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6. de Campos FG, Perez RO, Imperiale AR, Seid VE, Nahas SC, Cecconello I: Evaluating causes of death in familial adenomatous polyposis. J Gastrointest Surg; 2010 Dec;14(12):1943-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • At diagnosis, 57 patients (58.7%) already had CRC-associated polyposis.
  • CRC, other tumors (desmoid tumors, lymphoma, and gastric cancer), and other causes (complication of duodenal cancer surgery, complication after ileorectal anastomosis (IRA), and coronary disease) were responsible for 12 (63.1%), four (21.1%), and three (15.8%) of all deaths, respectively.
  • Desmoid disease was the second cause of death (10.5% of all causes), leading to a fatal outcome 22% of all patients who developed DT during the study period.
  • (3) long-term survival was also strongly related to the development of extracolonic neoplasia, especially desmoid tumors and gastroduodenal carcinoma;.

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  • (PMID = 20676788.001).
  • [ISSN] 1873-4626
  • [Journal-full-title] Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract
  • [ISO-abbreviation] J. Gastrointest. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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7. Geethamani V, Ravindra S, Reddy VV: Fine needle aspiration cytology of juvenile hyaline fibromatosis: a case report. Acta Cytol; 2007 Jul-Aug;51(4):624-6
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  • [Title] Fine needle aspiration cytology of juvenile hyaline fibromatosis: a case report.
  • BACKGROUND: Juvenile hyaline fibromatosis (YHF) is a rare inherited disorder characterized by tumorous growth of hyalinized fibrous tissue.
  • Fine needle aspiration of the nodule on the left ear revealed benign, spindle-shaped cells with an eosinophilic ground substance in the background.
  • The diagnosis of JHF was made following cytologic and histopathologic studies.
  • CONCLUSION: Fine needle aspiration cytology is reliable for the diagnosis of JHF.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Skin Neoplasms / pathology


8. Mazzocchi M, Onesti MG, Di Ronza S, Scuderi N: Breast desmoid tumor after augmentation mammoplasty: two case reports. Acta Chir Plast; 2009;51(3-4):73-8
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  • [Title] Breast desmoid tumor after augmentation mammoplasty: two case reports.
  • Desmoid tumor of the breast is a rare fibroblastic disease, a slow-growing and histologically benign lesion that accounts for 0.2% of all breast tumors.
  • These tumors do not metastatize, have no capsule but do have the propensity to aggressively invade local tissues and organs.
  • It has been suggested that these tumours are associated with surgical and non-surgical tissue trauma, certain hormones and genetic susceptibility.
  • We describe two cases of breast desmoid tumor after breast augmentation.
  • During surgery an inelastic, soft, capsulated mass, arising from the periprosthetic capsula in the area of pectoralis major muscle was found.
  • The results of this histological examination suggested an extra-abdominal desmoid tumor.
  • [MeSH-major] Breast Implantation. Breast Neoplasms / surgery. Fibromatosis, Aggressive / surgery. Postoperative Complications / surgery

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  • (PMID = 20514891.001).
  • [ISSN] 0001-5423
  • [Journal-full-title] Acta chirurgiae plasticae
  • [ISO-abbreviation] Acta Chir Plast
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Czech Republic
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9. Neri HA, Villagra EJ, Alvarez AC, Valencia P, Jiménez E, de la Torre C, Rodríguez MA, Espinosa R: Ethmoidal desmoid tumor in a pediatric patient. Otolaryngol Head Neck Surg; 2007 Jan;136(1):137-8
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  • [Title] Ethmoidal desmoid tumor in a pediatric patient.
  • [MeSH-major] Ethmoid Sinus. Fibromatosis, Aggressive / surgery. Paranasal Sinus Neoplasms / surgery

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  • (PMID = 17210352.001).
  • [ISSN] 0194-5998
  • [Journal-full-title] Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery
  • [ISO-abbreviation] Otolaryngol Head Neck Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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10. Tulchinsky H, Keidar A, Goldman G, Klausner JM, Rabau M: Surgical treatment and long-term outcome of patients with familial adenomatous polyposis: 16 years experience at the Tel Aviv Sourasky Medical Center. Isr Med Assoc J; 2005 Feb;7(2):82-5
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  • Complications and extra-intestinal manifestations are inherent to the procedure.
  • Extracolonic manifestations developed in 38 patients, including desmoid tumors (in 12), duodenal adenomas (in 9), pouch adenomas (in 5), and rectal stump adenomas (in 3).
  • Two patients died (4%) because of desmoid tumor and malignant fibrous histiocytoma.


11. Choi JY, Kang KM, Kim BS, Kim TH: Mesenteric fibromatosis causing ureteral stenosis. Korean J Urol; 2010 Jul;51(7):501-4
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  • [Title] Mesenteric fibromatosis causing ureteral stenosis.
  • Mesenteric fibromatosis is a rare benign disease characterized by proliferating fibrous tissue in the bowel mesentery.
  • We report a case of aggressive mesenteric fibromatosis with ureteral stenosis arising in a 46-year-old woman who suffered from intermittent right abdominal pain.
  • Pathological examination confirmed mesenteric fibromatosis.

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  • (PMID = 20664786.001).
  • [ISSN] 2005-6745
  • [Journal-full-title] Korean journal of urology
  • [ISO-abbreviation] Korean J Urol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC2907502
  • [Keywords] NOTNLM ; Aggressive fibromatosis / Retroperitoneal neoplasms
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12. González MA, Menéndez R, Ayala JM, Herrero M, Cuesta J, Domínguez A, Martínez M, Graña JL, Pozo F: [Intra-abdominal desmoid tumor]. Cir Esp; 2005 Jun;77(6):362-4
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  • [Title] [Intra-abdominal desmoid tumor].
  • [Transliterated title] Tumor desmoide intraabdominal.
  • Aggressive fibromatosis (desmoid tumor) are rare connective tissue tumors that occur sporadically or in association with familial adenomatous polyposis.
  • Biopsy is required to establish the diagnosis.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / surgery. Retroperitoneal Neoplasms / pathology. Retroperitoneal Neoplasms / surgery

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  • (PMID = 16420952.001).
  • [ISSN] 0009-739X
  • [Journal-full-title] Cirugía española
  • [ISO-abbreviation] Cir Esp
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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13. Sakorafas GH, Nissotakis C, Peros G: Abdominal desmoid tumors. Surg Oncol; 2007 Aug;16(2):131-42
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Abdominal desmoid tumors.
  • Desmoid tumors are rare, benign, fibromatous lesions that are the result of abnormal proliferation of myofibroblasts.
  • Desmoid tumors can be classified as extra-abdominal and abdominal.
  • Abdominal desmoid tumors are either superficial or intraabdominal.
  • These tumors are associated with a high recurrence rates, even if their microscopic characters indicate a benign disease; their biologic behavior often indicates rather a "malignant" disease, which can cause even the death.
  • Intraabdominal desmoid tumors can engulf surrounding viscera and vessels, thereby greatly complicating their surgical treatment.
  • Simple observation is a reasonable management option for asymptomatic patients; spontaneous regression of these tumors may be observed.
  • Complete excision is the treatment of choice for tumors causing symptoms or complications.
  • [MeSH-major] Abdominal Neoplasms / pathology. Abdominal Neoplasms / therapy. Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / therapy
  • [MeSH-minor] Algorithms. Diagnosis, Differential. Diagnostic Imaging. Genetic Testing. Humans. Medical History Taking

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  • (PMID = 17719772.001).
  • [ISSN] 0960-7404
  • [Journal-full-title] Surgical oncology
  • [ISO-abbreviation] Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 86
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4. Spiridakis K, Panagiotakis G, Grigoraki M, Kokkinos I, Papadakis T, Kokkinakis T, Kandylakis S: Isolated giant mesenteric fibromatosis (intra-abdominal desmoid tumors). Case report. G Chir; 2008 Oct;29(10):413-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Isolated giant mesenteric fibromatosis (intra-abdominal desmoid tumors). Case report.
  • A rare case of isolated giant mesenteric fibromatosis is presented.
  • The tumor originated from the fibrous mesenteric tissue.
  • The patient underwent laparotomy because of abdominal discomfort and sub-occlusive symptoms due to the giant mass.
  • Differential diagnosis of mesenteric masses is discussed and the Authors also review the literature concerning this rare disease.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis. Fibromatosis, Abdominal / surgery. Peritoneal Neoplasms / diagnosis. Peritoneal Neoplasms / surgery
  • [MeSH-minor] Adult. Diagnosis, Differential. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / surgery. Humans. Male. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 18947463.001).
  • [ISSN] 0391-9005
  • [Journal-full-title] Il Giornale di chirurgia
  • [ISO-abbreviation] G Chir
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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15. Tehranchinia Z, Rahimi H: Juvenile hyaline fibromatosis in two siblings. Eur J Dermatol; 2010 Sep-Oct;20(5):631-2
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  • [Title] Juvenile hyaline fibromatosis in two siblings.
  • [MeSH-major] Fibromatosis, Aggressive / pathology
  • [MeSH-minor] Child, Preschool. Consanguinity. Contracture / complications. Dermis / pathology. Fibromatosis, Gingival / complications. Fibromatosis, Gingival / pathology. Humans. Male. Syndrome

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  • (PMID = 20627857.001).
  • [ISSN] 1167-1122
  • [Journal-full-title] European journal of dermatology : EJD
  • [ISO-abbreviation] Eur J Dermatol
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] France
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16. Liu QY, Chen JY, Liang BL, Li HG, Gao M, Lin XF: [Imaging manifestations and pathologic features of soft tissue desmoid-type fibromatosis]. Ai Zheng; 2008 Dec;27(12):1287-92
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Imaging manifestations and pathologic features of soft tissue desmoid-type fibromatosis].
  • BACKGROUND & OBJECTIVE: Soft tissue desmoid-type fibromatosis is a type of benign, infiltrative tumors which are rarely seen.
  • This study was to analyze CT and MR manifestations and pathologic features of soft tissue desmoid-type fibromatosis to improve its diagnostic accuracy.
  • METHODS: A total of 34 soft tissue desmoid-type fibromatosis from 29 patients, including 20 primary and 14 recurrent tumors were analyzed.
  • Features of the growth pattern, CT or MRI findings and pathologic appearances of the tumors were studied.
  • RESULTS: The mean size of the 34 tumors was 6.5 cm.
  • Of the 34 tumors, 31(91.2%) had an ill-defined or partially ill-defined margin, 31(91.2%) had a lobulated or irregular contour, 17(50.0%) had neurovascular involvement, 15(44.1%) had bone involvement, 23(67.6%) had extra-compartmental extension.
  • Among the eight tumors scanned by CT, six showed slight hypodensity on the non-enhanced CT scan, and seven were inhomogeneously enhanced after contrast injection.
  • Twenty-six tumors scanned by MRI appeared either iso-or slightly hyperintense on T1W images, hyperintense on T2W images, and moderate or intense enhancement after gadolinium administration.
  • Heterogeneous signal intensity was detected in 88.5%(23/26) tumors.
  • Linear and curvilinear areas of signal void interspersed throughout the tumors were found in 22 tumors(84.6%) on both T1W and T2W images.
  • Histologic analysis revealed that the tumor was composed of spindle cells and collagen bundles, with a variable amount of intermingled collagen surrounding the spindle cells.
  • Nuclear atypia was not seen,and occasional mitoses were present in the tumor cells.
  • CONCLUSION: The characteristic manifestations of CT or MR images of desmoid-type fibromatosis provide important evidence to discriminate benign and malignant soft tissue tumors.
  • [MeSH-major] Extremities / radiography. Fibromatosis, Aggressive / diagnosis. Magnetic Resonance Imaging / methods. Soft Tissue Neoplasms / diagnosis. Tomography, X-Ray Computed / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Female. Humans. Image Enhancement. Infant. Male. Middle Aged. Neoplasm Recurrence, Local. Retrospective Studies. Thoracic Wall / pathology. Thoracic Wall / radiography. Young Adult

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  • (PMID = 19079995.001).
  • [Journal-full-title] Ai zheng = Aizheng = Chinese journal of cancer
  • [ISO-abbreviation] Ai Zheng
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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17. Shapeero LG, De Visschere PJ, Verstraete KL, Poffyn B, Forsyth R, Sys G, Uyttendaele D: Post-treatment complications of soft tissue tumours. Eur J Radiol; 2009 Feb;69(2):209-21
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  • [Title] Post-treatment complications of soft tissue tumours.
  • PURPOSE: To identify local and distant complications of patients with soft tissue tumours and evaluate their relationships to types of therapy.
  • METHODS AND MATERIALS: Fifty-one patients (29 males and 22 females, ages 14-80 years) with 34 malignant and 17 benign soft tissue tumours were evaluated for local and distant complications after resection or amputation only (26 patients) or after the addition of radiotherapy (25 patients: 17 patients had external beam therapy, 7 patients had external beam therapy and brachytherapy, and one patient had extracorporeal irradiation and reimplantation).
  • Duration of follow-up averaged 3.75 years for malignant tumours and 2.79 years for benign tumours.
  • Follow-up studies included radiography, T1- and T2-weighted magnetic resonance (MR) imaging, dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI), computed tomography for thoracic and abdominal metastases, and 3-phase technetium-99m-labeled-methylene-diphosphonate scintigraphy for bone metastases.
  • RESULTS: Recurrent tumours were 2.2 times more frequent in patients who had undergone their initial resection at an outside hospital as compared with those first treated at the university hospital.
  • Metastases from soft tissue sarcomas, most commonly to lung (nine patients) and to bone and muscle (five patients), showed no specific relationship to type of therapy.
  • DCE-MRI differentiated rapidly enhancing soft tissue recurrences (11 patients) and residual tumours (6 patients) from slowly enhancing muscle inflammation, and non-enhancing fibrosis and seromas that usually did not enhance.
  • Irrespective of the type of treatment, inflammatory changes in muscle and subcutaneous and cutaneous tissue and the majority of seromas were evident at the first follow-up study.
  • Inflammatory changes in muscle and cutaneous and subcutaneous tissue after resection alone disappeared by the second follow-up study, whereas these changes after radiotherapy resolved months to years after treatment.
  • Except for one patient with aggressive fibromatosis, bone and nerve complications occurred in patients with soft tissue malignancy.
  • In nerve entrapment, DCE-MRI demonstrated the rapidly enhancing recurrent tumour or non-enhancing fibrosis surrounding the slowly enhancing nerve.
  • CONCLUSION: This study suggests a possible relationship between types of treatment of soft tissue tumours and subsequent complications.
  • Incomplete or difficult surgery resulted in residual or recurrent tumours and heterotopic ossification.
  • Diligent follow-up of patients with soft tissue tumours with recognition of these complications and their differentiation from recurrent or residual tumour can help guide clinical care and may negate the need for surgery when benign disease is defined.
  • [MeSH-major] Postoperative Complications / diagnosis. Postoperative Complications / etiology. Radiation Injuries / diagnosis. Radiation Injuries / etiology. Radiotherapy, Conformal / adverse effects. Soft Tissue Neoplasms / complications. Soft Tissue Neoplasms / therapy

