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Myoung Ja Chung - One of the best experts on this subject based on the ideXlab platform.
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Abdominal Fibromatosis in a young child: a case study and review of the literature.
Korean Journal of Pathology, 2013Co-Authors: Pyoung Han Hwang, Yeon Jun Jeong, Myoung Ja ChungAbstract:Fibromatoses comprise many different entities of well-differentiated fibroblastic proliferation with variable collagen production and form a firm nodular mass. Abdominal Fibromatosis is distinguishable from other forms of Fibromatosis because of its location and its tendency to occur in women of childbearing age during or following pregnancy. Abdominal Fibromatosis in children is an extremely rare condition. A 15-month-old boy presented with an Abdominal wall mass that had recently increased in size. Mass excision was perfomed. The tumor was 4.3×4.1 cm and partly circumscribed. Histologically, the tumor was composed of parallel long fascicles of spindle-cells with a uniform appearance. The edges of the resected mass were infiltrative, and the surgical margins were positive. Mitotic figures were
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Abdominal Fibromatosis in a young child a case study and review of the literature
Korean Journal of Pathology, 2013Co-Authors: Pyoung Han Hwang, Yeon Jun Jeong, Myoung Ja ChungAbstract:Fibromatoses comprise many different entities of well-differentiated fibroblastic proliferation with variable collagen production and form a firm nodular mass. Abdominal Fibromatosis is distinguishable from other forms of Fibromatosis because of its location and its tendency to occur in women of childbearing age during or following pregnancy. Abdominal Fibromatosis in children is an extremely rare condition. A 15-month-old boy presented with an Abdominal wall mass that had recently increased in size. Mass excision was perfomed. The tumor was 4.3×4.1 cm and partly circumscribed. Histologically, the tumor was composed of parallel long fascicles of spindle-cells with a uniform appearance. The edges of the resected mass were infiltrative, and the surgical margins were positive. Mitotic figures were <1/10 high power fields. No cellular atypia or necrosis was present. The tumor cells were positive for vimentin and nuclear β-catenin staining.
S S Zaveri - One of the best experts on this subject based on the ideXlab platform.
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intra Abdominal Fibromatosis of the jejunum and mesentery
Journal of Clinical Pathology, 2004Co-Authors: S S ZaveriAbstract:A 24 year old woman presented with a painless Abdominal lump of six months’ duration. She had no history of colonic polyps. A mobile, non-tender, globular mass was felt in the umbilical region. A computed tomography scan showed a homogenous, non-enhancing mass, possibly arising in the small bowel mesentery. The tumour was resected entirely with a loop of jejunum. The tumour measured 14 × 12 × 10 cm and was intimately related to the bowel wall. The cut surface was tan, whorled, and firm, without necrosis, cystic change, or haemorrhage. Microscopy showed loosely and haphazardly arranged spindle cells with bland, oval nuclei and minimal cytoplasm (figs 1 and …
P Terrier - One of the best experts on this subject based on the ideXlab platform.
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frequency of β catenin heterozygous mutations in extra Abdominal Fibromatosis as a surrogate marker for disease management
Journal of Clinical Oncology, 2016Co-Authors: J Dômont, S Salas, L Lacroix, V Brouste, P Terrier, A Dufresne, J Y Blay, Le A Cesne, J M Coindre, Jean BenardAbstract:10501 Background: Fibromatosis, a rare soft-tissue and locally invasive neoplasm, consist of distinct clinical entities including sporadic extra-Abdominal Fibromatosis (EAF). The potential morbidity of loco-regional treatments combined with the high local recurrence rate have led investigators to assess the possibility to design a “wait and see” policy in a subset of patients (pts). Up to date, molecular biomarkers of local recurrence of EAF remain unknown. As dysregulation of β-catenin (βC) expression hallmarks Fibromatosis, herein we searched for βC mutations in a large retrospective European multi-centric tumors series to relate with clinicopathologic parameters. Methods: From 1987 to 2007, 155 pts with Fibromatosis from all sites were gathered by the Conticanet Network were analysed. Direct sequencing of exon 3 βC gene was performed for 155 frozen tissues. All mutated cases were confirmed by an independent analysis. From this cohort, 101 pts with EAF surgically treated with curative intent and follow-...
