Desmoid tumour: a pleomorphic lesion

Kulaylat, Mahmoud N.; Karakousis, Constantine P.; Keaney, C.M.; McCorvey, D.; Bem, Jiri; Ambrus, J L

doi:10.1053/ejso.1999.0684

Cited by 75 publications

(68 citation statements)

References 93 publications

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“…[2][3][4] Prior trauma or surgery, 8 -13 as well as endogenous or exogenous estrogen exposure, also appear to play a contributory role. 7,8,14,15 Although the overwhelming majority of FAP-associated diagnoses occur in the abdomen or abdominal wall, approximately only 50% of the reported sporadic cases occur in this anatomic location. The soft tissues of the shoulder, neck, and chest wall constitute the majority of the remaining sites of occurrence, with the extremities being involved in a minority of patients.…”

Section: Results Both Patients Demonstrated Ongoing Radiographic Andmentioning

confidence: 99%

“…The soft tissues of the shoulder, neck, and chest wall constitute the majority of the remaining sites of occurrence, with the extremities being involved in a minority of patients. 14 Although desmoid tumors do not demonstrate metastatic potential, the morbidity that results from this disease and its treatment cannot be understated. Local infiltrative growth and tissue invasion can result in pain, deformity, functional impairment, and death when vital organs are involved.…”

Section: Results Both Patients Demonstrated Ongoing Radiographic Andmentioning

confidence: 99%

“…Local infiltrative growth and tissue invasion can result in pain, deformity, functional impairment, and death when vital organs are involved. 14,16,17 The attendant morbidity and mortality from these tumors is highly sitedependent. Intraabdominal desmoid infiltration of vital organs reportedly leads to a 10-year mortality rate of approximately 37%.…”

Section: Results Both Patients Demonstrated Ongoing Radiographic Andmentioning

confidence: 99%

“…Intraabdominal desmoid infiltration of vital organs reportedly leads to a 10-year mortality rate of approximately 37%. 14 Mortality is rare among patients with extraabdominal desmoid tumors; however, the disfigurement and loss of function that result from tumor progression or its treatment is significant.…”

Section: Results Both Patients Demonstrated Ongoing Radiographic Andmentioning

confidence: 99%

“…A variety of other agents also have been employed in the treatment of desmoid tumor with variable success, including interferon, 45 ascorbic acid, theophylline, chlorthiazide, megestrol, and other progesterone formulations. [13][14][15]20,26,31 Imatinib mesylate (Gleevec™; Novartis Pharmaceuticals, Hanover, NJ) represents a selective tyrosine kinase inhibitor. In addition to the antagonistic action against the dysregulated bcr-Abl fusion protein observed in patients with chronic myelogenous leukemia, 46,47 imatinib mesylate also possesses inhibitory activity against multiple class 3 receptor tyrosine kinases (RTKs), including PDGFR-␣ and PDGFR-␤, as well as the c-kit subtype.…”

Section: Results Both Patients Demonstrated Ongoing Radiographic Andmentioning

confidence: 99%

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Response of extraabdominal desmoid tumors to therapy with imatinib mesylate

Mace

Biermann

Sondak

et al. 2002

Cancer

182

110

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BACKGROUNDDesmoid tumor represents a rare monoclonal neoplasm arising from deep musculoaponeurotic structures and may occur sporadically or in association with the familial adenomatous polyposis and Gardner syndromes. Desmoid tumors do not appear to demonstrate metastatic potential; however, local infiltrative growth results in significant morbidity and potential mortality. Although the delineation of optimal therapy for desmoid tumors has been confounded by several factors, surgical resection with adjuvant radiotherapy for a positive surgical margin remains the standard approach. Responses have been demonstrated to nonsteroidal antiinflammatory agents, antiestrogen compounds, and a variety of other agents in small series. Imatinib mesylate appears to demonstrate inhibitory activity against multiple class 3 receptor tyrosine kinases, including platelet‐derived growth factor receptor (PDGFR)‐α and PDGFR‐β, as well as c‐kit.METHODSThe authors performed immunohistochemical and qualitative real‐time polymerase chain reaction analysis on nine desmoid tumor specimens that demonstrated consistent positivity for c‐kit as well as PDGFR‐α and PDGFR‐β. At the time of last follow‐up, 2 patients had received therapy with imatinib mesylate at a dose of 400 mg twice daily.RESULTSBoth patients demonstrated ongoing radiographic and clinical responses with a duration of 9 months and 11 months, respectively.CONCLUSIONSImatinib mesylate has been reported to have activity against desmoid tumor, most likely because of c‐kit and PDGFR receptor tyrosine kinase activity inhibition, and warrants further study. The relative novelty of this agent and the lack of long‐term toxicity data should prompt its use only in the salvage setting in which established local and systemic approaches fail to control disease. In addition, the use of imatinib mesylate in the treatment of this neoplasm preferably should be in the context of a formal prospective clinical trial. Cancer 2002;95:2373–9. © 2002 American Cancer Society.DOI 10.1002/cncr.11029