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  • (PMID = 19101106.001).
  • [ISSN] 1872-7727
  • [Journal-full-title] European journal of radiology
  • [ISO-abbreviation] Eur J Radiol
  • [Language] eng
  • [Publication-type] Controlled Clinical Trial; Journal Article
  • [Publication-country] Ireland
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18. Domínguez Menéndez JA, Aguirrezábal Arredondo A, del Cura Rodríguez JL, Zabala Landa RM: [Management of a desmoid tumor using percutaneous injection of 50% acetic acid]. Farm Hosp; 2006 Sep-Oct;30(5):320-1
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  • [Title] [Management of a desmoid tumor using percutaneous injection of 50% acetic acid].
  • [Transliterated title] Tratamiento de un tumor desmoide mediante inyección percutánea de ácido acético 50%
  • [MeSH-major] Abdominal Neoplasms / drug therapy. Acetic Acid / administration & dosage. Fibromatosis, Aggressive / drug therapy. Indicators and Reagents / administration & dosage

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  • (PMID = 17166071.001).
  • [ISSN] 1130-6343
  • [Journal-full-title] Farmacia hospitalaria : órgano oficial de expresión científica de la Sociedad Española de Farmacia Hospitalaria
  • [ISO-abbreviation] Farm Hosp
  • [Language] spa
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Spain
  • [Chemical-registry-number] 0 / Indicators and Reagents; Q40Q9N063P / Acetic Acid
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19. Signoroni S, Frattini M, Negri T, Pastore E, Tamborini E, Casieri P, Orsenigo M, Da Riva L, Radice P, Sala P, Gronchi A, Bertario L, Pierotti MA, Pilotti S: Cyclooxygenase-2 and platelet-derived growth factor receptors as potential targets in treating aggressive fibromatosis. Clin Cancer Res; 2007 Sep 1;13(17):5034-40
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  • [Title] Cyclooxygenase-2 and platelet-derived growth factor receptors as potential targets in treating aggressive fibromatosis.
  • PURPOSE: To explore the molecular bases of potential new pharmacologic targets in aggressive fibromatosis (desmoid tumor).
  • EXPERIMENTAL DESIGN: Tumor specimens from 14 patients surgically treated for aggressive fibromatosis (6 familial adenomatous polyposis and 8 sporadic cases), analyzed for adenomatous polyposis coli (APC) and CTNNB1 (beta-catenin) mutations, were further investigated for beta-catenin, cyclooxygenase-2 (COX-2), platelet-derived growth factor (PDGF) receptor alpha (PDGFRA)/PDGF receptor beta (PDGFRB), their cognate ligands (PDGFA and PDGFB), and KIT using a comprehensive immunohistochemical, biochemical, molecular, and cytogenetic approach.
  • CONCLUSIONS: Aggressive fibromatosis is characterized by WNT/oncogene pathway alterations triggering COX-2-mediated constitutive coactivation of PDGFRA and PDGFRB, and may therefore benefit from combined nonsteroidal anti-inflammatory drug + tyrosine kinase inhibitor treatment.
  • [MeSH-major] Cyclooxygenase 2 Inhibitors / therapeutic use. Fibromatosis, Aggressive / drug therapy. Receptors, Platelet-Derived Growth Factor / antagonists & inhibitors

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  • [ErratumIn] Clin Cancer Res. 2008 Jul 1;14(13):4354
  • (PMID = 17785554.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cyclooxygenase 2 Inhibitors; 0 / RNA, Messenger; 0 / beta Catenin; EC 1.14.99.1 / Cyclooxygenase 2; EC 2.7.10.1 / Receptors, Platelet-Derived Growth Factor
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20. Rüdiger HA, Ngan SY, Ng M, Powell GJ, Choong PF: Radiation therapy in the treatment of desmoid tumours reduces surgical indications. Eur J Surg Oncol; 2010 Jan;36(1):84-8
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  • [Title] Radiation therapy in the treatment of desmoid tumours reduces surgical indications.
  • BACKGROUND: While several modalities have been proposed for the treatment of desmoid tumour/aggressive fibromatosis, high local recurrence rates have been reported.
  • In patients who did not receive surgery but only radiation therapy, MRI showed a complete response in 20%, a partial response in 20%, and stable disease in 53% of cases.
  • [MeSH-major] Fibromatosis, Aggressive / radiotherapy
  • [MeSH-minor] Adult. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Magnetic Resonance Imaging. Male. Neoplasm Recurrence, Local. Positron-Emission Tomography. Radiotherapy Dosage. Radiotherapy, High-Energy

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  • [Copyright] Copyright (c) 2009 Elsevier Ltd. All rights reserved.
  • (PMID = 19682833.001).
  • [ISSN] 1532-2157
  • [Journal-full-title] European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
  • [ISO-abbreviation] Eur J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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21. Zisis C, Dountsis A, Nikolaides A, Dahabreh J: Desmoid tumors of the chest wall. Asian Cardiovasc Thorac Ann; 2006 Oct;14(5):359-62
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  • [Title] Desmoid tumors of the chest wall.
  • Chest wall desmoid tumors (DT) are rare pathologic entities with microscopic features similar to, or undistinguishable from, fibromas or fibrosarcomas.
  • A resection of the lesion was performed with negative margins of 4 cm around the tumor (wide resection).
  • One patient had a recurrence 15 months later, and was admitted for complementary resection, and remains disease-free for 5 years.
  • The rest 3 patients are disease-free for 6 months to 5 years.
  • Resection must include all adjacent, overlying and underlying musculature as well as soft tissues and any spare skin from the procedure should be used.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Neoplasm Recurrence, Local / surgery. Soft Tissue Neoplasms / surgery

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  • (PMID = 17005879.001).
  • [ISSN] 1816-5370
  • [Journal-full-title] Asian cardiovascular & thoracic annals
  • [ISO-abbreviation] Asian Cardiovasc Thorac Ann
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biocompatible Materials
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22. Dalén BP, Meis-Kindblom JM, Sumathi VP, Ryd W, Kindblom LG: Fine-needle aspiration cytology and core needle biopsy in the preoperative diagnosis of desmoid tumors. Acta Orthop; 2006 Dec;77(6):926-31
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  • [Title] Fine-needle aspiration cytology and core needle biopsy in the preoperative diagnosis of desmoid tumors.
  • BACKGROUND: Desmoid tumors have a tendency to recur locally, and traditionally they have been treated surgically.
  • No treatment is sometimes indicated, however; this requires a morphological diagnosis that is not based on a surgical specimen.
  • In this study we aimed to identify the diagnostic accuracy of needle and core biopsy for the morphological diagnosis of desmoid.
  • METHODS: We compared the diagnostic accuracy of fine-needle aspiration (FNA) and core needle biopsy (CNB) in 69 and 26 patients, respectively, who had had surgical resections for desmoid.
  • We also reviewed 15 additional cases that had been incorrectly diagnosed as desmoid on FNA but which had different diagnoses after surgery.
  • RESULTS: FNA-based diagnoses of desmoid/fibromatosis were rendered in 35 of 69 cases, and other benign spindle cell proliferations in 26 cases and spindle cell sarcoma in the remaining 4 cases.
  • All 26 CNBs were either suggested to correspond to desmoid (24) or other benign spindle cell lesions (2).
  • Of the 15 FNAs incorrectly diagnosed as desmoid, 2 were found to be sarcomas.
  • INTERPRETATION: FNA is fairly reliable for recognition of the benign nature of desmoids.
  • Occasional over- and under-diagnosis of malignancy can occur, however.
  • [MeSH-major] Fibromatosis, Abdominal / pathology. Fibromatosis, Aggressive / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biopsy, Fine-Needle. Biopsy, Needle. Child. Child, Preschool. Diagnosis, Differential. Humans. Male. Middle Aged. Sensitivity and Specificity

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  • (PMID = 17260203.001).
  • [ISSN] 1745-3674
  • [Journal-full-title] Acta orthopaedica
  • [ISO-abbreviation] Acta Orthop
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Sweden
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23. Janitzky A, Porsch M, Daher M, Küster D, Liehr UB: [Aggressive fibromatosis (desmoid tumor) : A rare differential diagnosis of metastasis of renal cell carcinoma]. Urologe A; 2010 Jan;49(1):81-3
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  • [Title] [Aggressive fibromatosis (desmoid tumor) : A rare differential diagnosis of metastasis of renal cell carcinoma].
  • [Transliterated title] Aggressive Fibromatose (Desmoidfibromatose) : Seltene Differentialdiagnose einer Nierenzellkarzinommetastase.
  • We report the case of a 65-year-old woman with an aggressive fibromatosis of the rectus abdominis muscle suspicious for a metastasis of renal cell carcinoma after tumor nephrectomy 3 years previously.
  • Aggressive fibromatoses (desmoid tumors) are rare semimalignant tumors of the connective tissue with local infiltration and destruction of tissue.
  • Complete resection is essential to avoid tumor relapse.
  • Aggressive fibromatosis must be considered in the differential diagnosis of renal cell carcinoma metastasis.
  • [MeSH-major] Abdominal Muscles / pathology. Carcinoma, Renal Cell / diagnosis. Carcinoma, Renal Cell / secondary. Fibromatosis, Abdominal / diagnosis. Kidney Neoplasms / diagnosis. Muscle Neoplasms / pathology
  • [MeSH-minor] Aged. Diagnosis, Differential. Female. Humans


24. Mendenhall WM, Zlotecki RA, Morris CG, Hochwald SN, Scarborough MT: Aggressive fibromatosis. Am J Clin Oncol; 2005 Apr;28(2):211-5
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  • [Title] Aggressive fibromatosis.
  • The purpose of this article is to review the pertinent literature and to define the optimal treatment of patients with aggressive fibromatosis.
  • Moderate-dose radiotherapy alone for gross disease or after a microscopically incomplete resection yields local control rates of approximately 75% to 80%.
  • Thus, the optimal management for aggressive fibromatosis depends on tumor location and extent.
  • Radiotherapy is indicated for patients with unresectable tumors; those with positive margins after resection should be considered for adjuvant radiotherapy depending on the location and extent of the tumor.
  • Pharmacologic treatment should be considered for patients with progressive disease after unsuccessful local-regional therapy.
  • [MeSH-major] Fibromatosis, Aggressive / therapy

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  • (PMID = 15803019.001).
  • [ISSN] 1537-453X
  • [Journal-full-title] American journal of clinical oncology
  • [ISO-abbreviation] Am. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 31
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25. Raja TA, Albadri S, Hood C: Case report: Rutherfurd syndrome associated with Marfan syndrome. Eur Arch Paediatr Dent; 2008 Sep;9(3):138-41
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  • BACKGROUND: Rutherfurd syndrome is a rare genetic disorder that is primarily characterised by the classical triad of gingival fibromatosis, delayed tooth eruption and corneal dystrophy.
  • Associated features of the condition include abnormally shaped teeth, mental retardation and aggressive behaviour.
  • The child and his father had a diagnosis of Rutherfurd Syndrome; a rare autosomal-dominant condition featuring corneal dystrophy, gingival hypertrophy, abnormally shaped teeth and delayed eruption.
  • [MeSH-major] Abnormalities, Multiple / diagnosis. Anodontia / diagnostic imaging. Fibromatosis, Gingival / diagnosis. Jaw, Edentulous, Partially / rehabilitation. Tooth, Unerupted / surgery
  • [MeSH-minor] Child, Preschool. Corneal Dystrophies, Hereditary / diagnosis. Denture, Partial. Follow-Up Studies. Humans. Male. Marfan Syndrome. Radiography. Syndrome. Tooth Abnormalities / diagnostic imaging. Tooth Abnormalities / surgery. Treatment Refusal

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  • (PMID = 18793596.001).
  • [ISSN] 1818-6300
  • [Journal-full-title] European archives of paediatric dentistry : official journal of the European Academy of Paediatric Dentistry
  • [ISO-abbreviation] Eur Arch Paediatr Dent
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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26. Arena S, Salamone S, Cianci R, Scollo C, Masucci R, Giannone G, Manusia M, Vigneri R, La Rosa GL: Aggressive fibromatosis of the neck initiated after thyroidectomy. J Endocrinol Invest; 2006 Jan;29(1):78-81
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  • [Title] Aggressive fibromatosis of the neck initiated after thyroidectomy.
  • Fine needle aspiration biopsy revealed the presence of fibroblast-like cells, partially with atypical features and no colloid: the cytological diagnosis was suspicious for an indeterminate (mesenchymal) neoplasm.
  • Histological diagnosis, after extensive surgery, indicated aggressive fibromatosis.
  • [MeSH-major] Fibromatosis, Aggressive / etiology. Goiter, Nodular / surgery

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  • (PMID = 16553038.001).
  • [ISSN] 0391-4097
  • [Journal-full-title] Journal of endocrinological investigation
  • [ISO-abbreviation] J. Endocrinol. Invest.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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27. Jensen SG, Krogdahl A, Godballe C: [Aggressive fibromatosis of the frontal sinus]. Ugeskr Laeger; 2009 Jan 26;171(5):324-5
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  • [Title] [Aggressive fibromatosis of the frontal sinus].
  • [Transliterated title] Aggressiv fibromatose i sinus frontalis.
  • Aggressive fibromatosis (AF) is a benign tumour with expansive and locally invasive growth.
  • The condition was initially mistaken for chronic sinusitis however computed tomography indicated tumour.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Paranasal Sinus Neoplasms / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Female. Frontal Sinusitis / diagnosis. Humans. Middle Aged


28. Kilçiksiz S, Gökçe T, Somali I, Duransoy A, Aydin A, Yiğit S: Combined administration of ethodolac, ascorbic acid and radiotherapy as adjuvant therapies in an extrathoracic desmoid tumor with gross postoperative residual disease; case report and review of the literature. J BUON; 2006 Jul-Sep;11(3):355-8
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  • [Title] Combined administration of ethodolac, ascorbic acid and radiotherapy as adjuvant therapies in an extrathoracic desmoid tumor with gross postoperative residual disease; case report and review of the literature.
  • A 37-year-old man was admitted with an extrathoracic desmoid tumor invading the brachial plexus.
  • Biopsy revealed desmoid tumor which was resected subtotally with the brachial plexus.
  • The tumor was clinically indiscernible 35 months post-radiotherapy.
  • [MeSH-major] Anti-Inflammatory Agents, Non-Steroidal / therapeutic use. Ascorbic Acid / therapeutic use. Etodolac / therapeutic use. Fibromatosis, Aggressive / therapy. Thoracic Neoplasms / therapy
  • [MeSH-minor] Adult. Brachial Plexus. Combined Modality Therapy. Humans. Male. Neoplasm, Residual. Thoracic Wall

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  • (PMID = 17309163.001).
  • [ISSN] 1107-0625
  • [Journal-full-title] Journal of B.U.ON. : official journal of the Balkan Union of Oncology
  • [ISO-abbreviation] J BUON
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Anti-Inflammatory Agents, Non-Steroidal; 2M36281008 / Etodolac; PQ6CK8PD0R / Ascorbic Acid
  • [Number-of-references] 26
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29. Mazeh H, Nissan A, Simanovsky N, Hiller N: Desmoid tumor causing duodenal obstruction. Isr Med Assoc J; 2006 Apr;8(4):288-9
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  • [Title] Desmoid tumor causing duodenal obstruction.
  • [MeSH-major] Duodenal Diseases / etiology. Duodenal Neoplasms / complications. Fibromatosis, Aggressive / complications. Intestinal Obstruction / etiology


30. Wunder JS, Nielsen TO, Maki RG, O'Sullivan B, Alman BA: Opportunities for improving the therapeutic ratio for patients with sarcoma. Lancet Oncol; 2007 Jun;8(6):513-24
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  • The ideal sequence of surgery and radiation for local management of soft-tissue sarcoma remains controversial on the basis of early versus late treatment complications, although preoperative radiation can provide the best results for improved long-term function.
  • New methods for radiation delivery and tumour sensitisation might provide further improvements.
  • However, metastatic disease is common, and conventional chemotherapy provides for only a narrow therapeutic window outside of a few responsive pathological subtypes.
  • Targeting underlying molecular events in specific sarcomas can provide for dramatic benefits, as has been seen with imatinib treatment for gastrointestinal stromal tumours and dermatofibrosarcoma protuberans.
  • Trials of agents targeting the cell cycle and angiogenesis in soft-tissue sarcomas, and of those targeting osteoclasts in bone sarcomas, are currently underway.
  • Biological data and preclinical studies support trials using inhibitors of hedgehog signalling in chondrosarcoma, inhibitors of wnt/beta-catenin in osteosarcoma and aggressive fibromatosis, and inhibitors of histone deacetylases in synovial sarcoma and Ewing sarcoma.

