Marc and Ljungman, Gustaf and Bielack, Stefan S and Niggli, Felix and Ladenstein, Ruth and Klingebiel, Thomas and Fuchs, Joerg and Koscielniak, Ewa and CWS Study Grp and CWS Study Group and On Behalf of the CWS Study Group and Medicinska fakulteten and Forskargrupper (Inst. för kvinnor och barns hälsa) and Medicinska och farmaceutiska vetenskapsområdet and Institutionen för kvinnors och barns hälsa and Barnneurologi/Barnonkologi and Uppsala universitet
Pediatric Blood & Cancer, ISSN 1545-5009, 05/2018, Volume 65, Issue 5, pp. e26943 - n/a
Background Treatment algorithms for patients with aggressive fibromatosis (AF) are challenging. There are limited data available about the use of systemic... 
children and adolescents | CWS Group | systemic therapy | aggressive fibromatosis | MANAGEMENT | EFFICACY | TNM CLASSIFICATION | METHOTREXATE | PHASE-II | BETA-CATENIN | TUMORS | CHILDHOOD | ONCOLOGY | DESMOID-TYPE FIBROMATOSIS | PEDIATRICS | HEMATOLOGY | SOFT-TISSUE SARCOMA | Fibromatosis, Aggressive - surgery | Prognosis | Prospective Studies | Follow-Up Studies | Humans | Child, Preschool | Infant | Male | Survival Rate | Combined Modality Therapy | Young Adult | Antineoplastic Combined Chemotherapy Protocols - therapeutic use | Adolescent | Adult | Female | Child | Fibromatosis, Aggressive - pathology | Fibromatosis, Aggressive - drug therapy | Infant, Newborn | Algorithms | Children | Health aspects | Analysis | Adolescence | Vinblastine | Complications | Clinical trials | Remission | Radiation therapy | Adolescents | Methotrexate | Patients | Survival | Clinical Medicine | Medical and Health Sciences | Hematology | Hematologi | Medicin och hälsovetenskap | Klinisk medicin
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3. Full Text Radiation Therapy for Aggressive Fibromatosis: The Association Between Local Control and Age
by Bates, James E and Morris, Christopher G and Iovino, Nicole M and Rutenberg, Michael and Zlotecki, Robert A and Gibbs, C. Parker and Scarborough, Mark and Indelicato, Daniel J
International Journal of Radiation Oncology, Biology, Physics, ISSN 0360-3016, 03/2018, Volume 100, Issue 4, pp. 997 - 1003
Radiation therapy (RT) is often used in the treatment of unresectable or recurrent aggressive fibromatosis (also known as desmoid tumor) typically with... 
PHASE-II TRIAL | EFFICACY | ONCOLOGY | DESMOID-TYPE FIBROMATOSIS | IMATINIB | METHOTREXATE | VINBLASTINE | RADIOTHERAPY | SURGICAL-TREATMENT | TUMORS | RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING | CHILDREN | Medical records | Children | Radiotherapy | Health aspects | Analysis | Radiation | Index Medicus
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4. Full Text Locally aggressive connective tissue tumors
by Gounder, Mrinal M and Thomas, David M and Tap, William D
Journal of Clinical Oncology, ISSN 0732-183X, 01/2018, Volume 36, Issue 2, pp. 202 - 209
In this review, we highlight the complexities of the natural history, biology, and clinical management of three intermediate connective tissue tumors: desmoid... 