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High frequency of β-catenin heterozygous mutations in extra-Abdominal Fibromatosis: a potential molecular tool for disease management
British Journal of Cancer, 2010Co-Authors: J Dômont, S Salas, L Lacroix, V Brouste, P Saulnier, P Terrier, D Ranchère, A Neuville, A Leroux, L GuillouAbstract:Background: Fibromatosis comprises distinct clinical entities, including sporadic extra-Abdominal Fibromatosis, which have a high tendency for recurrence, even after adequate resection. There are no known molecular biomarkers of local recurrence. We searched for β -catenin mutations in a European multicentre series of Fibromatosis tumours to relate β -catenin mutational status to disease outcome. Methods: Direct sequencing of exon 3 β -catenin gene was performed for 155 frozen Fibromatosis tissues from all topographies. Correlation of outcome with mutation rate and type was performed on the extra-Abdominal Fibromatosis group (101 patients). Results: Mutations of β -catenin were detected in 83% of all cases. Among 101 extra-Abdominal Fibromatosis, similar mutation rates (87%) were observed, namely T41A (39.5%), S45P (9%), S45F (36.5%), and deletion (2%). None of the clinico-pathological parameters were found to be significantly associated with β -catenin mutational status. With a median follow-up of 62 months, 51 patients relapsed. Five-year recurrence-free survival was significantly worse in β -catenin-mutated tumours regardless of a specific genotype, compared with wild-type tumours (49 vs 75%, respectively, P =0.02). Conclusion: A high frequency (87%) of β -catenin mutation hallmarks extra-Abdominal Fibromatosis from a large multicentric retrospective study. Moreover, wild-type β -catenin seems to be an interesting prognostic marker that might be useful in the therapeutic management of extra-Abdominal Fibromatosis.
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high frequency of β catenin heterozygous mutations in extra Abdominal Fibromatosis a potential molecular tool for disease management
British Journal of Cancer, 2010Co-Authors: J Dômont, S Salas, L Lacroix, V Brouste, P Saulnier, P Terrier, D Ranchère, A Neuville, A Leroux, L GuillouAbstract:Fibromatosis comprises distinct clinical entities, including sporadic extra-Abdominal Fibromatosis, which have a high tendency for recurrence, even after adequate resection. There are no known molecular biomarkers of local recurrence. We searched for β-catenin mutations in a European multicentre series of Fibromatosis tumours to relate β-catenin mutational status to disease outcome. Direct sequencing of exon 3 β-catenin gene was performed for 155 frozen Fibromatosis tissues from all topographies. Correlation of outcome with mutation rate and type was performed on the extra-Abdominal Fibromatosis group (101 patients). Mutations of β-catenin were detected in 83% of all cases. Among 101 extra-Abdominal Fibromatosis, similar mutation rates (87%) were observed, namely T41A (39.5%), S45P (9%), S45F (36.5%), and deletion (2%). None of the clinico-pathological parameters were found to be significantly associated with β-catenin mutational status. With a median follow-up of 62 months, 51 patients relapsed. Five-year recurrence-free survival was significantly worse in β-catenin-mutated tumours regardless of a specific genotype, compared with wild-type tumours (49 vs 75%, respectively, P=0.02). A high frequency (87%) of β-catenin mutation hallmarks extra-Abdominal Fibromatosis from a large multicentric retrospective study. Moreover, wild-type β-catenin seems to be an interesting prognostic marker that might be useful in the therapeutic management of extra-Abdominal Fibromatosis.
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extra Abdominal primary Fibromatosis aggressive management could be avoided in a subgroup of patients
Ejso, 2008Co-Authors: S Bonvalot, P Terrier, J Y Blay, H Eldweny, V Haddad, G Missenard, D Vanel, O Oberlin, F Rimareix, Le A CesneAbstract:OBJECTIVE: To evaluate the impact of surgery as first-line treatment on event-free survival (EFS) of primary aggressive Fibromatosis. PATIENTS AND METHODS: Treatments were categorized into: surgery with or without radiotherapy and nonsurgical strategies with systemic treatment alone or wait and see policy. Eighty-nine patients had initial resection of their primary tumour followed by postoperative radiotherapy in 13 cases. Twenty-three did not undergo surgery but received systemic treatment or watch and wait policy. RESULTS: Median follow-up was 76 months. Overall 3 years EFS was 49%. In the univariate analysis, patients with microscopically complete surgery had a similar outcome to patients in the no-surgery group (3 years EFS of 65% and 68%, respectively). Gender, age, tumour size, treatment period and strategy (surgery versus no-surgery) were not statistically significant. Quality of resection according to margins and the tumour site were the only prognostic factors. There was a significant correlation between tumour site and quality of surgery (p=0.0002). CONCLUSIONS: A subset of patients with extra-Abdominal Fibromatosis could be managed with a nonaggressive policy, as growth arrest concerned 2/3 of nonoperated patients. When surgery is finally necessary, it should be performed with the aim of achieving negative margins.