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  • [CommentIn] Lancet Oncol. 2007 Aug;8(8):667-8; author reply 668-9 [17679078.001]
  • [ErratumIn] Lancet Oncol. 2007 Aug;8(8):670
  • (PMID = 17540303.001).
  • [ISSN] 1470-2045
  • [Journal-full-title] The Lancet. Oncology
  • [ISO-abbreviation] Lancet Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P01-CA47179
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 92
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31. Suresh CS, Ali AA: Desmoid tumor of the tongue. Med Oral Patol Oral Cir Bucal; 2008 Dec;13(12):E761-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumor of the tongue.
  • Desmoid tumors are rare neoplasms accounting for 0.03% of all neoplasms and have an estimated incidence of 2 to 4 per million per year.
  • World Health Organization currently refers to all of the deep types of fibromatosis as desmoid-type of fibromatoses.
  • The term "desmoid" refers to the hard, tendon-like appearance of the tumor.
  • About fifty percent of desmoid tumors arise in the abdominal region.
  • The extra-abdominal desmoid tumors present a difficult problem in recognition and management especially because of the striking discrepancy between its deceptively harmless microscopic appearance and its potential to attain a large size, to recur, and to infiltrate neighboring tissues in the manner of a fibrosarcoma.
  • Desmoid tumors are very rare in the oral cavity with less than 5% of cases constituting oral soft tissue fibromatosis.
  • A 22-year old mentally retarded female patient with desmoid tumor occurring in the tongue is presented here.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Tongue Neoplasms / pathology

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  • (PMID = 19047962.001).
  • [ISSN] 1698-6946
  • [Journal-full-title] Medicina oral, patología oral y cirugía bucal
  • [ISO-abbreviation] Med Oral Patol Oral Cir Bucal
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Spain
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32. Bonvalot S, Rimareix F, Bouzaiene H: [Surgical news of soft tissue sarcomas, fibromatosis and GIST]. Bull Cancer; 2010 Jun;97(6):645-56
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  • [Title] [Surgical news of soft tissue sarcomas, fibromatosis and GIST].
  • [Transliterated title] Actualités chirurgicales des sarcomes, des fibromatoses et des tumeurs stromales gastro-intestinales.
  • Concerning desmoids, authors address the question whether surgery and other aggressive treatments should systematically be part of first-line treatment, as growth arrest occurred in 2/3 of non-operated patients.
  • In the same way, surgical indications for gastrointestinal stromal tumors evolved: with the development of investigations, more micro-GIST are discovered, rising the question of wait and see policy for some of them.
  • Secondary excision of residual disease has been shown to be related to a good prognosis in responding patients to imatinib, but it is still not demonstrated whether this is due to surgery itself or to a selection bias.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Gastrointestinal Stromal Tumors / surgery. Retroperitoneal Neoplasms / surgery. Sarcoma / surgery

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  • (PMID = 20547479.001).
  • [ISSN] 1769-6917
  • [Journal-full-title] Bulletin du cancer
  • [ISO-abbreviation] Bull Cancer
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate
  • [Number-of-references] 54
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33. Dufresne A, Bertucci F, Penel N, Le Cesne A, Bui B, Tubiana-Hulin M, Ray-Coquard I, Cupissol D, Chevreau C, Perol D, Goncalves A, Jimenez M, Bringuier PP, Blay JY: Identification of biological factors predictive of response to imatinib mesylate in aggressive fibromatosis. Br J Cancer; 2010 Aug 10;103(4):482-5
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  • [Title] Identification of biological factors predictive of response to imatinib mesylate in aggressive fibromatosis.
  • BACKGROUND: Imatinib induces responses and disease stabilisations in non-resectable patients with aggressive fibromatosis (AF).
  • Pre-treatment lymphopenia (<1500/microl) and tumour size >120 mm correlated with shorter PFS in univariate and multivariate analyses.
  • CONCLUSION: Our findings show that a baseline biological characteristic of the patient is the major parameter influencing response to imatinib in aggressive fibromatosis.
  • Tumour characteristics, including the presence of a KIT exon 10 M541L variant, may influence tumour control but this needs to be confirmed and better explained.
  • [MeSH-major] Biomarkers, Tumor / analysis. Fibromatosis, Aggressive / drug therapy. Proto-Oncogene Proteins c-kit / analysis


34. Basdanis G, Papadopoulos VN, Panidis S, Tzeveleki I, Karamanlis E, Mekras A, Apostolidis S, Michalopoulos A: Desmoid tumor of mesentery in familial adenomatous polyposis: a case report. Tech Coloproctol; 2010 Nov;14 Suppl 1:S61-2
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumor of mesentery in familial adenomatous polyposis: a case report.
  • While a mass in the left lateral abdominal region was palpated.
  • Pathological examination revealed a desmoid tumor of the mesentery.
  • [MeSH-major] Adenomatous Polyposis Coli / surgery. Fibromatosis, Aggressive / surgery. Neoplasms, Second Primary / surgery. Peritoneal Neoplasms / surgery


35. de Bree E, Keus R, Melissas J, Tsiftsis D, van Coevorden F: Desmoid tumors: need for an individualized approach. Expert Rev Anticancer Ther; 2009 Apr;9(4):525-35
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  • [Title] Desmoid tumors: need for an individualized approach.
  • Desmoid tumor, also known as aggressive fibromatosis or desmoid-type fibromatosis, is a rare monoclonal, fibroblastic proliferation arising in musculoaponeurotic structures.
  • Although histologically benign, desmoids are often locally invasive and associated with a high local recurrence rate after resection.
  • Since it is a heterogeneous disease, in particular regarding clinical presentation, anatomic location and biological behavior, treatment should be individualized to reduce local tumor control failure with concurrently acceptable morbidity and preservation of quality of life.
  • Many issues regarding optimal treatment of desmoids remain controversial.
  • Radiotherapy for gross disease is considerably effective, but is associated with a relatively high rate of complications, which are usually mild or moderate and radiation dose dependent.
  • Risk factors for local tumor control failure include young age, large size, presentation as recurrent disease, limb/girdle or intra-abdominal location, involved surgical margins, omission of radiotherapy, radiation dose less than 50 Gy and insufficient radiation field size.
  • Increased comprehension of the pathogenesis and biological behavior of desmoids resulted in the emerging applicability of systemic therapies and a wait-and-see policy.
  • Considering the significant morbidity of surgery and/or radiotherapy for certain locations, especially mutilation and loss of function, and the tumor's natural history, which is often characterized by prolonged periods of stability or even regression, a period of watchful waiting may compose the most appropriate management in selected asymptomatic patients.
  • Attempts to complete eradication of the disease may be worse than the disease itself.
  • [MeSH-major] Fibromatosis, Aggressive / therapy
  • [MeSH-minor] Adult. Age Factors. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Case Management. Child. Combined Modality Therapy. Cyclooxygenase 2 Inhibitors / therapeutic use. Female. Humans. Male. Neoplasm Recurrence, Local. Pregnancy. Pregnancy Complications, Neoplastic / surgery. Radiotherapy / adverse effects. Radiotherapy, Adjuvant. Referral and Consultation. Risk Factors

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  • (PMID = 19374605.001).
  • [ISSN] 1744-8328
  • [Journal-full-title] Expert review of anticancer therapy
  • [ISO-abbreviation] Expert Rev Anticancer Ther
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Cyclooxygenase 2 Inhibitors
  • [Number-of-references] 89
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36. Meneu Díaz JC, Moreno González E, García García JI, Moreno A, Montejo JC, Colina F, Pérez B, Rodríguez S, Abradelos de Usera M, Garfia C, Fundora Y, Jiménez Galanes S, Lumbreras C, León M, Pérez Cerdá F, Solís-Herruzo JA: First Spanish series of intestinal transplantation in adult recipients. Rev Esp Enferm Dig; 2006 Oct;98(10):723-39
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  • RESULTS: to this date 5 transplants have been carried out in 4 patients (2 retransplants, 2 desmoid tumors, 1 short bowel syndrome after excision as a result of mesenteric ischemia).

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  • (PMID = 17094721.001).
  • [ISSN] 1130-0108
  • [Journal-full-title] Revista española de enfermedades digestivas : organo oficial de la Sociedad Española de Patología Digestiva
  • [ISO-abbreviation] Rev Esp Enferm Dig
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Spain
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37. Coco DP, Hirsch MS, Hornick JL: Smoothelin is a specific marker for smooth muscle neoplasms of the gastrointestinal tract. Am J Surg Pathol; 2009 Dec;33(12):1795-801
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  • Smoothelin expression in mesenchymal tumors of the gastrointestinal (GI) tract has not been evaluated earlier.
  • The purpose of this study was to determine whether immunostaining for smoothelin could help distinguish smooth muscle neoplasms from their morphologic mimics, particularly KIT-negative gastrointestinal stromal tumors (GISTs), desmin-positive GISTs, and desmoid fibromatosis.
  • A total of 150 mesenchymal neoplasms of the GI tract, abdominal cavity, and retroperitoneum were retrieved from consult and surgical pathology archives, including 54 GISTs (8 KIT-negative; 13 desmin-positive), 17 GI leiomyosarcomas (LMS), 11 GI mural leiomyomas, 13 leiomyomas of the muscularis mucosae, 12 gastric schwannomas, 15 inflammatory myofibroblastic tumors, 9 cases of mesenteric desmoid fibromatosis, 10 dedifferentiated liposarcomas, and 9 malignant peripheral nerve sheath tumors.
  • Cytoplasmic expression of smoothelin was present in all 24 (100%) benign smooth muscle tumors (mural leiomyomas and leiomyomas of the muscularis mucosae).
  • None of the GISTs, desmoid tumors, inflammatory myofibroblastic tumors, schwannomas, dedifferentiated liposarcomas, or malignant peripheral nerve sheath tumors showed cytoplasmic reactivity for smoothelin.
  • Nuclear expression of smoothelin was not detected in any of the other tumor types examined.
  • In summary, diffuse cytoplasmic staining for smoothelin is highly sensitive and specific for benign leiomyomas of the GI tract.
  • Aberrant nuclear expression is common in GI LMS and may also be seen in GISTs, especially epithelioid and mixed-type tumors.
  • These findings suggest that the extent and pattern of smoothelin expression may help differentiate between benign and malignant mesenchymal tumors of the GI tract, and may be useful in distinguishing leiomyomas from KIT-negative and/or desmin-positive GISTs.
  • [MeSH-major] Biomarkers, Tumor / analysis. Cytoskeletal Proteins / analysis. Gastrointestinal Neoplasms / chemistry. Muscle Proteins / analysis. Smooth Muscle Tumor / chemistry
  • [MeSH-minor] Cell Differentiation. Cell Nucleus / chemistry. Cytoplasm / chemistry. Desmin / analysis. Diagnosis, Differential. Humans. Immunohistochemistry. Neoplasm Staging. Predictive Value of Tests. Proto-Oncogene Proteins c-kit / analysis. Sensitivity and Specificity

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  • (PMID = 19950405.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Cytoskeletal Proteins; 0 / Desmin; 0 / Muscle Proteins; 0 / SMTN protein, human; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
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38. Shimoyama T, Hiraoka K, Shoda T, Hamada T, Fukushima N, Nagata K: Multicentric extra-abdominal desmoid tumors arising in bilateral lower limbs. Rare Tumors; 2010;2(1):e12
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  • [Title] Multicentric extra-abdominal desmoid tumors arising in bilateral lower limbs.
  • Extra-abdominal desmoid tumors preferentially affect the shoulders, arms, backs, buttocks, and thighs of young adults.
  • Multicentric occurrence is rather rare but seems to be another distinctive feature of extra-abdominal desmoid tumors.
  • In this article we report a rare case of multicentric extra-abdominal desmoid tumors arising in bilateral lower limbs.

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  • (PMID = 21139941.001).
  • [ISSN] 2036-3613
  • [Journal-full-title] Rare tumors
  • [ISO-abbreviation] Rare Tumors
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
  • [Other-IDs] NLM/ PMC2994500
  • [Keywords] NOTNLM ; bilateral limbs / desmoid / radiation
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39. Teo HE, Peh WC, Shek TW: Case 84: desmoid tumor of the abdominal wall. Radiology; 2005 Jul;236(1):81-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Case 84: desmoid tumor of the abdominal wall.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Humans. Magnetic Resonance Imaging. Tomography, X-Ray Computed

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  • (PMID = 15987965.001).
  • [ISSN] 0033-8419
  • [Journal-full-title] Radiology
  • [ISO-abbreviation] Radiology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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40. Ekwueme KC, McCarthy E, Healey C, Ellis A, Rooney PS: A novel frameshift mutation in the APC gene at exon 15 in familial adenomatous polyposis (FAP) with desmoid tumour. Scand J Gastroenterol; 2007 Jun;42(6):788-90
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  • [Title] A novel frameshift mutation in the APC gene at exon 15 in familial adenomatous polyposis (FAP) with desmoid tumour.
  • [MeSH-major] Adenomatous Polyposis Coli / genetics. Exons. Fibromatosis, Aggressive / complications. Frameshift Mutation. Genes, APC

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  • (PMID = 17506007.001).
  • [ISSN] 0036-5521
  • [Journal-full-title] Scandinavian journal of gastroenterology
  • [ISO-abbreviation] Scand. J. Gastroenterol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Norway
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41. Joyner DE, Trang SH, Damron TA, Aboulafia AJ, Cummings JE, Randall RL: Desmoid cell motility is induced in vitro by rhEGF. J Orthop Res; 2009 Sep;27(9):1258-62
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  • [Title] Desmoid cell motility is induced in vitro by rhEGF.
  • Desmoid tumors are benign but locally invasive myofibroblastic lesions that arise predominantly in the abdominal wall or shoulder and are prone to aggressive local recurrences.
  • A perceived association between desmoid activity and the expression of growth factors during pregnancy or following trauma suggests a cause-and-effect relationship between growth factor stimulation and desmoid invasiveness.
  • We used Boyden Chambers to quantify cell motility in order to determine the effect of growth factor stimulation on desmoid cell migration.
  • Desmoid cell cultures were treated under serum-free conditions with epidermal growth factor (rhEGF) or transforming growth factor alpha (rhTGFalpha).
  • The experiments demonstrated a direct dose-dependent relationship between rhEGF stimulation and desmoid motility.
  • [MeSH-major] Cell Movement / drug effects. Epidermal Growth Factor / pharmacology. Fibromatosis, Aggressive / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Antigens, CD29 / genetics. Benzamides / pharmacology. Cell Division / drug effects. Cell Line, Tumor. Culture Media, Serum-Free / pharmacology. Dioxoles / pharmacology. Enzyme Inhibitors / pharmacology. Gene Expression Regulation, Neoplastic. Humans. In Vitro Techniques. Matrix Metalloproteinase 1 / genetics. Matrix Metalloproteinase 3 / genetics. Quinazolines. RNA, Messenger / metabolism. RNA, Small Interfering. Receptor, Epidermal Growth Factor / genetics. Receptors, Transforming Growth Factor beta / antagonists & inhibitors. Recombinant Proteins / pharmacology. STAT3 Transcription Factor / genetics. Transforming Growth Factor alpha / genetics. Transforming Growth Factor alpha / pharmacology. Tyrphostins / pharmacology