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Section: Results Both Patients Demonstrated Ongoing Radiographic Andmentioning

confidence: 99%

Section: Results Both Patients Demonstrated Ongoing Radiographic Andmentioning

confidence: 99%

Section: Results Both Patients Demonstrated Ongoing Radiographic Andmentioning

confidence: 99%

Section: Results Both Patients Demonstrated Ongoing Radiographic Andmentioning

confidence: 99%

Section: Results Both Patients Demonstrated Ongoing Radiographic Andmentioning

confidence: 99%

See 3 more Smart Citations

Response of extraabdominal desmoid tumors to therapy with imatinib mesylate

Mace

Biermann

Sondak

et al. 2002

Cancer

182

110

View full text Add to dashboard Cite

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Epidermal growth factor up‐regulates expression of transforming growth factor β receptor type II in human dermal fibroblasts by phosphoinositide 3‐kinase/Akt signaling pathway: Resistance to epidermal growth factor stimulation in scleroderma fibroblasts

Yamane¹,

Ihn²,

Tamaki³

2003

Arthritis & Rheumatism

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Objective. Transforming growth factor ␤ receptors (TGF␤Rs) are known to be expressed at high levels in several fibrotic diseases, including systemic sclerosis. In the present study, we investigated the mechanism of up-regulation of TGF␤R expression.Methods. The levels of expression of TGF␤R type II (TGF␤RII) messenger RNA (mRNA), with or without stimulation by epidermal growth factor (EGF), were evaluated by Northern blot analysis, and the protein levels were determined by immunoblotting. The transcription activity of the TGF␤RII gene was examined with luciferase assays using the ؊1670/؉35 TGF␤RII promoter luciferase construct.Results. EGF up-regulates the expression of TGF␤RII mRNA and protein in human dermal fibroblasts. Actinomycin D, an RNA synthesis inhibitor, significantly blocked the EGF-mediated up-regulation of TGF␤RII mRNA expression, whereas cycloheximide, a protein synthesis inhibitor, did not block this upregulation. In addition, EGF treatment did not significantly affect the TGF␤RII mRNA half-life. EGFmediated induction of TGF␤RII expression was inhibited by treatment of fibroblasts with the selective phosphoinositide 3-kinase (PI 3-kinase) inhibitors wortmannin or LY294002, and Akt inhibitor also blocked EGF-induced expression of TGF␤RII. In addition, EGF induced TGF␤RII promoter activity, and this induction was significantly blocked by wortmannin, LY294002, or Akt inhibitor. Cotransfection with a dominant-negative mutant of p85 (the regulatory component of PI 3-kinase) or Akt significantly reduced the induction of TGF␤RII promoter activity by EGF. Moreover, a constitutive active form of p110 (a catalytic component of PI 3-kinase) induced TGF␤RII promoter activity. In addition, scleroderma fibroblasts expressed increased levels of TGF␤RII but did not show further up-regulation of TGF␤RII expression by EGF.Conclusion. These results indicate that EGFmediated induction of TGF␤RII expression occurs at the transcription level, does not require de novo protein synthesis, and involves the PI 3-kinase/Akt signaling pathway, and that abnormal activation of EGFmediated signaling pathways, including PI 3-kinase or Akt, might play a role in the up-regulation of TGF␤RII in scleroderma fibroblasts.Transforming growth factor ␤ (TGF␤) is a multifunctional protein that plays an important role in regulating cellular growth, differentiation, adhesion, and apoptosis in many biologic systems (1-3). TGF␤ inhibits the growth of most cell types. In addition, TGF␤ causes the deposition of extracellular matrix (ECM) by simultaneously stimulating dermal fibroblasts to increase the production of ECM proteins such as collagen, fibronectin, or proteoglycan, decrease the production of matrix-

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Full-term gestation and transvaginal delivery after wide resection of an abdominal desmoid tumor during pregnancy

et al. 2005

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A 29-year-old female was diagnosed with a symptomatic, extra-abdominal desmoid tumor during the first trimester of pregnancy. Computerized tomography (CT) and transabdominal ultrasound (US) noted a mass within the left rectus sheath measuring up to 15 cm in greatest diameter, with mild compression of the uterus. Preoperative diagnosis was confirmed by core-needle biopsy of the lesion. At 20-weeks gestation, wide local resection of the tumor with disease-free margins, as well as abdominal wall reconstruction with polytetrafluoroethylene (PTFE) mesh was successfully undertaken. Histological examination of the tumor ex vivo confirmed that the lesion was a desmoid tumor consisting of spindle cells with dense infiltrating collagenous fibers. Subsequent to her resection, the patient completed a full-term pregnancy without complication, and proceeded with a complication-free transvaginal delivery at 39 weeks. This case illustrates the probable contribution of estrogens towards desmoid tumor development, the durability of abdominal wall reconstruction when subjected to the extraordinary strain of both a gravid uterus and labor, as well as the safety and efficacy of aggressive surgical therapy during pregnancy.

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Desmoid tumour: a pleomorphic lesion

Cited by 75 publications

References 93 publications

Response of extraabdominal desmoid tumors to therapy with imatinib mesylate

Response of extraabdominal desmoid tumors to therapy with imatinib mesylate

Epidermal growth factor up‐regulates expression of transforming growth factor β receptor type II in human dermal fibroblasts by phosphoinositide 3‐kinase/Akt signaling pathway: Resistance to epidermal growth factor stimulation in scleroderma fibroblasts

Full-term gestation and transvaginal delivery after wide resection of an abdominal desmoid tumor during pregnancy

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