EFFICACY | ONCOLOGY | GIANT-CELL TUMOR | DESMOID-TYPE FIBROMATOSIS | DENOSUMAB | IMATINIB | METHOTREXATE | PHASE-II | BONE | EXPRESSION | VILLONODULAR SYNOVITIS | Outcome Assessment (Health Care) - methods | Fibromatosis, Aggressive - diagnostic imaging | Bone Neoplasms - therapy | Bone Neoplasms - diagnostic imaging | Humans | Magnetic Resonance Imaging - methods | Neoplasms, Connective Tissue - diagnostic imaging | Bone Neoplasms - pathology | Giant Cell Tumor of Tendon Sheath - diagnostic imaging | Fibromatosis, Aggressive - therapy | Giant Cell Tumor of Bone - therapy | Outcome Assessment (Health Care) - statistics & numerical data | Giant Cell Tumor of Bone - pathology | Giant Cell Tumor of Bone - diagnostic imaging | Giant Cell Tumor of Tendon Sheath - pathology | Giant Cell Tumor of Tendon Sheath - therapy | Neoplasms, Connective Tissue - therapy | Quality of Life | Neoplasms, Connective Tissue - pathology | Fibromatosis, Aggressive - pathology | Index Medicus | Sarcoma | Special Series
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5. Full Text The treatment of desmoid tumors: A stepwise clinical approach
by Bonvalot, S and Desai, A and Coppola, S and Le péchoux, C and Terrier, P and Dômont, J and Le cesne, A
Annals of Oncology, ISSN 0923-7534, 09/2012, Volume 23, Issue 10, pp. x158 - x166
Aggressive fibromatosis (AF) is a monoclonal proliferative disease but does not metastasize and does not dedifferentiate to a high-grade malignancy in case of... 
Desmoid | Fibromatosis | fibromatosis | SURGERY | SOFT-TISSUE SARCOMAS | SERIES | PROGNOSTIC-FACTORS | FOLLOW-UP | AGGRESSIVE FIBROMATOSIS | RADIATION-THERAPY | PHASE | ONCOLOGY | IMATINIB | desmoid | ABDOMINAL-WALL | Adenomatous Polyposis Coli Protein - genetics | Neoplasm Recurrence, Local - radiotherapy | Humans | Neoplasm Recurrence, Local - drug therapy | Combined Modality Therapy | Fibromatosis, Aggressive - genetics | Fibromatosis, Aggressive - therapy | Biopsy | Protein Kinase Inhibitors - therapeutic use | Germ-Line Mutation | Retrospective Studies | Radiotherapy, Adjuvant | Fibromatosis, Aggressive - pathology
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6. Full Text Management of sporadic desmoid-type fibromatosis: A European consensus approach based on patients’ and professionals’ expertise – A Sarcoma Patients EuroNet and European Organisation for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group initiative
by Kasper, B and Baumgarten, C and Bonvalot, S and Haas, R and Haller, F and Hohenberger, P and Moreau, G and van der Graaf, W.T.A and Gronchi, A and Desmoid Working Grp and Desmoid Working Group
European Journal of Cancer, ISSN 0959-8049, 2014, Volume 51, Issue 2, pp. 127 - 136
Abstract Desmoid-type fibromatosis (DF) is a rare monoclonal, fibroblastic proliferation characterised by a variable and often unpredictable clinical course.... 
Hematology, Oncology and Palliative Medicine | Patient advocacy groups | SPAEN | Consensus | Treatment algorithm | Desmoid-type fibromatosis | EORTC/STBSG | REGRESSION | PROGNOSTIC-FACTORS | SEE POLICY | AGGRESSIVE FIBROMATOSIS | TUMORS | RADIATION-THERAPY | PHASE-II TRIAL | LOCAL RECURRENCE | ONCOLOGY | IMATINIB | MUTATION | Fibromatosis, Aggressive - diagnosis | Bone Neoplasms - therapy | Fibromatosis, Aggressive - prevention & control | Europe | Humans | Osteosarcoma - prevention & control | Patient Care Management - methods | Sarcoma - therapy | Fibromatosis, Aggressive - therapy | Algorithms | Bone Neoplasms - prevention & control | Biomedical Research - methods | Sarcoma - prevention & control | Osteosarcoma - therapy | Patient Care Management - organization & administration | Medicine, Experimental | Medical research | Care and treatment | Sarcoma
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7. Full Text Prognostic Factors Influencing Progression-Free Survival Determined From a Series of Sporadic Desmoid Tumors: A Wait-and-See Policy According to Tumor Presentation
by Sébastien Salas and Armelle Dufresne and Binh Bui and Jean-Yves Blay and Philippe Terrier and Dominique Ranchere-Vince and Sylvie Bonvalot and Eberhard Stoeckle and Louis Guillou and Axel Le Cesne and Odile Oberlin and Véronique Brouste and Jean-Michel Coindre
Journal of Clinical Oncology, ISSN 0732-183X, 09/2011, Volume 29, Issue 26, pp. 3553 - 3558
Purpose Desmoid tumors are mesenchymal fibroblastic/myofibroblastic proliferations with locoregional aggressiveness and high ability to recur after initial... 