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  • [Copyright] (c) 2009 Orthopaedic Research Society.
  • (PMID = 19242968.001).
  • [ISSN] 1554-527X
  • [Journal-full-title] Journal of orthopaedic research : official publication of the Orthopaedic Research Society
  • [ISO-abbreviation] J. Orthop. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / 4-(5-benzo(1,3)dioxol-5-yl-4-pyridin-2-yl-1H-imidazol-2-yl)benzamide; 0 / Antigens, CD29; 0 / Benzamides; 0 / Culture Media, Serum-Free; 0 / Dioxoles; 0 / Enzyme Inhibitors; 0 / Quinazolines; 0 / RNA, Messenger; 0 / RNA, Small Interfering; 0 / Receptors, Transforming Growth Factor beta; 0 / Recombinant Proteins; 0 / STAT3 Transcription Factor; 0 / STAT3 protein, human; 0 / Transforming Growth Factor alpha; 0 / Tyrphostins; 170449-18-0 / tyrphostin AG 1478; 62229-50-9 / Epidermal Growth Factor; EC 2.7.10.1 / Receptor, Epidermal Growth Factor; EC 3.4.24.17 / Matrix Metalloproteinase 3; EC 3.4.24.7 / Matrix Metalloproteinase 1
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42. Nieuwenhuis MH, Hartgrink HH, Meijer S, Menko FH, Vasen HF: [Desmoid tumour as indication of familial adenomatous polyposis]. Ned Tijdschr Geneeskd; 2010;154:A2235
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Desmoid tumour as indication of familial adenomatous polyposis].
  • In two patients, a man aged 43 and a woman aged 40 years, who presented with a desmoid tumour, familial adenomatous polyposis (FAP) was diagnosed three and six years later, respectively.
  • Desmoid-type fibromatoses usually develop sporadically, but may also be an extracolonic manifestation of FAP.
  • All patients with desmoids diagnosed who are under age 60, or with desmoids located intra-abdominally or in the abdominal wall, should be referred for colonic and genetic evaluation.
  • In all further patients with a desmoid tumour, the possibility of FAP should be considered and patient data and the family history should be evaluated thoroughly.
  • [MeSH-major] Adenomatous Polyposis Coli / diagnosis. Adenomatous Polyposis Coli / pathology. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / pathology
  • [MeSH-minor] Adult. Colonic Neoplasms / diagnosis. Colonic Neoplasms / secondary. Colonic Neoplasms / surgery. Female. Genes, APC. Genetic Predisposition to Disease. Humans. Male. Mutation. Neoplasm Metastasis. Rectal Neoplasms / diagnosis. Rectal Neoplasms / secondary. Rectal Neoplasms / surgery

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  • (PMID = 20977806.001).
  • [ISSN] 1876-8784
  • [Journal-full-title] Nederlands tijdschrift voor geneeskunde
  • [ISO-abbreviation] Ned Tijdschr Geneeskd
  • [Language] dut
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Netherlands
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43. Roeder F, Timke C, Oertel S, Hensley FW, Bischof M, Muenter MW, Weitz J, Buchler MW, Lehner B, Debus J, Krempien R: Intraoperative electron radiotherapy for the management of aggressive fibromatosis. Int J Radiat Oncol Biol Phys; 2010 Mar 15;76(4):1154-60
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  • [Title] Intraoperative electron radiotherapy for the management of aggressive fibromatosis.
  • PURPOSE: We analyzed our experience with intraoperative electron radiotherapy (IOERT) followed by moderate doses of external beam radiotherapy (EBRT) after organ-sparing surgery in patients with primary or recurrent aggressive fibromatosis.
  • Median tumor size was 9 cm.
  • RESULTS: After a median follow-up of 32 months (range, 3-139 months), no disease-related deaths occurred.
  • CONCLUSION: Introduction of IOERT into a multimodal treatment approach in patients with aggressive fibromatosis is feasible with low toxicity and yielded good local control rates even in patients with microscopical or gross residual disease.
  • [MeSH-major] Electrons / therapeutic use. Fibromatosis, Aggressive / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Feasibility Studies. Female. Humans. Intraoperative Period. Male. Middle Aged. Neoplasm Recurrence, Local / mortality. Neoplasm Recurrence, Local / surgery. Neoplasms, Multiple Primary / surgery. Radiotherapy Dosage. Salvage Therapy / methods. Survival Rate. Young Adult

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
  • (PMID = 19647952.001).
  • [ISSN] 1879-355X
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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44. Oguz M, Bedirli A, Gultekin A, Dursun A, Mentes BB: Desmoid tumor arising at the colostomy site after abdominoperineal resection for rectal carcinoma: report of a case. Dis Colon Rectum; 2006 Sep;49(9):1445-8
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  • [Title] Desmoid tumor arising at the colostomy site after abdominoperineal resection for rectal carcinoma: report of a case.
  • A 69-year-old male who developed a desmoid tumor at the site of his end colostomy after abdominoperineal resection for rectal carcinoma is reported.
  • The tumor was resected with wide margins.
  • Histopathology revealed desmoid tumor of the anterior abdominal wall with no malignant features.
  • To our knowledge, this is the first case of solitary desmoid tumor of anterior abdominal wall at the colostomy site reported in the literature.
  • [MeSH-major] Abdominal Wall. Colostomy. Fibromatosis, Aggressive / pathology. Muscle Neoplasms / pathology. Neoplasms, Second Primary / pathology. Rectal Neoplasms / surgery

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  • (PMID = 16897327.001).
  • [ISSN] 0012-3706
  • [Journal-full-title] Diseases of the colon and rectum
  • [ISO-abbreviation] Dis. Colon Rectum
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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45. Douira-Khomsi W, Mascard E, Adamsbaum C: [A lumbar cutaneous mass in a child]. Arch Pediatr; 2009 Sep;16(9):1295-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Skin Neoplasms / diagnosis
  • [MeSH-minor] Child. Diagnosis, Differential. Female. Gardner Syndrome / diagnosis. Humans. Lumbosacral Region / pathology

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  • (PMID = 19586759.001).
  • [ISSN] 1769-664X
  • [Journal-full-title] Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
  • [ISO-abbreviation] Arch Pediatr
  • [Language] fre
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] France
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46. Desai SR, Dombale VD, Janugade HB: Infantile fibromatosis (desmoid type)--a case report. Indian J Pathol Microbiol; 2005 Jul;48(3):379-80
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Infantile fibromatosis (desmoid type)--a case report.
  • Infantile fibromatosis represents the childhood counter part of musculoaponeurotic fibromatosis & arises as a solitary mass in skeletal muscle, adjacent fascia, aponeurosis or periosteum.
  • Microscopically it exists in two forms diffuse (mesenchymal) & desmoid.
  • The less common desmoid form rarely occurs in infancy.
  • The differential diagnosis of this type is infantile fibrosarcoma.
  • The tumor may locally recur if inadequately excised.
  • We report a case of infantile fibromatosis of desmoid type occurring in 10 months male child for its extreme rarity.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Neck Muscles / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 16761760.001).
  • [ISSN] 0377-4929
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
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47. Lefevre JH, Parc Y, Kernéis S, Goasguen N, Benis M, Parc R, Tiret E: Risk factors for development of desmoid tumours in familial adenomatous polyposis. Br J Surg; 2008 Sep;95(9):1136-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Risk factors for development of desmoid tumours in familial adenomatous polyposis.
  • BACKGROUND: Desmoid tumours (DTs) are the primary cause of death of patients with familial adenomatous polyposis (FAP) following restorative proctocolectomy.
  • DT sites were mesenteric (73 tumours), abdominal wall (44) and extra-abdominal (seven).
  • Although DTs appeared after colectomy in 34 patients, the type of surgery did not influence the risk of DT.
  • [MeSH-major] Abdominal Neoplasms / etiology. Adenomatous Polyposis Coli / complications. Fibromatosis, Abdominal / etiology. Fibromatosis, Aggressive / etiology. Proctocolectomy, Restorative


48. Eralp L, Toker B, Akgül T, Ozger H, Kocaoğlu M, Hayat S: [Applications of external fixation for management of complications associated with musculoskeletal tumors and related surgery]. Acta Orthop Traumatol Turc; 2009 May-Jul;43(3):219-28
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  • [Title] [Applications of external fixation for management of complications associated with musculoskeletal tumors and related surgery].
  • OBJECTIVES: We evaluated the results of, and the course of treatment with, external fixation (EF) in treating complications associated with bone tumors and related surgery.
  • Histologic diagnoses were osteosarcoma (n=3), Ewing's sarcoma (n=3), hereditary multiple exostosis (n=3), chondrosarcoma (n=2), synovial sarcoma (n=2), Ollier's disease, giant cell tumor of bone, desmoid fibroma, chondromyxoid fibroma, and enchondroma.
  • Complications secondary to bone tumors (n=4) and occurring following limb salvage surgery (n=14) were treated with Ilizarov circular EF in nine patients, unilateral EF in six patients, and both in three patients.
  • The second group consisted of six patients who had shortening secondary to tumor surgery.
  • The third group included four patients with deformity and shortening secondary to multiple exostosis (n=3) and Ollier's disease.
  • CONCLUSION: The use of EF in the management of complications associated with bone tumors and related surgery yields successful results especially in young patients.

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  • (PMID = 19717939.001).
  • [ISSN] 1017-995X
  • [Journal-full-title] Acta orthopaedica et traumatologica turcica
  • [ISO-abbreviation] Acta Orthop Traumatol Turc
  • [Language] tur
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Turkey
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49. Min KW: Interstitial cells of Cajal (pICC) in the pancreas. J Cell Mol Med; 2005 Jul-Sep;9(3):737; author reply 738-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-minor] Animals. Antigens, CD / analysis. Fibromatosis, Aggressive / pathology. Humans

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  • [CommentOn] J Cell Mol Med. 2005 Jan-Mar;9(1):169-90 [15784175.001]
  • (PMID = 16202221.001).
  • [ISSN] 1582-1838
  • [Journal-full-title] Journal of cellular and molecular medicine
  • [ISO-abbreviation] J. Cell. Mol. Med.
  • [Language] eng
  • [Publication-type] Comment; Letter
  • [Publication-country] Romania
  • [Chemical-registry-number] 0 / Antigens, CD; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
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50. Li CF, MacDonald JR, Wei RY, Ray J, Lau K, Kandel C, Koffman R, Bell S, Scherer SW, Alman BA: Human sterile alpha motif domain 9, a novel gene identified as down-regulated in aggressive fibromatosis, is absent in the mouse. BMC Genomics; 2007 Apr 03;8:92
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  • [Title] Human sterile alpha motif domain 9, a novel gene identified as down-regulated in aggressive fibromatosis, is absent in the mouse.
  • In the mesenchymal neoplasm, aggressive fibromatosis, subtractive hybridization identified sterile alpha motif domain 9 (SAMD9) as a substantially down regulated gene in neoplasia.
  • SAMD9 is expressed at a lower level in a variety of neoplasms associated with beta-catenin stabilization, such as aggressive fibromatosis, breast, and colon cancers.
  • Over expression of SAMD9 in the colon cancer cell line, SW480, reduces the volume of tumors formed when transplanted into immune-deficient mice.
  • [MeSH-major] Down-Regulation / genetics. Fibromatosis, Aggressive / genetics. Gene Expression Regulation, Neoplastic. Proteins / genetics. Proteins / physiology
  • [MeSH-minor] Animals. Base Sequence. Breast Neoplasms / pathology. Cell Line. Cell Proliferation. Colonic Neoplasms / pathology. Cytoplasm / chemistry. Female. Humans. Male. Mice. Molecular Sequence Data. Rats. Species Specificity. Tumor Cells, Cultured. Tumor Suppressor Proteins / genetics

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  • (PMID = 17407603.001).
  • [ISSN] 1471-2164
  • [Journal-full-title] BMC genomics
  • [ISO-abbreviation] BMC Genomics
  • [Language] eng
  • [Databank-accession-numbers] GENBANK/ AF445355/ AF453311/ AF474973/ AY195582/ AY195583/ AY195584/ AY195585/ AY195586/ AY195587/ DQ068177
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Proteins; 0 / SAMD9 protein, human; 0 / SAMD9L protein, human; 0 / Tumor Suppressor Proteins
  • [Other-IDs] NLM/ PMC1855325
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51. Ramos-Font C, Santiago Chinchilla A, Rebollo Aguirre AC, Rodríguez Fernández A, Medina Benítez A, Llamas Elvira JM: [Desmoid tumor of the thoraco-abdominal wall characterized with 18F-fluorodeoxyglucose PET/ CT scan. Correlation with magnetic resonance and bone scintigraphy. Review of the literature]. Rev Esp Med Nucl; 2009 Mar-Apr;28(2):70-3
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  • [Title] [Desmoid tumor of the thoraco-abdominal wall characterized with 18F-fluorodeoxyglucose PET/ CT scan. Correlation with magnetic resonance and bone scintigraphy. Review of the literature].
  • [Transliterated title] Tumor desmoide de la pared torácico-abdominal. Caracterización con PET-TAC con 18F-fluorodesoxiglucosa y correlación con resonancia magnética y gammagrafía ósea. Revisión de la literatura.
  • Desmoid tumours are uncommon benign tumours but with aggressive behaviour, resulting from the proliferation of well-differentiated fibroblasts.
  • Clinically, they present as a painless firm soft tissue mass.
  • When planning treatment, it is important to assess the correct tumour extent to permit curative radical surgery minimizing possible local recurrence.
  • Different imaging techniques help to characterise these tumours.
  • At present, magnetic resonance is the tool of choice for studying these tumours.
  • We present the case of a patient with a desmoid tumour of the abdominal-chest wall and we review the related literature.
  • [MeSH-major] Abdominal Neoplasms / radionuclide imaging. Abdominal Wall / radionuclide imaging. Bone Neoplasms / radionuclide imaging. Fibromatosis, Aggressive / radionuclide imaging. Fluorine Radioisotopes. Fluorodeoxyglucose F18. Muscle Neoplasms / radionuclide imaging. Positron-Emission Tomography. Radiopharmaceuticals. Rectus Abdominis / radionuclide imaging. Ribs / radionuclide imaging. Tomography, X-Ray Computed
  • [MeSH-minor] Adult. Humans. Magnetic Resonance Imaging. Male. Neoplasm Invasiveness. Thoracic Wall / pathology. Thoracic Wall / radiography. Thoracic Wall / radionuclide imaging

