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Abdominal Fibromatosis

The Experts below are selected from a list of 363 Experts worldwide ranked by ideXlab platform

Myoung Ja Chung – 1st expert on this subject based on the ideXlab platform

  • Abdominal Fibromatosis in a young child: a case study and review of the literature.
    Korean Journal of Pathology, 2013
    Co-Authors: Pyoung Han Hwang, Yeon Jun Jeong, Myoung Ja Chung

    Abstract:

    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

  • Abdominal Fibromatosis in a young child a case study and review of the literature
    Korean Journal of Pathology, 2013
    Co-Authors: Pyoung Han Hwang, Yeon Jun Jeong, Myoung Ja Chung

    Abstract:

    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 – 2nd expert on this subject based on the ideXlab platform

  • intra Abdominal Fibromatosis of the jejunum and mesentery
    Journal of Clinical Pathology, 2004
    Co-Authors: S S Zaveri

    Abstract:

    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 – 3rd expert on this subject based on the ideXlab platform

  • frequency of β catenin heterozygous mutations in extra Abdominal Fibromatosis as a surrogate marker for disease management
    Journal of Clinical Oncology, 2016
    Co-Authors: J Dômont, S Salas, L Lacroix, V Brouste, P Terrier, A Dufresne, J Y Blay, Le A Cesne, J M Coindre, Jean Benard

    Abstract:

    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-…

  • High frequency of β-catenin heterozygous mutations in extra-Abdominal Fibromatosis: a potential molecular tool for disease management
    British Journal of Cancer, 2010
    Co-Authors: J Dômont, S Salas, L Lacroix, V Brouste, P Saulnier, P Terrier, D Ranchère, A Neuville, A Leroux, L Guillou

    Abstract:

    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.

  • high frequency of β catenin heterozygous mutations in extra Abdominal Fibromatosis a potential molecular tool for disease management
    British Journal of Cancer, 2010
    Co-Authors: J Dômont, S Salas, L Lacroix, V Brouste, P Saulnier, P Terrier, D Ranchère, A Neuville, A Leroux, L Guillou

    Abstract:

    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.