SURGERY | FOLLOW-UP | AGGRESSIVE FIBROMATOSIS | MANAGEMENT | ONCOLOGY | RADIATION-THERAPY | Fibromatosis, Aggressive - diagnosis | Multivariate Analysis | Prognosis | Europe | Humans | Middle Aged | Child, Preschool | Infant | Male | Fibromatosis, Aggressive - therapy | Adolescent | Survival Analysis | Aged, 80 and over | Adult | Female | Aged | Retrospective Studies | Child
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8. Full Text Update on desmoid tumors
by Escobar, C and Munker, R and Thomas, J.O and Li, B.D and Burton, G.V
Annals of Oncology, ISSN 0923-7534, 03/2012, Volume 23, Issue 3, pp. 562 - 569
Desmoid tumors (DTs) are histologically benign proliferations of stromal cells but may grow locally aggressive. Overall, DTs are rare (0.03% of all neoplasms).... 
Desmoid tumors | Aggressive fibromatosis | Treatment | Diagnosis | treatment | GENOTYPE-PHENOTYPE CORRELATIONS | PROGNOSTIC-FACTORS | diagnosis | CATENIN MUTATIONS | desmoid tumors | RADIATION-THERAPY | ESTROGEN-RECEPTOR-BETA | ONCOLOGY | APC GENE | FAMILIAL ADENOMATOUS POLYPOSIS | EXPRESSION | aggressive fibromatosis | IMATINIB MESYLATE | Fibromatosis, Aggressive - diagnosis | Fibromatosis, Aggressive - genetics | Fibromatosis, Aggressive - therapy | Humans
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9. Full Text Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients
by Bonvalot, S and Eldweny, H and Haddad, V and Rimareix, F and Missenard, G and Oberlin, O and Vanel, D and Terrier, P and Blay, J.Y and Le Cesne, A and Le Péchoux, C
European Journal of Surgical Oncology, ISSN 0748-7983, 2007, Volume 34, Issue 4, pp. 462 - 468
Abstract Objective To evaluate the impact of surgery as first-line treatment on event-free survival (EFS) of primary aggressive fibromatosis. Patients and... 
Hematology, Oncology and Palliative Medicine | Surgery | Aggressive fibromatosis | Desmoid tumour | Soft tissue tumour | soft tissue tumour | RE-EXCISION | desmoid rumour | PROLIFERATION | MARGINS | RADIATION-THERAPY | ONCOLOGY | EXTREMITY | ADJUVANT RADIOTHERAPY | MUTATIONS | aggressive fibromatosis | SOFT-TISSUE SARCOMA | surgery | DESMOID TUMORS | Fibromatosis, Aggressive - surgery | Humans | Middle Aged | Child, Preschool | Infant | Male | Antineoplastic Agents - therapeutic use | Combined Modality Therapy | Fibromatosis, Aggressive - therapy | Soft Tissue Neoplasms - surgery | Adolescent | Soft Tissue Neoplasms - pathology | Survival Analysis | Adult | Female | Aged | Radiotherapy, Adjuvant | Child | Fibromatosis, Aggressive - pathology | Soft Tissue Neoplasms - therapy
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10. Full Text Desmoid Tumors: Clinical Features and Treatment Options for Advanced Disease
by Kasper, Bernd and Ströbel, Philipp and Hohenberger, Peter
The Oncologist, ISSN 1083-7159, 05/2011, Volume 16, Issue 5, pp. 682 - 693
Desmoid tumors describe a rare monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Although... 