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  • (PMID = 19406052.001).
  • [ISSN] 0212-6982
  • [Journal-full-title] Revista española de medicina nuclear
  • [ISO-abbreviation] Rev Esp Med Nucl
  • [Language] spa
  • [Publication-type] Case Reports; Comparative Study; English Abstract; Journal Article; Review
  • [Publication-country] Spain
  • [Chemical-registry-number] 0 / Fluorine Radioisotopes; 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
  • [Number-of-references] 22
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52. Ferchichi L, Kourda N, Zermani R, Aouem J, Zaouche A, Abdjellil Z, Najah N, Baltagi Ben Jilani S: [Extragastrointestinal stromal tumors: a report of 4 cases]. Ann Chir; 2006 Apr;131(4):271-5
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  • [Title] [Extragastrointestinal stromal tumors: a report of 4 cases].
  • [Transliterated title] Les tumeurs stromales extradigestives: à propos de quatre observations.
  • Gastrointestinal stromal tumors (GIST) are mesenchymal tumors that arise from the wall of the gastrointestinal tract expressing CD117 and/or the CD34.
  • Similar tumors were described in the soft tissue of the abdomen and are so-called extragastrointestinal stromal tumors (EGIST).
  • The tumors occurred in 2 women and 2 men, who ranged in age from 42 to 71 years.
  • Three tumors arose from the soft tissue of the abdominal cavity, and the remainder arose from the retroperitoneum.
  • Three cases were composed purely of short fusiform cells tumors, the last case showed an epithelioid pattern.
  • All the tumors expressed CD117.
  • Immunohistochemistry is useful in distinguishing EGIST from other mesenchymal tumors.
  • Intra-abdominal aggressive fibromatosis may express actin and CD117 but catenin is also positive, leiomyosarcoma expresses the actin and/or desmin but CD117 is usually negative, retroperitoneal dedifferentiated liposarcoma is CD117 negative and PS100 positive, inflammatory myofibroblastic tumor is negative for CD117 and CD34.
  • The solitary fibrous tumor expresses CD34 and is negative to CD117.
  • Some tumors expressing CD117 such desmoplastic round cell tumor should not be confused with EGIST.
  • On the occasion of these four observations, we will discuss the clinical aspects and the main differential diagnoses of this tumor.
  • [MeSH-major] Abdominal Neoplasms. Soft Tissue Neoplasms
  • [MeSH-minor] Adult. Aged. Female. Gastrointestinal Stromal Tumors. Humans. Male. Middle Aged. Retrospective Studies

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  • (PMID = 16472759.001).
  • [ISSN] 0003-3944
  • [Journal-full-title] Annales de chirurgie
  • [ISO-abbreviation] Ann Chir
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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53. Dalal AK, Singal R, Dalal U, Attri AK, Sahu P, Gupta A: An unusual case of chest wall desmoid tumor. Indian J Surg; 2010 Jul;72(Suppl 1):336-8
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  • [Title] An unusual case of chest wall desmoid tumor.
  • Desmoid tumor of the chest wall are uncommon fibromatous tumors characterized by their local invasion and frequent recurrences.
  • Extra-abdominal sites are mainly the shoulder girdle, the pelvic girdle and distal ends of the lower limbs.
  • The chest wall represents 8-10% of cases and the tumor is exceptionally intrathoracic.
  • We present here a rare case of a large desmoid tumor of left antero-lateral chest wall.
  • In the presented case, wide excision of the tumor and chest wall reconstruction was done.

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  • (PMID = 23133290.001).
  • [ISSN] 0972-2068
  • [Journal-full-title] The Indian journal of surgery
  • [ISO-abbreviation] Indian J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] India
  • [Other-IDs] NLM/ PMC3451859
  • [Keywords] NOTNLM ; Chest wall / Extra abdominal tumor / Surgery
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54. Rix TE: A 10-year review of surgery for desmoid disease associated with familial adenomatous polyposis (Br J Surg 2006; 93; 1258-1264). Br J Surg; 2007 Feb;94(2):250-1
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  • [Title] A 10-year review of surgery for desmoid disease associated with familial adenomatous polyposis (Br J Surg 2006; 93; 1258-1264).
  • [MeSH-major] Abdominal Neoplasms / therapy. Adenomatous Polyposis Coli / complications. Antineoplastic Agents / adverse effects. Fibromatosis, Aggressive / therapy. Sulindac / adverse effects. Tamoxifen / adverse effects

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  • [CommentIn] Br J Surg. 2007 Mar;94(3):385-6 [17315281.001]
  • [CommentOn] Br J Surg. 2006 Oct;93(10):1258-64 [16952208.001]
  • (PMID = 17256816.001).
  • [ISSN] 0007-1323
  • [Journal-full-title] The British journal of surgery
  • [ISO-abbreviation] Br J Surg
  • [Language] eng
  • [Publication-type] Comment; Letter
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 094ZI81Y45 / Tamoxifen; 184SNS8VUH / Sulindac
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55. Ibrahim M, Sandogji H, Allam A: Huge intrathoracic desmoid tumor. Ann Thorac Med; 2009 Jul;4(3):146-8
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  • [Title] Huge intrathoracic desmoid tumor.
  • Desmoid tumors are soft-tissue neoplasms arising from fascial or musculo-aponeurotic structures.
  • Most reported thoracic desmoid tumors originate from the chest wall.
  • However, intrathoracic desmoid tumors are rare.
  • The patient was diagnosed to have a huge intrathoracic desmoid tumor, which was successfully resected.

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  • (PMID = 19641648.001).
  • [ISSN] 1817-1737
  • [Journal-full-title] Annals of thoracic medicine
  • [ISO-abbreviation] Ann Thorac Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] India
  • [Other-IDs] NLM/ PMC2714571
  • [Keywords] NOTNLM ; Chest wall / desmoid / fibromatosis / intrathoracic / tumor
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61. Chummun S, McLean NR, Abraham S, Youseff M: Desmoid tumour of the breast. J Plast Reconstr Aesthet Surg; 2010 Feb;63(2):339-45
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  • [Title] Desmoid tumour of the breast.
  • Desmoid tumour of the breast is a rare fibroblastic tumour whose spectrum ranges from being locally inert to aggressive and destructive, and represents 0.2% of all breast tumours.
  • Ultrasound showed a well-defined, hypoechoic mass arising within the muscles of the anterior chest, deep beneath the implant and not involving the underlying rib.
  • A provisional diagnosis of scarring and fibroblastic proliferation was made.
  • A final diagnosis of aggressive fibromatosis was made.
  • Although the association of desmoid tumour and breast implants has been described, this case is unique as the FNA was highly suggestive of a silicone granuloma and the diagnosis of desmoid tumour was made on definitive pathology.
  • The aetiology of desmoid tumours is reviewed and current treatment modalities discussed.
  • [MeSH-major] Breast Neoplasms / diagnosis. Breast Neoplasms / surgery. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / surgery

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  • [Copyright] 2008 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.
  • (PMID = 19059821.001).
  • [ISSN] 1878-0539
  • [Journal-full-title] Journal of plastic, reconstructive & aesthetic surgery : JPRAS
  • [ISO-abbreviation] J Plast Reconstr Aesthet Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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62. Spree E, Niemann U, Wenisch H: [Sporadical extraperitoneal desmoid tumors--review and report on 4 cases]. Zentralbl Chir; 2005 Oct;130(5):449-53
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  • [Title] [Sporadical extraperitoneal desmoid tumors--review and report on 4 cases].
  • INTRODUCTION: Desmoid tumors are very rare mesenchymal tumors with a partially aggressive growth pattern and high relapse rates.
  • Without metastases, they are classified as semi-malignant tumours.
  • CASE REPORTS: We report the follow-up of 4 patients who were treated for desmoid tumours.
  • In our opinion wide surgical resection should be performed in primary occurring cases with resectable tumours.
  • On account of the previous experiences, it can be concluded that therapy of primary non respectable or recurrent desmoid tumours is not only a domain of a surgical approach.
  • For generally accepted guidelines for this rare disease, general therapeutic options based on prospectively randomised studies have to be elaborated.
  • [MeSH-major] Abdominal Neoplasms / surgery. Fibromatosis, Aggressive / surgery. Neoplasm Recurrence, Local / radiotherapy. Neoplasm Recurrence, Local / surgery. Thoracic Neoplasms / surgery
  • [MeSH-minor] Abdominal Wall / pathology. Abdominal Wall / surgery. Adult. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Radiotherapy, Adjuvant. Reoperation

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  • (PMID = 16220442.001).
  • [ISSN] 0044-409X
  • [Journal-full-title] Zentralblatt für Chirurgie
  • [ISO-abbreviation] Zentralbl Chir
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
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63. Erzen D, Sencar M, Novak J: Retroperitoneal sarcoma: 25 years of experience with aggressive surgical treatment at the Institute of Oncology, Ljubljana. J Surg Oncol; 2005 Jul 1;91(1):1-9
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  • [Title] Retroperitoneal sarcoma: 25 years of experience with aggressive surgical treatment at the Institute of Oncology, Ljubljana.
  • BACKGROUND AND OBJECTIVES: Retroperitoneal sarcomas are rare malignant tumors with an aggressive course of disease and high local recurrence rate.
  • Local disease is the main cause of death.
  • A retrospective study was undertaken to evaluate the results of aggressive surgical treatment in a series of patients of primary and recurrent retroperitoneal sarcomas.
  • PATIENTS AND METHODS: From 1975 to 2000, 155 patients with retroperitoneal tumors were operated on at the Institute of Oncology, Ljubljana.
  • Patients with fibromatosis and children less than 16 years were excluded from further calculation.
  • Out of the rest 139 patients, 102 patients had localized retroperitoneal sarcoma, and 37 patients had retroperitoneal sarcoma with distant metastases Leiomyosarcoma was the most common type of histology, followed by liposarcoma and schwannoma.
  • Our treatment approach was aggressive.
  • We removed surgically primary tumor, recurrent sarcoma and metastases, whenever possible.
  • In 118, patients at least one organ was resected completely or partially en bloc with tumor.
  • High tumor grade was associated with poor survival.
  • Until there are no other treatment modalities, aggressive surgery for recurrent sarcoma is recommended.
  • [MeSH-major] Neoplasm Recurrence, Local / surgery. Retroperitoneal Neoplasms / surgery. Sarcoma / surgery
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Humans. Male. Middle Aged. Neoplasm Metastasis. Retrospective Studies. Slovenia / epidemiology. Survival Rate

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  • (PMID = 15999353.001).
  • [ISSN] 0022-4790
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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64. Lakranbi M, Smahi M, Maidi M, Bouchikh M, Msougar Y, Ouadnouni Y, Fenan H, Achir A, Caidi M, Alaziz A, Benosman A: [Desmoid tumors of the chest wall: report of 12 cases]. Pan Afr Med J; 2009;3:13
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  • [Title] [Desmoid tumors of the chest wall: report of 12 cases].
  • [Transliterated title] Les tumeurs desmoides de la paroi thoracique : à propos de 12 cas.

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  • [ISSN] 1937-8688
  • [Journal-full-title] The Pan African medical journal
  • [ISO-abbreviation] Pan Afr Med J
  • [Language] fre
  • [Publication-type] Journal Article
  • [Publication-country] Uganda
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65. Skubitz KM, Pambuccian S, Manivel JC, Skubitz AP: Identification of heterogeneity among soft tissue sarcomas by gene expression profiles from different tumors. J Transl Med; 2008 May 06;6:23
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  • [Title] Identification of heterogeneity among soft tissue sarcomas by gene expression profiles from different tumors.
  • The heterogeneity that soft tissue sarcomas (STS) exhibit in their clinical behavior, even within histological subtypes, complicates patient care.
  • Morphologic features are generally good predictors of biologic behavior, however, metastatic propensity, tumor growth, and response to chemotherapy may be determined by gene expression patterns that do not correlate well with morphology.
  • One approach to identify heterogeneity is to search for genetic markers that correlate with differences in tumor behavior.
  • We have reported gene expression patterns that distinguish two subgroups of clear cell renal carcinoma (ccRCC), and other gene expression patterns that distinguish heterogeneity of serous ovarian carcinoma (OVCA) and aggressive fibromatosis (AF).
  • All three gene sets also recognized the same two subsets of the fibromatosis samples that we had found in our earlier study of AF.
  • In addition, several genes that are targets of some anti-tumor drugs were found to be differentially expressed in particular subsets of STS.

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  • (PMID = 18460215.001).
  • [ISSN] 1479-5876
  • [Journal-full-title] Journal of translational medicine
  • [ISO-abbreviation] J Transl Med
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA106878-03; United States / NCI NIH HHS / CA / R01 CA106878; United States / NCI NIH HHS / CA / R01 CA106878-03; United States / NCI NIH HHS / CA / R01CA106878
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / RNA, Neoplasm
  • [Other-IDs] NLM/ PMC2412854
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66. Chaturvedi R: Idiopathic gingival fibromatosis associated with generalized aggressive periodontitis: a case report. J Can Dent Assoc; 2009 May;75(4):291-5
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  • [Title] Idiopathic gingival fibromatosis associated with generalized aggressive periodontitis: a case report.
  • Idiopathic gingival fibromatosis, a benign, slow-growing proliferation of the gingival tissues, is genetically heterogeneous.
  • This condition is usually part of a syndrome or, rarely, an isolated disorder.
  • Aggressive periodontitis, another genetically transmitted disorder of the periodontium, typically results in severe, rapid destruction of the toothsupporting apparatus.
  • The increased susceptibility of the host population with aggressive periodontitis may be caused by the combined effects of multiple genes and their interaction with various environmental factors.
  • Functional abnormalities of neutrophils have also been implicated in the etiopathogenesis of aggressive periodontitis.
  • We present a rare case of a nonsyndromic idiopathic gingival fibromatosis associated with generalized aggressive periodontitis.
  • We established the patient"s diagnosis through clinical and radiologic assessment, histopathologic findings and immunologic analysis of neutrophil function with a nitro-blue-tetrazolium reduction test.
  • [MeSH-major] Aggressive Periodontitis / complications. Fibromatosis, Gingival / complications
  • [MeSH-minor] Adult. Combined Modality Therapy. Diagnosis, Differential. Humans. Indicators and Reagents. Male. Neutrophils / metabolism. Nitroblue Tetrazolium

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  • (PMID = 19422753.001).
  • [ISSN] 1488-2159
  • [Journal-full-title] Journal (Canadian Dental Association)
  • [ISO-abbreviation] J Can Dent Assoc
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Canada
  • [Chemical-registry-number] 0 / Indicators and Reagents; 298-83-9 / Nitroblue Tetrazolium
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67. Joyce M, Mignanelli E, Church J: Ureteric obstruction in familial adenomatous polyposis-associated desmoid disease. Dis Colon Rectum; 2010 Mar;53(3):327-32
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  • [Title] Ureteric obstruction in familial adenomatous polyposis-associated desmoid disease.
  • PURPOSE: Intra-abdominal desmoid disease is the second leading cause of death in familial adenomatous polyposis patients.
  • The aim of this study was to identify the incidence, management, and outcomes for familial adenomatous polyposis associated intra-abdominal desmoids causing ureteric obstruction.
  • RESULTS: Of 107 patients identified with familial adenomatous polyposis related desmoid disease, 30 (28%) had documented CT scan evidence of ureteric obstruction.
  • Preceding surgery was the most prominent risk factor for development of desmoid disease (28 of 30 patients); 2 patients were diagnosed with desmoids before abdominal surgery.
  • Overall, 11 patients had ureteric obstruction at the time of diagnosis.
  • In the other 19 patients, median time from desmoid diagnosis to ureteric obstruction was 2 years.
  • One-third of patients required more than one urologic procedure, and 63% had extensive small-bowel involvement with desmoid.
  • CONCLUSIONS: The majority of patients with familial adenomatous polyposis associated desmoid disease who develop hydronephrosis require stenting.
  • Physicians treating patients with familial adenomatous polyposis and desmoid disease must be aware of the potential for development of ureteric obstruction and available treatment options.
  • [MeSH-major] Adenomatous Polyposis Coli / complications. Fibromatosis, Aggressive / complications. Ureteral Obstruction / etiology