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extra Abdominal Fibromatosis can aggressive management be avoided in a subgroup of patients
Journal of Clinical Oncology, 2007Co-Authors: S Bonvalot, P Terrier, Le A Cesne, H Eldweny, V Haddad, G Missenard, D Vanel, O Oberlin, Le C PechouxAbstract:10077 Background: The objective was to evaluate the impact of surgery as first-line treatment on event -free survival (EFS) of primary aggressive Fibromatosis. Methods: Treatments were categorized into: surgery with or without radiotherapy and non surgical strategies with systemic treatment alone or wait and see policy. Event-free survival curves were estimated using the Kaplan-Meier method. Results: The sex ratio was 39 males/73 females. The median age at the time of the first diagnosis was 30 years. The median size of the primary was 60 mm. Eighty-nine patients (79.5%) had initial resection of their primary tumor followed by postoperative radiotherapy in 13 cases. Twenty-three patients (20.5%) did not undergo surgery but received systemic treatment or watch and wait policy. Median follow-up was 76 months. Three years EFS was 49%. In the univariate analysis, patients with microscopically complete surgery had a similar outcome to that of patients in the non-surgery group (65% vs 68% for 3-yr EFS respectiv...
Massimo Roncalli - One of the best experts on this subject based on the ideXlab platform.
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localized intra Abdominal Fibromatosis of the small bowel mimicking a gastrointestinal stromal tumor a case report
World Journal of Gastroenterology, 2005Co-Authors: Pierigiuseppe Colombo, Daoud Rahal, Fabio Grizzi, Vittorio Quagliuolo, Massimo RoncalliAbstract:Intra-Abdominal Fibromatosis (IAF) is a benign mesenchymal lesion that can occur throughout the gastrointestinal tract. Although rare, it is the most common primary tumor of the mesentery and can develop at any age. We describe a rare case of primary IAF involving the mesentery and small bowel which clinically, macroscopically and histologically mimicked malignant gastrointestinal stromal tumor (GIST). This report highlights the fact that benign IAF can be misdiagnosed as a malignant GIST localized in the mesentery or arising from the intestinal wall. Their diagnostic discrimination is essential because of their very different biological behaviors and the fact that the introduction of effective therapies involving tyrosine kinase inhibitor STI571 (imatinib mesylate) has greatly changed the clinical approach to intra-Abdominal stromal spindle cell tumors.
Pyoung Han Hwang - One of the best experts on this subject based on the ideXlab platform.
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Abdominal Fibromatosis in a young child: a case study and review of the literature.
Korean Journal of Pathology, 2013Co-Authors: Pyoung Han Hwang, Yeon Jun Jeong, Myoung Ja ChungAbstract:Fibromatoses comprise many different entities of well-differentiated fibroblastic proliferation with variable collagen production and form a firm nodular mass. Abdominal Fibromatosis is distinguishable from other forms of Fibromatosis because of its location and its tendency to occur in women of childbearing age during or following pregnancy. Abdominal Fibromatosis in children is an extremely rare condition. A 15-month-old boy presented with an Abdominal wall mass that had recently increased in size. Mass excision was perfomed. The tumor was 4.3×4.1 cm and partly circumscribed. Histologically, the tumor was composed of parallel long fascicles of spindle-cells with a uniform appearance. The edges of the resected mass were infiltrative, and the surgical margins were positive. Mitotic figures were
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Abdominal Fibromatosis in a young child a case study and review of the literature
Korean Journal of Pathology, 2013Co-Authors: Pyoung Han Hwang, Yeon Jun Jeong, Myoung Ja ChungAbstract:Fibromatoses comprise many different entities of well-differentiated fibroblastic proliferation with variable collagen production and form a firm nodular mass. Abdominal Fibromatosis is distinguishable from other forms of Fibromatosis because of its location and its tendency to occur in women of childbearing age during or following pregnancy. Abdominal Fibromatosis in children is an extremely rare condition. A 15-month-old boy presented with an Abdominal wall mass that had recently increased in size. Mass excision was perfomed. The tumor was 4.3×4.1 cm and partly circumscribed. Histologically, the tumor was composed of parallel long fascicles of spindle-cells with a uniform appearance. The edges of the resected mass were infiltrative, and the surgical margins were positive. Mitotic figures were <1/10 high power fields. No cellular atypia or necrosis was present. The tumor cells were positive for vimentin and nuclear β-catenin staining.