Advanced disease | Aggressive fibromatosis | β‐catenin | Individualized treatment | Desmoid tumor | β-catenin | beta-catenin | SOFT-TISSUE SARCOMAS | MANAGEMENT | GENOTYPE-PHENOTYPE CORRELATIONS | ISOLATED LIMB PERFUSION | RADIATION-THERAPY | ONCOLOGY | GRADE FIBROMYXOID SARCOMA | APC GENE | FAMILIAL ADENOMATOUS POLYPOSIS | Adenomatous Polyposis Coli Protein - genetics | Diagnosis, Differential | Humans | Combined Modality Therapy | Fibromatosis, Abdominal - therapy | beta Catenin - genetics | Fibromatosis, Aggressive - genetics | Fibromatosis, Aggressive - therapy | DNA Mutational Analysis | Fibromatosis, Abdominal - pathology | Mesentery - pathology | Neoplasm Staging | Fibromatosis, Aggressive - pathology | Sarcomas
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11. Full Text Desmoid Tumor: Analysis of Prognostic Factors and Outcomes in a Surgical Series
by Mullen, John T and DeLaney, Thomas F and Kobayashi, Wendy K and Szymonifka, Jackie and Yeap, Beow Y and Chen, Yen-Lin and Rosenberg, Andrew E and Harmon, David C and Choy, Edwin and Yoon, Sam S and Raskin, Kevin A and Petur Nielsen, G and Hornicek, Francis J
Annals of Surgical Oncology, ISSN 1068-9265, 12/2012, Volume 19, Issue 13, pp. 4028 - 4035
Desmoid tumors are rare and exhibit a highly unpredictable natural history. We sought to analyze prognostic factors associated with recurrence in a large... 
Oncology | Medicine & Public Health | Surgical Oncology | Surgery | SURGERY | TRUNK | MANAGEMENT | ONCOLOGY | EXTREMITY | AGGRESSIVE FIBROMATOSIS | ADJUVANT RADIOTHERAPY | RADIATION-THERAPY | Fibromatosis, Aggressive - mortality | Prognosis | Follow-Up Studies | Humans | Neoplasm Recurrence, Local - therapy | Male | Survival Rate | Combined Modality Therapy | Fibromatosis, Abdominal - therapy | Neoplasm Recurrence, Local - mortality | Neoplasm Recurrence, Local - pathology | Fibromatosis, Aggressive - therapy | Fibromatosis, Abdominal - pathology | Adult | Female | Fibromatosis, Abdominal - mortality | Retrospective Studies | Radiotherapy, Adjuvant | Fibromatosis, Aggressive - pathology | Postoperative Complications | Nuclear radiation | Analysis | Risk factors | Tumors
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12. Full Text Guidelines for the clinical management of familial adenomatous polyposis (FAP)
by Vasen, H F A and Möslein, G and Alonso, A and Aretz, S and Bernstein, I and Bertario, L and Blanco, I and Bülow, S and Burn, J and Capella, G and Colas, C and Engel, C and Frayling, I and Friedl, W and Hes, F J and Hodgson, S and Järvinen, H and Mecklin, J-P and Møller, P and Myrhøi, T and Nagengast, F M and Parc, Y and Phillips, R and Clark, S K and de Leon, M Ponz and Renkonen-Sinisalo, L and Sampson, J R and Stormorken, A and Tejpar, S and Thomas, H J W and Wijnen, J
Gut, ISSN 0017-5749, 05/2008, Volume 57, Issue 5, pp. 704 - 713
Background: Familial adenomatous polyposis (FAP) is a well-described inherited syndrome, which is responsible for <1% of all colorectal cancer (CRC) cases. The... 