68. Castellazzi G, Vanel D, Le Cesne A, Le Pechoux C, Caillet H, Perona F, Bonvalot S: Can the MRI signal of aggressive fibromatosis be used to predict its behavior? Eur J Radiol; 2009 Feb;69(2):222-9
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  • [Title] Can the MRI signal of aggressive fibromatosis be used to predict its behavior?
  • PURPOSE: Aggressive fibromatosis is an invasive non-metastasizing soft-tissue tumor.
  • The aim of this study was to analyze and correlate the size and MR imaging signal features of aggressive fibromatosis with its behavior in order to choose the best treatment.
  • CONCLUSION: Fibromatoses are a group of soft-tissue tumors with variable characteristics on MRI, but it is not possible to predict their behavior based on the MRI signal.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Magnetic Resonance Imaging / methods. Soft Tissue Neoplasms / diagnosis


69. Alebouyeh M, Moussavi F, Tabari AK, Vossough P: Aggressive intra-abdominal fibromatosis in children and response to chemotherapy. Pediatr Hematol Oncol; 2005 Sep;22(6):447-51
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  • [Title] Aggressive intra-abdominal fibromatosis in children and response to chemotherapy.
  • Intra-abdominal fibromatosis (IAF) is a rare benign neoplasm arising from the abdominal fibrous tissue, mostly in the mesentery.
  • Within 6 months after initial presentation, he underwent 4 subsequent abdominal explorations for diagnosis, tumor reduction, and intestinal obstructions.
  • Due to accelerated tumor growth and deteriorated general condition, as a last resort, a chemotherapy trial with vincristin and methotrexate was carried out.
  • This regimen proved to be effective in reducing the tumor burden and improving the patient's general condition.
  • Outcome of IAF depends on early diagnosis and complete tumor resection, and, if indicated, timely employment of neo/adjuvant chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Fibromatosis, Abdominal / drug therapy. Fibromatosis, Aggressive / drug therapy
  • [MeSH-minor] Child, Preschool. Humans. Male. Methotrexate / administration & dosage. Tumor Burden / drug effects. Vincristine / administration & dosage

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  • (PMID = 16169811.001).
  • [ISSN] 0888-0018
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; YL5FZ2Y5U1 / Methotrexate
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70. Huang K, Fu H, Shi YQ, Zhou Y, Du CY: Prognostic factors for extra-abdominal and abdominal wall desmoids: a 20-year experience at a single institution. J Surg Oncol; 2009 Dec 1;100(7):563-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Prognostic factors for extra-abdominal and abdominal wall desmoids: a 20-year experience at a single institution.
  • BACKGROUND AND OBJECTIVE: Previous reports even large studies discussing the prognosis of desmoids have included tumors from intra- and extra-abdominal sites as well as incomplete resection.
  • The purpose of this study was to explore prognostic factors associated with the recurrence free survival (RFS) rate in surgically treated extra-abdominal and abdominal wall desmoids.
  • PATIENTS AND METHODS: A total of 198 consecutive desmoid patients were treated with surgery over a 20-year period at a single institution.
  • Of these, 151 patients with extra-abdominal and abdominal wall tumors were retrospectively reviewed.
  • One hundred thirteen patients were referred for the primary tumor and the other 38 for recurrent disease initially treated elsewhere.
  • No patients died of the disease.
  • Admission status, gender, tumor size, margin status, location, and number, were predictors of LR in univariate analysis.
  • Tumor size and margin status were independent prognostic factors in multivariate analysis.
  • Positive margins were predictive of recurrence of primary disease, and also showed a trend for recurrent disease, which was not statistically significant.
  • CONCLUSIONS: Regardless of primary or recurrent disease, microscopically negative margins should always be the goal for extra-abdominal desmoids surgery, if no cosmetic defects or function demolition is encountered.
  • Extra-abdominal desmoids deserve more attention and should be treated more aggressively, especially when leaving positive margins.
  • [MeSH-major] Abdominal Neoplasms / mortality. Fibromatosis, Abdominal / mortality. Fibromatosis, Aggressive / mortality
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Extremities / pathology. Female. Follow-Up Studies. Head and Neck Neoplasms / pathology. Head and Neck Neoplasms / therapy. Humans. Male. Middle Aged. Neoplasm Recurrence, Local / epidemiology. Prognosis. Radiotherapy, Adjuvant. Retrospective Studies. Survival Rate. Thoracic Neoplasms / pathology. Thoracic Neoplasms / therapy. Young Adult

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  • (PMID = 19722232.001).
  • [ISSN] 1096-9098
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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71. Guadagnolo BA, Zagars GK, Ballo MT: Long-term outcomes for desmoid tumors treated with radiation therapy. Int J Radiat Oncol Biol Phys; 2008 Jun 1;71(2):441-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Long-term outcomes for desmoid tumors treated with radiation therapy.
  • PURPOSE: To evaluate long-term outcomes in patients with desmoid fibromatosis treated with radiation therapy (RT), with or without surgery.
  • METHODS AND MATERIALS: Between 1965 and 2005, 115 patients with desmoid tumors were treated with RT at our institution.
  • Of the patients, 41 (36%) received RT alone (median dose, 56 Gy) for gross disease, and 74 (64%) received combined-modality treatment (CMT) consisting of a combination of surgery and RT (median dose, 50.4 Gy).
  • On univariate analysis, LC was significantly influenced by tumor size (< or =5 cm vs. 5-10 cm vs. >10 cm) (p = 0.02) and age (< or = 30 vs. >30 years) (p = 0.02).
  • There was no significant difference in LC for patients treated with RT alone for gross disease vs. CMT.
  • For patients treated with CMT, only tumor size significantly influenced LC (p = 0.02).
  • CONCLUSIONS: Desmoid tumors are effectively controlled with RT administered either as an adjuvant to surgery when resection margins are positive or alone for gross disease when surgical resection is not feasible.
  • Doses >56 Gy may not be necessary to control gross disease and are associated with high rates of radiation-related complications.
  • [MeSH-major] Fibromatosis, Aggressive / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Aged. Analysis of Variance. Child. Combined Modality Therapy / methods. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local. Radiation Injuries / complications. Radiotherapy Dosage. Salvage Therapy / methods. Time Factors. Treatment Outcome

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  • (PMID = 18068311.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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72. Hatamochi A, Sasaki T, Kawaguchi T, Suzuki H, Yamazaki S: A novel point mutation in the gene encoding capillary morphogenesis protein 2 in a Japanese patient with juvenile hyaline fibromatosis. Br J Dermatol; 2007 Nov;157(5):1037-9
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  • [Title] A novel point mutation in the gene encoding capillary morphogenesis protein 2 in a Japanese patient with juvenile hyaline fibromatosis.
  • [MeSH-major] Fibromatosis, Aggressive / genetics. Membrane Proteins / genetics. Skin Neoplasms / genetics

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  • (PMID = 17725672.001).
  • [ISSN] 0007-0963
  • [Journal-full-title] The British journal of dermatology
  • [ISO-abbreviation] Br. J. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / ANTXR2 protein, human; 0 / Membrane Proteins; 0 / Receptors, Peptide
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73. Singh N, Sharma R, Dorman SA, Dy VC: An unusual presentation of desmoid tumor in the ileum. Am Surg; 2006 Sep;72(9):821-4
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  • [Title] An unusual presentation of desmoid tumor in the ileum.
  • Desmoid tumors are nonencapsulated, locally invasive tumors of fibrous origin.
  • A wide variety of locations have been documented in the literature with respect to the origin of these tumors.
  • Intra-abdominal desmoids are commonly found in the mesentery.
  • We present the report of a case in which a sporadic desmoid tumor originated from the wall of the ileum.
  • Of all the options described, surgery with tumor-free margins has been defined as the best treatment modality.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis. Ileal Neoplasms / diagnosis

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  • (PMID = 16986393.001).
  • [ISSN] 0003-1348
  • [Journal-full-title] The American surgeon
  • [ISO-abbreviation] Am Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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74. Bhosale PR, Patnana M, Viswanathan C, Szklaruk J: The inguinal canal: anatomy and imaging features of common and uncommon masses. Radiographics; 2008 May-Jun;28(3):819-35; quiz 913
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  • A variety of benign and malignant masses can be found in the inguinal canal (IC).
  • Benign causes of masses in the IC include spermatic cord lipoma, hematoma, abscess, neurofibroma, varicocele, desmoid tumor, air, bowel contrast material, hydrocele, and prostheses.
  • Metastases to the IC can occur from alveolar rhabdomyosarcoma, monophasic sarcoma, prostate cancer, Wilms tumor, carcinoid tumor, melanoma, or pancreatic cancer.
  • In patients with a known malignancy and peritoneal carcinomatosis, the diagnosis of metastases can be suggested when a mass is detected in the IC.
  • When peritoneal disease is not evident, a mass in the IC is indicative of stage IV disease and may significantly alter clinical and surgical treatment of the patient.
  • [MeSH-major] Abdominal Neoplasms / diagnosis. Inguinal Canal / pathology. Inguinal Canal / radiography. Peritoneal Diseases / diagnosis
  • [MeSH-minor] Adult. Aged. Female. Humans. Male. Middle Aged. Rare Diseases / diagnosis

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  • [Copyright] Copyright RSNA, 2008.
  • (PMID = 18480486.001).
  • [ISSN] 1527-1323
  • [Journal-full-title] Radiographics : a review publication of the Radiological Society of North America, Inc
  • [ISO-abbreviation] Radiographics
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 88
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75. Goto T, Nemoto T, Ogura K, Hozumi T, Funata N: Successful treatment of desmoid tumor of the chest wall with tranilast: a case report. J Med Case Rep; 2010;4:384
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  • [Title] Successful treatment of desmoid tumor of the chest wall with tranilast: a case report.
  • INTRODUCTION: Desmoid tumor is characterized by infiltrative growth and local recurrence often occurs after surgery.
  • In addition, these non-surgical treatments are also performed in patients with unresectable desmoid tumors.
  • We successfully treated a patient with a desmoid tumor with tranilast; an anti-allergic agent.
  • CASE PRESENTATION: A 48-year-old Japanese man with a slow-growing desmoid tumor on his chest wall was treated with an oral administration of tranilast (300 mg per day, three times a day).
  • Two years and two months after the commencement of his therapy, the tumor became impalpable.
  • Two years after discontinuation of the treatment, a physical examination showed no recurrence of the tumor and he continued in a state of remission.
  • We were successfully able to reduce the size of the tumor and thereafter maintain the reduced size.
  • Because tranilast has substantially fewer adverse effects than cytotoxic agents, it could be a very useful therapeutic agent for desmoid tumor.

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  • (PMID = 21114809.001).
  • [ISSN] 1752-1947
  • [Journal-full-title] Journal of medical case reports
  • [ISO-abbreviation] J Med Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2999613
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76. Solazzo M, Marcianò P, Benzi F, Puccio F: Ileal resection and small bowel transplantation for large mesenteric desmoid tumor. Tech Coloproctol; 2005 Apr;9(1):72-3
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  • [Title] Ileal resection and small bowel transplantation for large mesenteric desmoid tumor.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Ileum / surgery. Intestine, Small / transplantation. Mesentery. Peritoneal Neoplasms / surgery

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  • (PMID = 15884154.001).
  • [ISSN] 1123-6337
  • [Journal-full-title] Techniques in coloproctology
  • [ISO-abbreviation] Tech Coloproctol
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Italy
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77. Gould CF, Ly JQ, Lattin GE Jr, Beall DP, Sutcliffe JB 3rd: Bone tumor mimics: avoiding misdiagnosis. Curr Probl Diagn Radiol; 2007 May-Jun;36(3):124-41
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  • [Title] Bone tumor mimics: avoiding misdiagnosis.
  • Whether discovered incidentally or as part of a focused diagnostic evaluation, the finding of a benign osseous lesion that has radiologic features resembling a bone tumor is not uncommon.
  • Some of the more common benign and nonneoplastic entities that can sometimes be confused with tumors are the following: cortical desmoid, Brodie abscess, synovial herniation pit, pseudocyst, enostosis, intraosseous ganglion cyst, fibrous dysplasia, stress fracture, avulsion fracture (healing stage), bone infarct, myositis ossificans, brown tumor, and subchondral cyst.
  • Accurate diagnosis and management of these lesions require a basic understanding of their epidemiology, clinical presentations, anatomic distributions, imaging features, differential considerations, and therapeutic options.
  • This in-depth review of 13 potential bone tumor mimics will assist the radiologist in correctly identifying these benign lesions and in avoiding misdiagnosis and related morbidity.
  • [MeSH-major] Bone Diseases / diagnosis. Bone Neoplasms / diagnosis. Magnetic Resonance Imaging
  • [MeSH-minor] Ankle Joint / radiography. Bone Cysts / diagnosis. Bone Cysts / pathology. Bone and Bones / blood supply. Diagnosis, Differential. Femur Neck / pathology. Fibromatosis, Aggressive / diagnosis. Fibrous Dysplasia of Bone / diagnosis. Fractures, Stress / diagnosis. Ganglion Cysts / diagnosis. Humans. Infarction / diagnosis. Myositis Ossificans / diagnosis. Tomography, X-Ray Computed

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  • (PMID = 17484955.001).
  • [ISSN] 0363-0188
  • [Journal-full-title] Current problems in diagnostic radiology
  • [ISO-abbreviation] Curr Probl Diagn Radiol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 48
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78. Maeda R, Isowa N, Onuma H, Miura H, Tokuyasu H, Kawasaki Y: Desmoid tumor of the chest wall in an elderly woman. Gen Thorac Cardiovasc Surg; 2009 Oct;57(10):554-7
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  • [Title] Desmoid tumor of the chest wall in an elderly woman.
  • We report a case of desmoid tumor of the chest wall in a 79-year-old woman.
  • Magnetic resonance imaging of the chest revealed a soft tissue tumor in the right lateral chest wall with unclear margins that extended into the intercostal muscles.
  • Because open biopsy suggested a desmoid tumor, full-thickness chest wall resection with reconstruction was performed.
  • The final diagnosis was desmoid tumor of the chest wall.
  • Wide surgical resection during the initial operation is an essential element in the treatment of this tumor.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Intercostal Muscles / pathology. Ribs / pathology. Thoracic Neoplasms / diagnosis. Thoracic Wall / pathology
  • [MeSH-minor] Aged. Biopsy. Female. Fluorodeoxyglucose F18. Humans. Magnetic Resonance Imaging. Neoplasm Invasiveness. Positron-Emission Tomography. Radiopharmaceuticals. Thoracotomy. Tomography, X-Ray Computed

