Platelet‐derived growth factor receptors and ligands are up‐regulated in paediatric fibromatoses

Gibson, Sian; Sebire, Neil J.; Anderson, John

doi:10.1111/j.1365-2559.2007.02868.x

Cited by 12 publications

(6 citation statements)

References 38 publications

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“…12 Both PDGFR and the PDGF ligands have been shown to play important roles in both normal and pathological cell proliferation. 13 PDGF-B, in particular, has been shown to induce tumorigenesis in a number of animal models. 14 In view of the fact that our patient had two very rare metachronous tumors, we investigated the possibility of a link between the two.…”

Section: Discussionmentioning

confidence: 99%

GIST with a Twist—Upregulation of PDGF-B Resulting in Metachronous Gastrointestinal Stromal Tumor and Dermatofibrosarcoma Protuberans

McCarthy

O’Brien

Cummins

et al. 2010

Journal of Gastrointestinal Surgery

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Section: Discussionmentioning

confidence: 99%

GIST with a Twist—Upregulation of PDGF-B Resulting in Metachronous Gastrointestinal Stromal Tumor and Dermatofibrosarcoma Protuberans

McCarthy

O’Brien

Cummins

et al. 2010

Journal of Gastrointestinal Surgery

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“…Desmoids are rarely positive for CD117, and lack KIT mutations. They show immunoreactivity for PDGFa and PDGRFa, 67 although corresponding genetic mutations have not been demonstrated. 17 However, therapy with tyrosine kinase inhibitors including imatinib [68][69][70][71][72][73] and sorafenib 74 has been effective in some cases.…”

Section: Behaviour and Managementmentioning

confidence: 97%

Aggressive fibromatosis

Fisher

Thway

2014

Pathology

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Aggressive (deep or desmoid-type) fibromatoses are locally infiltrative collagen-forming tumours with potential for recurrence but not metastasis. They exert their clinical effects primarily in relation to location and have variable biological behaviour. In sporadic cases there are somatic mutations in the β-catenin (CTNNB1) gene on 3p21, resulting in immunohistochemically demonstrable overexpression in nuclei. Fibromatosis in patients with familial adenomatous polyposis (FAP) harbours inactivating germline mutations in the desmoid region of the adenomatous polyposis coli (APC) gene on 5q21-q22. The differential diagnosis includes other myofibroblastic lesions, perineurioma, low grade fibromyxoid sarcoma and, in the abdomen, gastrointestinal stromal tumour and liposarcoma with 'low-grade' dedifferentiation. The primary management is surgical, though some desmoids cease to grow and can be watched. Other therapies have a role in stabilising growth or shrinking tumours. Although no single therapy is effective in all cases, available modalities including irradiation, hormonal therapy, chemotherapy, and receptor tyrosine kinase inhibition can be of value in appropriate clinicopathological subgroups.

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“…DFSP is thought to be intimately associated with the PDGFB paracrine-autocrine system, as to the tumorigenesis [3,[17][18][19], and the excessive secretion of PDGFB is considered to be a result of COL1A1-PDGFB fusion gene products. Imatinib, a tyrosine kinase inhibitor, which is well known as a specific treatment for gastrointestinal stromal tumors, has been adopted for recurrent or unresectable cases of DFSP [5,17], having a therapeutic effect on 36% to 50% of these tumors [4,5].…”

Section: Discussionmentioning

confidence: 99%

Alteration of PDGFRβ-Akt-mTOR pathway signaling in fibrosarcomatous transformation of dermatofibrosarcoma protuberans

et al. 2017

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Dermatofibrosarcoma protuberans (DFSP) is a cutaneous mesenchymal tumor of intermediate malignancy and fibroblastic/myofibroblastic differentiation. Fibrosarcomatous (FS) component is a high-grade component of DFSP. The detailed oncogenic difference between DFSP and FS components is not clear. We thus investigated the Akt-mTOR pathway in both components. We used 65 tumor samples obtained from 65 patients. The phosphorylation of Akt-mTOR pathway proteins (Akt, mTOR, 4EBP1, and S6RP) and PDGFRα/β was assessed by immunohistochemical staining, the results of which were confirmed by Western blotting. The immunohistochemical results were as follows: in ordinary DFSP components, p-PDGFRα-positive tumors were 41.9% (18/43 cases), p-PDGFRβ 55.8% (24/43 cases), p-Akt 51.2% (22/43 cases), p-mTOR 39.5% (17/43 cases), p-4EBP1 46.5% (20/43 cases), and p-S6RP 41.8% (18/43 cases); in DFSP components of FS-DFSP, 52.6% (10/19 cases), 47.4% (9/19 cases), 52.6% (10/19 cases), 36.8% (7/19 cases), 52.6% (10/19 cases), and 52.6% (10/19 cases); and in FS components, 45.5% (10/22 cases), 36.4% (8/22 cases), 72.7% (16/22 cases), 54.5% (12/22 cases), 72.7% (16/22 cases), and 68.2% (15/22 cases), respectively. There were significant positive correlations of the phosphorylation of most of the Akt-mTOR pathway proteins (p-Akt, p-mTOR, p-4EBP1, and p-S6RP) with each other (P < .05). Phospho-PDGFRβ was well correlated with the phosphorylation of Akt-mTOR pathway proteins in DFSP components of ordinary and FS-DFSPs, but these correlations were weaker in FS components. This study suggested the association of activation of Akt-mTOR pathway proteins and PDGFR with the progression of DFSP to FS. The Akt-mTOR pathway is thus a potential therapeutic target in imatinib-resistant DFSP/FS.

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Platelet‐derived growth factor receptors and ligands are up‐regulated in paediatric fibromatoses

Abstract: Increased expression of PDGF/Ralphabeta may play an important role in the mechanism of growth of these paediatric fibromatous lesions and warrants further investigation, since novel therapeutic interventions could potentially be developed in the light of the expression patterns.

Cited by 12 publications

References 38 publications

GIST with a Twist—Upregulation of PDGF-B Resulting in Metachronous Gastrointestinal Stromal Tumor and Dermatofibrosarcoma Protuberans

GIST with a Twist—Upregulation of PDGF-B Resulting in Metachronous Gastrointestinal Stromal Tumor and Dermatofibrosarcoma Protuberans

Aggressive fibromatosis

Alteration of PDGFRβ-Akt-mTOR pathway signaling in fibrosarcomatous transformation of dermatofibrosarcoma protuberans

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