RESTORATIVE PROCTOCOLECTOMY | GERMLINE MUTATIONS | THYROID-CARCINOMA | AGGRESSIVE FIBROMATOSIS | MUTYH-ASSOCIATED POLYPOSIS | ILEAL POUCH | GASTROENTEROLOGY & HEPATOLOGY | CYCLOOXYGENASE-2 INHIBITOR | POUCH-ANAL ANASTOMOSIS | DESMOID TUMORS | COLORECTAL ADENOMAS | Fibromatosis, Aggressive - diagnosis | Genetic Predisposition to Disease | Humans | Risk Factors | Male | Duodenal Neoplasms - therapy | Duodenal Neoplasms - diagnosis | Genetic Testing - methods | Genes, APC | Fibromatosis, Aggressive - therapy | Anti-Inflammatory Agents, Non-Steroidal - therapeutic use | Age of Onset | Female | Adenomatous Polyposis Coli - therapy | Adenomatous Polyposis Coli - genetics | Adenomatous Polyposis Coli - diagnosis | Prevention | Evaluation | Care and treatment | Colorectal cancer | Practice guidelines (Medicine) | Polyposis, Familial | Risk factors | Studies | Mutation | Genes | Medical protocols | Polyps (Pathology) | Intestines | Intestins | Intestinal diseases | Malalties intestinals | Pòlips (Patologia)
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13. Full Text Radiation therapy for aggressive fibromatosis (desmoid tumors): Results of a national Patterns of Care Study
by Micke, Oliver and Seegenschmiedt, M. Heinrich and German Cooperative Grp Radiotherap and German Cooperative Group on Radiotherapy for Benign Diseases
International Journal of Radiation Oncology, Biology, Physics, ISSN 0360-3016, 2005, Volume 61, Issue 3, pp. 882 - 891
After a general Patterns of Care Study (PCS) the German Cooperative Group on Radiotherapy for Benign Diseases (GCG-BD) initiated a multicenter cohort study to... 
Desmoid tumors | Patterns of Care Study | Aggressive fibromatosis | Benign disease | Radiotherapy | Surgery | MANAGEMENT | patterns of care study | desmoid tumors | MUSCULO-APONEUROTIC FIBROMATOSIS | LOW-DOSE CHEMOTHERAPY | SOFT-TISSUE | COMBINED SURGERY | GARDNERS-SYNDROME | ONCOLOGY | benign disease | radiotherapy | LOCAL-CONTROL | EXPERIENCE | ADJUVANT RADIOTHERAPY | RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING | aggressive fibromatosis | BENIGN DISEASES | surgery | Radiotherapy Dosage | Humans | Female | Male | Surveys and Questionnaires | Treatment Outcome | Statistics as Topic | Germany | Health Care Surveys | Fibromatosis, Aggressive - radiotherapy | Health aspects | Analysis | Radiation | SURGERY | IRRADIATION | PATIENTS | NEOPLASMS | RADIOLOGY AND NUCLEAR MEDICINE | EVALUATION | TOXICITY | RADIOTHERAPY | SIDE EFFECTS | DOSE-RESPONSE RELATIONSHIPS
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14. Full Text Extra-abdominal desmoid fibromatosis: A review of management, current guidance and unanswered questions
by Eastley, N and McCulloch, T and Esler, C and Hennig, I and Fairbairn, J and Gronchi, A and Ashford, R
European Journal of Surgical Oncology, ISSN 0748-7983, 2016, Volume 42, Issue 7, pp. 1071 - 1083
Abstract Extra abdominal desmoid fibromatosis is a complex condition with many recognised treatments including active observation, hormonal therapy,... 
Hematology, Oncology and Palliative Medicine | Surgery | Review | Management | Aggressive | Extra-abdominal | Desmoid | Fibromatosis | SURGERY | WALL | PROGNOSTIC-FACTORS | AGGRESSIVE FIBROMATOSIS | LOW-DOSE CHEMOTHERAPY | TUMORS | RADIATION-THERAPY | ONCOLOGY | FRENCH SARCOMA GROUP | COMBINATION CHEMOTHERAPY | RECURRENCE | SURGICAL RESECTION | Fibroma - diagnosis | Fibromatosis, Aggressive - diagnosis | Biopsy, Needle - instrumentation | Chromosomes, Human, Pair 8 - genetics | Fibromatosis, Aggressive - diagnostic imaging | Prognosis | Biomarkers, Tumor - analysis | Humans | Risk Factors | Fibroma - therapy | Patient Care Team | Clinical Trials as Topic | Molecular Targeted Therapy | Biopsy, Needle - methods