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  • (PMID = 19830521.001).
  • [ISSN] 1863-6713
  • [Journal-full-title] General thoracic and cardiovascular surgery
  • [ISO-abbreviation] Gen Thorac Cardiovasc Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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79. Nakayama T, Tsuboyama T, Toguchida J, Hosaka T, Nakamura T: Natural course of desmoid-type fibromatosis. J Orthop Sci; 2008 Jan;13(1):51-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Natural course of desmoid-type fibromatosis.
  • BACKGROUND: Desmoid-type fibromatosis is a locally aggressive tumor known to have high potential for local recurrence after resection, while it exhibits self-limiting behavior and shows growth arrest or spontaneous regression in many patients.
  • METHODS: We retrospectively reviewed clinical outcome and changes in tumor size in 11 consecutive patients with extremity and trunk desmoid-type fibromatoses, who were basically observed without any treatment after diagnosis.
  • RESULTS: For two patients in whom the tumors were initially incorrectly diagnosed as other tumors, surgical resection was performed.
  • For another two patients, surgical resections were performed in the follow-up periods due to tumor enlargement or joint contracture.
  • In all four patients who underwent surgery, tumors recurred shortly after resection and re-resection was not performed.
  • During the follow-up periods with a median length of 56 months, ten tumors eventually stopped growing, and three of them regressed spontaneously.
  • At the time of final follow-up, ten patients were alive with residual disease without severe morbidity.
  • In one patient, the tumor enlarged to over 30 cm in diameter with a substantial functional deficit.
  • CONCLUSIONS: Simple observation is a noninvasive and function-preserving treatment for desmoid-type fibromatosis.
  • [MeSH-major] Fibromatosis, Aggressive / pathology
  • [MeSH-minor] Adolescent. Adult. Cohort Studies. Female. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Regression, Spontaneous. Outcome and Process Assessment (Health Care). Retrospective Studies. Tomography, X-Ray Computed. Tumor Burden

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  • (PMID = 18274856.001).
  • [ISSN] 0949-2658
  • [Journal-full-title] Journal of orthopaedic science : official journal of the Japanese Orthopaedic Association
  • [ISO-abbreviation] J Orthop Sci
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Japan
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80. Yu YH, Son BK, Jun DW, Kim SH, Jo YJ, Park YS, Hong BW, Joo JE: [A case of desmoid tumor presenting as intra-abdominal abscess]. Korean J Gastroenterol; 2009 May;53(5):315-9
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  • [Title] [A case of desmoid tumor presenting as intra-abdominal abscess].
  • Desmoid tumor is a rare benign tumor derived from fibrous sheath or musculoaponeurotic structure.
  • The tumor is benign histologically but considered as malignant clinically because it has high propensity on infiltrative growth with local invasion and tendency to recurrence after local excision.
  • Especially, when this tumor happens to be in the intra-abdomen, the prognosis is worse because it can cause intestinal obstruction, ureter obstruction and, fistula formation.
  • This tumor occurs more frequently in patients with familial adenomatous polyposis (FAP), in post-partume women, and at old surgical incision site.
  • We report a rare case of the young male patient who presented with an acute abdomen and underwent laparotomy and was found to have an intra-abdominal desmoid tumor with abscess formation.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis. Peritoneal Neoplasms / diagnosis
  • [MeSH-minor] Abdominal Abscess / diagnosis. Adult. Diagnosis, Differential. Humans. Male. Tomography, X-Ray Computed

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  • (PMID = 19458469.001).
  • [ISSN] 1598-9992
  • [Journal-full-title] The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi
  • [ISO-abbreviation] Korean J Gastroenterol
  • [Language] kor
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Korea (South)
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81. Croner RS, Brueckl WM, Reingruber B, Hohenberger W, Guenther K: Age and manifestation related symptoms in familial adenomatous polyposis. BMC Cancer; 2005 Mar 2;5:24
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  • BACKGROUND: To identify early symptoms of familial adenomatous polyposis with a view to improve early diagnosis and treatment.
  • Diagnosis on the basis of genetic testing is usually limited to where there is a known family history, so FAP is more usually diagnosed on clinical grounds.
  • Except for those identified via FAP registers, the majority of patients are symptomatic at the time of diagnosis.
  • Non-specific symptoms such as abdominal pain, fatigue and bloating were less frequent and were mainly reported by patients older than 40.
  • Patients with these findings need immediate endoscopy to allow prompt diagnosis and prophylactic surgery.
  • [MeSH-major] Adenomatous Polyposis Coli / diagnosis
  • [MeSH-minor] Abdominal Pain / epidemiology. Adolescent. Adult. Aged. Anemia / epidemiology. Child. Child, Preschool. Colonic Neoplasms / diagnosis. Colonic Neoplasms / epidemiology. Comorbidity. Constipation / epidemiology. Diarrhea / epidemiology. Female. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / epidemiology. Humans. Male. Middle Aged. Occult Blood. Retroperitoneal Neoplasms / diagnosis. Retroperitoneal Neoplasms / epidemiology. Retrospective Studies

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  • (PMID = 15740631.001).
  • [ISSN] 1471-2407
  • [Journal-full-title] BMC cancer
  • [ISO-abbreviation] BMC Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC1079798
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82. de Jong WK, van der Graaf WT, van der Jagt EJ, Klinkenberg TJ, Groen HJ, Timens W, Douma WR: A 20-year-old male with thoracic pain and a lower thoracic mass. Diagnosis: intrathoracal desmoid tumour with microscopically incomplete resection. Eur Respir J; 2005 Oct;26(4):740-3
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  • [Title] A 20-year-old male with thoracic pain and a lower thoracic mass. Diagnosis: intrathoracal desmoid tumour with microscopically incomplete resection.
  • [MeSH-major] Chest Pain / etiology. Fibromatosis, Aggressive / diagnosis. Thoracic Neoplasms / diagnosis

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  • (PMID = 16204607.001).
  • [ISSN] 0903-1936
  • [Journal-full-title] The European respiratory journal
  • [ISO-abbreviation] Eur. Respir. J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
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83. Lips DJ, Barker N, Clevers H, Hennipman A: The role of APC and beta-catenin in the aetiology of aggressive fibromatosis (desmoid tumors). Eur J Surg Oncol; 2009 Jan;35(1):3-10
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  • [Title] The role of APC and beta-catenin in the aetiology of aggressive fibromatosis (desmoid tumors).
  • BACKGROUND: Aggressive fibromatosis (syn. desmoid tumor) is a sporadically occurring neoplastic proliferation of fibroblasts originating from musculoaponeurotic planes, forming invasively growing masses without the capability to metastasize.
  • Better understanding of the aetiology of aggressive fibromatosis is needed to be able to develop new treatment strategies to cope with the high recurrence rates.
  • The following search terms were used: 'aggressive fibromatosis', 'desmoid tumor', 'adenomatous polyposis coli', 'APC', 'beta-catenin', 'Wnt', 'Wingless' and 'Wnt/Wingless'.
  • RESULTS: The neoplastic nature of aggressive fibromatosis and the role of the adenomatous polyposis coli (APC) and beta-catenin signaling cascade in driving the onset and progression of this disease are discussed.
  • CONCLUSION: Mutations in either the APC or beta-catenin genes are likely to be a major driving force in the formation of these desmoid tumors.
  • [MeSH-major] Adenomatous Polyposis Coli Protein / genetics. Fibromatosis, Aggressive / genetics. Fibromatosis, Aggressive / metabolism. Signal Transduction / genetics. beta Catenin / genetics


84. Koeda S, Nagasaka H, Kumamoto H, Kawamura H: Extra-abdominal fibromatosis of the cheek: report of a case. J Oral Maxillofac Surg; 2005 Aug;63(8):1222-6
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  • [Title] Extra-abdominal fibromatosis of the cheek: report of a case.
  • [MeSH-major] Cheek. Fibromatosis, Aggressive / pathology. Mouth Neoplasms / pathology
  • [MeSH-minor] Child. Follow-Up Studies. Humans. Male. Mouth Mucosa / pathology. Neoplasm Recurrence, Local / pathology

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  • (PMID = 16094595.001).
  • [ISSN] 0278-2391
  • [Journal-full-title] Journal of oral and maxillofacial surgery : official journal of the American Association of Oral and Maxillofacial Surgeons
  • [ISO-abbreviation] J. Oral Maxillofac. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 20
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85. Abikhzer G, Bouganim N, Finesilver A: Aggressive fibromatosis of the head and neck: case report and review of the literature. J Otolaryngol; 2005 Aug;34(4):289-94
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  • [Title] Aggressive fibromatosis of the head and neck: case report and review of the literature.
  • [MeSH-major] Fibromatosis, Aggressive. Head and Neck Neoplasms. Neck / radiation effects. Neck / surgery


86. Li CF, Wei RY, Baliko F, Bapat B, Alman BA: An association between the 4G polymorphism in the PAI-1 promoter and the development of aggressive fibromatosis (desmoid tumor) in familial adenomatous polyposis patients. Fam Cancer; 2007;6(1):89-95
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] An association between the 4G polymorphism in the PAI-1 promoter and the development of aggressive fibromatosis (desmoid tumor) in familial adenomatous polyposis patients.
  • Aggressive fibromatosis is a mesenchymal neoplasm associated with mutations resulting in beta-catenin mediated transcriptional activation.
  • Plasminogen activator inhibitor-1 (PAI-1) is expressed at a high level in aggressive fibromatosis, and using transgenic mice, we found that PAI-1 plays an important role in aggressive fibromatosis tumor formation.
  • Familial adenomatous polyposis is associated with Adenomatous Polyposis Coli gene mutations resulting in beta-catenin mediated transcriptional activation, yet only some patients develop aggressive fibromatosis.
  • Since PAI-1 expression is influenced by a promoter 4G/5G polymorphism, we investigated the incidence of this polymorphism in familial adenomatous polyposis patients who did and who did not develop aggressive fibromatosis, as well as sporadic aggressive fibromatosis patients.
  • There was a trend towards association of the 4G allele (associated with high PAI-1 expression) with the development of aggressive fibromatosis in familial adenomatous polyposis patients (50% vs. 19%, P = 0.1).
  • In familial adenomatous polyposis patients who did not develop aggressive fibromatosis, there was a significantly lower proportion of patients with a 4G allele compared to the healthy control (19% vs. 51%, P = 0.0286).
  • The lower incidence of 4G polymorphism in the PAI-1 promoter may be preventive against the development of aggressive fibromatosis.
  • This data provides additional evidence supporting an important role for PAI-1 in the pathogenesis of aggressive fibromatosis.
  • [MeSH-major] Adenomatous Polyposis Coli / genetics. Fibromatosis, Aggressive / genetics. Gene Expression Regulation, Neoplastic. Plasminogen Activator Inhibitor 1 / genetics. Polymorphism, Genetic. Promoter Regions, Genetic / genetics
  • [MeSH-minor] Adult. Causality. Comorbidity. Genetic Predisposition to Disease. Humans. Incidence. Severity of Illness Index. Transcriptional Activation


87. Maseelall P, Robins JC, Williams DB, Thomas MA: Stabilization and regression of a recurrent desmoid tumor with the antiestrogen toremifene. Fertil Steril; 2005 Aug;84(2):509
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  • [Title] Stabilization and regression of a recurrent desmoid tumor with the antiestrogen toremifene.
  • OBJECTIVE: To report a case of a pelvic desmoid tumor that was treated with the antiestrogen toremifene after a failed attempt at surgical excision.
  • PATIENT(S): A reproductive-aged woman with a recurrent desmoid tumor.
  • INTERVENTION(S): After surgical excision of a desmoid tumor that presented during childbirth, subsequent recurrence resulted in the use of toremifene for tumor stabilization.
  • MAIN OUTCOME MEASURE(S): Magnetic resonance imaging was used to monitor desmoid tumor size.
  • RESULT(S): One year after postsurgical recurrence of the desmoid tumor, the patient began treatment with the antiestrogen toremifene.
  • Tumor stabilization and regression with symptomatic relief was observed.
  • Nine years of antiestrogen use revealed no progression in tumor size or patient symptoms.
  • After the patient demonstrated perimenopausal symptoms, toremifene administration was discontinued without a return of symptoms or tumor growth after 3 years.
  • CONCLUSION(S): Our case demonstrates that toremifene is a safe and effective therapy that can be used for the stabilization and regression of desmoid tumors.
  • An antiestrogen should be considered as adjuvant therapy after surgery and as a first-line treatment with disease recurrence.
  • [MeSH-major] Estrogen Antagonists / therapeutic use. Fibromatosis, Abdominal / drug therapy. Fibromatosis, Aggressive / drug therapy. Neoplasm Recurrence, Local / prevention & control. Toremifene / therapeutic use

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  • (PMID = 16086575.001).
  • [ISSN] 1556-5653
  • [Journal-full-title] Fertility and sterility
  • [ISO-abbreviation] Fertil. Steril.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Estrogen Antagonists; 7NFE54O27T / Toremifene
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88. Hara R, Matsuguma H, Suzuki H, Ishikawa Y, Nakahara R, Yamaguchi T, Hirabayashi K: Desmoid tumor presenting as a superior sulcus tumor: a unique bone change in the vertebral body. Ann Thorac Surg; 2007 Nov;84(5):1752-4
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  • [Title] Desmoid tumor presenting as a superior sulcus tumor: a unique bone change in the vertebral body.
  • Desmoid tumor, also referred to as aggressive fibromatosis, is a relatively rare, locally infiltrative, histologically benign tumor.
  • This report details a case of desmoid tumor presented as a superior sulcus tumor, which showed a unique manifestation in the vertebral bodies on computed tomography.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Lung Neoplasms / pathology. Spine / pathology

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  • (PMID = 17954107.001).
  • [ISSN] 1552-6259
  • [Journal-full-title] The Annals of thoracic surgery
  • [ISO-abbreviation] Ann. Thorac. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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89. Lucas DR, Shukla A, Thomas DG, Patel RM, Kubat AJ, McHugh JB: Dedifferentiated liposarcoma with inflammatory myofibroblastic tumor-like features. Am J Surg Pathol; 2010 Jun;34(6):844-51
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  • [Title] Dedifferentiated liposarcoma with inflammatory myofibroblastic tumor-like features.
  • The dedifferentiated component of dedifferentiated liposarcoma shows wide histologic variation including tumors with heterologous differentiation.
  • However, tumors closely resembling inflammatory myofibroblastic tumor have not.
  • We report the clinicopathologic, immunohistochemical, and molecular finding in 6 cases of dedifferentiated liposarcoma with inflammatory myofibroblastic tumor-like features treated at our institution.
  • The tumors occurred mostly in middle age or elderly men, involved mostly the inguinal/scrotal region or retroperitoneum, and behaved aggressively.
  • Microscopically, the dedifferentiated component closely resembled or, if taken out of context, was indistinguishable from inflammatory myofibroblastic tumor.
  • All 3 major patterns seen in inflammatory myofibroblastic tumor (myxoid, cellular, and hypocellular fibrous) were represented.
  • Areas resembling nodular fasciitis or desmoid fibromatosis were frequent findings.
  • One tumor had heterologous osseous differentiation.
  • In 4 tumors the inflammatory myofibroblastic tumor-like areas were diffuse, whereas in 2 they were combined with noninflammatory myofibroblastic tumor-like high-grade sarcoma.
  • Five tumors stained for smooth muscle actin or desmin, none stained for ALK-1, 5 stained for MDM2, and 5 had amplified MDM2 by fluorescence in situ hybridization.
  • Well-differentiated liposarcomatous components were present in every tumor.
  • All patients developed locally recurrent or metastatic disease.
  • At last follow-up 2 patients had died of disease and 2 were alive with disease.
  • Dedifferentiated liposarcoma can have prominent inflammatory myofibroblastic tumor-like features, a finding that further expands its histologic spectrum.
  • Awareness of this finding can prevent one from misdiagnosing dedifferentiated liposarcoma as inflammatory myofibroblastic tumor, a much less aggressive neoplasm.
  • [MeSH-major] Liposarcoma / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 20431481.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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90. Roa JA, Martínez GR, González LM, Cedillo EA, Pineda NF: [Muscle-aponeurotic aggressive fibromatosis management. 10 years of experience]. Acta Ortop Mex; 2008 Mar-Apr;22(2):85-9
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  • [Title] [Muscle-aponeurotic aggressive fibromatosis management. 10 years of experience].
  • [Transliterated title] Manejo de la fibromatosis musculoaponeurótica agresiva. 10 años de experiencia.
  • INTRODUCTION: This work presents clinical evolution and functional outcome of patients with histologic diagnosis of Muscle-aponeurotic aggressive fibromatosis in 10 years of follow up in the National Rehabilitation Institute.
  • MATERIAL AND METHODS: We performed a descriptive, retrospective, cross sectioned clinical trial in the Bone Tumour Department.
  • INCLUSION CRITERIA: both genres, any age, clinical and histological diagnosis.
  • EXCLUSION CRITERIA: incomplete file, lost to follow up, other diagnosis.
  • CONCLUSIONS: If clinical suspicion of Muscle-aponeurotic fibromatosis appears, one should start protocol work up: laboratory, radiographic assessment, nuclear medicine, biopsy and histologic and immune-histochemistry.
  • One should perform broad resections aiming to preserve the most possible extremity tissue disease free.
  • [MeSH-major] Fibromatosis, Aggressive. Muscle Neoplasms

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  • (PMID = 18669308.001).
  • [ISSN] 2306-4102
  • [Journal-full-title] Acta ortopédica mexicana
  • [ISO-abbreviation] Acta Ortop Mex
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Mexico
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91. Jeblaoui Y, Bouguila J, Haddad S, Helali M, Zaïri I, Zitouni K, Mokhtar M, Adouani A: [Mandibular aggressive fibromatosis]. Rev Stomatol Chir Maxillofac; 2007 Apr;108(2):153-5
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  • [Title] [Mandibular aggressive fibromatosis].
  • [Transliterated title] Fibromatose agressive mandibulaire.
  • INTRODUCTION: Aggressive fibromatosis is a rare histologically benign fibrous tumor with a potential for locoregional aggression.
  • Surgical treatment is the reference, chemotherapy, radiotherapy and homonotherapy being proposed as complementary treatment or for inoperable tumors.
  • CASE REPORT: A three-year-old patient underwent surgery for removal of a mandibular tumor.
  • Pathology reported aggressive fibromatosis.
  • DISCUSSION: Conservative surgery for aggressive mandibular fibromatosis appears to be preferable to radical mutilating surgery which would have a major impact on facial growth in children.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Mandibular Neoplasms / pathology

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  • (PMID = 17350660.001).
  • [ISSN] 0035-1768
  • [Journal-full-title] Revue de stomatologie et de chirurgie maxillo-faciale
  • [ISO-abbreviation] Rev Stomatol Chir Maxillofac
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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92. Nakagiri T, Koseki M, Nakamoto K, Taniyama K: Paraesophageal mediastinal desmoid tumor: case report. Gen Thorac Cardiovasc Surg; 2007 Mar;55(3):125-9
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  • [Title] Paraesophageal mediastinal desmoid tumor: case report.
  • Computed tomography and magnetic resonance imaging showed an abnormal shadow in the paraesophageal mediastinum, which a thoracoscopic biopsy revealed to be a desmoid tumor that had infiltrated the surrounding tissues.
  • We attempted esophageal stent placement; however, the esophageal stenosis could not be dilated, and bypass surgery was performed first, with tumor resection postponed, and radiation and hormonal therapy were added.
  • Mediastinum desmoid tumors are rare, with only 22 other cases reported in the English language literature.
  • [MeSH-major] Esophageal Neoplasms. Fibromatosis, Aggressive. Mediastinal Neoplasms

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  • [Cites] Cancer. 1960 Jul-Aug;13:825-36 [14405434.001]
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  • (PMID = 17447511.001).
  • [ISSN] 1863-6705
  • [Journal-full-title] General thoracic and cardiovascular surgery
  • [ISO-abbreviation] Gen Thorac Cardiovasc Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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93. Wong SL: Diagnosis and management of desmoid tumors and fibrosarcoma. J Surg Oncol; 2008 May 1;97(6):554-8
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  • [Title] Diagnosis and management of desmoid tumors and fibrosarcoma.
  • Fibrous tumors represent a diverse subtype of soft tissue tumors and can represent benign conditions as well as frankly malignant sarcomas.
  • Desmoid tumors and dermatofibrosarcoma protuberans are more difficult to classify and tend to be considered in the intermediate risk category.
  • They are distinct entities, but both are locally aggressive processes which are plagued with attendant morbidity and high recurrence rates.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / therapy. Fibrosarcoma / diagnosis. Fibrosarcoma / therapy

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  • [Copyright] (c) 2008 Wiley-Liss, Inc.
  • (PMID = 18425778.001).
  • [ISSN] 0022-4790
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 37
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94. Altmann S, Lenz-Scharf O, Schneider W: [Therapeutic options for aggressive fibromatosis]. Handchir Mikrochir Plast Chir; 2008 Apr;40(2):88-93
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Therapeutic options for aggressive fibromatosis].
  • [Transliterated title] Therapieoptionen bei der aggressiven fibromatose.
  • INTRODUCTION: Aggressive fibromatosis, e.g., desmoid tumour, is a rare neoplasm of the connective tissue with local infiltrative growth.
  • Because of the high recurrence rates and destruction of the surrounding tissue, these tumours are classified as semi-malignant.
  • However, desmoid tumors tend not to metastasise.
  • Arising from deep musculoaponeurotic structures, a monoclonal proliferation of fibroblasts occurs.
  • Radical surgical treatment with tumour excision accompanied by radiotherapy is the current standard therapy that can be supplemented by pharmacological treatment in a few cases.
  • PATIENTS AND METHOD: We report on 9 patients (5 males and 4 females) with surgical therapy for aggressive fibromatosis.
  • The neoplasm was located on the extremities in 7 cases (4 x upper extremity, 3 x lower extremity), one tumour was situated in the chin and one in the rectus abdominis muscle.
  • All patients were treated with radical tumour resection.
  • RESULTS: In 6 cases complete tumour resection (R0) was achieved.
  • In 3 cases a total tumour resection was impossible (R1 resection).
  • The aggressive fibromatosis had infiltrated the pelvis in 2 cases implying a hemipelvectomy for R0 resection which was not practicable.
  • DISCUSSION: Aggressive fibromatosis is a semimalignant neoplasm of the connective tissue with an extremely high recurrence rate.
  • Radiotherapy is indicated for patients with non-resectable tumours.
  • [MeSH-major] Fibromatosis, Aggressive / therapy. Soft Tissue Neoplasms / therapy

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  • (PMID = 18437666.001).
  • [ISSN] 0722-1819
  • [Journal-full-title] Handchirurgie, Mikrochirurgie, plastische Chirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft für Handchirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft für Mikrochirurgie der Peripheren Nerven und Gefässe : Organ der Vereinigung der Deutschen Plastischen Chirurgen
  • [ISO-abbreviation] Handchir Mikrochir Plast Chir
  • [Language] ger
  • [Publication-type] Comparative Study; English Abstract; Evaluation Studies; Journal Article
  • [Publication-country] Germany
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95. Skubitz KM, Manivel JC, Clohisy DR, Frolich JW: Response of imatinib-resistant extra-abdominal aggressive fibromatosis to sunitinib: case report and review of the literature on response to tyrosine kinase inhibitors. Cancer Chemother Pharmacol; 2009 Aug;64(3):635-40
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  • [Title] Response of imatinib-resistant extra-abdominal aggressive fibromatosis to sunitinib: case report and review of the literature on response to tyrosine kinase inhibitors.
  • PURPOSE: Aggressive fibromatosis (AF) is usually a slowly growing locally invasive tumor, but may exhibit a much more aggressive phenotype.
  • METHODS: We report a case of an aggressive multicentric extra-abdominal AF that was responsive to sunitinib, but resistant to imatinib.
  • After 13 months, tumors recurred.
  • [MeSH-major] Fibromatosis, Aggressive / drug therapy. Indoles / therapeutic use. Protein Kinase Inhibitors / therapeutic use. Pyrroles / therapeutic use
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Benzamides. Drug Resistance, Neoplasm. Female. Follow-Up Studies. Humans. Imatinib Mesylate. Neoplasm Recurrence, Local. Piperazines / therapeutic use. Pyrimidines / therapeutic use. Treatment Outcome. Young Adult

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  • (PMID = 19404642.001).
  • [ISSN] 1432-0843
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Benzamides; 0 / Indoles; 0 / Piperazines; 0 / Protein Kinase Inhibitors; 0 / Pyrimidines; 0 / Pyrroles; 0 / sunitinib; 8A1O1M485B / Imatinib Mesylate
  • [Number-of-references] 40
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96. Chiang YY, Cheng KL, Hu CH: Desmoid tumour occurring after ambulatory phlebectomy. J Eur Acad Dermatol Venereol; 2008 Nov;22(10):1270-2
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  • [Title] Desmoid tumour occurring after ambulatory phlebectomy.
  • [MeSH-major] Ambulatory Surgical Procedures. Fibromatosis, Aggressive / etiology. Veins / surgery

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  • (PMID = 18462297.001).
  • [ISSN] 1468-3083
  • [Journal-full-title] Journal of the European Academy of Dermatology and Venereology : JEADV
  • [ISO-abbreviation] J Eur Acad Dermatol Venereol
  • [Language] eng
  • [Publication-type] Letter
  • [Publication-country] Netherlands
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97. Röpke M, Kalinski T, Wördehoff H, Aumann V, Bürger T: [Multicentric extra-abdominal fibromatosis: a rare case]. Z Orthop Ihre Grenzgeb; 2006 Mar-Apr;144(2):223-7
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  • [Title] [Multicentric extra-abdominal fibromatosis: a rare case].
  • [Transliterated title] Multizentrische extraabdominale Fibromatose -- ein seltener Fall.
  • Extra-abdominal aggressive fibromatosis is a benign fibroblastic neoplasia with an infiltrative nature and a high tendency of local recurrence.
  • Here, we report on a very rare case of multicentric fibromatosis.
  • Low complaints led to considerable size of the tumours.
  • [MeSH-major] Fibroma / diagnosis. Fibroma / surgery. Leg / surgery. Limb Salvage. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Abdomen. Adult. Chemotherapy, Adjuvant. Combined Modality Therapy. Female. Humans. Radiotherapy, Adjuvant. Rare Diseases / diagnosis. Rare Diseases / surgery. Treatment Outcome

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  • (PMID = 16625455.001).
  • [ISSN] 0044-3220
  • [Journal-full-title] Zeitschrift für Orthopädie und ihre Grenzgebiete
  • [ISO-abbreviation] Z Orthop Ihre Grenzgeb
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
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98. McCormack D, Kesha K, Tittle SL, Saldinger PF: Mesenteric fibromatosis mimicking a gastrointestinal stromal tumor. Conn Med; 2010 Apr;74(4):197-200
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  • [Title] Mesenteric fibromatosis mimicking a gastrointestinal stromal tumor.
  • Mesenteric fibromatosis is a locally aggressive tumor of the mesentery with a high propensity for bowel involvement.
  • Mesenteric fibromatosis often mimics gastrointestinal stromal tumors in size, location and immunohistochemical features.
  • We report the case of a 30-year-old male who underwent resection of a mesenteric tumor, initially diagnosed as gastrointestinal stromal tumor.
  • The tumor was categorized as high-risk and the patient was treated with chemotherapy.
  • The tumor did not respond to medical management.
  • The patient underwent a second en bloc resection and pathology results were conclusive for mesenteric fibromatosis.
  • This case highlights the significance of accurately differentiating mesenteric fibromatosis from gastrointestinal stromal tumor.
  • Making a concrete diagnosis is often difficult because both gastrointestinal stromal tumors and mesenteric fibromatosis share a number of morphological and immunohistochemical features including CKIT expression.
  • [MeSH-major] Diagnostic Errors. Fibromatosis, Abdominal / diagnosis. Gastrointestinal Stromal Tumors / diagnosis
  • [MeSH-minor] Adult. Antineoplastic Agents / therapeutic use. Diagnosis, Differential. Humans. Male

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  • (PMID = 20440999.001).
  • [ISSN] 0010-6178
  • [Journal-full-title] Connecticut medicine
  • [ISO-abbreviation] Conn Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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99. Ferenc T, Wroński JW, Kopczyński J, Kulig A, Sidor M, Stalińska L, Dziki A, Sygut J: Analysis of APC, alpha-, beta-catenins, and N-cadherin protein expression in aggressive fibromatosis (desmoid tumor). Pathol Res Pract; 2009;205(5):311-24
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  • [Title] Analysis of APC, alpha-, beta-catenins, and N-cadherin protein expression in aggressive fibromatosis (desmoid tumor).
  • The aims of this study were to analyze the cadherin/catenin adhesion complex in cells from abdominal and extra-abdominal aggressive fibromatosis tumors, and to estimate the correlation between the expression of the tested proteins and the clinical data of the desmoid patients.
  • Immunohistochemistry was used to examine the expression of the cadherin/catenin adhesion complex: APC protein, alpha-, beta-catenin, and N-cadherin in archival material derived from 15 cases of extra-abdominal desmoid tumor (E-AD) and 20 cases of abdominal (AD) desmoid tumor.
  • In the E-AD group, in both cases of recurrent tumors, no alpha-catenin expression was observed but the expression of this protein was detected in primary tumors.
  • In the groups investigated, no statistically significant correlation was found between alpha-catenin, beta-catenin (c), (n) and (c+n) expression, and tumor size (p>0.1).
  • The results regarding beta-catenin expression obtained in our study confirm the previous findings that nuclear accumulation of this protein plays a crucial role in the pathogenesis of aggressive fibromatosis.
  • [MeSH-major] Adenomatous Polyposis Coli Protein / biosynthesis. Cadherins / biosynthesis. Fibromatosis, Aggressive / metabolism. alpha Catenin / biosynthesis. beta Catenin / biosynthesis
  • [MeSH-minor] Adult. Age Factors. Aged. Biomarkers, Tumor / analysis. Female. Gene Expression. Humans. Immunohistochemistry. Male. Young Adult


100. Pojchamarnwiputh S, Muttarak M, Na-Chiangmai W, Chaiwun B: Benign breast lesions mimicking carcinoma at mammography. Singapore Med J; 2007 Oct;48(10):958-68
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  • [Title] Benign breast lesions mimicking carcinoma at mammography.
  • Many benign breast lesions pose diagnostic challenges.
  • These lesions include abscess, haematoma, radial scar, post surgical scar, diabetic mastopathy, focal fibrosis, sclerosing adenosis, granular cell tumour, extra-abdominal desmoid tumour, medial insertion of pectoralis muscle and sternalis muscle, and axillary lymphadenopathy (due to HIV infection, collagen vascular lesions, tuberculous and bacterial lymphadenitis).
  • Radiologists should be familiar with the characteristic imaging features of these benign lesions, and should include these benign lesions in the differential diagnosis whenever malignant-appearing findings are encountered.
  • Correlation of the patient's clinical features with the mammographical findings and additional use of ultrasonography, fine-needle aspiration biopsy or core biopsy are helpful in establishing the final diagnosis and obviating unnecessary surgical intervention.
  • [MeSH-minor] Adult. Biopsy, Needle. Breast Neoplasms / pathology. Breast Neoplasms / radiography. Breast Neoplasms / ultrasonography. Carcinoma / pathology. Carcinoma / radiography. Carcinoma / ultrasonography. Diagnosis, Differential. Female. Histocytochemistry. Humans. Middle Aged

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  • (PMID = 17909685.001).
  • [ISSN] 0037-5675
  • [Journal-full-title] Singapore medical journal
  • [ISO-abbreviation] Singapore Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Singapore
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